Blog Archive

Archived posts for 2018

Shadow Jumpers

Saturday - December 29, 2018 @ 06:30:07

SHADOW JUMPERS

LongSleeveCrew.jpgWelcome to Shadow Jumpers! This is a page for EPP kids and parents by EPP kids and parents. Shadow Jumpers was created to help give kids with Erythropoietic Protoporphyria (EPP) and their families a place to learn about this rare disease, read tips and tricks learned over time and to hear from fellow kids.  Through spreading awareness, fellow EPP interviews, tips to protecting yourself outside and some insight for parents, we hope all families living with EPP will look at this condition as a challenge they can overcome.  Be sure to check out all the Shadow Jumper resources! Wed love to hear from you. Reach out to us anytime on shadowjumpers@porphyriafoundation.org!!
A main goal of Shadow Jumpers is to help kids living with Erythropoietic Protoporphyria do things they have always wanted to do but have not been able because of the sun. There are only several hundred diagnosed cases of this ultra-rare genetic disorder in the United States so we need to learn from and support one another! Pain from the sun combined with the mental discomfort kids can feel from having to cover up may lead kids to avoid certain activities, relationships and paths in life.
Shadow Jumpers wants to show (and remind) kids that they can still follow their dreams! Whether thats trying to play a sport outdoors, going on a family vacation, camping or whatever comes to mind, Shadow Jumpers wants to help. Click around to learn about some awesome EPP kids, how we are trying to do our part and how you can too!
Here are ways to connect with Shadow Jumpers!



Importance of Medical IDs

Thursday - December 27, 2018 @ 06:31:52

Importance of Medical IDs

why it is important to wear your medical id
  • Your medical ID provides for a quick recognition of your medical conditions, allergies, medications, or treatment wishes; this leads to faster and more effective medical treatment.
  • Medical ID bracelets reduce treatment errors which may result from not having a patients health record during an emergency situation or upon hospital admission.
  • A medical ID speaks for you in the event of an emergency if you become unresponsive
  • First responders and medical personnel are trained to first look for medical identification jewelry in an emergency. Medical IDs will immediately alert emergency medical professionals to your critical health and personal information.
  • There are unlimited reasons for you and your loved ones to wear a medical ID when living with common or unusual medical ailments. A medical ID will save your life and the lives of those you love.
https://www.medicalert.org/importance-of-medical-ids

Who should wear a medical ID

  • Alzheimers/Dementia
  • ADD/ADHD
  • Anemia
  • Hypertension
  • Diabetes
  • Cancer
  • Porphyria
  • Food Allergies
  • Dialysis
  • Blood thinners (Coumadin
    Warfarin)
  • Lung disease
  • Autism
  • Heart disease
  • Asthma
  • COPD
  • Blood disorders
  • Children with special needs
  • Drug allergies
  • Pacemaker
  • Stroke risks
  • Hypothermia
  • Epilepsy, seizures
  • Multiple Sclerosis


8 Tips for Managing Caregiver Stress

Tuesday - December 25, 2018 @ 06:30:10

8 Tips for Managing Caregiver Stress

Caring for a loved one suffering from a medical condition can strain even the most resilient people. Caregiving can have many rewards while also taking a substantial toll on both the caregiver and their loved ones.

Common Signs of Caregiver Stress

Because caregivers are most commonly altruistic in nature, you may be completely focused on the health and welfare of your loved one before realizing your own overall well-being is suffering. When taking upon the role as a family caregiver, try to be cognizant of early warning signs of caregiver stress such as:
  • Feeling overwhelmed or constantly worried
  • Feeling tired most of the time
  • Sleeping too much or too little
  • Gaining or losing a lot of weight
  • Becoming easily irritated or angry
  • Losing interest in activities you used to enjoy
  • Feeling sad
  • Having frequent headaches, bodily pain or other physical problems
  • Abusing alcohol or drugs, including prescription medications
Excess stress over an extended period of time can do more than cause increased depression and anxiety. Lack of sleep, eating a poor diet, and not getting enough physical activity can increase your chance of developing serious medical problems including heart disease and diabetes.

Strategies for Managing Caregiver Stress

  1. Ask for help- dont automatically assume you have to take on everything. Make a list of priorities and reach out to friends, other family members and even distant relatives.
  2. Take daily breaks- You deserve it. Enjoy some you time throughout the day whether its going to the gym, reading a book, or engaging in another hobby.
  3. Just say no- Accept the fact that you simply cant do everything. Resist the urge to take on more activities, projects or financial obligations that you feel you can handle.  Be honest when feeling you are being stretched too thin.
  4. Get organized- try to keep your responsibilities prioritized. Dont stress too much if you cant manage everything and just take care of the most important things one at a time.
  5. Stay connected with friends and family- try to keep yourself from being isolated from family and friends. It can be easy to completely absorb yourself into your role as caregiver increasing your stress level. Make time for social gatherings- dinner with friends, attending family events and holiday celebrations, etc.
  6. Keep a positive attitude- When caring for another living with a chronic medical condition, it can be easy to fall into a negative mindset. Instead of dwelling on what you cant control, give yourself praise for how much you are impacting the life of your loved one in need.
  7. Set personal health goals- establishing a normal sleep pattern, regularly finding time to be physically active and fueling your body with a balanced diet are great ways to battle stress while maintaining your own emotional and physical health.
  8. Join a caregiver support group- others in a support group can understand your trials and frustrations while offering alternative solutions. A support group can present opportunities to interact with others in order to avoid isolation; while giving you the chance to create meaningful relationships.  
https://www.caring.com/support-groups offers many featured online groups for caregivers, offering you an outlet to share, vent, laugh, and feel less alone. 


Porphyria Consortium 101

Monday - December 24, 2018 @ 06:32:32

Overview of the Porphyrias

Disorder Definitions
The porphyrias are inherited genetic conditions, which means that people with a porphyria have changes to certain genes that affect their bodys ability to regulate itself.  When genes are copied, either to make new cells or to make a child, sometimes the body makes an imperfect copy.  There can be little changes in the genes, called mutations, which can occur randomly.  Sometimes these changes do not make any difference in how well the gene works, but other times they can keep the gene from working properly (referred to as mutations) and are disease causing.
In the porphyrias, these mutations are in the genes involved in a certain chemical pathway, called the heme biosynthetic pathway. Heme is a compound that the body needs to make hemoglobin and there are several steps to make this compound in the body. Each type of porphyria is caused by a defect in a specific enzyme in the heme biosynthetic pathway. Without these enzymes working properly, the body is not able to finish making heme and it causes a buildup of other compounds, called porphyrins. It is the buildup of different types of porphyrins that causes the different types of porphyria.
Most commonly the porphyrias are divided into the acute and cutaneous porphyrias, depending on the primary symptoms. The acute porphyrias [acute intermittent porphyria (AIP), hereditary coproporphyria (HCP), variegate porphyria (VP), and ALA-dehydratase deficiency porphyria (ALD)] present with sudden attacks of severe stomach pain that last for several days; VP and HCP may also have skin symptoms of blistering after sun exposure. The cutaneous porphyrias present with blistering and scarring of the skin, pain, and/or redness and swelling in sun-exposed areas. The porphyrias may also be classified as hepatic or erythropoietic, depending on the organ where the porphyrins accumulate, the liver for the hepatic porphyrias [AIP, HCP, VP, porphyria cutanea tarda (PCT), and hepatoerythropoietic porphyria (HEP)] or the bone marrow for the erythropoietic porphyrias [congenital erythropoietic porphyria (CEP), erythropoietic protoporphyria (EPP), and X-linked protoporphyria (XLP)].

The Acute Porphyrias

There are four types of acute porphyrias; acute intermittent porphyria (AIP), hereditary coproporphyria (HCP), variegate porphyria (VP), and δ-aminolevulinic acid dehydratase porphyria (ADP), and they have similar symptoms. These are genetic disorders that are very rare and may be difficult to diagnose for this reason. It is estimated that about 1 in 10,000 Europeans or people of European ancestry have a mutation in one of the genes that cause AIP, VP or HCP. These mutations have been found in all races and many other ethnicities in addition to Europeans.
Approximately 80-90% of individuals who carry a gene mutation for acute intermittent porphyria, variegate porphyria, and hereditary coproporphyria, remain asymptomatic, and others may have only one or a few acute attacks throughout life. The most frequent symptom is severe abdominal pain and is often accompanied by nausea, vomiting, and constipation. Other symptoms may include heart palpitations, seizures, and hallucinations. People with VP and HCP may also have skin symptoms of blistering after sun exposure.

The Cutaneous Porphyrias

All but one of the cutaneous porphyrias cause skin blistering and fragility on sun-exposed areas of the body, most commonly the backs of the hands, forearms, face, ears and neck. The cutaneous porphyrias are porphyria cutanea tarda (PCT), hepatoerythropoietic porphyria (HEP), congenital erythropoietic porphyria (CEP), erythropoietic protoporphyria (EPP), and X-linked protoporphyria (XLP).
CEP and HEP occur in childhood with severe blistering skin lesions. PCT occurs in adulthood generally and less severe blistering skin lesions after sun exposure. Erythropoietic protoporphyria (EPP) and X-linked protoporphyria (XLP) have the same symptoms of painful, but nonblistering, reactions to sunlight. There can also be swelling and redness of the sun exposed areas of the skin with EPP and XLP. 
Each type of porphyria is caused by a mutation, or change, in the gene coding for a specific enzyme in the heme pathway. PCT is unique as it is the only porphyria where most patients do not have mutations in a gene, but instead have acquired, or sporadic, PCT.    

Types of porphyria, their patterns of inheritance, and the enzyme that is deficient in each.

TypeInheritanceDeficient EnzymeGene
ALA-Dehydratase Porphyria (ADP)
Autosomal recessiveALA-DehydrataseALAD
Acute Intermittent Porphyria (AIP)
Autosomal dominantHydroxymethylbilane synthase (Porphobilinogen deaminase)
HMBS
Congenital Erythropoietic Porphyria (CEP)
Autosomal recessiveUroporphyrinogen III synthaseUROS
Porphyria Cutanea Tarda (PCT), familial form
Autosomal dominantUroporphyrinogen decarboxylaseUROD
Hepatoerythropoietic Porphyria (HEP)
Autosomal recessiveUroporphyrinogen decarboxylaseUROD
Hereditary Coproporphyria (HCP)
Autosomal dominantCoproporphyrinogen oxidaseCPOX
Variegate Porphyria (VP)
Autosomal dominantProtoporphyrinogen oxidasePPOX
Erythropoietic Protoporphyria (EPP)
X-linked Protoporphyria (XLP)
Autosomal recessive
X-linked
Ferrochelatase
δ-Aminolevulinate synthase 2
FECH
ALAS2
The inherited porphyrias are either autosomal dominant (inherited from one parent), autosomal recessive (inherited from both parents), or X-linked (the gene is located on the X-chromosome). "Autosomal" genes always occur in pairs, with one coming from each parent. Individuals with an autosomal dominant form of porphyria have one mutated gene paired with a normal gene, and there is a 50% chance with each pregnancy that the mutated gene will be passed to a child. 
Individuals with an autosomal recessive type of porphyria have mutations on both copies of a specific gene, one passed to them from each of their parents. Each of their children will inherit one mutated gene for that porphyria, and the child will be a carrier but will not have symptoms.
In X-linked disorders, the gene is located on one of the sex chromosomes, called the X-chromosome. Females have two X-chromosomes, and males have one X-chromosome and one Y-chromosome. Both males and females will likely have symptoms from a mutated gene on the X-chromosome, but females, with a normal gene on the other X-chromosome, usually are less severely affected than males. The risk for children depends on the gender of the affected parent. A female with an X-linked gene mutation will have a 50% risk of passing that mutation to any of her children with each pregnancy. However, a male will pass the mutation to all of his daughters but none of his sons.
There are many laboratory tests available for the porphyrias, and the right tests to order depend on the type of porphyria the doctor suspects. When abdominal and neurological symptoms suggest an acute porphyria, the best screening tests are urinary aminolevulinic acid (ALA) and porphobilinogen (PBG). When there are cutaneous symptoms that suggest porphyria, the best screening test is a plasma porphyrin assay. If one of these screening tests is abnormal, more extensive testing, including urinary, fecal, and red blood cell porphyrins, are often indicated.
DNA testing to identify the specific mutation in an individuals porphyria-causing gene is also recommended. Before requesting DNA testing, it is helpful that patients have biochemical testing. However, many patients have not had an acute attack or are not symptomatic at present, so biochemical testing may be inconclusive.
In contrast, DNA testing is the most accurate and reliable method for determining if a person has a specific porphyria and is considered the "gold standard" for the diagnosis of genetic disorders. If a mutation (or change) in the DNA sequence is found in a specific Porphyria-causing gene, the diagnosis of that Porphyria is confirmed. DNA analysis will detect more than 97% of disease-causing mutations. DNA testing can be performed whether the patient is symptomatic or not. Once a mutation has been identified, DNA analysis can then be performed on other family members to determine if they have inherited that Porphyria, thus allowing identification of individuals who can be counseled about appropriate management in order to avoid or minimize disease complications.

