Blog Archive

Archived posts for 2011


Tuesday - December 20, 2011 @ 23:06:18

Desiree Lyon has been invited to participate in a teleconference with officials of the Social Security Administration (SSA) on Thursday, January 19. The topic will be: "Overview of SSA's Disability Programs." She will hear a presentation and then participate in a questions and answers session.

Desiree requests that you send the APF your questions and concern regarding the Social Security Disability. Also, if you applied for disability and were turned down, please tell us about your experience . This is a rare opportunity to have our problems known directly to SSA officials and have our questions answered immediately . We will be soliciting their suggestions, as well.

To make this opportunity more fruitful, your input is very important. Please email your comments and questions or experiences to the Desiree at or call the APF office at our toll free number 866.APF.3635.

Remember to join the National Registry on the APF website and follow the instructions on the Registry section on the top of the home page or go to

There you will find informatjon about the Porphyrias Consortium, which is a team of porphyria experts /researchers who aim to expand knowledge about the porphyrias. The Porphyrias Consortium enables a large scale collaborative effort to develop new strategies and methods for diagnosis, treatment, and prevention of illness and disability resulting from the porphyrias

The Porphyria Consortium is conducting a Longitudinal Study on all porphyrias and they need YOU. After joining , one of the research coordinators will contact you to discuss the research project.

All that is needed is your blood and some of your health records, which is not much on your effort to join in the group of other patients willing to devote a little time for such an important research project.

Chronic Illness and the Holidays

Sunday - December 11, 2011 @ 19:24:36

Speak Up:

"Many chronic illnesses, such as diabetes, depression, arthritis, fibromyalgia, etc., are 'invisible,'" Fennell says. "People go to work or volunteer or shuttle kids to school. Most of the time, they don't look sick. When illness flares up, their pain is invisible. Or they have bone-numbing fatigue, so bad that they can't take a shower and go to the store in the same day. There's a cultural misperception that says you're not sick unless you look sick. They need to make their illness visible by talking about it."
Fennell, who is president and CEO of Albany Health Management, Inc., in Albany, N.Y., coaches patients on how to negotiate needs. "People don't know how to ask for what they need. They'll stay home from a holiday party because they can't stand that long. We need a new social etiquette for people with chronic illness."

Party Strategies: Ask for What You Need in Advance

Fennell describes a typical holiday scenario. "You're invited to Aunt Jane's. Let her know that you'll do your best to attend her party, but that if your illness flares up, you may have to bow out. Ask her how much lead time she needs. She'll say, 'Anything's fine.' Tell her you'll call her 48 hours in advance to let her know. Uncle Bob will still be annoyed if you don't come, but if you predict that you're unpredictable, people will generally handle it better."
She advises stating your needs in behavioral rather than general terms. "Don't just tell Aunt Jane you'll have to leave early. Tell her you've been feeling fatigued and can stay only two or three hours. Also tell her that standing tires you out, and ask her to have a seat for you. Putting it in behavioral terms makes it easier for Aunt Jane to conceptualize and to accommodate."
Many hosts and restaurants have become accustomed to considering various dietary needs for guests who have heart disease or diabetes or another condition that requires a restricted diet. "They should be offering options for people,"  "If you don't know what's being served, carry a large handbag with snacks and water, or offer to bring a dish that can be shared with others."
When you're the host, whatever you do don't wait till the last minute to ask for help, says Joffe. "You may not get the help you need. And if people do help, they might resent it. Become an expert at planning. Asking in advance allows people to help gracefully."

Managing the Handicap Parking Space

Shopping and gift giving present special challenges, not the least of which is managing the mall. If your illness is invisible, the challenge can start when you get out of your car. Some less-than-jolly shopper who parked way out in left field will let you know that you have no business parking in a handicap space. Try to think of a humorous retort, like that of a cancer patient who plucks off her wig and smiles.
Joffe advises not letting presents and errands get out of control. "Many people with chronic illness aren't in the best financial situation but don't have the energy to shop for bargains. Plan in advance. Take a day off work so you can shop yet avoid the weekend crowds. The key is what matters most to you. Is it going into your bank account? Would a simple note do? Don't go into lock-step motion."

SSA to Expand Compassionate Allowances List in 2012

Tuesday - November 29, 2011 @ 19:37:25

Social Security Administration (SSA) has notified the APF that it hopes to expand its Compassionate Allowances list in 2012. 

As most of you know, Compassionate Allowances are diagnoses so severely disabling that affected individuals, almost always, ultimately are approved for Social Security disability assistance, when they apply. However, the approval process may take a long time, particularly with rare diseases. For that reason, Social Security Commissioner, Michael Astrue, worked with his staff to create the Compassionate Allowances program early in his tenure as commissioner. 

Having a disease that is on the Compassionate Allowances list doesn't guarantee that a patient will be given approval for disability assistance. However, it does guarantee a timely review of his/her application for assistance.

To give SSA time to conduct a thorough review of all suggested diagnoses, the APF will be submitting its suggestions to SSA immediately. We must have information by Tuesday, December 13. The requirements are very specific.

SSA's definition of disability is as follows:

Adults: You must be unable to do any substantial work because of your medical condition(s); and your medical condition(s) must have lasted, or be expected to last, at least 1 year, or be expected to result in your death.

Children: The child must have a physical or mental condition(s) that very seriously limits his or her activities; and the condition(s) must have lasted, or be expected to last, at least 1 year or result in death.

Please contact the APF or email Desiree Lyon at

Your input is very important!

Your Not ALONE!

Tuesday - November 22, 2011 @ 16:25:36

Thank You APF!

Tuesday - November 8, 2011 @ 16:03:54

I wanted to take a moment to Thank everyone for their support to the APF.
What a difference you all make.  We have made some headway with all the types of Porphyria.
With your continued support, fundraising opportunities, support for fellow members we are all playing an important role.  The Doctors are working so hard for us all lets show our appreciation and we cant forget those who work in the office many hours a day.  So please take a moment a just say Thanks to all.
I hope everyone has a fine weekend and takes care of themselves.  If you have not signed up for the blog take a moment to do so today.  If your already on have you donated to we have so many ways to donate Rob is walking 100 miles outside, many are walking, some are setting up a band benefit and the wristbands are still coming in it all goes to the best cause the APF, training new Doctors for the future, and so much more won't you do your part?

Wristbands for Sale

Monday - November 7, 2011 @ 18:19:47

We Have Wristbands for Sale to Support
Dr. Continuing Education Program for the
American Porphyria Foundation

Your Choice is Light Blue OR Purple and they read: www.Porphyria
Please take a moment and ask your friends and family to help donate to this very special cause.  I will be taking orders by Email they will be 2/4.50$ US residents.  They are all 100% Silicone.  I where mine all the time it shows how important the APF is to me, it serves as a reminder to me that I have a rare disease and also helps to show the Medical Staff that I have this disease to.
But lets be supportive together to raise awareness for PORPHYRIA

Please email me @
Please include the following:  Name Full Address City State Zip Phone
Quantity of Each of the Blue or Purple.  You can send your deposit directly to and click under American Porphyria Foundation and click under Amy Chapman~Wristbands
You will receive a receipt that you have made a donation to the APF

Thank you for your support!

Patient Empowerment

Tuesday - November 1, 2011 @ 17:30:51

Patient Empowerment:

What does this mean to you?

·                     Health, as Attitude, can be defined as being successful in coping with pain, sickness, and death as a Normal Vicissitude of Life.  And, successful coping requires always being in control of your life.

·                     Health belongs to the individual. And, the individual has the prime responsibility for their own health.

·                     The individual's capacity for growth and self-determination is paramount.

·                     Health care professionals cannot empower people; only people can only empower themselves.

·                     You Hold the Power, If You But Grasp It!

We Need Your Feedback

Saturday - October 22, 2011 @ 12:41:10

We Need Your Feedback.An Important Request for Medicare Beneficiaries

Dear Friends,
The APF is working on an effort to ensure access to life-saving porphyria medications and ..We need your help!
Over the past several months, we have heard that some of our members may have experienced problems obtaining needed medical treatment in the hospital inpatient setting, and we are concerned that this may be limiting patients' access to Panhematin, a crucial but costly medication that is the only approved drug for the treatment and prevention of acute porphyric attacks.
As a result, the APF has recently appealed to Medicare to change its policy so that hospitals can continue providing porphyria patients with the medications they need most.  However, to support this effort, we are asking APF members to provide their feedback about access problems faced in the hospital inpatient setting.
The APF will share some of your feedback with Medicare but, as always, will preserve the confidentiality of its members (i.e., all responses will be completely anonymous).  We hope that the comments will demonstrate the very real problems that are faced by patients who suffer from porphyria and will prompt a policy change.
If you are a Medicare beneficiary and have experienced access problems in the inpatient setting, please email your story to Desiree at the APF office,
 As Always, we thank our members for taking the time to help in this very important effort.
Thank You!

Checklists You Can Take To Your Doctor

Friday - October 7, 2011 @ 21:19:23

An important part of getting properly diagnosed or treated is maintaining clear communication with your practitioner. If you suspect that you might have a Particular health or related problems, your first step should be to schedule an appointment with your practitioner. A risks and symptoms checklist can be an important tool to help communicate your concerns. Even if you've been diagnosed and are receiving treatment, a risks and symptoms checklist can be useful in discussing unresolved symptoms and concerns that need to be addressed.  Dont forget to be PREPARED.  Have your list of questions written down ahead of time and prioritize your questions in relation to your Dr.s visit.  Some things may apply immediately while other questions may have to wait till another appointment.  Take a moment to Thank Your Practitioner for their time and effort.  They are very busy every day to get everyone seen so youre in and out but by being prepared having questions you will be able to spend more time with your Dr.

By A.I.P Patient ~Amy Chapman

Why Do We Beat Ourselves Up? A Look at Self-Destructive Thoughts

Sunday - September 25, 2011 @ 23:06:51

If we had friends that treated us the way that many of us treat ourselves, they wouldnt be our friends for very long. Imagine a friend who calls up just to complainabout you. Or an alleged buddy who quickly says I told you so when you screw up. Or someone who encourages you to give up instead of encouraging you to do your best.

Why do we do this? Why do we treat ourselves in such horrible ways? For some, the negative self-talk is so bad that it would literally be considered verbal abuse if coming from another person. Do any of these sound familiar? Im not good enough. I knew Id fail. I cant believe I messed it up again. Why cant I be more like (fill in the blank)? I dont deserve to be happy. If someone said these things to you, imagine the impact it would have on your confidence.

Negative self-talk can easily turn into a damaging self-fulfilling prophecy where you live downinstead of upto expectations. Its a bad habit that could strangle any growth and needs to be dealt with now. This is not friend talk. Yet its exactly the kind of destructive feedback we give ourselves.
Why? What did we do to deserve this? Is our self-esteem so low that we think we need to be talked toeven by ourselveslike this? Hopefully not.

Youre a fantastic person on an exciting journey, in the middle of creating a life that you want. If I were you, I wouldnt put up with that negative voice in your head. You deserve better. If anything, your self-esteem needs to be built up, not torn down. Whats so wrong with reminding yourself of how wonderful you are? Isnt that what you would do for a friend who needed a word of encouragement?

Sometimes, this can be easier said than done. Any self-bashing you might do may actually have its roots in something other people have told you over the years. Harsh words can leave scars that never go away. Still, that doesnt mean we have to carry on the legacy of beating ourselves up.

You deserve to be treated with respect, encouragement and patience. You demand that much from people you know. Demand it from yourself.  So do yourself a favor when a family, friend, or another person says something you do like or take offense, dont say anything back your better than that.
Maybe theyre going through some problem we dont know about, maybe they havent gotten the same help that we have, maybe they dont have a diagnose, they can be mad about that.  We are a special group of people who need to come together and stay positive to each other no matter what our opinion is, right or wrong, there are so many serious things going on in this world~ So lets not be destructive, tear each other down, be the bigger person and be nice.

Meet Expert Dr. Joseph R Bloomer, MD

Monday - September 19, 2011 @ 00:35:56

Description: Dr. Joseph BloomerJoseph R. Bloomer, MD

Porphyria specialist, Dr. Joseph Bloomer recently won the most prestigious Presidential LIVER DISEASE AWARD given at the American Association for the Study of Liver Disease.

Dr. Bloomer was also honored as the Cecil J. Watson Lecturer  at the University of Minnesota.  The lecture series was named for Dr. Cecil Watson,   who was the first Chairman of the Department of  Medicine at the University of Minnesota.  In Dr. Watsons honor, this yearly lecture brings in outstanding scientists and clinicians that are interested in liver disease, porphyria and heme metabolism. Dr. Bloomers work in porphyria and liver disease exemplified those passions for science and clinical care.

Dr. Bloomer served as Professor and Director of the Division of Gastroenterology , Hepatology and Nutrition at the University of Minnesota from 1983 until 1995 when he was recruited to the University of Alabama (UAB) to begin the UAB Liver Center, which has come one of the top liver centers in the country.  He also serves as a Professor of Medicine and Genetics .  Dr. Bloomer has many honors and awards including being listed as Americas Top Physician from 2004 to present.  Dr Bloomer has served on numerous UAB committees during his tenure at UAB, including the Liver Transplant Evaluation Committee, Research Development for the Depart-Special Interests: Porphyria, Genetics, Hepatitis B and C and therapy for complications in advanced liver disease. 