Porphyrias Defined

 

Genetics 101

Have a Tour of Basic Geneticsfrom the Genetic Science Learning Center at the University of Utah

    Acute Porphyrias

    Cutaneous Porphyrias

What is Acute Intermittent Porphyria?

Acute Intermittent Porphyria (AIP) is an inherited genetic condition. The genetic mutations that cause AIP are in the HMBS gene. They result in the genes to produce too little of the enzyme hydroxymethylbilane synthase (also called porphobilinogen deaminase). Without enough of this enzyme, the body is not able to finish converting porphobilinogen into heme chains, causing them to build up to much higher levels than usual. This buildup can cause the pain attacks of AIP, but about 80-90% of patients with AIP mutations will not develop symptoms.
When patients do suffer an attack, they will usually experience severe abdominal pain. This is often extremely painful and patients may need to go to the hospital for help. Because these attacks will often not involve any visible symptoms, the ER staff may not know to treat patients for porphyria if they are unaware of the diagnosis. It is very important for patients to speak with their local hospital and health care provider to make sure a plan is in place before an attack so they are able to receive care as quickly as possible.
Patients can also experience numbness, weakness, nausea, constipation, confusion, restlessness, hallucination, seizures, and difficulty with urination during acute attacks. These symptoms can be very severe and hard to treat if a doctor does not know to suspect AIP. It is important for family members of AIP patients to get tested even if they have never had an attack before. If they do have a mutation in one of their copies of the HMBS gene, knowing this will allow their doctor to give them the appropriate care if any symptoms arise.
Patients with AIP also have a slightly increased risk than the general population of developing liver cancer, called hepatocellular carcinoma.

How is Acute Intermittent Porphyria diagnosed?

There are two types of testing; biochemical, meaning looking for biomarkers in the blood or urine, and genetic, meaning looking at the gene we know causes the disease directly from a blood sample.
Biochemical: To diagnose AIP the most important test is to measure the level of porphobilinogen (PBG) in the urine. This test is often combined with measuring the total amount of porphyrins in the urine and another biomarker called aminolevulinic acid (ALA). The level of PBG in the body can vary so the best time to take samples is during an acute attack (e.g. when someone is having abdominal pain, etc). In people with AIP the level of PBG is very high.
Genetic: A blood sample is used to look at a persons genes and by doing this it is possible to see if their genes have mutations that can cause disease. The gene that causes AIP is called HMBS. Genetic testing is recommended for patients even if they have very high PBG levels.
If a patient has a mutation, their immediate family members should be tested for that same mutation as well. This includes their parents, their siblings, and any children they may have. This will allow all family members to receive appropriate care and counseling even though 80-90% of people with a mutation will not develop symptoms of AIP.
Also see FAQ: What diagnostic tests are available?

What are treatments for Acute Intermittent Porphyria?

AIP attacks can be triggered by a number of factors. One known trigger is progesterone, a hormone which naturally increases in women during their menstrual cycle. Female AIP patients are more likely to have attacks in the second half of their menstrual cycle, when their uterine lining is thickening but before it begins to shed (when they begin bleeding). Dieting can also be a trigger, so patients should avoid fasting and dieting. Patients with AIP should eat a balanced diet. Drugs can be another trigger, especially barbiturates, sulfonamide antibiotics, anti-seizure drugs, and oral contraceptives (progesterone in particular). There is an online drug database to check which medications may be unsafe for people with AIP. The American Porphyria Foundation offers a mobile phone app that pulls up this information online (porphyriadrugs.com).
During an attack, patients may often need to be hospitalized. This will allow them to receive medications to handle their pain and IV fluids if they are unable to stop vomiting or are too nauseous to eat. If the attack was triggered by using drugs for a long time, the muscles which control breathing may be weak and the patient may need respiratory support.
Patients can receive heme therapy through an IV. Panhematin is an FDA approved medication which can help decrease the severity and length of the attack, and is more effective the earlier they receive it.
Attacks can be prevented in many cases by avoiding harmful drugs and fasting or dieting. Wearing a Medic Alert bracelet is recommended for patients who have had attacks. Very frequent premenstrual attacks can be prevented by a gonadotropin-releasing hormone (GnRH) analogue administered with expert guidance. In some cases, frequent attacks can be prevented by regularly scheduled infusions of hemin.
Individuals with AIP who are prone to attacks should eat a normal balanced diet and should not fast or diet, even for short periods of time. If weight loss is desired, it is advisable to consult a physician and a dietitian to have them an individualized diet plan created.

How is AIP Inherited?

AIP is an autosomal dominant condition. Autosomal means that the defect is not on the chromosomes that determine sex, and dominant means that you only need to inherit one mutated gene to manifest the disease. The gene that causes AIP is called HMBS.
Genes are inherited randomly, so a parent has an equal chance of passing on either of their two copies of each gene. Since most AIP patients have one mutated copy and one normal copy, this means that each of their children will have a 50% chance of inheriting the mutated copy and 50% chance of inheriting the working copy.


Who Make Up Our Scientific Advisory Board

Friday - December 21, 2018 @ 06:30:06

SCIENTIFIC ADVISORY BOARD

Our Scientific Advisory Board is made up of the world's foremost experts in Porphyria management, diagnosis, and research. They have written or approved the medical information on this website.  Many of the American Porphyria Foundation's SAB members have over 40 years experience working on Porphyria - conducting cutting-edge research, and writing peer-reviewed article for major medical journals and authoring the chapters on Porphyria in medical school textbooks.  Doctors worldwide consult with these Porphyria specialists for help with diagnosis and treatment of their patients.
Karl E. Anderson, MD, Chairman
University of Texas Medical Branch
Manisha Balwani, MD
Mount Sinai School of Medicine
D. Montgomery Bissell, MD
University of California
Joseph R. Bloomer, MD
University of Alabama
Herbert L. Bonkovsky, MD
Wake Forest Baptist Medical Center Winston-Salem, North Carolina
Sylvia S. Bottomley, MD
University of Oklahoma
Robert J. Desnick, PhD, MD
Mount Sinai School of Medicine
Micheline M. Mathews-Roth, MD
Harvard University School of Medicine
John Phillips, PhD
University of Utah
Claus A. Pierach, MD
University of Minnesota
Neville Pimstone, MD, PhD
University of California
Maureen B. Poh-Fitzpatrick, MD
University of Tennessee
Steven Shedlofsky, MD
University of Kentucky
Samuel M. Silver, MD, PhD
University of Michigan Medical School
Ashwani Singal, MD
University of Alabama, Birmingham
Manish Thapar, MD
Sidney Kimmel Medical College of Thomas Jefferson University
Peter V. Tishler, MD
Harvard University School of Medicine
Bruce Trippe, MD
Montgomery, AL
Bruce Wang, MD
University of California, San Francisco

Protect the Future

Simon Beaven, MD, PhD
University of California, Los Angeles
Brendan Chen, MD
Mount Sinai School of Medicine
Maria A. de Lima, MD
Rio de Janerio, Brazil
Angelika Erwin, MD
Cleveland, Ohio
Bradley Freilich, MD
Kansas City, Missouri
Eric Gou, MD
University of Texas Medical Branch
Jennifer Guy, MD
University of California, San Francisco
Sajid Jilil, MD
Fargo, North Dakota
Sioban Keel
Seattle Cancer Care Alliance, Univ. WA
Cynthia Levy, MD
University of Miami
Charles Marques Lourenço, MD
University of São Paulo, Brazil
Brendan McGuire, MD
University of Alabama-Birmingham
Sahil Mittal, MD
Houston, Texas
Akshata Moghe, MD, PhD
University of Pittsburgh
John G Quigley
University of Illinois at Chicago
Sean Rudnick, MD
Wake Forest Medical Center
Behnam Saber, MD
Mount Sinai School of Medicine
Jonathan Ungar, MD
Mount Sinai School of Medicine


Happy Winter Season!

Wednesday - December 19, 2018 @ 17:19:53

                  Happy Winter Season!


Porphyria Post

Friday - December 14, 2018 @ 06:30:00

Porphyria Post

Happy Holidays from the APF!

The American Porphyria Foundation will be closed for the Christmas holiday beginning on Monday, December 24, 2018 and will re-open for normal business hours on Wednesday, January 2, 2019.

For emergency medical attention, please go to your nearest emergency room and contact your primary care physician. For all other issues, please direct your needs to the APF office staff via email. Your messages will be answered accordingly.

Desiree Lyon - lyonapf@aol.com
Kristen Wheeden - kristen@porphyriafoundation.org

We hope that you enjoy this Christmas and New Years holiday with your loved ones.
Reminders!

EPP Clinical Trial Participation - We need YOU!
Please note: CANADIANS ARE NOW ELIGIBLE TO PARTICIPATE!

A clinical trial with an oral investigational drug intended to reduce phototoxicity in people living with EPP is underway.

Please note that a Phase 3 study is very unlikely if Phase 2 is not completed. To date, this drug has proven nontoxic and there have been essentially no dropouts in the Phase 2 study to date.

This is an oral drug which makes administration simple.

This study in adults will pave the way for a near-future pediatric trial. EPP is a rare disease and patients must participate in order to get the drug approved.

To date there is no public information on the availability of Scenesse, and if approved, two drugs on the market may lower their cost.

We will connect you with a Research Coordinator that will answer all your questions and concerns. We will work to make this process as easy as possible for you.

Eligibility Criteria:
â?¢ Confirmed diagnosis of EPP
â?¢ Provide written informed consent to participate
â?¢ Be willing and able to travel to all study sites for scheduled visits
â?¢ 18 - 70 years of age at the time of screening.

Sites enrolling:
â?¢ Mount Sinai - New York City
â?¢ Wake Forest Baptist University - Winston Salem, NC
â?¢ University of Miami - Miami, FL
â?¢ University of Texas Medical Branch - Galveston, TX
â?¢ University of Utah - Salt Lake City, UT
â?¢ University of California San Francisco - San Francisco, CA

We need YOU! Please dig deep and consider being part of this changing moment for all with EPP.

Contact us here at the APF office to get in contact with a research coordinator.
301.347.7166 or 1.866.APF.635
porphyrus@porphyriafoundation.com

"RememberResearch is the key to YOUR cure!"
Each Step Toward Finding an Effective Treatment is Important!
Pet Calendars on Sale!
Order your APF 2019 Pet Calendar TODAY! This is a great way to start your year off right and support the APF.

Calendars are $12 including shipping and can be ordered through the APF Store or by emailing autumnlee@porphyriafoundation.org.

The calendar is filled with your fluffy companions, dates, and porphyria facts.

Click on the link below to order today!
https://porphyriafoundation.org/store

Harvoni Study - PCT
Do you have PCT? Are you interested in participating in research? Do you have Hepatitis C? If you answered YES to these questions, this is for you.

We need YOU for a clinical trial!

The purpose of this clinical trial is to assess whether Harvoni alone is an effective therapy in active PCT patients with Chronic Hepatitis C.

Who can participate?

1. Adult patients with PCT who also have Hepatitis C

If you are interested in participating please contact Edrin Williams, Director of Patient Services at the APF office at 301.347.7166 for additional information.

Get Involved

â?¢ Research â?¢

Contact Information

Is your contact information up to date?
If not please give us a call @ 866-APF-3635 or Email to porphyrus@porphyriafoundation.com


American Porphyria Foundation 1.866.APF.3635 porphyriafoundation.org


STAY CONNECTED
"Remember.Research is the Key to Your Cure!"


The Porphyria story of Victor LaFae with HCP

Thursday - December 13, 2018 @ 18:38:10


Porphyria story - HCP - Victor LaFae
Im told that I was a typical happy baby for the first few months of my life. I reached all my milestones on time or even a little bit early. It was not until I was three months old that something seemed amiss. One Saturday afternoon in mid-July 1993 while my father was at work my mother decided to take me and my older sister out to a park and to run some errands. My sister ran around the park having a blast for a couple of hours while my mother sat in on a bench with me partially shaded by a large oak tree. As the sun began to set my sister was called from the playground and my mother began the walk to the grocery store. I was getting fussy, and she assumed that I was tired and ready to eat and sleep.

About halfway to the store, I let out a screeching noise. Thinking I must have been bitten by a spider or stung by a bee my mother stopped to shake out my blanket and check me over for bites. She found nothing to explain my screaming cry. When I could not be comforted my mother grew more and more concerned. That night we ended up sitting in a hospital for several hours. The doctors ran blood tests and found nothing unusual. They told my mother that I was a colicky baby and that I would outgrow it. Not having raised a colicky child before, my parents grudgingly accepted the doctors' diagnosis and took me home.

A few weeks went by and I continued to scream and cry every time my mother tried to take me out of the house. It was the start of August and my older sister was getting sick of being cooped up at home. So, I was taken to the park to let my sister play. My mother decided that perhaps I just needed to cry it out as she sat under the oak tree talking with another mother whose child was off playing with my sister. When the other woman inquired about my cries, my mother told her I was just colicky. That is not a colicky baby! My boy was colicky, the mother declared. As if to prove the woman's point, I began having my first of many seizures.

I only seized for a moment or two, but it was enough to have my mother rush off to the ER. The doctors again did blood work and asked many questions. They found nothing to explain the seizure. They told her that I was colicky and that I was likely just trembling from having exhausted myself crying. Over the next few months, things would spiral out of control with new and more bizarre symptoms popping up every few days. Each symptom dismissed and my mother became known as the over dramatic nervous mother. With each new visit to the ER, the doctors took my mother less seriously than the last.

I became lethargic. My arms and legs that had once danced around lay limp at my side. I stopped trying to nurse and spit up anything my mother managed to get into me. My weight decreased until I was not even on the growth curve. I didnt react to the things I once loved, like peek a boo. I developed tremors and muscle twitches, small blisters, and dark urine. My mother was told again that it was all from being a colicky baby, I was probably dehydrated, and that the blisters were from the laundry soap. This went on for a few years. Slowly some of the symptoms cleared up and so my parents believed that perhaps the doctors had been right.

At around 4 years old, the first signs of my Autism became clear, and I was later diagnosed with Asperger's syndrome. I was minimally communicative which meant I could not explain my own symptoms to the doctors or to my parents. I would only cry when forced outside. I could not explain the burning pain or the itching. My stomach felt like a hot sword was being twisted around in my gut, but I didnt have the words to tell anyone. My heart would beat so hard and fast I was afraid it would burst out of my chest.

I remember so clearly the exams. Every few months, scopes were shoved up one end or down the other as they looked for things like appendicitis and ulcers. My mother was told that with my autism it was hard to really tell why I was having so many meltdowns and that I was likely overstimulated. This is common for autistic children, so she neednt worry. I developed dizziness and began having regular falls. My coordination declined dramatically, I started dropping things and running into walls. This clumsiness was also attributed to my autism and my mother's concerns dismissed.

When I was 10 years old, I had visual hallucinations of red lights sliding around on the walls. I could hear ripping metal like a car being crushed by a compactor and the sound of electric sparks echoing in my head. The pain was back like my insides were being ripped out. The best way I could explain this terrifying and painful experience to the doctors was to say, The demon eyes are hurting my belly and heart. This led to a 72-hour psychiatric hold on the pediatric psych unit. After three days in the unit, I was discharged with a diagnosis of Autism, PTSD, and Somatic Symptom Disorder. Three weeks later in another ER with another blistering rash, the doctors decided I had scarlet fever from an untreated strep infection. There was no strep test done, they just put me on antibiotics. I took the full course of the meds and began having more of the same symptoms only now they were more severe.

This is when it was decided that my parents must be behind it all. They accused my parents of causing my blisters with boiling water and telling me to tell doctors I was sick. They said my mother had Munchausen's By Proxy. With these new abuse allegations, I was placed in foster care where of course my illness continued only now it was being attributed to anxiety and PTSD from the supposed abuse. I have bounced around from one foster home to the next and overlooked for years. This was a very lonely and dark time for me. I felt guilty that my illness was causing my family so much suffering. The pain and guilt lead me to attempt suicide on several occasions.

My suicide attempt led to another psychiatric hospitalization where I met someone with the same symptoms that I had, at this point my communication skills were improving and I was able to sit and speak with the girl. When I told her, what had been happening to me she told me it sounded like her condition but that It couldnt be the same thing because I was too young, and the disease is rare. She had Hereditary Coproporphyria. Even being in the same hospital and treated by the same doctors I was still not diagnosed or even tested for this disease. Hearing how rare the condition was, I dismissed the idea of telling myself the odds were astronomical.

I spent several more years suffering from symptoms no one could explain, and many refused to believe. In 2015, I contracted Pneumonia and an ear infection. One of the medications I was given was Bactrim, which is a medication that is not safe for Porphyria patients. I ended up being admitted to the hospital. For three days no one could figure out what was happening. I was deteriorating before their eyes and they had no answers. During rounds, one of the doctors looking over my medical records made a joke that it looked like I might just be a vampire. I had heard this joke before by doctors and nurses when they read that I could not be exposed to sunlight without blistering and burning.

Suddenly, one of the medical student's eyes grew super wide and he stuttered out one word, Porphyria. The lead doctor ordered a whole bunch of tests and, just in case, started me on a Dextrose drip. The tests were done several times during my stay, often mishandled, but the treatment was slowly working. The doctor informed me that he believed I had a form of Porphyria but could not offer me a definitive diagnosis at the time.

The word Porphyria sounded familiar, but I had long dismissed the idea. I had decided it must be something else and put the memory out of my mind. I didnt really remember the my earlier encounter until later when I was reading about the disease.

For a year and a half, they tested me and each time something else went wrong. It wasnt until December 2016 that they managed to collect, package, and ship the samples correctly. Two weeks later I got the call from Hematologist/ oncologist  confirming the diagnosis. I did, in fact, have Hereditary Coproporphyria, a disease that typically does not appear until after puberty or early adulthood. My biochemical testing had revealed elevated ALA, PBG, and Coproporphyrins in my urine. Coproporphyrins were also found in high levels in my stool samples.

I was hospitalized again a couple of months after receiving my diagnosis, this time it was much different. During prior hospitalizations, I had been treated with D10 which slowly helped me recover. However, during this hospitalization, I was given a treatment called Panhematin. When I started the treatment, I was in and out of consciousness, weak, and too lethargic to move. By my third treatment, I was hanging out at the nurse's station, though still in my wheelchair. I was not magically cured, but I was well on my way to recovering.

Though I was relieved to have an answer, I was terrified of what the disease would mean for the rest of my life. I felt lost until my doctor directed me to The American Porphyria Foundation. Through their Facebook support groups I was able to connect with others facing the same challenges and fears.

I receive Panhematin infusions, and I have reconnected with my family. When it is too sunny for me to go outside, I write stories and hang out in the Facebook groups. My life is far from perfect, but I have an answer, I have support, and I have hope that someday I might even have a cure.




Porphyria Post

Wednesday - December 5, 2018 @ 16:50:14


December 12, 2018


Porphyria Post


EPP Clinical Trials: Volunteers Needed!
We are close, but we need YOU!

A clinical trial with an oral investigational drug intended to reduce phototoxicity in people living with EPP is underway.

Please note that a Phase 3 study is very unlikely if Phase 2 is not completed. To date, this drug has proven nontoxic and there have been essentially no dropouts in the Phase 2 study to date.

This is an oral drug which makes administration simple.

This study in adults will pave the way for a near-future pediatric trial. EPP is a rare disease and patients must participate in order to get the drug approved. 

To date there is no public information on the availability of Scenesse, and if approved, two drugs on the market may lower their cost.

We will connect you with a Research Coordinator that will answer all your questions and concerns. We will work to make this process as easy as possible for you. 

Eligibility Criteria:
·    Confirmed diagnosis of EPP
·    Provide written informed consent to participate
·    Be willing and able to travel to all study sites for scheduled visits
·    18 - 70 years of age at the time of screening.

Sites enrolling:
·    Mount Sinai - New York City
·    Wake Forest Baptist University - Winston Salem, NC
·    University of Miami - Miami, FL
·    University of Texas Medical Branch - Galveston, TX
·    University of Utah - Salt Lake City, UT
·    University of California San Francisco - San Francisco, CA

We need YOU! Please dig deep and consider being part of this changing moment for all with EPP.

Contact us here at the APF office to get in contact with a research coordinator.
301.347.7166 or 1.866.APF.635

"RememberResearch is the key to YOUR cure!"
Each Step Toward Finding an Effective Treatment is Important!

Are you interested in participating in this clinical trial?

Yes

No


Harvoni Study - PCT
Harvoni Study - PCT

Do you have PCT? Are you interested in participating in research? Do you have Hepatitis C? If you answered YES to these questions, this is for you.

We need YOU for a clinical trial!

The purpose of this clinical trial is to assess whether Harvoni alone is an effective therapy in active PCT patients with Chronic Hepatitis C.

Who can participate?

1.   Adult patients with PCT who also have Hepatitis C

If you are interested in participating please contact Edrin Williams, Director of Patient Services at the APF office at 301.347.7166 for additional information.


Are you interested in participating in this clinical trial?

Yes

No

Panhematin Study - Participants Needed!
Do you have AIP, VP or HCP? Are you interested in participating in research? If so, we are recruiting volunteers for the Panhematin Study.

Our researchers need people to volunteer for the Panhematin study. Completing this study will help prevent insurance companies from not paying for this treatment.

The purpose of this study is to determine if Panhematin is safe and effective for prevention of acute attacks of porphyria.
·    Can you predict when your next attack will happen?
·    Are you currently receiving prophylactic heme treatment?

Please contact Edrin at the APF office (301.347.7166) if you are interested in participating.

Are you interested in participating in this clinical trial?

Yes

No

I have already participated

Panhematin approved in Canada!

Panhematin Approved in Canada to Treat Recurrent Attacks of Acute Intermittent Porphyria
Click on the link below to read this article:


2019 Pet Calendar
Order yours TODAY!
Order your APF 2019 Pet Calendar TODAY! This is a great way to start your year off right and support the APF. 

Calendars are $12 including shipping and can be ordered through the APF Store or by emailing autumnlee@porphyriafoundation.org.

The calendar is filled with your fluffy companions, dates, and porphyria facts.

Click on the link below to order today!


APF Website
If you have not already done so, please take a moment and bookmark the APF website as one of your favorites!

Here is the Link:


How do you like the newly updated APF website? We would love to hear your feedback!

I LOVE IT!

Still getting used to it!

Emailing my feedback to Edrin @ edrinw@porphyriafoundation.org




Get Involved



Contact Information

Is your contact information up to date?
If not please give us a call @ 866-APF-3635 or Email to porphyrus@porphyriafoundation.com

American Porphyria Foundation 1.866.APF.3635 porphyriafoundation.org

STAY CONNECTED

"Remember.Research is the Key to Your Cure!"





Porphyria Post

Wednesday - December 5, 2018 @ 06:30:05


Porphyria Post


The 2019 Pet Calendars are on Sale!!!

It is time is to order your 2019 APF Pet Calendar! Calendars will feature all of our contest pet submissions along with important dates and porphyria facts.  

Each calendar is $12 for orders shipped in the US. Want to give a gift that gives back? This is the perfect way to support the porphyria community while brightening the year of your friends and family. We can ship it to the home of a loved one with an included gift message. All funds raised support our Protect the Future program and training up the next generation of porphyria experts. 

This is a great chance to support the APF and the future of porphyria while enjoying 12-months of our furry, fuzzy, and even feathery friends.

To order call the APF office at 866.APF.3635 or email autumnlee@porphyriafoundation.org or visit the APF store using the link below.




Bookmark the APF Website as your favorite!


If you have not already done so, please take a moment and bookmark the APF website as one of your favorites! If this is something that you have done in the past, please remove the old bookmark and replace it with the newly updated website address.

Here is the Link: www.porphyriafoundation.org





Get Involved



Contact Information

Is your contact information up to date?
If not please give us a call @ 866-APF-3635 or Email to porphyrus@porphyriafoundation.com

American Porphyria Foundation 1.866.APF.3635 porphyriafoundation.org

STAY CONNECTED

"Remember.Research is the Key to Your Cure!"