Why We Need Your HELP NOW!

Thursday - September 8, 2011 @ 16:08:41

We are in need of your participation to help save the life saving medications for all Porphyria Types.
We may be shy, we may not want anyone to know we have a rare disease, but if you or someone you love or know has Porphyria and needs medication, what would happen if it was not available any more?  What would you do?  Coould you do anything?  We need people to open up, step up and share your story on how Porphyria has affected you personally, as a family member but most importantly if you have taken these orphan drugs we need you to write to your Congress men/women, email them, call them, write them to make this bill 340b come alive to see the need that they need to make a change and why.

Please really think about this and act quickly time is running out!  For more details contact: or call 866-apf-3635.


Friday - September 2, 2011 @ 17:49:47

This is the biggest legislative problem affecting people with porphyria that we have encountered in the last 30 years. A new bill was introduced in Congress at the end of July that will impact access to orphan medicines, H.R 2674 - The 340B Improvement Act. This legislation will expand government price controls to a wide range of locations, regardless of need, in some instances forcing medicines to be sold for as low as a penny, regardless of the costs to innovation and patient access. If the bill passes, a number of treatments for rare diseases, including porphyria, may no longer be available.

While well-intentioned, such an expansion of price controls could be devastating for those people who now take Panhematin or who may need to take it in the future. I will explain why.

This bill forces drug companies sell treatments to approximately 5000 hospitals for artificially low prices, regardless of the costs to manufacture and the cost to distribute. In some instances, orphan medicines, such as Panhematin, will be sold for as little as one penny, regardless of the losses incurred to make it available. The new expansion to the 340B program also expands the program beyond geographic needs, basically allowing affiliate programs of a central qualifying hospital to purchase price controlled products, regardless of location or need. In other words, a 340B hospital can purchase a rare disease drug for one penny and in turn and sell it to another non-qualified hospital for a large profit. To me, this is outrageous and puts those of us who use a rare disease treatment in terrible jeopardy

Under this new bill, 340B was expanded significantly to hospitals, rural and community clinics to include the addition of inpatient and outpatient services (1500 new hospitals, clinics, etc.). In the new bill, there are NO exemptions for rare disease drugs. As you can imagine, companies like the one that manufactures Panhematin, would be hard pressed to make this complex biologic and sell it for one penny. How could they or any other company that manufactures rare disease treatments continue to make those treatments available under such conditions?

Many rare disease treatments, like Panhematin, are life-saving. Patients can have attacks and lose their lives without this medicine. You may say, "This cannot be possible. Surely our Congressmen would not put us in such jeopardy?" But, it is true; some members of Congress have already signed on to support this legislation. Below are five ways you can help:

1. Contact your Congressman! Tell them we MUST have an exclusion for rare disease drugs included in H.R. 2674, the 340B Program Improvement Act.

2. If you cannot talk to your Representative personally, ask to speak with their Legislative Aid.

3. Write a letter to your Congressman. This is one of the most powerful things you can do. Write a handwritten letter (not an email) and tell them how important the orphan drug exclusion is and that it must be included in the 340B Program Improvement Act.

4. Contact your local newspaper and television station about this issue.

5. Ask your friends and family members to call and write their Congressman. The more voices that speak up, the stronger the message.

The APF cannot educate members of Congress on the unintended consequences of such legislation without your help. This legislation could create a life threatening situation that involves YOU. Even if you don't take such a treatment, your children may need it in the future, but it may NOT be available.

We must advise our Congressmen that expansions of 340B, no matter how well-intentioned, can have devastating consequences for patients who need orphan medicines. To find out who your Congressman is go to

To contact me, the APF office number, toll free, is: 866.APF.3635 or 713.266.9617. My email is: We want to hear about your successes spreading this important message. Call the office and let us know what you are doing to help. I look forward to speaking with you because together we can stop this bill from passing and save our future - the lives of our children.


Tuesday - August 30, 2011 @ 05:41:50

This request is of ultimate importance. I will explain everything better but in essence , congress has put forth a bill that could make certain drugs for rare diseases no longer available . Please call me if you are taking panhematin. I will be in the APF office tomorrow. 1 866 APF 3635.


We need you to tell us straight forward in a letter format ASAP if you have received Panhematin how many times you have received it, what setting hospital inpatient outpatient and would you say that it has helped save your life. Please think about your letters to inspire us to help gvt officials see the need for Panhematin and why it is so precious to you and how it has saved your life. Please include your name address and phone numbers, city and state, PLEASE BE DIRECT. Look at the announcement on the www.porphyriafoundation and send them to your Congressmen/woman The APF needs our help! They help us lets help them!

Arguing with Reality!

Monday - August 22, 2011 @ 18:55:36

Description: stop complaining1 The 21 Day No Complaining Challenge  Stop Arguing with Reality!
Imagine getting upset every time the sun goes down.
See your fist in rage at the sun while screaming there you go doing it again, just like yesterday! How dare you! and so on. You look crazy, which makes sense. I mean the sun is just doing its thing (actually the earth is the one doing most of the moving but you get what I mean). I am here to tell you that every time you complain you are arguing with reality and that is just as pointless and crazy as yelling at the sun.
I dont complain you might be thinking Its too hot its too bright and its not tall enough oh and traffic is awful! Shall I go on? This costs too much, shes horrible and this and that sucks I know you have heard it and I would bet some of you might have heard it out of you own mouth. Did you know that when you express these emotions the chemistry in your brain changes? It makes sense they change your mood and not for the better. And all that is; is arguing with reality.
Things happen objectively. They come with no labels. If traffic did not slow your progress it wouldnt be traffic! If poor drivers didnt drive poorly they wouldnt be fully expressing themselves. Think about it. The point of yoga is to accept life as is and by doing so you can shape the future. However, when the present is happening, its happening and we can choose to embrace it or complain about it. Complaining only hurts us and doesnt change a thing expect our mood.
Try this! As I work with future yoga teachers I challenge them to go 21 consecutive days without arguing with reality or complaining. It seems easy however it took me four months on my first try but it gets easier with practice. Let me clarify complaining, lets say you order a meal at a restaurant and it is cold when the server brings it to you. If you simply request the server to bring you a warm meal that is not complaining because you are speaking to a person who can directly adjust the situation. If you turn to the person who you are dinning with and say I cant believe they would serve me a cold dinner That is complaining.
To keep track simply put a rubber band or a hair tie around your wrist and every time you catch yourself complaining move the band to your other wrist. Try it. It is a life changer. Also you will find those around you very helpful so let them know what you are trying to do. I once had a second grade teacher try it and she had heard whole class wearing non complaint bands! Imagine twenty second graders trying not to complain, imagine if it caught on, what a pleasant world it could be.
How to go 21 Days Without Complaining:
It doesnt take that long after you put that rubber band around your wrist and attempt to go 21 consecutive days without complaining, switching the band to the opposite wrist when you do, to realize this challenge wont be as easy as you first thought. To be honest, I considered myself to be a pretty positive person but within an hour I caught myself complaining. I believe my first catch was that song (name withheld) is terrible. Yes, labeling is complaining. The song wasnt terrible, in fact all the artists, engineers and producers were extremely talented and the song itself had given joy to millions of people hence producing millions in revenue consequentially supporting thousands of jobs that supported thousands of families and so on. The song wasnt terrible it just wasnt the style of music I enjoyed. So my first tip to stop complaining is to stop labeling and take a moment to consider why and what you are saying. This practice alone will improve every relationship you have because you begin to communicate what you truly want to say.
The example above also illustrates my next tip. Get over yourself.  It is not all about you, so consider other people and other things. Here in Texas in the spring many people will complain about the rain. Oh darn, its raining again. The first thing I think of are the countless animals and plants that rejoice at the showers of spring, however in a moment I am also reminded of all the gardeners, botanists and even people like myself who love the rain! Then of course in the summer I hear I cant believe its so hot. Really? I think to myself. It has been hot in Texas for tens of thousands of years, what did you expect?
This last tip is my favorite; Look for the Good. What can you do with what life is currently offering? Isnt that always the question? Because the fact is, we live in the present no matter what that present moment brings? If there is a temporary delay in your life like illness, sickness or finding yourself on hold look for a good way to use it! In traffic you can catch up with an old friend you have been meaning to call, pop in a CD to learn a new language. On hold you can do a quick brief meditation that can relieve stress and perhaps add years to your life. If someone is short with you it is your opportunity to work on your compassion and understanding.
Bottom line, you want to wrap yourself up with the ever present silver lining of life. Not only will it bring you joy and peace. It makes you one of those people who seem to shine and attract the attention and affection of all around you. Shine on!

So why do we do this to ourselves?  Do we even notice it, does someone tell us?

Amy Chapman~AIP Patient

Do you know your Limit's?

Monday - August 22, 2011 @ 18:39:56

What limits are those you may ask yourself.  Everyday we make choices some good some bad.  We make decisions in our brains without evening taking the time to notice.  What am I going to drink?  What sounds good to eat?  How do I feel today?  What can I do today?

We may organize ourselves to death every day.  We have high goals and dreams.   When faced with an Illness or disability how do we cope from day to day?  We could do everything all at once and have no strength at the end of the day, we could choose not to do anything.  But what we should do is keep moving and setting good limit's/balance every day.  We have families, friends jobs, to do list's we may get tired.  But try this for one week and let me know how you do and how you feel.

What is most important for me to get done this week?  How do I feel?  What can I do?  What can wait?
We get so busy we don't stop and take a break, listen to the birds, feel the breeze, or enjoy a good book or dish.  So stop and smell, taste and see all the beautiful things in this world, and ask your self Do I know my own Limits?

Amy Chapman~AIP Patient


Thursday - August 4, 2011 @ 23:21:05


How to Stick to Your Goals When Life Disrupts Em

Thursday - July 21, 2011 @ 17:07:05

I cant change the direction of the wind, but I can adjust my sails to always reach my destination. Jimmy Dean
Have you ever been doing great with a goal or habit change exercise, waking early, becoming organized, eating healthy, anything and your progress was completely disrupted because of some major event in your life (death, marriage, illness, work, etc.)?
Of course you have. Its happened to all of us.
Life gets in the way.
Fortunately, although we often give up our goals when something like this happens, a life event that disrupts your progress doesnt have to sidetrack your goal completely. You can overcome this obstacle and it is an obstacle, just like any other.
Reader quench recently wrote in with a great question:
Oftentimes, I have just one goal, as you suggest, and I am focused fully on that one goal, the baby steps, etc. Then all of a sudden, out of the blue, theres some crisis in another part of my life my boyfriend will become enormously demanding, my brother will get hospitalized with a life-threatening illness, my boss will decide there is an emergency that I have to handle overtime, etc.! This happens will such frequency, that its almost predictable, every time I start really buckling down on what I need to do to accomplish my goal. Then when I thoroughly demoralized, and lying around unmotivated, everything else in my life seems to calm down, and Im just left wondering how to pick myself up again.
This is an awesome question, because its so true to life. Things happen to all of us â?¦ but the real determination is how do we deal with them. Do we get discouraged and give up, or do we figure out a way around and keep going?
Heres the thing: the path to every goal is littered with obstacles. You must, must, must, absolutely must anticipate that you will run into obstacles â?¦ figure out ways around them â?¦ and keep going.
Heres how to do that:
1. Anticipate, anticipate. Think ahead. What obstacles do you see on the horizon? What obstacles have you run into in the past? What will change your routine? Weekends are a mini-obstacle that often seem to derail people trying to create a habit. But there are other changes in routines â?¦ holidays, summer, big events, changes at work, birthdays â?¦ these are often things you can anticipate and plan for. When you see something coming up on the horizon that might derail you, plan for it. Make it a part of your plan.
An example: Ive been trying to learn to eat under my new meal plan â?¦ and I knew that a party was coming up. Well, I anticipated it, learned the restaurant, researched the menu, and incorporated it into my plan. But on another occasion, I just anticipated the family event and allowed it to be one of my two cheat meals for the week. You have to be able to let up a little if you want to stick to it for the long term.
2. Find the path. When you see an obstacle, are you just going to give up? I hope not. An obstacle is just something we have to get around, or over â?¦ its not a reason to quit. So instead of quitting, ask yourself: how do I get around this obstacle? Theres always a solution, if youre creative enough.
Sometimes there isnt just one path. And it can be hard to decide which solution to choose. But dont fret: choose one path, and see if it works. If it doesnt, try another. Life is an experiment.
3. Two steps forward, one step back. You will face some setbacks every now and then. Thats a part of achieving any goal worth achieving if it were too easy, its not a worthy goal.
But instead of seeing the setback as something discouraging, just accept it as a part of the journey. Two steps forward, one step back, is what I always tell myself â?¦ and its a realization that even with setbacks, Im still making forward progress over the long run.
For example, Ive had some setbacks when it comes to my exercise habit. I often seem to get sick after a few weeks of continuous exercise. But though illness has set me back a few times, Ive now learned to see it as a welcome break, allowing my body to recover. Think of it as a time out â?¦ youre allowed to rest when youre sick, and when you get better, youll be recharged and ready to start again.
4. If you fall, get up, and learn from it. All of us fail, from time to time. No one is successful all the time. But instead of letting failure stop us completely, you just need to get up and dust yourself off â?¦ and this part is important: learn from your experience. When you fail, ask yourself: why did I fail? What stopped me? What obstacles got in my way? And how can I get around them next time it happens (and yes, it will happen again). And plan for next time.
5. Find new motivation. Sometimes a setback will leave us not only derailed, but demotivated. So the real problem is finding the motivation to start again. So, it helps to get back to the basics: what made you want to start in the first place? What was your reason for change? What motivated you? Thinking about that, and the benefits it will have in your life, and the way things will be once youve accomplished the goal, can help motivate you.
But I also recommend adding additional motivators, to help jump start you.
6. Go with the flow. Change is good. Change is a part of life. We might like our routines, but there will always be something that comes along to disrupt them. Accept that, embrace it, and learn to flow with it. If we become too rigid, we will break in the face of the pressures of life. But if we learn to accommodate those changes that life throws at us, and still head towards our goal, well be happier and well get to where we want to be.
Be willing to be flexible. Change comes at you â?¦ dont despair, or get frustrated â?¦ take that change and make it a part of your plan.
Life will throw us curveballs. With practice, we can learn to hit them out of the park.