Winter Sale in the New APF Store Check us out!

Saturday - December 1, 2018 @ 06:30:01

Looking for a great gift this winter.  Share with a loved one an APF Hat or Tshirt!  Extra cold this winter throw on an APF Hoodie or Zipper Hoodie- Looking good & a great price.  Check us out!

Are you or your child a Shadow Jumper?
We got the goods here this season- what are you waiting for come see us.

Give yourself a gift by being prepared when you have a flare or an emergency!  Stop by and order your comprehensive emergency kit!  Cant put a price on this valuable tool.  We have Kits in Acute Porphyrias & Cutaneous Types EPP!  Check us out..

https://porphyriafoundation.org/store




Thanks for your support and stopping by


The Porphyria Research Consortium: A Partnership Between the American Porphyria Foundation and Physician Researchers

Friday - November 30, 2018 @ 06:30:03

The Porphyria Research Consortium: A Partnership Between the American Porphyria Foundation and Physician Researchers

AM Rounds Slider Master-03
Editors Note: For more on patient advocacy groups, like the Porphyria Research Consortium, check out our AM Last Page, entitled Realizing the Potential of Patient Organizations in Translational ResearchDelivering Treatments for Rare Diseases.
By: Desiree Lyon Howe, executive director, American Porphyria Foundation
The American Porphyria Foundation (APF) has been instrumental in facilitating porphyria research for over thirty years. The porphyrias are a group of disorders caused by enzyme deficiencies in the heme biosynthetic pathway that affect the Central Nervous System and/or the skin. The collaborative relationship we enjoy with our Scientific Advisory Board is vital to the success of our research projects. This relationship became even more productive when we formed the Porphyria Research Consortium with these porphyria experts. The Consortium is now funded by the National Institutes of Health Rare Disease Clinical Network.
The success of this partnership was evident during the recent Afamelnotide clinical trials. In only one month, we located and registered 90 Erythropoietic Protoporphyria patients for the Phase II trials and 100 more patients for the Phase III trials. This unprecedented accomplishment was achievable because we maintain a well-developed research strategy that includes the Consortium in every step. Their participation, in turn, substantiates our research efforts and gives credibility to the medical information we publish. Such research and educational validation is important to gain the respect of primary care physicians.
Together, we and the Consortium created the National Porphyria Research Registry as a source of research patients and a means to determine incidence. Prior to the initiation of research projects, we prepare patients by educating them on the value and process of clinical trials, introducing them to the researchers, and encouraging them to join the Registry. We reduce patients fears and provide reassurance about the Registry, clinical trials, the expertise of the researchers, and the merit of the research by publishing articles on our website, in our newsletters, and on social media, (e.g., Facebook, Twitter, and blogs). We use these same methods to tout former research patient volunteers, who by sharing their experience, increase the number of future research volunteers.
Researchers in turn assist us by preparing the clinical trials announcements for patients and physicians, which we then distribute via our vast database. Another unique and successful collaboration occurs at our patient education meetings held around the country, during which we locate and register research participants. Three to five experts volunteer for these gatherings to make presentations, answer questions, register patients for their research, and often collect DNA samples.
Also important to our research efforts are the young physicians who participate in the Protect the Future (PTF) program, an APF-supported, mentor project to train the next generation of porphyria experts. The Consortium educates these physicians so they become well versed in both the clinical and basic sciences of the porphyrias and become ready to take on the role of future academic leaders in the field. As part of their training, PTF physicians also conduct mentored research and serve as clinicians at our satellite clinics where patients are given the rare opportunity to meet with a physician with porphyria expertise. In turn, at these clinics, PTF physicians interact with a new pool of research participants.
Our synergistic relationship with our Scientific Advisory Board of experts is key to the success of our research programs.


GET INVOLVED WITH APF

Wednesday - November 28, 2018 @ 10:46:24

GET INVOLVED WITH APF

Become a member of the American Porphyria Foundation today, and join us in our work for your good health!
When you join the APF, you become an integral part of an organization that empowers patients and helps them on the road to accurate diagnosis, proper care, and some day a cure. Adding your voice to ours makes us all stronger as we address our needs to health care providers, local, state and federal agencies and Congress. If you feel you have porphyria but do not have tests indicating you have one of the diseases, you are welcome to join the APF to keep abreast of new discoveries in diagnostics and treatment.
Members of the American Porphyria Foundation receive:
  • Our quarterly newsletter  news about porphyria research and clinical studies, scientific meetings and member get-togethers, and stories about the doctors working in the porphyria field and about members like you;
  • Attend APF Patient Educational meetings with Experts Nationwide;
  • Receive weekly E-News in your email;
  • Free participation in telephone conference calls with top researchers in the porphyria field;
  • Knowledgethat you are an important part of keeping reliable medical information about porphyria available to those who are newly diagnosed, or at a crossroads in their porphyria treatment, and frightened;
  • And much more!
The American Porphyria Foundation relies on member support to sustain programs like this website and other literature written or approved by porphyria experts.
U.S. memberships are $35 annually; US membership suggested annual donation is $35. Donate and receive a FREE Porphyria Live DVD, as well as a number of pertinent brochures. International memberships are $45. The APF is a U.S. 501(c)(3) non-profit organization. All donations to the APF are tax deductible.
Join the APF today! Call us or join online.
To join by phone with your VISA or MasterCard, call us toll-free at 1-866-APF-3635 Monday-Friday from 9:00 a.m. to 4:00 p.m. Central Time. To join by mail or fax, please click here to complete and print the donation form.
Unable to pay? Membership is free to all.  The fee is only a suggested donation.
American Porphyria Foundation
4915 St. Elmo Avenue, Suite 105 
Bethesda, MD 20814

Work with us!

The American Porphyria Foundation welcomes donations of your time and talents too. Would you like to volunteer or raise money for the APF, organize or attend an event, in addition to informing yourself about the porphyrias? If so, welcome! Youll find lots more information in this section of our website on opportunities to get involved in our, and you can always call our office for help: 301-347-7166.
The APF is here to serve, and were proud to have you with us.


THE GLUCOSE EFFECT IN ACUTE PORPHYRIAS

Monday - November 26, 2018 @ 13:47:55

THE GLUCOSE EFFECT IN ACUTE PORPHYRIAS

vial.jpgThe disorders Acute Intermittent Porphyria (AIP), Hereditary Coproporphyria (HCP), Variegate Porphyria (VP) and ALAD Porphyria (ADP) are treated initially with the administration of carbohydrate/glucose. This therapy has its basis in the ability of glucose to decrease porphyrin biosynthesis in the liver.
Glucose can diminish excess excretion of heme precursors, which, in turn, can prevent an attack or can hasten recovery from an attack of the acute porphyrias. Therefore, it is suggested that when patients cannot consume carbohydrates due to nausea or vomiting, glucose should be administered intravenously. Some physicians have prepared a standing order for patients who are prone to attacks to help facilitate intravenous glucose in the emergency room. Often this prevents further hospitalization.
Most patients are knowledgeable about the deleterious affect the wrong drug can have on their bodies and are consequently careful about the medication they ingest, but some do not understand the importance of carbohydrates for prevention and treatment of a Porphyria attack.
Because it is a simple therapy, many patients ignore the significance of their carbohydrate (sugar) intake to suppress disease activity. When the Atkins Diet, which was a high protein/low carbohydrate diet, became popular a number of years ago, many Porphyria patients who adhered to this diet became ill. It soon became apparent that their severely reduced daily carbohydrate count exacerbated their Porphyria. In fact, complying with the Atkins plan precipitated attacks in some previously undiagnosed and non-symptomatic individuals.


Support Giving Tuesday

Wednesday - November 28, 2018 @ 19:10:14

Please support the American Porphyria Foundation today! #GivingTuesdayis a global day of giving focused on organizations that have an impact on something you care about. The APF cares about making each day better for individuals and families living with Porphyria. 
Every dollar counts as we work to educate physicians, raise awareness and promote research!! 
This year Facebook and PayPal are partnering together to match up to a total of $7 million in donations to US nonprofits on #GivingTuesday!
 We appreciate all that our members do each day to help us reach our goals - thank you for considering a donation today. Like, Tag and Share!
Click the link below to donate!
https://www.facebook.com/donate/360039071418367/



GLOBAL PORPHYRIA ALLIANCE

Wednesday - November 21, 2018 @ 14:53:24

GLOBAL PORPHYRIA ALLIANCE

The APF is proud to support our members that live across the globe. Many countries around the world have developed organizations to support their local communities. We hope that enjoy the same opportunity to communicate with one another, develop friendships, and learn about porphyria.
Here are links to patient advocacy groups worldwide that offer support to individuals impacted by porphyria.


WHAT IS NEUROPATHIC PAIN?

Wednesday - November 21, 2018 @ 14:51:32

What could it be?
Nerve Pain
Take a pain questionnaire by the My Pain Feels Like initiative
 

WHAT IS NEUROPATHIC PAIN?

Neuropathic or nerve pain (NP) is a long-term or chronic pain disease that results from nerve damage. It can be caused by different diseases or conditions. Worldwide nerve pain affects as many as 26 million people. Neuropathic or nerve pain may affect larger areas of the body or it can be restricted to a smaller area, in this case it is called localized neuropathic pain (LNP).

WHAT CAUSES NEUROPATHIC PAIN?

Neuropathic or nerve pain may occur in the absence of an obvious visible cause (e.g. an accident, an injury, a chemical burn). There are several external situations that can directly damage nerves and lead to neuropathic pain, such as:
Neuropathic pain is also a common complication of other diseases, including nerve damage after shingles or herpes zoster infection (postherpetic neuralgia or PHN), nerve damage after HIV infection, nerve damage resulting from diabetes mellitus (diabetic polyneuropathy or DPN), multiple sclerosis, or nerve damage in the spine or lower back (low back pain).

WHAT ARE THE TYPICAL NEUROPATHIC PAIN SYMPTOMS?

Neuropathic pain is a chronic pain condition often described by patients with symptoms such as shooting pain, burning pain, or stabbing pain. It can also feel like pins and needles. In addition, neuropathic or nerve pain patients can suffer from symptoms such as allodynia (when a normally unpainful stimulus such as a light touch or clothing becomes painful) and hyperalgesia (when a mild or moderate stimulus causes severe pain).

HOW IS NEUROPATHIC PAIN DIAGNOSED?

Neuropathic pain is often difficult to diagnose. This can lead to patients with neuropathic or nerve pain being insufficiently treated on a trial and error basis that may persist over many months or even years. A correct and early diagnosis is crucial to find the right treatment and to relieve symptoms of neuropathic pain. It is therefore important that patients describe their symptoms in as much detail as possible to their doctor. As part of this process, the doctor will conduct a physical examination and ask about the medical history of the symptoms.

WHAT CAN PATIENTS DO?

There are treatment options available. It is important to get active. Do you have symptoms that you would describe as burning pain, shooting pain, or stabbing pain? Do you have sensitive skin and/or feel a sensation like pins and needles on the skin?
If you have chronic pain and think that it might be neuropathic or nerve pain, please fill out the my painquestionnaire and see your doctor. It is a very useful tool to improve the communication between patients and doctors, as it supports the doctor in making an accurate diagnosis of the cause of chronic pain. It will be most useful for you to accurately describe your chronic pain, where it occurs on your body and if it is triggered by anything in particular. In preparation for a doctors appointment, patients should thoughtfully complete the my painquestionnaire, print out the results, and discuss them with their doctor. You can read more about possible treatment options here.


Porphyria Post

Thursday - November 15, 2018 @ 06:30:11

Porphyria Post

Happy Thanksgiving!

APF Office Closure
We would like to wish you all a very Happy Thanksgiving! For those of you traveling this Holiday, please have a safe and fun trip.

The American Porphyria Foundation will be closed on Thursday, November 22, 2018 and reopen for normal business hours on Monday, November 26, 2018. Please see below for some additional announcements from the APF.

Harvoni Study - PCT
Do you have PCT? Are you interested in participating in research? Do you have Hepatitis C? If you answered YES to these questions, this is for you.

We need YOU for a clinical trial!

The purpose of this clinical trial is to assess whether Harvoni alone is an effective therapy in active PCT patients with Chronic Hepatitis C.

Who can participate?

â?¢ Adult patients with PCT who also have Hepatitis C

If you are interested in participating please contact Edrin Williams, Director of Patient Services at the APF office at 301.347.7166 for additional information.
Clinical Trial for EPP - We NEED you!
The Mitsubishi Clinical Trial is in danger of being cancelled if we do not fully enroll with 102 participants. We must have full participation in order to push this new drug toward approval. We know that a Phase 3 trial is very unlikely if our small EPP community does not come together to fill this trial. Adults who participate will pave the way for a near-future pediatric trial.

Here are the facts. We need 102 total participants for this clinical trial. There are 500+ known patients with EPP in the US, including children and older adults. 1 in 4 patients meet the age requirement. Of those approximately 400 patients, another subset cannot participate due to medical exclusions (other needed medications, high liver enzymes, history of melanoma, etc.) To date, the drug has proven non-toxic and there have been essentially no dropouts in this study to date. The promise of a new treatment depends on you.
Call the APF on 866-APF-3635 or 301-347-7166.

We will connect you with a Research Coordinator that will answer all your Questions and concerns. We will work to make this process as easy as possible for you. It is 5 months of your life in order to give a future to ALL with EPP! To date there is no public information on the future of Scenesse. If we are able to get two FDA-approved treatments on the market, it may lower costs. If we do not do all we can as a community, we will have no treatment of EPP.

Bottom line: We need YOU! Please dig deep and consider being part of this changing moment for all with EPP.
Pet Calendar Update!
Thank you all for submitting your pets for the 2019 Pet Calendar Contest.

We have had an overwhelming response of submissions from our community. Please note that we are working diligently to have this calendar ready for you to purchase soon. Stay tuned for more details!

RSVP Today!

Patient Education and Support Meeting

LOCATION
Double Tree Hotel - San Diego - Hotel Circle
DATE AND TIME
12/01/18 6:00pm - 12/01/18 8:00pm
1515 Hotel Circle South San Diego, CA 92108 Room:Cortez Family and Friends are welcome to attend. We look forward to seeing you there.

Contact Information

Is your contact information up to date?
If not please give us a call @ 866-APF-3635 or Email to porphyrus@porphyriafoundation.com

American Porphyria Foundation 1.866.APF.3635 porphyriafoundation.org

STAY CONNECTED
"Remember.Research is the Key to Your Cure!"


Michelle Bridges AIP

Wednesday - November 7, 2018 @ 17:56:13

I spent my whole life with unexplained symptoms. Sometimes my legs would give out, and I would have back pain, abdominal pain, slurred speech, difficulties walking and so forth. When I was 16, doctors found a mass in my brain. After removing what they could, all my health problems were blamed on post surgical changes.
michellebridges.jpgIn 2005, the doctors began to wonder about other causes. I was sent to see a Gastroenteritis  and diagnosed with pancreatitis. But there was no explanation for my abdominal pains that would come out of nowhere and then disappear. The doctors decided that is was due to a problem with my digestion and cut part of my bile duct. However, I landed back in the hospital with pancreatitis. Then nothing happened for about eight months.  
At the end of July 2006, I began to have problems again. They put me on Reglan with the intention of running more tests and after about a week, I woke up feeling bizarre. I was in a haze and unable to function. I had called a friend and asked her to take me to the hospital. I turned to walk down the hall towards my bedroom so I could lie down, but my legs would not move. I remember standing there thinking you bend the knee and extend the leg. By the time I had dragged myself down the hall, my arms wouldn't cooperate and I fell over.
When the paramedics got the house I swear I asked if I was having a stroke. My son says I just lay there with my tongue hanging out, in and out of consciousness and seizure activity.  I don't remember. The ER released me to my husband with the diagnosis allergic reaction. The following day I could not speak. Again, I went to the ER, but this time it was to a different hospital, one covered through our insurance. The doctor told my husband I was being over-medicated, and then a student at the hospital noticed the color of my urine and suggested to the doctor that my condition might be porphyria. She was correct!
I don't remember anything from the end of 2006 to 2008. Treatment for my symptoms made some things worse. I had been active in my church and home schooled other peoples children as well as my own, but I had to stop educating others and withdrew from some activities at church.
We have had a hard time finding doctors who will treat me. This disease is rare and doctors aren't experienced with it. With my new oncologists gentle touch and the support of the staff at our new hospital, I have become more active. I am not spending as much time in the hospital, and I still home school my children, write curriculum and resource guides, and do crafts for craft fairs and whatever else I can to support the church and help others. I do all of these activities from my bed because I am too weak and have too much pain to leave the bedroom for long stretches and I get sick so easily. But I do like to have some level of activity in my life and so I try to contribute where I can while being careful of my health.


Porphyria Post

Wednesday - October 31, 2018 @ 16:27:14


Porphyria Post



Last Day to Vote!

Tomorrow is the last day to capture votes for your furry friends! We look forward to sharing the results with our community.

If you haven't already done so, please go to the APF website to see everyone's submissions!

Make sure that you see your share this link and your individual voting pages with your family and friends.

The final 12-month wall calendar will feature your wonderful animals, porphyria facts, tips, and important dates. Voting for the top slots will be held online and will benefit our critical Protect the Future program.


Heres how it works:





To Vote:
·    ALL photo entries will be included somewhere in the calendar, though the top dogs will be featured on the cover and in each month!
·    Starting October 23rd - Your pets photo will be posted on the APF website for all to see. Youll be able to share the link with family and friends to encourage a vote! Anyone can participate with a $1+ donation per vote.

Important Dates:

·    Voting: October 23-November 8
·    Calendars ready for sale and shipping on November 25th!


Please contact Autumnlee at the APF office with any questions at: autumnlee@porphyriafoundation.org or call (301) 347-7166.

GOOD LUCK TO EVERYONE and HAVE FUN!



EPP Clinical Trials - NEED PARTICIPANTS
Are you ready to be a medical hero? A new clinical trial with an oral investigational drug intended to reduce phototoxicity in people living with EPP has begun. Dont delay!

Pharmaceutical company: Mitsubishi Tanabe
Length on treatment: 16 weeks
Treatment: Oral (pills)
Number of appointments: 7-9 
Arrangements: Includes Travel/hotel/food
Stipend: Each participant will receive a stipend
Locations: New York City, NY/Winston-Salem, NC/Miami, FL/Galveston, TX/Salt Lake City, UT/San Francisco, CA 
How many participants do we need: 102!!!

Please contact the APF at 1.866.APF.3635 if you are interested in participating.

"RememberResearch is the key to YOUR cure!"
Each Step Toward Finding an Effective Treatment is Important!

Website Update!

You are now able to access the new website from any browser and on any device at www.porphyriafoundation.org

Thank you to our members, industry partners, and our brilliant physicians for making this update possible!

RSVP TODAY!
Patient Education and Support Meetings coming your way. Contact the APF today to RSVP! We look forward to seeing you there.

Patient Education and Support Meeting - San Francisco

LOCATION
UCSF Medical Center 505 Parnassus Ave. Room: S-157 San Francisco, CA 94143
DATE AND TIME
11/11/18 5:30pm - 11/11/18 7:00pm
Seating is limited. Please RSVP Today!!



Patient Education and Support Meeting - San Diego

LOCATION
Double Tree Hotel - San Diego - Hotel Circle 1515 Hotel Circle South San Diego, CA 92108
DATE AND TIME
11/11/18 5:30pm - 11/11/18 7:00pm
Room: SOLANA Please RSVP Today! Please note that there will be a Porphyria Specialist on site. We look forward to seeing you there!






Get Involved




Contact Information

Is your contact information up to date?
If not please give us a call @ 866-APF-3635 or Email to porphyrus@porphyriafoundation.com

American Porphyria Foundation 1.866.APF.3635 porphyriafoundation.org

STAY CONNECTED


"Remember.Research is the Key to Your Cure!"



Porphyria Post ~ BRAND NEW WEBSITE TOMORROW!~

Tuesday - October 30, 2018 @ 21:32:42



Porphyria Post


BRAND NEW WEBSITE TOMORROW!



We are excited to announce that we will launch our newly updated APF website TOMORROW. It is optimized to offer the patient community and healthcare professionals easy access to critical content - written by renowned experts - on all of the porphyrias.

On Thursday, November 1, 2018 you will be able to access the new website from any browser and on any device at

Thank you to our members, industry partners, and our brilliant physicians for making this update possible!

Mobile view of the New APF Homepage!




Pet Calendar Update
CALENDAR UPDATE: We are a week into voting and have seen an outstanding response! There is still a week left so remember to vote for your favorite pet picture and show your support of the APF. You can visit the APF website and see all of our amazing entries to pick who you want to see on the cover of the 2019 APF Pet Calendar! All photos will be included in the calendar though. Happy voting! 

Important Dates:

Voting: October 23rd - November 8th
Calendars ready for sale and shipping on November 25th!

Link to entries on APF Website: APF Website Contest Page


AMAZON SMILE
Starting your holiday shopping? Many of you purchase numerous items from Amazon. Did you know that your you can support the APF through the AmazonSmile program! Amazon donates 0.5% of the price of your eligible AmazonSmile purchases to the charitable organization of your choice.

Please make the APF your choice of a charitable donation. Support porphyria while shopping!

Note, this program to provide donations to the APF will ONLY be available to shoppers who visit Amazon via a special web address, namely, www.smile.amazon.com instead of the normal www.amazon.com homepage.

It is easy and free! AmazonSmile is the same Amazon you know - same products, same prices, same service.

Thank you for supporting us!  Please follow the link:  http://smile.amazon.com/ch/36-4401266 .

Once you are logged in you will see at the top of your screen that you are supporting the American Porphyria Foundation.

RSVP TODAY!
Patient Education and Support Meetings coming your way. Contact the APF today to RSVP! We look forward to seeing you there.

Patient Education and Support Meeting - San Francisco

LOCATION
UCSF Medical Center 505 Parnassus Ave. Room: S-157 San Francisco, CA 94143
DATE AND TIME
11/11/18 5:30pm - 11/11/18 7:00pm
Seating is limited. Please RSVP Today!!



Patient Education and Support Meeting - San Diego

LOCATION
Double Tree Hotel - San Diego - Hotel Circle 1515 Hotel Circle South San Diego, CA 92108
DATE AND TIME
12/01/18 6:00pm - 12/01/18 8:00pm
Room: SOLANA Please RSVP Today! Please note that there will be a Porphyria Specialist on site. We look forward to seeing you there!






Get Involved




Contact Information

Is your contact information up to date?
If not please give us a call @ 866-APF-3635 or Email to porphyrus@porphyriafoundation.com

American Porphyria Foundation 1.866.APF.3635 porphyriafoundation.org

STAY CONNECTED

"Remember.Research is the Key to Your Cure!"



Porphyria POST

Thursday - October 25, 2018 @ 22:22:25

VOTE: 2019 Pet Calendar, RSVP - Patient Education Meetings and EPP Clinical Trials

Let the voting begin!
Submissions have now closed for the 2019 Pet Calendar Contest. We appreciate all of your entries and can't wait to see how well everyone does in the contest.
If you haven't already done so, please go to the APF website to see everyone's submissions!
Make sure that you see your share this link and your individual voting pages with your family and friends.
The final 12-month wall calendar will feature your wonderful animals, porphyria facts, tips, and important dates. Voting for the top slots will be held online and will benefit our critical Protect the Future program.

Heres how it works:
To Vote:
ALL photo entries will be included somewhere in the calendar, though the top dogs will be featured on the cover and in each month!
Starting October 23rd - Your pets photo will be posted on the APF website for all to see. Youll be able to share the link with family and friends to encourage a vote! Anyone can participate with a $1+ donation per vote.
Important Dates:
  • Voting: October 23-November 8
  • Calendars ready for sale and shipping on November 25th!
Please contact Autumnlee at the APF office with any questions at:autumnlee@porphyriafoundation.org or call (301) 347-7166.
GOOD LUCK TO EVERYONE and HAVE FUN!

EPP Clinical Trials - NEED PARTICIPANTS
Are you ready to be a medical hero? A new clinical trial with an oral investigational drug intended to reduce phototoxicity in people living with EPP has begun. Dont delay!
Pharmaceutical company: Mitsubishi Tanabe
Length on treatment: 16 weeks
Treatment: Oral (pills)
Number of appointments: 7-9
Arrangements: Includes Travel/hotel/food
Stipend: Each participant will receive a stipend
Locations: New York City, NY/Winston-Salem, NC/Miami, FL/Galveston, TX/Salt Lake City, UT/San Francisco, CA
How many participants do we need: 102!!!
Please contact the APF at 1.866.APF.3635 if you are interested in participating.
"RememberResearch is the key to YOUR cure!"
Each Step Toward Finding an Effective Treatment is Important!