Amy Chapman~AIP Patient


Sunday - July 17, 2011 @ 11:23:54

LOL It's so hot.. Perhaps, as you stumbled in the house, clammy and damp, you said, "Man, it's hot," for what seems like the hundredth time. We know we have and we're tired of repeating ourselves. We figured if our bodies can't be fresh, at least our words can be.

Until the heat peaks in August, try out a hot phrase. They won't make the temperature drop, but they might make you smile. And that's pretty cool.

â?¢ It's so hot the birds have to use potholders to pull worms out of the ground.

â?¢ It's hotter than a half-bred fox in a forest fire.

â?¢ It's hotter than two bears fighting in a forest fire.

â?¢ It's hotter than a billy goat in a pepper patch.

â?¢ It's hotter than two cats fighting in a wool sock.

â?¢ It's so hot that I tied my mule in a field of corn, and the corn started popping and the mule thought it was snow and froze to death!

â?¢ It's so hot I saw two trees fighting over a dog.

â?¢ It was so hot today I saw a dog chasing a cat and they were both walking.

â?¢ It's hot enough to fry an egg on the sidewalk.

â?¢ It's so hot outside that you could fry an egg on the hood of my car.

â?¢ It's hotter than a depot stove.

â?¢ It's hot as love in August.

â?¢ It's hotter than a mother-in-law's kiss.

Pain Levels (EPP): by Victor Mejias

Wednesday - July 13, 2011 @ 12:36:02

Pain Levels (EPP): by Victor Mejias

In this posting I am going to attempt to document, on a Zero to 10 scale, differing degrees of pain and (with the exception of swelling - refer "note B") other symptoms involved in differing degrees of EPP reactions to sunlight.

The level zero will of course be defined as that state where there is no light exposure effect whatsoever, and a level of 10 will be the complete opposite, i.e. a totally out of control EPP
reaction after a great deal of exposure.

I attempt this in the full knowledge that both the speed of reactions as well as their severity, as hereinafter described, will and do differ from one EPP patient to another and therefore
some may not agree with everything that follows.

However the reasons I am attempting to do this basically are: - The degree of pain involved in EPP reactions has been "sadly under-articulated" in general (to say the least), and therefore not fully appreciated by the medical profession or some EPP parents and relatives.

- To assist all EPP patients (young and old alike) communicate with their physicians and parents/relatives the level of pain and type of reaction they are experiencing.

Several factors for each "Reaction Level"  level will
be included, these are:

a) Pain Description.
b) Skin Sensitivity Rating: On a scale of 1 to 10 skin surface sensitivity to touching, scraping, accidental knocks and exposure to heat etc.
c) "Sleep Disturbance" Rating (if any), from zero to  very significant 
d) A "psychology effect" factor on a "No Effect to the High" scale, whether behavior modifications are sought, levels of    stress experienced etc.
e) An "overall pain" level, also on a scale of Zero to    Very High.

So let's begin at the very base level of "Zero" i.e. the very inactive base level, defined as no reaction whatsoever.

Reaction Level - 0
a) Pain Description: This is the completely normal (un-agitated) state of the skin prior to any exposure to sunlight.
b) Skin Sensitivity: This also zero, i.e. same as any normal person. Completely pain and sensation free.
c) Sleep Disturbance Rating - Zero.
d) Psychology: Undetectable/No effect.
e) Overall Pain Level: Zero.

Reaction Level 1
a) Pain Description: Light tingling sensation.
b) Skin sensitivity: Rating 1, almost normal.
c) Sleep Disturbance Rating: Zero - Normal sleep pattern.
d) Psychology: Almost Zero - Detectable only.
e) Overall Pain Level: Low - Easily tolerable.

Reaction Level 2
a) Pain Description: Stronger tingling - including the nerves.
b) Skin Sensitivity: Two - getting a little sensitive.
c) Sleep Disturbance rating: Very slight - But normal sleep pattern.
d) Psychology: Still low - But noticeable.
e) Overall Pain Level: Low - Still relatively tolerable.

Reaction Level 3
a) Pain Description: Stinging sensation, light nerve vibration, light chills on the skin surface (particularly when exposed to cool air).
b) Skin Sensitivity: Three - getting sensitive.
c) Sleep Disturbance Rating: Slight - But normal sleep pattern possible.
d) Psychology: Low - But noticeable and still quite tolerable.
e) Overall Pain Level: Low/Medium - Still relatively tolerable.

Reaction Level 4
a) Pain Description: Warmer stinging sensation, nerve vibration, chills on skin surface.
b) Skin Sensitivity: Four - Sensitive to touching, scraping knocks etc.
c) Sleep Disturbance Rating: Still only slight - But normal sleep pattern usually possible.
d) Psychology: Low/Medium - Slightly intrusive but still tolerable.
e) Overall Pain Level: Medium - Still tolerable.

Reaction Level 5
a) Pain Description: Warmer stinging sensation, slightly painful deeper nerve vibration, more severe chills.
b) Skin Sensitivity: Five - Quite sensitive.
c) Sleep Disturbance Rating: Getting significant - normal sleep pattern often disturbed.
d) Psychology: Medium - Intrusive and now barely tolerable.
e) Overall Pain Level: Medium - Barely tolerable.

Reaction Level 6
a) Pain Description: Very warm (almost hot) sensation, painful & deeper nerve vibration, more severe chills.
b) Skin Sensitivity: Six - Getting very sensitive.
c) Sleep Disturbance Rating: Getting more significant - normal sleep pattern usually disturbed.
d) Psychology: Medium - Intrusive and now becoming bothersome, beginning to be stressful, methods of alleviation often sought from this level upwards.
e) Overall Pain Level: Medium/High - becoming intense.

Reaction Level 7
a) Pain Description: Hot sensation, painful & deep nerve vibration, severe chills (if any chills occur).
b) Skin Sensitivity: Seven - Very sensitive.
c) Sleep Disturbance Rating: Significant - normal sleep pattern almost always disturbed.
d) Psychology: Medium/High - Intrusive and now annoying and/or distracting, often stressful, methods of alleviation usually sought.
e) Overall Pain Level: Medium/High - relatively intense.

Reaction Level 8
a) Pain Description: Hot & burn like sensation, painful and very deep nerve vibration, severe chills (if any).
b) Skin Sensitivity: Eight - Highly sensitive, getting painful to touch, quite painful to knocks and scrapes.
c) Sleep Disturbance Rating: Very Significant - normal sleep pattern often/usually severely disturbed.
d) Psychology: High - Intrusive and now very annoying and/or frustrating, stressful in general, methods of alleviation more actively sought.
e) Overall Pain Level: High - Intense.

Reaction Level 9
a) Pain Description: Very hot & burning sensation, deep searing nerve vibration occasionally affecting other parts of nervous system, hot flushes, etc.
b) Skin Sensitivity: Nine - Very highly sensitive, now painful to touch, very painful to knocks and scrapes.
c) Sleep Disturbance Rating: Very Significant - normal sleep pattern almost always severely disturbed (if sleep is possible at all).
d) Psychology: High - Very Intrusive and now very annoying and/or very frustrating, quite stressful, methods of alleviation pro-actively sought.
e) Overall Pain Level: High/Very high - Very intense level of pain.

Reaction Level 10
a) Pain Description: Very hot & burning sensation, very deep searing nerve vibration (deep into the bone marrow) often affecting other parts of nervous system, hot flushes, etc.
b) Skin Sensitivity: Ten - Extraordinarily sensitive, now very painful to touch, incredibly painful to knocks and scrapes.
c) Sleep Disturbance Rating: Very Significant - normal sleep usually impossible until reaction declines in severity.
d) Psychology: Very high - Dominating and exasperating, very stressful, any method/s of alleviation pro-actively sought and adopted.
e) Overall Pain Level: Very high - Extremely intense level of pain.

A. Once again I must re-iterate that the speed at which one patient progresses from
Zero to 10 varies from one individual to another. This is to be expected with any qualitative rating system/analysis like this. So please feel free to respond to this posting with other symptoms that you experience but are not listed above etc.

B. I have deliberately omitted the swelling that often accompanies the above levels due to the vastly differeing levels of swelling experienced by different EPP patients. Those that find
swelling a major symptom are encouraged to pencil a description
of that in as an additional factor for each level above.

C. I probably should add another level (number 11) for those patients who have experienced liver problems, or even a total breakdown of liver function, because there will obviously be
factors involved in such a reaction that are not included in the ten levels above. Maybe one of them can give us an indication of what they went through in comparison?

D. EPP parents who are having trouble comprehending the degree of pain their EPP children are suffering should experiment with a table like this. Younger patients in particular are not able to articulate the level of pain and discomfort they are suffering. Parents will then gain a clearer understanding of what their children are experiencing and therefore whether to commence taking alleviating and/or preventative measures.

E. I included the "sleep disturbance rating" to remind relatives of EPP patients that just because they have gone to bed does not necessarily mean they are getting good night's sleep.

F. To assist communication with non-EPP relatives, all EPP patients (younger patients
in particular) should be encouraged to rate on this 0 to 10 scale events that often occur
in the lives of non-EPP patients. e.g. falling off a bicycle, spraining an ankle or wrist, breaking a limb, getting kicked in the leg during a football match etc. For example, I would rate falling off a bicylce and getting a few bruises and scratches a 2 or 3 (depending on the severity of the fall) which is a long way from the pain experienced in a full blown EPP reaction (9 or 10).

G. Personally, I would rate "normal" sunburn as being between Reaction Levels 1 and 4 on the above scale, well maybe a 5 for very serious sunburn. This is because it is mainly a superficial skin surface phenomenon in my experience. Yes, I have experienced normal sunburn a few times in my life, and it is much, much less painful than the levels of pain achievable by a "full blown" EPP burn.

H. I have never experienced the chills at the higher reaction levels, only up to about the Number 7 level. After a reaction has started subsiding from higher levels, yes for sure, but not at the very top levels, this is probably another reaction factor/symptom that varies from one individual to another.

I. I have a more simplified version of the above on an Excel spreadsheet, which I can send to anyone that needs it, or post in the "files" section of the website if required.

J. In view of the lack of appreciation (by the medical profession etc.) of the type of pain being experienced by the average EPP patient, I had decided to compose a table like this some time ago.

Miranda's Advice to College-Age Porphyria Sufferers

Wednesday - July 13, 2011 @ 12:17:48

Youve just turned 21, and you have a rare disease that forbids you from drinking. You go to parties where people pull a PBR from a bathtub or do keg stands. People are getting progressively drunker; even shy people who never talked in that English lit class you had are suddenly dancing on a table, and you are standing by, utterly confused. Youre holding a beer. You take a sip, but you dont actually drink it. You just kind of hold the beer, hoping no one expects you to consume. Later, you give the beer to someone when theres no more beer left in the bathtub. Someone offers you a cigarette. I dont smoke, you say. Neither does that person, just when drinking. You begin to wonder if youre an alien.

Dont worry, its totally normal if you have porphyria!

I went to a tiny womens college in mountains of Virginia, where theres not a lot to do besides drink. American college life sort of stipulates that you make a lot of poor decisions, including possibly getting your stomach pumped. Obviously, Im being facetious, but Im also being realistic about the drinking culture, especially where it applies to college life in the United States.

But, guys, as tempting as it is to join the legions of drunk people, eating eggs at an IHOP at 2 am, and dancing on tabletops, remember that alcohol can affect some of the porphyrias, if not all of them. As someone with AIP I could die if I drank.

Im not judging people who drink in college. A lot of times I envied them because in American culture drinking is such a coming-of-age experience, despite the fact that theres nothing inherently mature about getting drunk. (We really have a weird relationship with alcohol in this culture!) But I am saying that its important to realize that many of us just cannot drink alcohol, and basically, you have to suck it up and get over it.

I could try to provide a list of things you could do as an alternative to drinking, but you know what? It would be unrealistic and ring false. Instead, you should go to the parties and dance and talk to people (Ive been known to explain porphyria to people who are drunk) and be who you are-- pretend you are completely uninhibited, and you, too, can dance on a tabletop.

--Miranda Dennis

In Praise of Chicory Coffee (and Grandmothers)

Saturday - July 2, 2011 @ 14:46:21

As a child and a teenager I was lucky enough to live in the same city as my grandmother, who was a health nut and a bit of a snob when it came to good cooking, especially in restaurants. Up until I was a teenager I had no idea that my grandmother knew how to make sweets like fudge or divinity. I assumed that she got her sweet tooth fix from ripe blueberries and the occasional custard. It turns out she simply chose not to eat rich, unhealthy desserts, except very rarely.

Shes a great example of someone who was able to eat very healthy while maintaining the necessary carbs to prevent Acute Intermittent Porphyria attacks. Now that Ive grown older and can appreciate what I thought was rather fanatic as a kid (it wasnt) I like to follow the examples she set in my own health, as evidenced by previous posts of mine.

One thing she introduced me to, which I am forever grateful, is chicory coffee, served with steamed/heated milk and maybe a little sweetener, New Orleans style like at the famous Cafe Du Monde. One day she made it for me, and I got hooked. The great thing about chicory is it negates a lot of the caffeines effects; half of the drink is steamed skim milk, leaving only 25% caffeine intake compared to a normal cup of coffee.

I sometimes make chicory coffee with steamed milk as a dessert drink when I have a hankering for sweets. After all, skim milk can help satiate the craving for sweets (something my Grandma taught me, which has turned out to be true). So, I tend to heat up my milk, brew up some chicory coffee, and add agave nectar (or the occasional raw sugar, which isnt as processed and therefore better for you). Voila, I have a sweet drink thats not too sweet but satisfies my sweet tooth.

I think people with porphyria should be mindful of how theyre getting those sweets theyre craving. While it may feel great to eat a piece of cake (yum), dont forget about fruits, skim milk, a little bit of sugar, etc., going a long way. Remember, the healthier we eat, the healthier we are, porphyria or not.

--Miranda Dennis


Thursday - June 30, 2011 @ 09:30:17

Written All over His Face: Rare Disease Offers Clues to How We Read Emotions

People who feel what they see offer clues about how we read emotions and empathize
  • Understanding the thoughts and feelings of other individuals is essential for navigating the social world. But empathy is a complex process, based in part on fleeting facial expressions. Research suggests that we empathize by effectively putting ourselves in others shoes: for example, when we observe someone feeling sad, we simulate their experience by activating the same regions of the brain that are involved when we feel sad ourselves.
A study in the Journal of Neuroscience in February bolsters this idea using rare individuals with mirror-touch synesthesia. When watching another individual being touched, these people actually feel a touch on the same part of their own body. Neuroscientist Michael Banissy and his colleagues at University College London tested whether this heightened ability to simulate another persons experience would cause eight mirror-touch synesthetes to excel at recognizing the emotions embedded in facial expressions. They did, correctly identifying 92 percent of the facial expressions tested compared with the 81 percent identified by control subjects. Their success probably stemmed from their simulation expertise rather than a general agility with faces because further experiments showed they were no better than controls at recognizing a persons identity.
For the rest of us without mirror-touch syn­esthesia, the simulation process is the same but less pronounced, Banissy says. So the next time you find yourself sympathizing with someone who looks sad, thank the part of your brain that feels you frown.
I enjoyed reading this over, when we face trials, illness, over outward expressions can speak volumes and usually we say were doing ok or good!  So be honest with how you feel!
Thanks to Michele for letting me pass this on to you.
Amy Chapman

A Day At The FDA

Tuesday - June 21, 2011 @ 19:39:03

I was part of the Afamelanotide trial last summer and was fortunate enough to have received the real thing. Unless you have EPP, I cannot explain what a miracle that was. But I did get the chance recently to do just that at the FDA Office of Orphan Diseases. Another fellow EPPer, Matt Johnson, joined me. In my 62 years I had never met anyone with EPP outside of my family (I have two cousins with EPP). That was an experience in itself. Those of us with EPP know that we can never explain to someone without EPP what it is like, but listening to Matt Johnson was like reading my own thoughts. Matt and I spoke a couple of times during telephone conferences before we actually met at the FDA in Silver Spring, Maryland. Although our session was scheduled for 10:30am, we were asked to come early to meet with Dr.Timothy Cote, the head of the Office of Orphan Diseases. I arrived just before 9am and Matt was close behind. Dr. Cote had just returned from Israel two day prior and was sick the day before, so he was not in good spirits, but after a few minutes listening to Matt and me, he was beaming. He told us that his staff, like all bureaucratd, tends to lose site of their mission and was delighted that we were there to remind them that we are out there.

Dr. Bob Desnick, a member of the APF Advisory Board, was also to attend, but his flight from New York delayed him an hour. By the time we were ushered into the conference room over 30 doctors and health professionals were waiting to hear what we had to say. For an hour Matt and I regaled them with the inexplicable world of EPP and the miracle of Afamelanotide during which time we were videotaped. We tried to tell them of the unrelenting pain, the isolation and self-doubt, and ultimate despair. Then we told them about our experience Last Summer and how it had changed our lives. We weren't supposed to directly discuss this, but it just couldn't stay untold - so we did. Dr. Desnick translated what we had to say into meaningful medical speak, but I believe that our words had more impact.

Like most families, we have gone to the beach almost every summer, and every summer after 15 minutes, I am banished to the house and sit with my feet in a lobster pot of ice water for the next 5 days sipping copious amounts of adult beverages. Needless to say this is hardly endearing to my wife and makes everyone wonder. But last summer, I spent hours on the beach with virtually no pain a certified miracle if ever there was one. Like most EPPers, I have never told anyone outside of my immediate family about my condition. My colleagues at the office began referring to me as Mr. Tan without ever knowing why.

But the most compelling thing that day at FDA was a video that Matt had brought. It was a video of the episode that led to his diagnosis. He was 11 at the time and had gone skiing with his family. The video showed the progress of the effect of EPP over the period of about 10 days. As most of EPPers know, even though the pain has past, we are not a pretty site after 10 days. It had a profound effect on our audience.

Drs. Cote and Mueller thanked us for our time and promised that they would advocate our case to the extent they could, but could promise nothing. They explained that there is concern that the drug would be used for off label usage and that it would take time and effort to weigh the risks and benefits of a drug such as Afamelanotide. Dr. Mueller told us that she would share the video of our session with the FDA Center for Drug Evaluation and Research. I believe that this was a first and significant step in getting approval for the use of Afamelanotide, but I am not sure that the end is in sight. I live 45 minutes from the FDA campus and with the help of APF will continue to do whatever it takes to gain approval for use of Afamelanotide.


Monday - June 13, 2011 @ 23:42:53

       I would like to let you all know how wonderful the Porphyria foundation has helped me.  I always love to help in any way I can and I think most of you are that way to, but have you asked yourself what is your limit?  Do you know you limit?  Do many people pull you in all different directions?  When we feel great or good, we pack everything that needs to be done or want to do all in one day, reminds me of going on vacation to see and do everything in one trip because you may not get back there. You all know what I mean.  Many people that have a disease wear T-shirts, bracelets, raise money, run races and so much more.  I see so many people get depressed and have low spirits because with Porphyria there are different types that affect us in so many ways and does affect our everyday lives.  So if you find yourself in that downward state what can you do to be happy?
·        Find an activity to do, read a book, paint, crafts, write your thoughts down in a journal
·        Exercise, stretch, go for a short walk this you can do inside or out
·        Could you take a day trip, favorite place to shop, visit a museum, go to the zoo
·        Take a vacation give yourself a break even if its a stay home vacation
·        Reach out and call a friend or family member, write a letter and mail it  It would touch some ones heart (old fashioned I guess)
·        Do something with your friends inside or outside, grill make a lunch or dinner date
·        Spend quality time with your family
So if youre down today think of these fun things to do!  The most important thing is to get up and do something and have your life be in balance.  You can always volunteer to spread the message about the APF and send them a letter or note to thank them for all that they do.

Amy Chapman- AIP Patient

Searching for a Good Doctor.

Wednesday - June 8, 2011 @ 16:16:15

I was thinking today, Oh another new Doctor today.  I was not happy nor Sad, but I thought Ive got to explain Porphyria againâ?¦.I could do this in my sleepâ?¦. I was thinking.  I had to be honest I had a bit of a negative attitude.  I think we have all felt this way at one time or another, how is the new Dr. going to treat me are they going to understand me, believe me, look at 
me weird, think Im crazyâ?¦

I had all my Papers and booklets and Binder ready to go.  I was so nervous and this in turn made me not feel so well, but said to myself and prayed that I could have the strength and confidence that I could get through this.  I got there and waited they did everything so fast {get you in and out}.  Waited for a bit and then it was my time to see the Dr.

The Doctor shook my hand introduced herself to me and said you have a rare disease I was told, what could you possibly have? She asked!

I told her, she looked at me with the crazy look and then excused herself and said she would be right back.  She came back a bit later, she said she had to go look this Disease up because she had heard of it in school but never thought she would see anyone with this condition.

She said she could help me but she really did not want to.  I told her that we hear that sometimes.

But this was # 6 of searching for a new Dr. I was still sick.  My mom happened to be on the phone to find me someone to see.  Ok we may have someone.  All those feelings all over again.  Those of us that go through this so often.  Made an appointment for another new Dr.  Checked In for my Appointment.

I could not clearly understand him, but he had a sincere smile and seemed to be very concerned about me.  He listened to us without questioning us as my mom, sister also have AIP.  He looked at my test results from Dr. Anderson made contact with him, had me meet the Pharmacist and they ordered me what I needed to be well.  I had a total of 12 different nurses 2 Doctors and 2 Pharmacists we all learned together and now we have a system.

When you find that special Dr. dont ever take them for granted tell them how much you appreciate from the small things to the large items, they will help you for a long time.  That brings me to think of how we have Dr.s today for Protect the Future Program may we Thank old and new for all their time experience and investment for Porphyria patients everywhere.

Thank You!  Amy Chapman AIP Patient

Honesty is the Best Policy

Wednesday - June 8, 2011 @ 15:56:15

I was a much more private person before I got sick nearly two years ago. I didnt feel the need to tell too many people that I could get sick with porphyria-- after all, I assumed I wouldnt. And for what its worth, Im a very lucky person-- when I got sick, I was able to manage it through a number of activities (which I may detail later), and I got my life and health under control, for now.

But boy, was it ever so hard having to explain to friends, acquaintances, and bosses why I was sick. It was even more difficult because I didnt look sick. Someone once said to me, You look fine now. She was suspicious. I was as bubbly as ever, which was mostly an act-- and not everyone is an expert on health by looking at someone. For a brief second I felt badly that I didnt look sick enough, until I realized whether or not people believed me was irrelevant. The only people who needed to believe me were doctors, and thats another story, another issue for another day.

Yet I dont regret a single choice I made to actually talk about my sickness. If I was unloading too much information on one of my bosses, then they could deal with feeling awkward; so long as they understood that I was tired and anxious and sick all the time, then they could help make my life better. If friends didnt want to talk to a sick girl, then they werent friends. If I made acquaintances feel uncomfortable by being sick, then they had to deal with their own fear of mortality, not mine.

So, next time someone asks how youre doing, and you feel utterly terrible and sick, tell the truth. Its hard. But in the end its best if people know your situation, where youre coming from, and why you write gripey blog posts about honesty being the best policy!

--Miranda Dennis

Glamorous (Nonalcoholic) Drinking

Wednesday - June 8, 2011 @ 15:32:40

Sometimes I want to be like Audrey Hepburn in Breakfast at Tiffanys, decked out in Givenchy, throwing wild parties (and singing Moon River on my fire escape) and holding a glass of champagne while trying to chat up someone with a name like Rusty Trawler.

Of course, the reality is that 1.) smoking cigarettes (like in the picture), even on long elegant cigarette holders is terrible for everyones health, 2.) wild parties sometimes end with cops being called, as the guests often find out, and 3.) drinking alcohol is certainly a huge no-no for someone who has porphyria.

Still, I like to hold wine glasses.

So, why not be luxe and still invest in those nice glasses, even if you cant drink the wine? My favorite non-caffeinated drink is usually pure cranberry juice cut with seltzer water. It sparkles brightly, and it leaves me feeling pure and happy. Glamor, like so many things, is a cultural phenomenon constructed from objects and feelings; why not transfer those objects and feelings to a nonalcoholic world where no one ends up dead or in the hospital?

Do yourself a favor: scour or or just check Google. Look for nonalcoholic beverages. Or experiment in the kitchen. Invest in sparkling water (or even a machine that carbonates water for you), and live a life that's full of health. And in the credits to the movie adaptation of your life, thank me! I'll be the one in the audience, sipping lemonade from a goblet.

--Miranda Dennis

Om: Yoga For Managing Stress

Wednesday - June 8, 2011 @ 15:13:05

I was taking a walk in Fort Greene park in Brooklyn when I saw a man doing yoga outside. I stealthily snapped a picture of him, not because it was a bizarre sight (sometimes public yoga can be), but because I thought that was so awesome of him. If one of the best ways to lead is by example, then what an extraordinary leader!