New Publication - Journal of Pediatrics
Dr. Manisha Balwani, Porphyria Expert at Mount Sinai in New York City recently authored a publication that was recently released in the Journal of Pediatrics.
Please click on the link below to review.
RSVP TODAY!

Patient Education and Support Meetings coming your way. Contact the APF today to RSVP! We look forward to seeing you there.
Patient Education and Support Meeting - Missouri
LOCATION
THIS MEETING HAS BEEN CANCELLED
DATE AND TIME
10/27/18 12:00pm - 10/27/18 2:00pm
This meeting has been cancelled and will be rescheduled at a later date. If you have any questions, please reach out to Edrin at the APF!

Patient Education and Support Meeting - San Francisco
LOCATION
UCSF Medical Center 505 Parnassus Ave. Room: S-157 San Francisco, CA 94143
DATE AND TIME
11/11/18 5:30pm - 11/11/18 7:00pm
Seating is limited. Please RSVP Today!!

Patient Education and Support Meeting - San Diego
LOCATION
Double Tree Hotel - San Diego - Hotel Circle 1515 Hotel Circle South San Diego, CA 92108
DATE AND TIME
11/11/18 5:30pm - 11/11/18 7:00pm
Room: SOLANA Please RSVP Today! Please note that there will be a Porphyria Specialist on site. We look forward to seeing you there!


Emergency Room Guidelines for Acute Porphyrias

Thursday - October 25, 2018 @ 22:19:29


Emergency Room Guidelines for Acute Porphyrias
These Emergency Room Guidelines (download PDF) cover essential information for the emergency physician treating a patient in an acute porphyria attack, including common precipitating factors, typical presentation and other diagnostic clues, making the initial diagnosis, common sequelae and best practices for treatment. A PowerPoint presentation (as PDF) for instruction is also available.
Please note: These Guidelines are for Physician Use Only. The APF sells a separate, personalized Emergency Room and Primary Care Physician Kit that contains all the information acute porphyria patients need to have with them in the Emergency Room (medical journal articles, information about diagnostic labs and Panhematin, room for your own diagnostic test results).
Neville R Pimstone MD, PhD, Head Liver Diseases Greater West Los Angeles Veterans Affairs, Professor Emeritus UC Davis
Karl E. Anderson MD, Professor, Departments of Preventive Medicine and Community Health, Internal Medicine, Pharmacology and Toxicology; Associate Director, General Clinical Research Center, Director, Porphyria Center and Laboratory, University of Texas Medical Branch, Galveston, Texas
Bradley Freilich, MD, Kansas City Gastroenterology and Hepatology, LLC

KEY POINTS
1. The human porphyrias are clinical disorders reflecting defects in heme biosynthesis.
2. Acute porphyrias cause acute attacks of neurological symptoms that can be life-threatening.
3. Acute attacks are triggered by certain drugs, sex steroid hormones, reduced intake of calories and carbohydrate, alcohol and unknown factors.
4. Many of these factors stimulate heme synthesis in the liver, which in the face of a metabolic enzyme defect, leads to increased production of heme precursors that may be neurotoxic.
5. Delta-aminolevulinic acid (ALA) and porphobilinogen (PBG), are porphyrin precursors and intermediates in the heme biosynthetic pathway.
6. ALA and porphobilinogen (PBG) are almost always elevated in urine during an acute attack of porphyria.
7. The most common emergency room (ER) clinical presentation is acute abdominal pain. Other features may include seizures, confusion and hallucinations, and a progressive polyaxonal motor neuropathy, which can progress to paralysis and respiratory failure requiring a ventilator.
8. A high index of suspicion in the presence of nonspecific symptoms is important for diagnosis. A family history of porphyria, female sex, onset during the luteal phase of the menstrual cycle, or recent use of a porphyrinogenic drug may be diagnostic clues.
9. A new diagnosis of porphyria as the cause of acute symptoms must be substantiated by finding a substantial increase in urine porphobilinogen (PBG).
10. Treatment should start promptly after the diagnosis is made. Mild attacks are sometimes treated with glucose loading (e.g. 3L of 10% glucose daily by vein).
11. Most acute attacks should be treated with hemin (Panhematin, Recordati Rare Diseases at: www.recordatirarediseases.com or 866-654-0539) 3-4mg/kg into a large peripheral vein or venous access port daily for 4 days. Reconstituting Panhematin with human serum albumin rather than sterile water is recommended prior to infusion. This helps prevent phlebitis at the site of intravenous infusion.
12. Hospitalization is usually required for symptomatic treatment of pain, nausea and vomiting, correction of electrolyte imbalance and observation for respiratory impairment, either to a general medical service or ICU.



Pediatrics & EPP

Wednesday - October 24, 2018 @ 16:00:08

Share your article!

Dear Dr. Balwani,
We are pleased to let you know that the final version of your article Diagnostic Delay in Erythropoietic Protoporphyria is now available online, containing full bibliographic details.
To help you access and share this work, we have created a Share Link a personalized URL providing 50 days' free access to your article. Anyone clicking on this link before December 11, 2018 will be taken directly to the final version of your article on ScienceDirect, which they are welcome to read or download. No sign up, registration or fees are required.
Your personalized Share Link:
https://authors.elsevier.com/a/1Xx5s55CrsSvu
Click on the icons below to share with your network:


WRITTEN ALL OVER HIS FACE: RARE DISEASE OFFERS CLUES TO HOW WE READ EMOTIONS

Thursday - October 18, 2018 @ 17:11:02

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.


10/18/18 Porphyria Post

Thursday - October 18, 2018 @ 17:06:04

PLEASE READ IMPORTANT!
October 18, 2018

Porphyria Post
Harvoni Study - PCT
Do you have PCT? Are you interested in participating in research? Do you have Hepatitis C? If you answered YES to these questions, this is for you.
We need YOU for a clinical trial!
The purpose of this clinical trial is to assess whether Harvoni alone is an effective therapy in active PCT patients with Chronic Hepatitis C.
Who can participate?
â?¢ Adult patients with PCT who also have Hepatitis C
If you are interested in participating please contact Edrin Williams, Director of Patient Services at the APF office at 301.347.7166 for additional information.

WE NEED YOU FOR THE EPP CLINICAL TRIAL!
IF NOT YOU, THEN WHO??
1. Do you have a diagnosis of EPP?
2. Are you interested in participating in research?
If so, we NEED you!
Participants are desperately needed for participation in a Phase II clinical trial to study an investigational drug for EPP. CONTACT THE APF ON 1-866-APF-3635 or email porphyrus@porphyriafoundation.com to learn more.
An oral investigational drug has been developed with the potential to increase sunlight duration and tolerance in individuals with EPP. This phase II clinical trial is needed to understand if the investigational drug works and to ensure that it is safe. The study duration is 24 weeks including follow-up. You must be age 18-70 to participate and have a confirmed diagnosis of EPP. All travel is included and will be arranged by a concierge service.
â?¢ We need patients at all participating sites (NYC, Wake Forest, Miami, Utah and UCSF)
â?¢ Please note that the site at the University of Utah have just opened for enrollment.
â?¢ The site at the University of California San Francisco will be enrolling patients soon.
Contact the APF today! 1-866-APF-3635.
"RememberResearch is the key to YOUR cure!"
Each Step Toward Finding an Effective Treatment is Important!

Pet Calendar Update!
Thank you all for submitting your pets for the 2019 Pet Calendar Contest. Submissions are due by Sunday, October 21, 2018. See the instructions below on how you can get involved.
We have had an overwhelming response of submissions from our community. Please note that we are working diligently to upload your pet photos to the APF Website. Please be on the lookout for updates from the APF team regarding your profile.

CALLING ALL PETS!!!
Welcome to the 2019 APF Pet Calendar Contest YOUR pet can win a coveted spot on this fun and informational calendar! The final 12-month wall calendar will feature your wonderful animals, porphyria facts, tips, and important dates. Voting for the top slots will be held online and will benefit our critical Protect the Future program. Enter your pets then a link will be created to encourage voting! Pets are an important part of our lives and provide much-needed unconditional love in tough times. We look forward to showing off your special friends as your photos roll in!!
Heres how it works:
To Enter (free!):
â?¢ Email a picture of your pet(s) to the APF by October 21st.
â?¢ Send to autumnlee@porphyriafoundation.org with the subject heading APF Pet Calendar. Make sure to include:
â?¢ Pet type and name
â?¢ Owner name, email, and mailing address**
â?¢ Send a caption, too! (optional)
To Vote:
â?¢ ALL photo entries will be included somewhere in the calendar, though the top dogs will be featured on the cover and in each month!
â?¢ Starting October 17th - Your pets photo will be posted on the APF website for all to see. Youll be able to share the link with family and friends to encourage a vote! Anyone can participate with a $1+ donation per vote.
Rules:
â?¢ You may enter one photo that contains as many of your pets you wish to include. Remember that this photo will be voted on so choose your best!
â?¢ Anyone can enter
â?¢ No professional or copyrighted photos
â?¢ Any type of pet can be submitted; past and present
â?¢ No humans please
â?¢ Photos cannot be photo shopped or professionally edited
Important Dates:
â?¢ Submissions due by October 21st
â?¢ Voting: October 23-November 8
â?¢ Calendars ready for sale and shipping on November 25th!
Thank you to Nicole Castellano for leading this effort for the APF!
Please contact Autumnlee at the APF office with any questions at: autumnlee@porphyriafoundation.org or call (301) 347-7166.
GOOD LUCK TO EVERYONE and HAVE FUN!
Get Involved
â?¢ Research â?¢
â?¦ Donate â?¦
â?¢ Visit our Website â?¢
Contact Information
Is your contact information up to date?
If not please give us a call @ 866-APF-3635 or Email to porphyrus@porphyriafoundation.com
American Porphyria Foundation 1.866.APF.3635  porphyriafoundation.org
STAY CONNECTED

"Remember.Research is the Key to Your Cure!"




Newest PTF Amy Dickey

Monday - October 15, 2018 @ 06:30:01

Welcome our newest Protect The Future Doctor Amy Dickey. She will be training with our Expert Dr. Karl Anderson @ UTMB and she will have other mentors. Please give her a warm welcome and watch her presentation below:

https://partners.zoom.us/recording/play/k7MJO7Vr7gIW1qWufO9QkS1n0-zeDOeE0JVAR_i0w-L7yCk2U-LjjloMXiw6w1Av?startTime=1539360794000

Last week I was able to teach on porphyria to the internal medicine residents at Massachusetts General Hospital. Below is a link with the video if youre interested in hearing it. Its a talk to physicians, so be prepared for lots of medical jargon â?º

Im a physician at MGH trained in internal medicine, pulmonary, and critical care, and I have EPP. Padmini Pillai who shares at the end is a post-doc in biomedical engineering at MIT, and she has AIP.
#porphyria #awareness