His calm and poise reminded me to go home and unfold my own yoga mat and do stretches:

I'm a very lucky girl. I have gone to Golden Bridge Yoga in Los Angeles and seen Gurmukh herself instruct people on kundalini yoga. When I lived in Amherst, Massachusetts I had an excellent hatha yoga instructor at Yoga Center Amherst. I have great DVDs for my home viewing, and I've a good memory for certain moves and postures. I am able to do my own stretching on a budget! My own meditation on a budget!

The great thing is there's a lot of access to free yoga online ( has a free class each week online). If you have Netflix, request a video! Drop in on a class for a reduced fare. If you learn from books, get a book-- the library will have videos and books. Hurrah! And I really recommend it, especially for people who suffer from any illness where stress is a trigger-- like porphyria.

Whenever I start to feel ill or run-down, I take the time to stretch my body, to meditate, to practice on my breathing, to really try to center myself in my body. A lot of people may scoff and say, "That sounds really New Age and stupid!" But there's a reason people of all faiths, politics, and incomes have started to gravitate towards yoga; it works. The body wants to heal, so let it!

--Miranda Dennis

Sugar My Friend. Sugar My Enemy.

Tuesday - June 7, 2011 @ 16:58:43

It would be easy-- and probably a lot of fun-- to pretend that having acute intermittent porphyria gives me free reign to eat all the carbs and sugars I want. But interestingly enough, after being officially diagnosed with porphyria, I started to make a lot of lifestyle changes and dropped weight at a slow but steady pace. And I didn't alter my carbohydrate intake a single bit.

I owe much of this to my switch from processed sugars to more natural sweeteners like agave or honey, supplementing my choice to use raw turbinado sugar. Every morning I usually add a spoonful or two of sugar to my French Press coffee pot, to cut the bitter and to leave a light sweet taste on my tongue. The turbinado warms to an almost molasses taste-- you can tell because it's brown, not white, that it'll be closer to caramel than just pure white sugar. Life's good for me. I make tea or bake and use agave nectar, which (according to various sources on the internet, which isn't always right) has one of the lowest glycemic indexes of sweeteners out there.

And now that a heat wave has struck NYC where I live, I have a few ice cream sandwiches tucked away in the freezer. But here's the deal: they're made from soy products and have no cholesterol. They're tiny, too. That's why they're called Cuties:

I'm not saying this as diet advice-- eat a bunch of Cuties soy ice cream sandwiches and put raw sugar in your coffee. I'm saying that I try to focus on various forms of sweeteners, various ways of getting sweets. Not featured, of course, is the best way to get your sugar fix: a banana. Fruits and veggies offer the best sweets available, the kind that does the body good.

Just because we have porphyria doesn't mean we should eat really delicious donuts covered in frosting and delivered by unicorns (okay, every once in a blue moon is okay), but we can have our sweet moments that are good for us without compromising our health. After all, too much unhealthy eating and sugar can lead to type 2 diabetes and obesity, which would further complicate porphyria problems. We must stay vigilant! But sweet!

--Miranda Dennis


Tuesday - May 31, 2011 @ 18:28:33

Recently our Facebook group page got a virus. Please be aware of the malacious viruses that can slow your computers and infect them with Trojans and worms. As a result of the misfortune we have had with FB we have not been able to reach many of our group members. Some were even locked out. These are just a few of the issues we have encountered. Please continue to follow us as we appreciate your support.

Subject: Kindness Despite Being Ill Part 2

Tuesday - May 10, 2011 @ 10:22:23

Dear APF Members,

I wanted to share a thought when we have bad days and we have pets, do we
ignore them, love them, and pay any attention to them?  Well I have three
dogs and usually I don't feel my best.  They each have their own
personalities, funny things they do but if we don't feel good we may at
times put them aside.  They can sense when we don't feel right, remember
that we never need to get upset with them because all they want is love and
affection when we don't feel right and our approval just as we seek it from
our family and friends. In return by showing this kindness it brings all of
us happiness and some measure of contentment.

Show kindness through loving animals and the living world. Loving animals
and caring for pets is kindness in action. Nothing compels you to care about
beings of another species, especially in a day and age where the tools of
human domination are so powerful. And yet, the very act of loving an animal
and respecting the animal for its own value is an expression of deep
kindness. As well, being kind to the world that sustains and nurtures us is
sensible as well as kind, ensuring that we don't poison the very elements
that assure us a healthy life.

o    Adopt or foster a pet. Your kindness will be rewarded by letting
another being into your life that will bring you joy and love.

o    Offer to pet-sit for a friend who is going away. Give your friend the
reassurance that someone loving and caring will be tending to her pet while
she's away.

o    Respect the species you're caring for. Humans don't "own" animals;
rather, we stand in a relationship of being responsible for their well-being
and care.

o    Take time to restore parts of your local environment with the local
community. Go for walks in nature with family, friends, alone, and commune
with the world that you're a part of. Share your love for nature with
others, to help reawaken their sense of connection with nature.

Practice the kindness effect

o    Practice kindness and generosity toward others. Being out of practice,
being shy, or not knowing how to reach out to others can only be overcome in
the doing, by continually trying until it becomes a natural impulse to be
kind and giving to others.

o    Ask for nothing in return. The greatest kindness expects nothing, comes
with no strings attached, and places no conditions on anything done or said.

o    Meditate to help spread kindness

Extend your kindness to people who are not "in your neighborhood" Look at
all the wonderful things we have done with Porphyria Awareness Week Local,
National Media, we shared this with Dr's, family friends, neighbors, maybe
we went the extra mile got out of our comfort zone to spread the word.

"Be kind, for everyone you meet is fighting a hard battle". Attributed to
Plato, He was mentioned in this book I was just reading~ this saying is
recognition that everyone is undergoing some challenge or other in their
lives and that sometimes, it's all too easy for us to lose sight of that
when embroiled in our own problems or anger against them. Before committing
an action that might impact another person negatively, ask yourself a simple
question: "Is this kind?" If you cannot answer this in the affirmative, this
is a reminder to change your action and approach immediately.

o    Even where you're feeling at your very worst, remember that other
people are also feeling uncertainty, pain, hardship, sadness,
disappointment, and loss. In no way does this belittle your own feelings but
it does allow you to realize that people often react from their hurt and
pain rather than from their whole self, and kindness is the key to seeing
past the raging emotions and connecting with the real person inside.

o    Consider the adage "be cruel to be kind". Think about why this saying
is so popular. Do you think it is an appropriate way to view people's
situations? When you believe that someone truly needs to learn a lesson,
often one involving standing on their own two feet, one of the greatest
kindnesses that you can do for them is to withhold your judgment and to go
the extra mile to do things for them that will enable them to make the
changes or leaps of faith that they need to do, without actually trying to
make that change for them. We're all well aware that we cannot change
another human being. But kindness allows us to enable things to change
around them so that they can make the necessary changes for themselves.
Which means that we don't need to view our act as "cruel"; rather, it
becomes an act of "enabling".

o    If you're neglecting being kind to someone else just because you think
they can cope without your support or understanding, then you're practicing
selective kindness.

"Carry out a random act of kindness, with no expectation of reward, safe in
the knowledge that one day someone might do the same for you." These are the
words once said by Princess Diana. The practice of random acts of kindness
is alive and well as a conscious effort to spread more kindness, I know that
this reminder on kindness has helped me immensely in my battle with
Porphyria, how I speak with people how I view myself as a person and most am
I doing the Best that I can each and every day?  Do something today for
someone else your heart will be so happy!

From Amy Chapman AIP Patient


Friday - May 6, 2011 @ 10:46:16

DAY AT THE FDA with Mike Kenworthy,Ph.D and Matt Johnson

I was part of the Afamelanotide trial last summer and was fortunate enough to have received the real thing. Unless you have EPP, I cannot explain what a miracle that was. But I did get the chance recently to do just that at the FDA Office of Orphan Diseases. Another fellow EPPer, Matt Johnson, joined me. In my 62 years I had never met anyone with EPP outside of my family (I have two cousins with EPP). That was an experience in itself. Those of us with EPP know that we can never explain to someone without EPP what it is like, but listening to Matt Johnson was like reading my own thoughts. Matt and I spoke a couple of times during telephone conferences before we actually met at the FDA in Silver Spring, Maryland. Although our session was scheduled for 10:30am, we were asked to come early to meet with Dr.Timothy Cote, the head of the Office of Orphan Diseases. I arrived just before 9am and Matt was close behind. Dr. Cote had just returned from Israel two day prior and was sick the day before, so he was not in good spirits, but after a few minutes listening to Matt and me, he was beaming. He told us that his staff, like all bureaucratd, tends to lose site of their mission and was delighted that we were there to remind them that we are out there.

Dr. Bob Desnick, a member of the APF Advisory Board, was also to attend, but his flight from New York delayed him an hour. By the time we were ushered into the conference room over 30 doctors and health professionals were waiting to hear what we had to say. For an hour Matt and I regaled them with the inexplicable world of EPP and the miracle of Afamelanotide during which time we were videotaped. We tried to tell them of the unrelenting pain, the isolation and self-doubt, and ultimate despair. Then we told them about our experience Last Summer and how it had changed our lives. We weren't supposed to directly discuss this, but it just couldn't stay untold - so we did. Dr. Desnick translated what we had to say into meaningful medical speak, but I believe that our words had more impact.

Like most families, we have gone to the beach almost every summer, and every summer after 15 minutes, I am banished to the house and sit with my feet in a lobster pot of ice water for the next 5 days sipping copious amounts of adult beverages. Needless to say this is hardly endearing to my wife and makes everyone wonder. But last summer, I spent hours on the beach with virtually no pain a certified miracle if ever there was one. Like most EPPers, I have never told anyone outside of my immediate family about my condition. My colleagues at the office began referring to me as Mr. Tan without ever knowing why.

But the most compelling thing that day at FDA was a video that Matt had brought. It was a video of the episode that led to his diagnosis. He was 11 at the time and had gone skiing with his family. The video showed the progress of the effect of EPP over the period of about 10 days. As most of EPPers know, even though the pain has past, we are not a pretty site after 10 days. It had a profound effect on our audience.

Drs. Cote and Mueller thanked us for our time and promised that they would advocate our case to the extent they could, but could promise nothing. They explained that there is concern that the drug would be used for off label usage and that it would take time and effort to weigh the risks and benefits of a drug such as Afamelanotide. Dr. Mueller told us that she would share the video of our session with the FDA Center for Drug Evaluation and Research. I believe that this was a first and significant step in getting approval for the use of Afamelanotide, but I am not sure that the end is in sight. I live 45 minutes from the FDA campus and with the help of APF will continue to do whatever it takes to gain approval for use of Afamelanotide.


Tuesday - April 26, 2011 @ 09:44:26

Since Panhematin has beeen on the news for the last month, I thought I would tell you my first expereince with the drug. When I took it so many years ago, it had not been FDA approved and subsequently becem the first ORPHAN DRUG.

That story will come at a later date.

. My doctor at NIH, Dr. Lamon , arrived at my room . I was wide-awake and ready for what I hoped would be good news. Dr. Lamon said he thought that I might benefit from treatments with infusions of hematin; however, the FDA had not yet approved heme therapy for the acute Porphyrias. Evidence from years of prior research indicated that hematin was effective and might enhance my recovery.
Since I had been very ill for quite some time, Dr. Lamon warned me that hematin was sometimes not as effective when given far along in an attack as I was then. Having never been one to stand and wait for anything, I certainly didnt want to think more than that moment to tell him that I was willing to try the hematin as soon as possible. Participating as a research patient was also an intriguing proposal. I concluded that having the hematin infusions would provide me with the chance to benefit from the use of an experimental drug while being able to help someone else in the future. I liked that idea, too, so I was ready to begin the treatment.
I later learned that the rationale for administering heme therapy is to correct a heme deficiency in the liver and repress production of porphyrin precursors. Since the first report in 1971, numerous patients were given heme therapy and clinically benefited from the treatment. Although there have been no controlled studies, the reported results were impressive enough to advocate the early use of heme therapy for porphyric attacks. When heme therapy is given late in an attack, no substantial benefit may occur; but if given early, the heme therapy almost always normalizes ordinary porphyrin and porphyrin precursor values.
If a patient with signs and symptoms of a Porphyria attack is not stabilized within a day by glucose and other care, most specialists recommend the administration of heme therapy. Usually, a dose of 3-4 mg/kg, given once daily for four days, produces a highly beneficial effect in most patients. A decrease in pulse rate, blood pressure, abdominal pain and decreased levels of urinary porphobilinogen are commonly noted and can occur within a day. At the time, I didnt know what would happen, but I was certainly ready to try.
The following morning Dr. Lamon walked into my room holding an extremely large syringe full of hematin for me to see. I gasped and remarked that the concoction looked like swamp water, a black/green color. Some patients have since told me that they thought that hematin looked like crankcase oil or a prop in a Frankenstein movie. Regardless, I was ready to receive the infusion. At that time, Dr. Lamon infused the remedy into a vein in my arm with a butterfly needle and a very large syringe. He repeated the process daily for five days.
The hematin may have looked like swamp water or crankcase oil, but it was 24K gold to me. After only a few infusions, I improved dramatically despite the fact that I had been ill for a number of weeks. Relief from my constant pain and nausea occurred quickly, but the extreme weakness remained for months thereafter. (Hematin was the first drug approved by the FDA for rare diseases under the Orphan Drug Act many years ago. It became available from Abbott Laboratories in Chicago under the trade name Panhematin. It has since been sold and is now available through Lundbeck, Inc , which purchased Ovation Pharmaceuticals ..)
My doctor now gives me Panhematin when I have an attack. . Since many Porphyria patients asked how my doctor arranged for the treatments, I have included the following description of the usual scenario of the infusion process. The IV infusion is much different now than it was when Dr. Lamon infused the hematin by hand with a large syringe and a butterfly needle.
Now when symptoms of an attack of AIP begin, my docotr immediately orders Panhematin through the hospital pharmacy. Some people take it at local infusions centers , in their doctors offices and soem who are on home care take it at homeMost of the time, the hospital pharmacy or whatever the source does not maintain Panhematin on hand. Therefore, it is ordered from Lundbeck and is shipped immediately from their emergency supply. In fact, it can arrive within a matter of 12 hours since they keep a supply neaar the airport in Chicago.

More to come .

How to Be Kind Despite Having an Illness

Wednesday - April 6, 2011 @ 13:10:43

Being kind is a way of living that keeps giving long after the kind thoughts, words, and actions have taken place. Kindness is a force without force, and it goes well beyond manners to the very heart of how people respect and treat one another.
Being kind is a vital way of making our own lives, and the lives of others, meaningful. Being kind allows us to communicate better with others, to be more self-compassionate, and to be a positive force in other people's lives. Kindness has its true source deep within you, and while some people are innately kind, it's something that everyone can cultivate by choice.
Understand what kindness is and is not. At its most basic, kindness is about caring genuinely for others around you, wanting the best for them, and recognizing in them the same wants, needs, aspirations, and even fears that you have too. Kindness is warm, resilient, patient, trusting, loyal, and grateful. Sometimes when we are sick we may not show kindness in the usual way. Due to pain, suffering, lack of sleep and energy may all play a part.
Ultimately Kindness is deep caring for all beings.
Be kind to yourself. Many people make the error of trying to be kind to others while not focusing on being kind to themselves. Some of this can stem from not liking aspects of yourself, but more often than not, it's sourced in the inability to know yourself better. And unfortunately, when you don't feel rock solid within yourself, your kindness to others risks falling into the deluded types of kindness it can lead to burn-out and because you've put everyone else first. Self-knowledge allows you to see what causes you pain and conflict, and enables you to embrace your contradictions and inconsistencies. Take time to become more self aware and use this learning to be kinder to both yourself (remembering that we all have weaknesses) and to others. In this way, your inner angst is being dealt with rather than fueling your need to project the hurt and pain.
o Avoid viewing time taken to become more aware of your own needs and limits as an act of selfishness; far from it, it is a vital pre-condition to being able to reach out to other people with great strength and awareness.
o Ask yourself what you think it means to be kinder to yourself. For many people, being kinder to themselves includes monitoring the negative chatter that involves putting yourself down and stopping your negative thinking.
o If you have a tendency toward perfectionism, competitiveness, or a driven sense of urgency, self-kindness can often be a victim of your ambition and fast pace, as well as your fear of being seen to be lazy or selfish. Remember to slow down and to forgive yourself when things don't work out as wished.
o When I think of any of the Porphyrias, when were in an attack~TO DO LIST we wish to get things done and they race over and over in our minds. We simply have to let it go and think about getting well~ even though we always want to do the work ourselves and try to put the pain aside.
o Learn from your mistakes rather than beating yourself up over them, or comparing yourself to others. It is through self-compassionate responses that you can start to see other people's needs in a compassionate light.
Be present: Family, Friends and Caregivers and Medical Doctors and Nurses: The greatest gift of kindness to another person is to be in the moment in their presence, to be listening with care, and to be genuinely attentive to them.
o Be a good listener. Often said, yet the act of listening is easier said than done in our fast-paced world, where rushing and being busy are seen as virtues; where cutting someone off because you're too busy, or you need to get somewhere in a hurry, is the norm. Making being busy into a habit is no excuse for unkindness, however. When talking to someone, learn to listen with your whole being and sincerely pay attention to them until they're done revealing their thoughts and story.
o Schedule your day differently, so that you're not known as the person who always rushes off. Being present means being available; you can only do this if you're not rushing or squeezing in people and activities.
o Take time to connect with people face-to-face, or via an uninterrupted phone call. Send a letter instead of an email and surprise someone with the kindness of your having taken time out of your day to put pen to paper. How nice it is to see us encourage each other with Facebook send caring thoughts to each other to get better and stay well and most of us have never met each other in person! Because we took the time to listen.
2. Be happy, joyful, and grateful. And keeping your sense of humor ensures that you don't take yourself too seriously and take life's contradictory and contrary moments with good faith.
Reflect on the kindness of other people. Think about the truly kind people in your life and how they make you feel. Do you carry their warm glow around in your heart every time you think of them? It is likely that you do because kindness lingers, warming you even when the hardest challenges face you. When other people find a way to love you for who you are, it's impossible to forget such trust and confirmation of worthiness, and their kindness lives on forever. So ask yourself what was the last kind thing someone did for you? How did it make
Cultivate kindness for the good of your own health. Improved Health and happiness comes from thinking more positively, and kindness is a positive mental state. While kindness is about giving and being open to others, giving kindness returns a sense of well-being and connectedness to us that improves our own mental state and health. This is just part 1 of a series to be Happy Healthy show Kindness to everyone! Pass it on!
By Amy Chapman AIP Patient

Attack Questions #3

Wednesday - April 6, 2011 @ 12:55:39

Other patients have asked me why the attacks did not return right away after I ceased taking the injections. I do not know, but I have an idea that cannot be scientifically validated. I believe that my body got a break from the perpetual onslaught, thus, giving it time to recover some fighting ability. A better example is that a wound can heal completely over time but, if it receives repeated blows, it cannot heal until the blows stop. The injections kept me from the repeated strikes of the Porphyria attacks that inevitably would land me back in the hospital. As I indicated previously, the scientific validity of my supposition cannot be established, but I was finally enjoying my almost-pain-free life.

It was a wonderful time for me. Life was normal like other people. I could climb mountains, ski, walk three miles a day, go to the gym and work out, attend work-out classes, live like other people. It was wonderful. In fact, most patients who attain this stability in their lives are able to maintain the normal, happy, pain-free, symptom-free lifestyle.

Those days lasted about ten years. I had attacks that were painful, but they didnt last a long time and they decreased in intensity and frequency. If others of you are having trouble, take joy in the fact that this disease will let go. In fact, it may let go forever and you will be attack free. That is not a far fetched thought.

What I also learned during the experience of the bad years was that I had been amazingly blessed with brilliant, compassionate doctors and Dr. Anderson was one of them. If I had the next decade to tell all of the wonderful acts he has done for me, I could fill every second of every day telling about his goodness. He is such a humble genius that he takes no credit for any of his greatness. Personally, I owe my life to him, as do countless other pat

The Foundation of the APF.

Wednesday - March 23, 2011 @ 12:13:25

I want to tell you about the people who are the FOUNDATION of the APF. JJ My life and yours would have or could have had a tragic ending without the hard work of the people I have written about in the following paragraphs. There are so many other scientists, physicians and lab technicians that have enhanced our lives and improved our health but there is not room enough on the pages of this book to name them all and to enumerate their achievements. Know that they are in laboratories, hospitals and research centers all over the world and that without them, to be sure, your life would be filled with more sickness or pain.

You can support them first with your prayers. They are the most powerful and least used force in the universe. Next, you have the opportunity to support them with your finances. There are plenty of research projects that will change the Porphyria world but they need money to be completed. The APF has separate accounts for research projects. You can donate funds that will go specifically to those projects by noting your request in a letter. You can take joy in knowing that your participation by praying and funding one of these projects has lessened the suffering of Porphyria patients throughout the world and that you have helped the following individuals and those not listed to accomplish their life-changing goals. You can make donations by credit card or check.

Our first Chairman of the Scientific Advisory Board was Dr. Karl Anderson, who is board certified in both Internal Medicine and Gastroenterology, an ideal combination for the acute Porphyrias. He was at Rockefeller University when I met him and first became his patient. A few years later, Dr. Anderson left Rockefeller to become the Director of the Porphyria Center, the Director of the Division of Human Nutrition in the Department of Preventive Medicine and Community Health and the Associate Director of the General Clinical Research Center (GCRC) at the University of Texas Medical Branch in Galveston, Texas. In that capacity, he developed the GCRC's training curriculum in clinical investigation, including developing the course Methods in Clinical Investigation. Dr. Andersons research focused on the effects of diet on drug and hormone metabolism, the human Porphyrias and drug metabolism in cirrhosis.

His Porphyria-specific studies include treating the acute Porphyrias with heme arginate, GnRH analogues and erythropoietin, as well as studying the roles of vitamin C and hepatitis C in PCT. Recently he began researching the effect of an enzyme replacement for the Porphyrias, which is manufactured by Hemebiotech, a company based in Denmark. Dr. Anderson co-chaired an advisory panel of experts for the APF, which was established to enhance awareness of the Porphyrias among primary care physicians and to help them improve diagnosis and treatment of the disease.

He has been my doctor for over twenty years. I can hardly speak or write about him without becoming emotional. Even though we have also become friends and colleagues for twenty years, I continue to call him Dr. Anderson rather than Karl. For me, I call him Doctor, because it is a revered title and the most outwardly respectful thing I can do. To be a doctor is a high calling. It involves healing, a God-given ministry and that is what I feel about Dr. Anderson. He is a ministering physician, an instrument of God; the kind of man about whom the Lord can say, Well done, my good and faithful servant.

It would take another entire book to describe Dr. Andersons many kindnesses to patients all over this country and other parts of the world. In fact, I can say the same about all of our board members. They all have served the Porphyria patient community with very little compensation.
Dr. Anderson is also one of the physicians who are training future experts . To enlighten you on this very important program:

At the formation of the American Porphyria Foundation in 1980, the founders sought the most renowned porphyria experts in the country to serve on the Scientific Advisory Board. These individuals were identified and selected on the basis of their training, experience, peer-reviewed publications, research, attendance at international meetings and knowledge as experts among other physicians in the field of metabolic diseases. These have provided expert advice to the Foundation and its members continuously over many years.

Members of the Scientific Advisory Board of the American Porphyria Foundation are medical pioneers in the field of porphyria and have led porphyria research, testing and treatment for the past thirty years. This prestigious group of porphyria specialists are currently diagnosing and treating patients, as well as acting as consultants to their primary care physicians. These experts also developed the medical publications in the American Porphyria Foundation brochures, website, newsletters and E-news updates.

In addition, these experts have published the major medical texts and journal articles related to porphyria, including the well received article, Recommendations for the Diagnosis and Treatment of the Acute Porphyrias, published in the March 2005 issue of the Annals of Internal Medicine. The American Porphyria Foundation has received over one thousand requests for copies of this particular article.

Over the past few years, such publications have dramatically increased the level of porphyria diagnosis, which, in turn, increased the need for expert consultation, medical publications and educational programs among primary care physicians. These newly diagnosed porphyria patients and their treating physicians rely on the American Porphyria Foundation and the board of specialists for the best available approaches to diagnose and treat porphyria.

Most of the present porphyria experts received their training through government funding. However, because of budget cuts, all rare diseases had little access to the type of government funding that was available in times past. Since porphyria is categorized as a rare disease, the funding opportunities and financial incentives to enter the field are few. These and other financial restraints on medical funding will in the future create a potentially disastrous situation for patients and their primary physicians as the number of present experts decreases and the funding to train their replacements is very limited.

The American Porphyria Foundation is acutely concerned about the evolving scarcity of porphyria specialists. Therefore, in December 2004, the American Porphyria Foundation established the Protect The Future educational program to attract and train the next generation of porphyria specialists. Our Protect The Future program is a major means of expanding the present group of porphyria experts and practitioners while preventing a future lack of experts to serve the Foundation and its members on the Scientific Advisory Board.

At present, the APF has supported the training of ten experts via the PTF program. Most of the PTF doctors are on their way to Cardiff to attend the International Porphyria Meeting where experts gather from around the world to share their expertise and research projects.

*Dr. Anderson is co chair the Consortium of researchers

It is an exiting time!!!!

Awareness Week/ Conference Call

Tuesday - March 1, 2011 @ 11:30:43

Reminder ---Conference Call with an Expert

The APF will be hosting a Conference Call with a Porphyria Expert, Dr. Herbert Bonkovsky, who is renowned in the world of porphyria as a clinician and researcher. This is scheduled for Saturday, the 2nd of April, 2011. It will be from 1:00 eastern
until 3:00 p.m eastern time. This conference call is free to members of the APF and is $10 to all non members.
We encourage you to join, so as to benefit from a forum like this. Your support is
shown through the $40 suggested annual donation. To register, call the APF
TOLL FREE at (866) APF-3635 or (866) 273-3635. All wishing to participate
in the phone call must register prior to the conference call. Upon registering you
will receive dial in instructions.
After a short presentation about the porphyrias, Dr. Bonkovsky will field questions from the callers. If you are an APF member and for some reason you cannot be on the call, please send your questions to Annie Pangle at the APF :

The calle is free to APF members and $10.00 for nonmembers. We do encourage you to join the APF so that you can participate in the call for free but also help educate other patients and physicians, as well as support research and training future experts.