Treatment and Management of EPP

Saturday - October 13, 2018 @ 23:34:51

Treatment and Management
1.  Sunlight protection
Protection from sunlight is the mainstay of management of EPP, and this is necessary throughout life.  Disease severity and porphyrin levels in erythrocytes and plasma probably remain high and relatively constant throughout life in EPP.  However, this has been little studied and more longitudinal observations are needed.  Life style, employment, travel and recreation require adjustment in order to avoid painful reactions to sunlight and even from exposure to fluorescent lighting.  For these reasons EPP can substantially affect quality of life. 
Protective clothing, including broad-brimmed hats, long sleeves, gloves and trousers (rather than shorts), is beneficial.  Several manufacturers specialize on clothing made of closely woven fabrics for people with photosensitivity. 
2.  Other considerations
In an occasional patient, protoporphyrin causes liver problems, so monitoring liver function is important. EPP patients should also not use any drug or anesthetic which causes cholestasis (slowing down bile flow), and should also avoid alcohol. Women should avoid medications containing estrogen (birth-control pills, hormone replacement therapy), and men should avoid testosterone supplements, as these substances can also have deleterious effects on the liver of a person with EPP.
Consult a specialist.  Because EPP is a rare condition, most physicians are not knowledgeable about it.  Contact The American Porphyria Foundation, 713-266-9617 for contact with an expert and to provide further information.  A Medic Alert bracelet with instructions to contact a specialist if needed is a worthwhile precaution.
Yearly monitoring.  Testing to include erythrocyte total protoporphyrin, plasma porphyrin, complete blood counts, ferritin and liver function tests should be done yearly.  Porphyry levels are expected to be stable and liver tests to remain normal.  EPP patients may have evidence of iron deficiency, and an iron supplement may be advisable if the serum ferritin is below about 20 ng/mL. 
Vitamin D.  Because they avoid sunlight, EPP patients are likely to be deficient in vitamin D.  A vitamin D supplement with calcium is recommended for bone health. 
Liver protection.  It is important to avoid other causes of liver disease that might promote the development of liver complications from EPP.  Patients should avoid alcohol and other substances that might damage the liver, including many herbal preparations, and be vaccinated for hepatitis A and B. 
Surgical lights. Strong operating room lights can cause photosensitivity of the skin and even surfaces of internal organs.  Flexible membrane filters, such as CL5-200-X from Madico Co., are available to cover surgical lights and offer some protection.  This is especially important in EPP patients with liver failure, which causes even greater increases in protoporphyrin levels and photosensitivity. 
Drugs.  Drugs that are harmful in other porphyrias are not known to make EPP worse, but are best avoided as a precaution.  This may include estrogens and other drugs that might reduce bile formation.  A short course of a non-steroidal anti-inflammatory drug can provide some pain relief after an episode of photosensitivity, but can cause ulcerations of the digestive track especially with prolonged use. 
Laser treatment.  According to Dr. Roth, laser treatments for hair removal or eye surgery have not been a problem in EPP people.  But the doctor should be made aware of the diagnosis, and that laser output between 400 and 650 nanometers might be harmful. Before hair removal treatment, the doctor may irradiate a small area of the skin to be treated for the length of time it will take to do the hair removal to ascertain if the patient would react within the period of time that a reaction to sunlight would be expected in that patient.  



CALLING ALL PETS!!!

Thursday - October 11, 2018 @ 06:30:03

CALLING ALL PETS!!!
Welcome to the 2019 APF Pet Calendar Contest YOUR pet can win a coveted spot on this fun and informational calendar! The final 12-month wall calendar will feature your wonderful animals, porphyria facts, tips, and important dates. Voting for the top slots will be held online and will benefit our critical Protect the Future program. Enter your pets then a link will be created to encourage voting! Pets are an important part of our lives and provide much-needed unconditional love in tough times. We look forward to showing off your special friends as your photos roll in!!
Heres how it works:
To Enter (free!):
â?¢ Email a picture of your pet(s) to the APF by October 21st.
â?¢ Send to autumnlee@porphyriafoundation.org with the subject heading APF Pet Calendar. Make sure to include:
â?¢ Pet type and name
â?¢ Owner name, email, and mailing address**
â?¢ Send a caption, too! (optional)

To Vote:
â?¢ ALL photo entries will be included somewhere in the calendar, though the top dogs will be featured on the cover and in each month!
â?¢ Your pets photo will be posted on the APF website for all to see. Youll be able to share the link with family and friends to encourage a vote! Anyone can participate with a $1+ donation per vote.
Rules:
â?¢ You may enter one photo that contains as many of your pets you wish to include. Remember that this photo will be voted on so choose your best!
â?¢ Anyone can enter
â?¢ No professional or copyrighted photos
â?¢ Any type of pet can be submitted; past and present
â?¢ No humans please
â?¢ Photos cannot be photo shopped or professionally edited
Important Dates:
â?¢ Submissions due by October 21st
â?¢ Voting: October 23rd-November 8th
â?¢ Calendars ready for sale and shipping on November 25th!
Thank you to Nicole Castellano for leading this effort for the APF!
Please contact Autumnlee at the APF office with any questions at: autumnlee@porphyriafoundation.org or call (301) 347-7166.

GOOD LUCK TO EVERYONE and HAVE FUN!
Click below to watch Nicole's Video Introduction!


REVIEW FOR ACUTE HEPATIC PORPHYRIA

Tuesday - October 9, 2018 @ 06:30:03

Recordati Rare Diseases is launching a new 350mg dosage strength of Panhematin

Recordati Rare Diseases is launching a new 350mg dosage strength of Panhematin.  This is the first significant change to Panhematin since it was approved over 30 years ago.  The prescribing instructions have numerous updates including the removal of the boxed warning, addition of clinical studies section, updates to the adverse events section, and changes to dosing and administration.  The distribution process for Panhematin will stay the same.
The new dosage strength is being launched to better accommodate the current weight of patients and the current dosing of Panhematin in clinical practice.
A new copay assistance program for Panhematin will be available starting in July 2017.  This program can help eligible patients with their copay when they receive Panhematin on an outpatient basis.  Please see the Panhematin website (www.panhematin.com) for more information.


What is Variegate Porphyria?

Tuesday - October 2, 2018 @ 06:30:14

Variegate Porphyria (VP)


What is Variegate Porphyria?
VP is caused by a mutation in the enzyme protoporphyrinogen oxidase (PPOX), which is part of the pathway that produces porphyrins and heme. Acute attacks are similar to those in AIP and HCP but are unusual. A more common sign of the disease is blistering skin lesions, which are chronic in many people with VP.
Acute attacks almost always start with severe pain in the abdomen but sometimes in the chest, back, or thighs, and are often accompanied by nausea, vomiting, and constipation. Heart rate and blood pressure are commonly increased. These symptoms and signs are all due to the effects of the disease on the nervous system. Confusion, convulsions, and muscular weakness, due to impairment of the nerves controlling the muscles, may lead to paralysis. An acute attack usually lasts for days or weeks. Recovery from severe paralysis is generally slow.

Who gets Variegate Porphyria?
VP is especially common in South Africa in individuals of Dutch ancestry, where it has been estimated that 3 in 1,000 of the white population are affected. It is much less prevalent in other countries. Like AIP and HCP, it is an autosomal dominant disorder, meaning that a mutation is present in only one of the pair of PPOX genes.

What causes Variegate Porphyria?
As in HCP, acute attacks of VP are unusual except in the presence of environmental activating factors, such as drugs, hormones, and dietary changes. See HCP for discussion of these issues.

How is Variegate Porphyria Diagnosed?
Urine ALA and PBG are increased during attacks, but as in HCP, these may increase less and decrease more rapidly than in AIP. Plasma porphyrins are frequently increased in VP, in contrast to AIP and HCP, and the plasma of VP patients displays a distinctive fluorescence peak, which is diagnostic. Fecal porphyrins are also elevated and are predominantly coproporphyrin III and protoporphyrin.
What are treatments for Variegate Porphyria?
Management and prevention are the same as in AIP and HCP. Blistering skin lesions are much more common than in HCP and are not readily treated. The only effective preventive measure is use of protective clothing.

What is the long-term outlook after an attack of Vairegate Porphyria?
The prognosis is usually good if the disease is recognized and treated promptly, before nerve damage develops. Although symptoms usually resolve after an attack, recovery of neuromuscular function (in a severe case) may require several months. Mental symptoms may occur during attacks but are not chronic. Premenstrual attacks often resolve quickly with the onset of menses.

Can attacks be prevented?
Yes, particularly with regard to drugs and diet. Genetic VP carriers should become informed on medications to avoid (see information on AIP and HCP) and should be prepared to point their healthcare providers to on-line drug lists that are regularly updated. The American Porphyria Foundation offers a mobile phone app that pulls up this information on line (http://porphyriadrugs.com/). A Medic Alert bracelet is useful for a situation in which the patient is incapacitated. Very frequent premenstrual attacks can be prevented by a gonadotropin-releasing hormone (GnRH) analogue (Lupron, Zoladex, others) administered with expert guidance. In selected cases, frequent noncyclic attacks can be prevented by once- or twice-weekly infusions of hemin.
Individuals who are prone to attacks should consume a normal balanced diet. Despite on-line discussion, there is no evidence that pushing carbohydrate prevents attacks, and it has the side effect of weight gain, which is undesirable for most people. Fasting, fad diets (for example, high protein) and gastric reduction surgery should be avoided. If weight loss is desired, it is advisable to consult a physician and a dietitian about an individualized diet with modest caloric restriction (ca. 10%), which will produce gradual weight loss without increasing the risk of an attack of porphyria. Exercise is safe in porphyria, and recommended.


Porphyria Post

Monday - October 1, 2018 @ 06:30:10

Porphyria Post
CLINUVEL INITIATES VARIEGATE PORPHYRIA PROOF OF CONCEPT STUDY
·    Today CLINUVEL announced it has initiated a new clinical trial in variegate porphyria (VP), set to commence in Europe in 2019. 

Click on the link below to view the announcement. 

RSVP TODAY!
Patient Education and Support Meetings coming your way. Contact the APF today to RSVP! We look forward to seeing you there.
Patient Education and Support Meeting - Missouri
LOCATION
Mound Ridge Retreat and Mission Center 31 Agape Lane Cook Station, MO 65449
DATE AND TIME
10/27/18 12:00pm - 10/27/18 2:00pm
Question and Answer session by Porphyria Expert via Skype. Family and Friends are welcome to attend. Lunch will be provided.

Patient Education and Support Meeting - San Francisco
LOCATION
UCSF Medical Center 505 Parnassus Ave. Room: S-157 San Francisco, CA 94143
DATE AND TIME
11/11/18 5:30pm - 11/11/18 7:00pm
Question and Answer session by Dr. Bruce Wang. Seating is limited. Please RSVP Today!!



Contact Information

Is your contact information up to date?
If not please give us a call @ 866-APF-3635 or Email to porphyrus@porphyriafoundation.com
American Porphyria Foundation 1.866.APF.3635  porphyriafoundation.org
STAY CONNECTED

"Remember.Research is the Key to Your Cure!"


APF Merchandise SALE BOGO & Free Gratis Gift

Friday - September 28, 2018 @ 16:11:29

  • ***ANY PURCHASE THIS WEEK WILL RECEIVE FREE APF Gratis***


APF Merchandise for Sale!
Our hope is that you all will buy and wear APF merchandise to help promote and raise awareness about Porphyria.  Please note that all orders will go through APF employee Amy Chapman.  Amy@porphyriafoundation.org
****We DO NOT accept orders through FB****
 Please submit your order and payment toAmy@porphyriafoundation.org or call 1-616-213-0030 directly and leave a detailed message.
Please send an email and include the following:  Full Name, complete mailing address, phone number.
Order: Please send your items that you would like to order by quantity & size for each T-shirt.  For privacy purposes, please send in the email either the Visa/MC Number, Expiry Date, & CVV code on the back of your card.  If you prefer I can contact you by phone.  NO CC info is EVER kept on file!
All orders include shipping prices for USPS only **International orders may be priced higher allow 48 hours**

Please review this link for color and size selection:

 http://www.porphyriafoundation.com/content/apf-merchandise

ITEMS FOR SALE:  

BOGO - Buy One Get One Free! 
V-Neck Short Sleeve Shirts - Deep Purple
Sizes available: Adults Only- S, M , LG, X-LG, 2XL, 3XL- (X-LG. 2XL, 3XL are $3.00 each more)
Price Each- S, M, L $20.00 Each ** X-LG. 2XL, 3XL are $3.00 each more**

Long Sleeve Shirts~ are 6 ounces each 100% Cotton.  Colors are Medium Gray or Purple
Sizes available Adults Only- S, M , LG, X-LG, 2XL, 3XL- (X-LG. 2XL, 3XL are $3.00 each more)
Price Each- S, M, L $20.00 Each ** X-LG. 2XL, 3XL are $3.00 each more**


Long Sleeve 100% Cotton Thick Zip Up Hoodie  Colors are Medium Gray or Royal Purple.
Price Each- S, M, L  $45.00 Each ** X-LG. 2XL, 3XL are $5.00 each more**


Long Sleeve 100% Cotton Pull Over Hoodie  Colors are Medium Gray or Purple
Price Each- S, M, L $40.00 Each ** X-LG. 2XL, 3XL are $5.00 each more**


Gray Soft Baseball cloth hats adjustable hat backside- $22.00 Each  (Fits Adults Sizes)


Wristbands- 2/$6.00 or $3.00 each Colors are Blue or Purple


Porphyria Car or Fridge Magnet $8.00 each



September is Pain Awareness Month

Friday - September 28, 2018 @ 06:00:03

September is Pain Awareness Month

The month of September has been declared Pain Awareness Month. Pain Awareness Month is a time when various organizations work to raise public awareness of issues in the area of pain and pain management.
The first Pain Awareness Month was in 2001, when the ACPA led a coalition of groups to establish September as Pain Awareness Month. ACPA established Partners for Understanding Pain and 80 organizations, both health care professionals and consumer groups, including the NAACP supported the effort.


http://www.theacpa.org/wp-content/uploads/2017/08/Pain_Awareness_Month_History.pdf
The key to raising awareness is to get involved. There are many things that you can do to help promote Pain Awareness Month.
·      Talk with Friends & Family: Let them know that September is Pain Awareness Month. Like the ACPA on Facebook. Encourage your friends to do the same.
·      Talk with your Healthcare Provider: Let them know that September is Pain Awareness Month. You also can share the tools to better communicate with your healthcare team found at the links below:
·       
o   Videos
o   Resources
o   FAQs
o   Surveys


·      Call your local government and community leaders to let them know about Pain Awareness Month and issues of pain and pain management

·      Call your local media and ask them if they are doing a story on Pain Awareness Month.