Listen for Radio Public Service Announcements on Porphyria
If you are a radio listener, listen for the Public Service Announcements (PSA) on porphyria. In connection with National Porphyria Awareness Week, Desiree Lyon Howe, Executive Director of the APF , was selected to tape a commercial encouraging people with unexplained abdominal pain to "Think Porphyria" and get tested.

APF members and National Porphyria Awareness Week

APF members are already working hard to promote porphyria awareness. Some are setting up medical seminars, grand rounds, hospital in-service, and other physician education programs. Others are approaching their local media to tell their own stories. For example, the Cooks in Texas are

Several of the television programs featuring porphyria recently aired. Within a 24 hour period, we received a rash of calls from people who thought that they might have the disease. In turn, the office sent them information on the disease and sent their doctor a packet on diagnosis and treatment. Hopefully, the television exposure was responsible for finding more people with porphyria and ending their cycle of pain and illness.

One woman who called mentioned her horrible bouts of abdominal pain, rapid pulse and "brownish /reddish/purple, tea like " urine. She went on to tell us that her doctors had given her almost every tests known to man and that she had been in the hospital more than home for the past year. After viewing a recent Medical Mysteries, she contacted her doctor and is being tested for AIP.The media is POWERFUL

Your local media is often looking for a story, particularly around an event like Porphyria Awareness Week. You can use the local media to promote porphyria awareness by telling them about your own case. A number of APF members are participating with their local media to either film a daytime show or a newspaper article as part of Porphyria Awareness Week.

If you would like to receive materials for your event or media venue, please contact the APF.

About Sypmtoms.

Tuesday - February 22, 2011 @ 10:54:50

As a review , my attacks continued intermittently for years, which made my doctors wonder even more about my illness. How could I be so sick one day and a week later be overcome with pain and illness. About 20 doctors later, I gave up. Fortunately, Dr. George Penton believed that I was ill and encouraged me to press forward to a diagnosis. He was further confused when his myriad of tests provided him with NOTHING upon which he could find a diagnosis.
To make matters worse, he gave me a dilantin , a "NO NO " for acute porphyrias. The dilantin caused me to have an attack so severe I was med evacuated me to the National Institutes of Health where I remained off and on for over two years. Then is when the idea of the APF was born in my heart and mind.
A gentleman from Illinois had the same idea , so we started the APF together. The two of were the first members and it has now grown to 4000 members, not withstanding the thousands of other potential patients who reached out to the APF for porphyria information.
Almost all of the people who contacted us had several major questions. First, how can I get diagnosed and where can I find a doctor to treat me. Although we hear those same questions almost 30 years later, we have educated thoussands and thousands of doctors. Even now they can take a free CME course of the acute pophyrias, they receive a very comprehensive kit about diagnosis and treatment , they receive info at targeted medical conventions and via the APF website .
The good news is that we have physicians as a primary focus of our educational programs and our board of experts are willing to consult with them and have been doing this gratis for 30 years. We thank them for this amazing and generous service and for their compassion for porphyria patients that would motivate them to do this for each of us.
We would like to hear your comments on finding a doctor with some knowledge of porphyria. Desiree

More King George III

Tuesday - February 15, 2011 @ 10:47:18

On that same note about King George III, we had another recent occurrence. that gave the APF and porphyria more publicity. Recently, the movie, The King's Speech, was the story of one of the heirs of King George who became King after his brother abdicated, "for the woman he loved." He was the grandfather of the present Queen Elizabeth. Remember that story!!!

Several years ago , producers of the movie called us regarding the porphyria connection to the royal family. The King has a stammer, so they wanted to know if pophyria was a cause. I told them this was another family issue.

Regardless, they gave the APF credit in the movie credits. Which reminds me, we have had more television exposure than any other rare disease. If you watch HOUSE, you will have seen porphyria featured many times. To illustrate , here is a list of shows below, as well as two movies:
*The Madness of King George (1994) & (2005) Movie
* The King's Speech ( 2010) Movie


*House, Honeymoon, Season 1: episode 22

*House, Fools for Love, Season 3: episode 5 (mentions porphyria)

*House, Finding Judas, Season 3: episode 9

*House, Dont ever Change, Season 4: episode 12 (mentions porphyria)

*House, Guardian Angels, Season 4: episode 4 (mentions porphyria)

*House, Whatever it takes, Season 4: episode 6 (mentions porphyria)

*House, Itch, Season 5: Episode 7

*House, Emancipation, Season 5: episode 8 (mentions porphyria)

*House, Let them eat cake, Season 5: Episode 10

*Scrubs, My Ocardial Infarction: Season 4, Episode 13

*Scrubs, Kelsos last stand,Season7: episode 9

*Scrubs, My Dumb Luck, Season 9: episode 7

*Scrubs: Outta here like Vladimir: Season 8, Episode 4

*CSI Las Vegas, Justice Served Season 1 Episode 21

* CSI Las Angles, Once Bitten , Twice Damned Season 2 Episode ?

*ABC News Specials, Medical Mysteries Series, episode 2*

*ABC News Desiree Lyon interview

*ABC Prime Time CEP segment

*Mystery Diagnosis, The Sickest Patient in the Hospital and Terrifying Tremors, Season 5

*Mystery Diagnosis, The Boy Who Kept Swelling, Season 6 episode 6

*Mystery ER, Seeping through the cracks/Purple Haze, Season 1: episode 3

*Secret History: Purple Secret in Search of Royal Madness, Season 6: episode 6

*Montel Williams: Porphyria with Lauren Warren

*Montel Williams, Rare Diseases, Leppert Family

*Travel Channel, Documentary Romania

*Learning Channel Dec 09 Porphyria, another myth in the making.

*Fox News Desiree Lyon interview

*Fox News Vampires , A Medical Myth Nov 2009

*Fox News King George, Madness or Arsenic July 2005

*BBC news Desiree Lyon

*Sanjay Gupta CEP patient, Kasey Knauf CEP

*Anderson Cooper 360 CEP

*CNN documentary on CEP patient, Kasey Knauf

*CNN interview Desiree Lyon (CNN HERO)

*Dr. Oz EPP patient, Craig Leppert

*National Geographic; Feb 2010 Six Ways to Stop A Vampire

*Court TV

*Grays Anatomy, Time Warp Season 6 episode 15

*Castle, , Vampire Weekend, Season 2 , Episode 6


Parade Magazine, Desiree Lyon Howe story

Parade Magazine, Porphyria, Rare Disease Feature

New York Time Magazine-Sunday, Perplexing Pain : Porphria Article

King George III and Porphyria and You

Tuesday - February 8, 2011 @ 10:09:34

Since our last blog mentioned that one way to gain media attention is to enlighten them on the significance of Porphyria and the Revolutionary War. Namely, King George III, often known as Mad King George, was the reigning king of Great Britian and Ireland from 1760-1820. The APF has a letter signed GR to the Lord Chancellor, Kew, Feb. 23rd 1789 and is the one single document in English history in which medical and political history are joined. It is difficult to imagine a more important autograph letter marking a happier occasion in George IIIs life-long battle with the illness that entirely unjustly, saddled him with the epithet Mad King George.

George III was born in 1738, first son of Frederick, Prince of Wales and Augusta. He married his beloved wife, Charlotte of Mecklinburg-Strelitz in 1761. The couple produced a prolific fifteen children: nine sons and six daughters. George III succeeded his grandfather, George II, in 1760 and became one of the longest ruling Monarch of the British crown. Sadly, George III died blind, deaf and mentally ill at Windsor Castle on January 29, 1820.

Since George III ruled during the American Revolution, he was thought by many historians to have had a significant impact on Britain's loss to the revolutionaries. His mental and physical lapses were blamed for much of the mishandling of the war.

King George lost his American colonies south of Canada by the Peace of Paris in 1783 and was notified by his Lord Chancellor, Edward Thurlow. During that period, George had a number of bouts of illness which made it difficult for the leaders to take timely action, including military decisions. His illness created enormous complications for his kingdom. Much pressure was also placed on the royal physicians, who were distressed over their utter inability to diagnose or alleviate his painful malady. The physicians meticulously recorded each attack and in some, described him as arbitrary, capricious, given to fits of rage and to long periods of depression. Occasionally he even had to be restrained physically.

Although he had his first attack at age twenty six, the illness began again when he as about 50 years old. Sir George Baker was the main royal physician at the time, so when King George began suffering another severe bout, he summoned Baker. The

king first experienced severe abdominal pain and constipation, followed by weak limbs, a fast pulse, hoarseness, fever and strange dark red urine. Over a short period of time, he began experiencing a multitude of other symptoms: headaches, visual problems, restlessness, delirium, convulsions, and insomnia. Because his attacks waxed and waned, Parliament was continually debating his ability to rule and continually threatening to wrest his kingdom form him. All of his symptoms were similar to those described by other porphyrics; severe abdominal pain, breathing problems, a rash and muscle cramps.

Part of the problem was that in George III's time, doctors were permitted to do very little to what they called the royal body, so they had to base their diagnoses on what the king told them. Dr. Baker prescribed the standard purgative of that day: castor oil and senna. Instead of the purgatives relieving King George of his symptoms, they made his condition far worse. When the pain intensified, he was prescribed laudanum, which also did not ease the pain. During later attacks, according to notes made by the physician, King George became so agitated and was talking so incessantly and violently that his physicians had to confine him to a straitjacket.

Quizzically, they also mentioned that he had dark reddish-purple stains in his bed clothes during these sieges of physical pain and what they referred to as periods of madness. Since the royal physicians were not permitted to conduct extensive physical examinations, they had to greatly depend on King Georges descriptions of his symptoms. On one particular occasion when he was suffering an exceptionally bad mental relapse, Parliament openly debated his ability to maintain his position as King. Shortly thereafter, he spontaneously recovered, further validating the idea in the minds of many that he was emotionally ill.

By the middle of November, 1788, the accumulated symptoms of the Kings illness had led to government-wide fears for his life and sanity. This forced the Kings first minister, William Pitt the Younger, to propose a Regency Bill, which would have transferred the Kings power to the improvident Prince of Wales, who was the enemy to both Pitt and the King. As this debate proceeded, George IIIs ministers, convened in a cabinet meeting by the Prince of Wales, decided to move the King from Windsor to Kew, a luxurious place of confinement and attempted marginalization nonetheless. Through all this, Lord Chancellor Thurlow, who had been one of the Kings staunchest proponents of his absolute sovereignty over his American colonies during the Revolutionary War, kept his options open with both sides.

Months passed and the King remained at Kew Palace. While there, he endured a humiliating regime at the hands of Dr. Francis Willis, including confinement in a straight jacket and fixed restraint in a chair if he did not precisely follow Willis directions. Because of the nature of porphyria, George III recovered naturally from this attack, which had begun to recede noticeably by January, 1789. When the Lord Chancellor saw that the Kings recovery was likely to prove complete, he jumped fully into the King and Pitts camp, and moved to adjourn the Regency Bills third reading on February 19, 1789. Four days later George III decided that he could notify all his allies and serve notice on all his political enemies that he had fully recovered and was ready to resume full sovereignty.

He suffered a relapse thirteen years later, then again three years after that. The symptoms appeared in the same order, beginning with abdominal pain, fever, and weakness and progressing to mental difficulties. Finally, an attack in 1811 placed him in an apparently permanent stupor. That was the proverbial last straw for the Lords of Parliament. At that reoccurrence his son, George, the Prince of Wales had him dethroned. While he lived for several more years, he continued to experience further episodes of porphyria.

In the letter George III wrote to the Lord Chancellor, in which he relates the details of a meeting he had just held that very day with the Prince of Wales (with whom a successful meeting, in particular, implicitly testified to the Kings complete recovery of his nerves) and intimates that Thurlow should convey the same to Mr. Pitt whom I propose seeing tomorrow Morning. By conveying these and related details, and at the same time thanking the Nation at large to whom I am so much indebted for the Support and anxiety shewn during my illness, George III consolidated the support that saw him successfully through the Regency Crisis, and through his next two porphyria attacks (1801 and 1804), as well.

In other words, the letter, written from Kew, which was the scene of both the worst of George IIIs ordeal, and his recovery, explicitly marks George IIIs forceful, yet diplomatic re-entry into politics and the business of government. His final phrase of the letter concerns supplies for the Current year, i.e., the 1789 royal budget. Within days of his letter, George III had fully resumed his duties as King.

Medical historians Ida Macalpine and Richard Hunter first proposed that the King George had porphyria in their historical monograph, George III and the Mad-Business. It had long been suspected that King George III had porphyria, but this assumption was not proven until scientists exhumed the bodies of family members and performed DNA studies.

I also had a personal experience over this questions. A British Lord came to visit a friend of mine in Houston. He wanted to have a typical Texas experience, so she ask Dick and me to take him to the hill country of Texas where we had a lake home. He was a lovely guest and was thrilled when he we took him to the first dance hall, in Gruene . He had fund doing the two step and watching all the terrific dancers whirling in a circle.

The next morning over coffee, he told me he loved the dance hall and the Wimberly, Texas "pie social" better than all other experiences. I was so sick that I could not share his delight. He told me that he didn't share his delight and my sickness was porphyria..not a hangover as I don't drink. He asked me what was wrong and I said my usual, "Nothing." But he insisted that something was wrong at which point I said it was a disease that he most likely had never heard of and I said, "Porphyria. "

" I know it well , " he said. " My wife was in the lineage of George III and her brother had pophyria. " What about that for a coincident !!!!!

You can Google the subject and even see paintings of King George and others in the Royal Family who are deemed to have the disease. It makes for an interesting story for the media so it is something to gain the attention of a local reporter.

There was Fun, Too!!!

Tuesday - February 1, 2011 @ 10:47:24

I wanted to include in the materials we were developing for doctors and patients the issues that were key to patients. So I interviewed many of them and asked them their thoughts on the subject. I would like to take a moment to share with you a few special patients who were in the Rockefeller Research program with me.

Herta was another extraordinary Porphyria patient at Rockefeller. When Herta arrived at the hospital, everyone knew it. She walked down the halls with a bigger than life personality. Everyone was a friend. Herta was vivacious, smart, interesting and had such a commanding presence that she drew instantaneous attention. I liked her and her husband, Herman, from the moment we met. Unfortunately, Herta was also very sick with Porphyria at times and often had to visit the outpatient clinic at the hospital.

Herman drove Herta into the city from New Jersey for her appointments. Since he was making the round-trip to and from the city, he took on the job of bringing urine samples from other New Jersey patients to the research lab at Rockefeller. You can imagine all the names we created for his automobile and his new volunteer service, our favorite of which was The PeeWee Delivery Service. Laughter , the Good Book says , is the best medicine and in this case it was definitely a major factor. The arrival of the PeeWee Delivery Service, was a much awaited event that precipitated howls of laughter each time. Plus, Herta and Herman transported more than urine when they came to the hospital. They brought overflowing love and if we were lucky, they also brought oodles of goodies that Herta had cooked. I never asked, however, if they kept the food and urine samples in the same ice chest.

Hertas son, Roland, was producing videos at the time and approached me about filming a video on Porphyria. I jumped at the chance and assured him that we would distribute it through our new American Porphyria Foundation. After retrieving the specific permissions from the patients and doctors who were going to participate in the film, we finalized all of the other details before starting the video. Interestingly , the basics are still true today, so although we a new DVD , Porphyria Live, the older version is a treasure, too.

I was so impressed with Roland that I told him, "You are such a special young man that I think one day, you will be a producer on the Today Show or a similar program. Sure enough, not too many years later, he became one of the one of the Today Show producers and is still in television production.

Roland first set up the camera in the recreation room at Rockefeller Hospital. Most of the patients in the hospital gathered to watch the filming, whether they had Porphyria or not. I was the films narrator and Herta and Frank were the key patient interviews. They conversed about their respective cases of Porphyria and other men and women in the New York area who were patients at Rockefeller discussed the photosensitive Porphyrias. In years to come, we have tried to keep this as a prime feature at the APF, namely, working together, making what patients have to say very important , and soliciting the opinions of our members.

Our APF members are the reason we are a foundation. Since I don't take a salary and never have , I can say it is not for funding on my part. Also, our porphyria experts have very limited research funds and don't receive any funds for consultation with doctors or patients, so no one can say they benefit either. What we do is because we know pophyria is rare and many patients need help desperately.

Thus, we all need to help each other. Certainly, your donations help. But also it is helpful for you to do whatever you can to help educate your doctor , other patients and , of course, your community. You may ask , "How can I accomplish this? "

Let me give you a few suggestions:
Contact your local media and offer your story, particularly if it is National Porphyria Awareness Week or if there is a very exceptional angle to it . For example, near the 4th of July, you might suggest a story on pophyria because of the historical significance with Mad King George III and the Revolutionary War as historians feel that his ignoring the colonies was partly responsible for the British defeat. The APF will provide suggestions, materials and other help you may need to garner media attention to your story or to porphyria.

Another suggestion is to advance awareness of porphyria in the medical community. gain medical attention at your hospital , like a seminar at your hospital and teach your own doctor.

Watch the next blog to learn about King George and what he has to do with porphyria.

The APF and You.

Tuesday - January 18, 2011 @ 10:27:59

When the two of us decided to move forward to start an American Porphyria Foundaiton, we first discussed the fact that without a group of experts behind us, we would have absolutely no validation. Fortunately, we knew some of the porphyria experts in the country, so we began contacting them to discover the others.

We set the parameters to determine what consitiutes an expert and we decided on certain parameters. the following were a few of the parameters:

They wrote the medical text book chapters on poprhyria.

They did the porphyria research.

They already had a reputation in the medical world as the "pophyria experts."

They attened the national and international porhyria conferences .

They were known by their european counterparts as porphyria experts.

They had publications in the most prestigious medical journals, like New England Journal of Medicine.

They were known as porphyria experts amoung their peers.

They had many porphyria patients and not just a few .

With these very strict parameters in place, we were easily able to identify THE EXPERTS. They were an elite group and were amazingly knowledgeable about the porphyrias . Our next step was to contact them and arrange a meeting. This was not easy as they were spread across the country and we ha no funds to bring them together. So we investigated where the next medical meeting would be held that many of them would attend. Chicago was the place. The onew who were already attending were not a problem. Those doctors who were not attending the convention paid their own way.

The plan we devised that day turned out better than we could have imagined. Our first step was to invite a group of prominent Porphyria experts to gather in Chicago to discuss our objectives and to solicit their input and participation. We both agreed that the effectiveness of our future foundation was dependent upon the caliber of the physicians/researchers involved and the quality and reliability of the educational materials we would produce. The name we chose for the new foundation would be The American Porphyria Foundation.

The financial burden of starting such a foundation was on us, including gathering the names and support of Porphyria specialists around the country to start the plan of action. This would be a very costly but necessary endeavor. Fortuitously, the Digestive Disease Week convention was scheduled to convene in Chicago shortly after James and I started planning our mini-conference. Digestive Disease Week is the major convention for gastroenterologists, liver specialists and other related medical specialties, which meant that most of the Porphyria specialists who were involved with the acute Porphyrias would be in attendance. This was not only a huge financial break for us; it also saved us from the logistical quandary of trying to bring doctors from different coasts to one location.

We quickly contacted the list of specialists, introduced ourselves and invited them to join us to discuss our dream of an American Porphyria Foundation. They all accepted. At the time, I did not know the rigors of a medical convention, so I wasnt sensitive to the great sacrifice of time and energy that the experts shared with us.

Our meeting was held in one of Chicagos lakeside high-rises. The view was spectacular, which added to the ambiance of our first meeting with a group of strangers. James knew one of the specialists very well, but, like me, he never had met most of the other attendees. The Porphyria specialists were a wonderfully congenial group of men and women. When we presented our plan to start the American Porphyria Foundation, each of them was very receptive to the idea and agreed to be members of our scientific advisory board. Almost thirty years later, they continue to volunteer their time and expertise, a contribution not often duplicated in other educational or medical foundations.

Before I proceeed, I want to add here that this has been the way our experts have been for the next thirty years. They have volunteered their time , etc. This is why I am so bothered when I see commments about our board of experts making money out of porphyria or getting money from a drug company. that is so ludicrious. No one makes money on a rare disease. They have consulted with thousands of doctors over the past thirty years and have never changed one red cent..not one penny. So those people who ae saying differently are not only ill informed, i my book, they should be ashamed. You see we need to be appreciative of this group of doctors who are so caring and who have volunteered their time for 30 years. to enhance that thought, one of the original experts, donated the major funding for the APF to move forward.

To continue, when we met with Dr.s Anderson, Bissell, Bloomer, Desnick, Pierach, Pimstone, Shedlofsky, Tishler, Kushner, Bonkovsky and Sassa ( who has since died). they were glad that we wanted to start a foundation and heartily agreed to serve on our Scientific Advisory Board.

This gave us the impetus to continue. If your are seeking information from any group or organization, if they do not have experts at the forefront, then it might be best to find another information source.

With this group, we developed educational materials and even got names of patients from the experts and NIH. Unfortunately , however, shortly after we started the APF, NIH stopped their porphyria research program and the government almost completely stopped their funding of rare disease research. That brought important research to an almost stop. This was severely disappointing.

Until recently, for over 25 years, research funding was very difficult, if not impossible, to find. You can imagine how I felt and how hard it was for the porphyria researchers. Nonetheless, we pressed onward and upward. We grew each year and instituted major patient and physician educational programs. WE knew both were of ultimate importance. As many of you havc experienced, most doctors have NEVER had a porphyria patient and did not know how to diagnose and treat each of the porphyrias.

Therefore, we have put as much emphasis on the doctors educational program as the patient education program knowing that unless patients are diagnosed and treated properly,we are still at square one. We feel the same way today , so the APF makes these educational programs a very important funding priority.

How you can help us is to send in your doctor's name and address and we will send him or her a very comprehensive packet of info all written by pophyria experts and we will put the doctors on the database to keep them updated. We also attend targeted medical conventions like , The Liver meeting, where 8000 doctors attend to learn the latest in liver disease. You can volunteer to help man our APF exhibit booth and distribute our brochures etc to the attendees. What an opportunity to make a difference. So check the APF news letters and E-NEWS to find out when these conferences will take place.

My First Support Group that Grew Into the APF

Thursday - January 6, 2011 @ 10:58:16

Like most good ideas, the American Porphyria Foundation, began out of necessity, namely I was terribly lonesome and needed someone with whom I could communicate about porphyria. When I first arrived at NIH, I was the only patient who had Porphyria on the metabolic wing. I yearned to meet someone else, so I could talk with them about their experience. Although there were a few other patients on the wing, I could not identify with their symptoms as they were so vastly different from my own. However, I could identify with their feelings of isolation and anxiety on one hand and their feelings of gratefulness for each new day.

One morning, Dr. Lamon came into my room with the news that two more Porphyria patients had arrived that morning. Valerie was an Acute Intermittent Porphyria (AIP) patient from Utah, and Maureen was a patient from Baltimore, who had Variegate Porphyria (VP). Both had been patients at NIH for treatment on several other occasions. We met within hours of their arrival at NIH. I did not feel that I should interrupt them, because I did not know if they were very ill or there for a research project. I had learned during my times in the laboratory that some patients returned to NIH to participate as volunteers for specific research projects. I was anxious to meet them whatever the case may be.

On my daily walk around the halls on our floor, I got my chance to meet them. They too were roaming the halls of the metabolic wing, and we greeted one another in passing. It did not take us long to introduce ourselves, strike up a conversation and discover that we had the diagnosis of Porphyria in common.

Rather than gab on in the middle of the hallway, we quickly searched for a quiet place to sit and discuss our respective cases. It was soul soothing to be able to compare symptoms and treatments, discuss our emotional states, share our yearning for our families and talk about the ways in which we were able to cope with our illness. Like most patients with a rare disorder, we felt as if we were the only ones in the country suffering from this rare disorder. The opportunity to meet each other and then mutually support each other was crucial to our improvement. For me, it was important to see other patients who were still alive several years after their diagnosis. Although I had read a great deal about the disease and knew that patients could live long lives after attacks, I was wary that this was not true, particularly since my symptoms were so harsh. Gladly, I had two examples of living, breathing, happy individuals to prove that it was the truth.

Valerie was the mother of several children and, like me, was depressed over not being with her offspring. We comforted one another with photos and stories about each child. Our interchanges helped both of us bear the long days without them. Valerie also had AIP like me, but she had been diagnosed for five years. This helped me considerably. I told her that she was living proof that AIP was not a degenerative disease and that a person could have a relatively normal life when the disease was not active. Mostly, I was glad to see that she was alive several years after her diagnosis.

Maureen was single and was in the process of becoming a nun. The three of us spent long hours talking about the Lord and the Bible, thus fulfilling our need to share what was happening to us spiritually, as well as physically. Those conversations were more valuable than we fully understood. By sharing many of the powerful promises in the Bible, we were empowering ourselves. Each of us was responsible for bringing a passage to our daily meeting, like bear one anothers burdens or God is a very present help in time of trouble. I had heard from years listening to sermons in church that the spoken word is very powerful but I did not understand how powerful until we began to take those words and repeat them until they became alive in our souls.

In the course of my many conversations with Valerie and Maureen, we agreed that what was needed was a patient education organization to distribute understandable information on Porphyria and act as a point of contact and support system for other patients. At that time, nothing was available in the way of patient information other than a very basic print-out that Dr. Lamon gave me and my own handwritten pamphlets. Our many discussions precipitated the thought that I might have my more comprehensive educational information for patients approved by Porphyria specialists and then used as the first brochure for a support network. This thought flickered at my minds edge until on the early gentlemen I met gave birth to the American Porphyria Foundation a number of years later. I hope most of you understand the importance of sharing your experience. In time, we will open the blog up to others to share their stories or comments.