·      Donate to the APF: Your contributions allow us to help fulfill our mission and work year-round to raise awareness and support for those with chronic pain. www.Porphyriafoundation.org


·       Take care of yourself! Take time out for yourself this month.



NEW! Member story of Jeannine Keith HCP

Thursday - September 27, 2018 @ 06:30:06


What is your name?Jeannine Keith

Type of Porphyria?Hereditary Coproporphyria (HCP)

When & how long before you were diagnosed? I started having symptoms in March of 2010 and got my formal diagnosis June of 2010. In May of 2010 my Gastroenterology Dr. Peter Heit sent me for a urinalysis of the porphyrin levels in my body after I had every other organ tested with no answers to my pain. When the urinalysis can back positive he found me my savior Dr. Bari.

Who was able to diagnose you? Name/place? Dr. Fazal Bari, Oncology and Hematology Specialists

What has been your experience with porphyria? It is an unknowing disorder and can sneak up on you at any time day or night. I have had more good days than bad thankfully.

What symptoms do you have and how long do they last for? I have dealt with pain in my side, back, and chest. I have also had experienced nausea, neuropathy, and suffer from anxiety. 

What treatment do you get?  How often do you have to get treatment? Since 2010 I have been doing IV therapy of D5W monthly. When I started this, I was going to my doctors office 3 days out of the month during my menstrual cycle and spent 4 hours getting my hydration in the office. After doing this for a few months and knowing that this type of treatment was working for me I got a PICC line. I had that for 6 weeks and was able to have a nurse come to my house and I could run my IVs from home. After I had the PICC line taken out, I had my first port in 2011. I had my port in for 2 years before opting to have it moved from its original position. I have had my second port since November 2012 and still do my IV therapy from home, work or wherever I am.

Do you think a new medication would help you? What I am doing has been helping me for the past 8 years. I am not sure if new medication would help me.

How does having porphyria affect your daily activities? It really doesnt affect my daily activities unless I am having an attack. I live my life the way any person would, and I make sure I stay hydrated and attack free as best I can.
How has porphyria affected your life & family living? It is my way of living and I try not to let it affect my life or family living. I have this disorder, but I am not this disorder.

What are the positives of knowing you have a rare disorder? The positives are that when I have pain, nausea, vomiting or an anxiety attack I know what could most likely be the cause. I can also educate others about porphyria.

How do you keep yourself encouraged from day to day? I think about all the positives in my life and know that I am blessed. My 4-year-old son Ayden and my husband Sean are also my loves, my support and my heart.

If you could give an advice to another one suffering what would you say? Dont let this disorder define who you are. You are strong and can fight this. I know having this disorder is terrible and we all have our good days and our bad days, but we have to keep our heads held high and smile because some days that is all that might get us through. Remember, you never know what someone is going through, and I like many, smile and say everything is ok but sometimes it is ok to be truthful and say when it is not ok.

How has the APF been there to assist you?  (Materials, videos, calls, FB) There website has many articles, blogs and useful information. I have met so many people through groups on FB and they are wonderful!

What else would you like to mention? I am very lucky to have an amazing support system of Family, friends and medical professionals. I want to thank my husband Sean for always being by my side, my son Ayden for always making me laugh & My parents for always being there for me through learning about this disorder and getting answers. I also want to give a huge thank you to Dr. Bari for giving me my life back because without you I dont know where I would be with this disorder.


Thank you Jeannine Keith for sharing your inspiring journey with HCP!  You always encourage us.






Baby Harmoni's Diagnosis of CEP Congenital Erythropoietic Porphyria.

Wednesday - September 26, 2018 @ 18:17:43


Last year in October 2017, Me and my fiance found out we were pregnant with our first child & we could not have been more excited. After waiting so long at 38 weeks our sweet little girl Harmoni Grace was here weighing 8 pounds 1 oz and twenty one and a half inches long.

A short few hours after Harmoni was born she had what the nurses thought was what they call a brick dust diaper, which is normal in babies just not nearly as dark as Harmonis was or the quantity.

 We soon learned she was also born jaundice, so she was sent to the NICU to spend two days to help with the jaundice and to get to the bottom of the brick dust diaper. After two very long days and some testing doctors could not find anything wrong with Harmonis urine so we were cleared to head home.

 As excited as we were to bring our baby girl home we just knew something wasn't right. For two whole months after that me and my fiance spent almost every day in Valley Children's Hospital trying to get to the bottom of all of her problems and symptoms.

Almost every time we took her in for vomiting, the red diaper, her being lethargic, and not eating they sent us home and told us we have a colic baby and we should hold her more and we would have to let her cry it out sometimes, that answer was never good enough for me. As a mother I knew something just wasn't right.   Finally, after many trips to the emergency department we get admitted due to her hemoglobin being extremely low.

I can remember that day was a nightmare being questioned left and right and having a whole team of doctors come in to tell you your two-month-old has a hemoglobin that should be at her max limit which is a 13.6 but she is at the lowest a person should get a 7.0.

Doctors began to tell us that she will need a blood transfusion and we will be in the hospital until we can find the source of the bleed. This admission we spent almost two weeks in the hospital with the best doctors on our team to find some possible answers to what is going on with our baby girl.

On the fifth day the team of doctors came in, sat us down, & let us know they might have an answer to all of this madness going on with our sweet girl. They informed us they think Harmoni has Porphyria, and in that moment,  I think we reacted in any way any parents would with hearing a big medical term without knowing any knowledge of what it is or what comes with it.

After a little more information from several different doctors we were able to take a deep breath and brace ourselves for whatever was to come next. The next few days consisted of meeting with a hematologist and genetics doctor and TONS of testing.

The results would take some time to come back.

We were finally discharged when harmonies hemoglobin was at a safe level. We were advised her levels would have to be checked every two weeks to make sure her hemoglobin stays at a safe level and to make sure she wont need another transfusion.


On September 6th she went in to test her levels and sure enough they were low again, so we were once again admitted for a transfusion and a Broviac Line placed. After two days Harmoni was safe enough to come home once again with the same routine of checking her blood levels every two weeks. On September 11th we got her results and it was confirmed she has Congenital Erythropoietic Porphyria.

Now, we are waiting for calls back to get into UCSF to meet with specialists to take the next step to helping her.

Even though she was diagnosed with porphyria will do everything in our power as her parents to make sure she in no way shape or form feels any different than others. And at only three months old our little girl is the strongest little girl I have ever met, we refuse to let this kick her butt, so we will always make sure she is Porphyria strong.

Thank you for sharing through a parents eye how special and wonderful your baby Harmoni is to you.  Harmoni you are very special to us and how wonderful it is to have such loving and caring parents fighting for you.

To learn more about Congenital Erythropoietic Porphyria (CEP) please call our office at 1-866-APF-3635 or view our website www.porphyriafoundation.org








Porphyria Post

Tuesday - September 25, 2018 @ 06:30:00

Porphyria Post

Volunteers Needed!
The APF will be exhibiting at the two conventions this fall. Are you interested in volunteering at our booth? If so, please contact Edrin Williams, Director of Patient Services for additional information (edrinw@porphyriafoundation.org or 301.347.7166). This is an incredible opportunity to raise awareness about porphyria to those with little knowledge about this rare disease.

AASLD: San Francisco, CA
â?¢ Sunday, November 11 9:30 am - 3:00 pm time slots available
â?¢ Monday, November 12 9:30 am - 3:00 pm time slots available

ASH: San Diego, CA
â?¢ Saturday, December 11:00 a.m. - 5:00 p.m. time slots available
â?¢ Sunday, December 210:00 a.m. - 5:00 p.m. time slots available
â?¢ Monday, December 310:00 a.m. - 2:00 p.m. time slots available

EPP Clinical Trials
Are you ready to be a medical hero? A new clinical trial with an oral investigational drug intended to reduce phototoxicity in people living with EPP has begun. Dont delay contact the APF to learn more at 1-866-APF-3635 or porphyrus@porphyriafoundation.org

"RememberResearch is the key to YOUR cure!"
Each Step Toward Finding an Effective Treatment is Important!

Access to Care Toolkit for the Acute Porphyrias
A downloadable Access to Care Toolkit is a resource designed to help patients living with Acute Intermittent Porphyria (AIP), Hereditary Coproporphyria (HCP), and Variegate Porphyria (VP) or their caregivers, loved ones and healthcare providers secure access to Panhematin at their preferred health facility. We have recently learned of patients who are being denied this treatment from some hospitals and directed to secure another healthcare provider. If this has happened to you or someone you know or care for, please use these tools to request help from your state and local representatives and health advocacy organizations. We understand the debilitating effects of acute porphyria and hope these resources will help you secure access to Panhematin when you need it most.

The Toolkit contains the following materials:

â?¢ Healthcare Conversation Tracker is a simple form to record your conversations with doctors, insurance agents, etc.
â?¢ Customizable letter templates to record your details to use for doctors, state departments, insurance, etc.
â?¢ Access to Care Fact Sheet defines AIP, its symptoms and why it's important for patients to get immediate care
â?¢ Patient Bill of Rights can be used to support your appeal for access to treatment

This Toolkit can be found HERE. Contact the APF office today if you have questions!

Panhematin Study - Participants Needed!
Do you have AIP, VP or HCP? Are you interested in participating in research? If so, we are recruiting volunteers for the Panhematin Study.

Our researchers need people to volunteer for the Panhematin study. Completing this study will help prevent insurance companies from not paying for this treatment.

The purpose of this study is to determine if Panhematin is safe and effective for prevention of acute attacks of porphyria.

Please contact Edrin at the APF office (301.347.7166) if you are interested in participating.

Safe/Unsafe Drug Questionnaire for the Acute Porphyrias (AIP,VP,HCP & ADP) - VERY IMPORTANT
The APF is collaborating with researchers to identify new safe and unsafe drugs. We need your help. Are you experiencing adverse effects with any of your new medications? If so, please let us know in the questionnaire below how it affected you. We will share these results with our team of renowned Porphyria experts/researchers. They are in the process of updating our safe and unsafe drug list for the acute porphyrias. Your donations will help us educate physicians about the dangerous effects of unsafe drugs. Please click on the link below for the Questionnaire.

Safe and Unsafe Drug Questionnaire

RSVP TODAY!
Patient Education and Support Meetings coming your way. Contact the APF today to RSVP! We look forward to seeing you there.

Patient Education and Support Meeting - Cincinnati

LOCATION
Cincinnati Children's Hospital 3333 Burnett Ave. Room D2.28 Cincinnati, OH 45229
DATE AND TIME
09/29/18 11:00am - 09/29/18 2:00pm
We appreciate everyone who sent in their RSVP! We hope to see you on Saturday!!

See you on Saturday!

Patient Education and Support Meeting - Missouri

LOCATION
Mound Ridge Retreat and Mission Center 31 Agape Lane Cook Station, MO 65449
DATE AND TIME
10/27/18 12:00pm - 10/27/18 2:00pm
Question and Answer session by Porphyria Expert via Skype. Family and Friends are welcome to attend. Lunch will be provided.

I'll be there!

Maybe

I can't make it

Patient Education and Support Meeting - San Francisco

LOCATION
UCSF Medical Center 505 Parnassus Ave. Room: S-157 San Francisco, CA 94143
DATE AND TIME
11/11/18 5:30pm - 11/11/18 7:00pm
Seating is limited. Please RSVP Today!!

I'll be there!

Maybe

I can't make it

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