Epidermolysis bullosa or EB is a genetic condition that causes the skin to be fragile and blister easily.
The CEO of Scioderm, Robert Ryan has spent more than two years approaching capitalists with a strategy for treating this rare skin disease and finally raised $16 million to continue the experimental breakthrough treatment. Robert Ryan has continuously showed images of children who look like burn victims, covered in blisters, with open and festering sores. The experimental treatment by Scioderm for Epidermolysis bullosa, or EB, the rare skin condition, which has no known cure is generating attention from nonprofit organizations dedicated to EB and the Research Association of America.
Scioderm have provisionally called the cream SK-101 and this cream causes the wounds to heal quicker than normally.
Reported EB cases in America are estimated at 40,000, some have tried genetic therapy such as bone marrow transplants but only show a limited success. The company Scioderm currently has six people on its team, and is planning to conduct another trial on 28 patients this summer. Definite quality of life changes for these young children through the breakthrough therapy of this rare skin disorder by the company Scioderm, and their determination to bring positive healing change to a serious medical need that so few know about. Nothing, but nothing, is easy in the life of 14-year-old Jonathan Pitre — save for the sleep that descends on him each evening in a cloud of methadone. Jonathan suffers from one of the most painful conditions known to medicine, Epidermolysis bullosa (EB), a rare genetic disease that causes the skin to endlessly blister, shear and scar. His skin is so sensitive that his body must be wrapped in gauze to protect it from the mildest friction. Jonathan’s pain threshold is unimaginable for most, but still antibiotics and pain relievers play a daily role in his life. Russell teenager Jonathan Pitre suffers from Epidermolysis Bullosa (EB) — a rare disease defined by severe blistering and shearing of the skin that leaves Jonathan in constant pain. The pain has become so hard to manage that Jonathan has advanced through Advil and Tylenol, to morphine, and now methadone. Although the powerful opioid allows him to sleep, it has complicated his introduction to high school.
Jonathan has never known a day without pain, and for a time, he wondered about the purpose of that suffering.
Jonathan is now an EB ambassador, one of two young people who have stepped forward to educate Canadians about the disease. DEBRA Canada is raising money for a wish campaign so that Jonathan and his fellow ambassador, Toronto’s Deanna Molinaro, can fulfil one of their dreams. Through Facebook, the teenager has come to understand the hard facts of life for EB patients: some of those whom he befriended in Toronto have since died. Those with Dystrophic EB — Jonathan’s EB — suffer high rates of squamous cell carcinoma, an aggressive form of skin cancer. People with Dystrophic EB are missing key structural proteins that connect their two upper layers of skin. Teacher’s aide, Nathalie Courillon-Chevalier, helps Jonathan with homework during his spare and usually at lunch in a small room off one of the main hallways. A Kindle is another modern tool that really helps kids with EB, allowing them to read without holding books. In his Embrun high school, Jonathan avoids the cafeteria because the jostling of other teenagers can tan him with blisters. The highlight of his day usually comes on his return home, when his dogs, Molly and Gibson, greet him at the door. Despite painful bath time, Jonathan happily chirps away to his mom and anyone else who will listen — until the bandages come off.
Like a surgical unit, Tina has everything laid out in her bedroom next to the bathroom: bandages, tape, cushy mats and a warm heater to help Jonathan stay warm until all the bandages are reapplied for another day. A few blisters must be pierced with a pin to stop the wound from getting bigger on his hand. When he was younger, Jonathan liked to dream about playing hockey and baseball, but the progress of his disease has shattered that hope.
Send Home Our method Usage examples Index Statistics Advertise with us ContactWe do not evaluate or guarantee the accuracy of any content in this site. Aout two years ago, her own 1-year-old son Charlie became very ill and was thought to have a viral infection.


You present with all these symptoms, you get a lot of repeated misdiagnoses, you keep getting sent home [by doctors]… We had a really hard time getting him diagnosed.
And with Kawasaki Disease, there is an added wrinkle- treatment needs to be started in the first 10 days to prevent more serious complications (see below). We thought, ‘What can we do to help other parents and kids not have to go through what we went through? Chalke also filmed a PSA aired at the end of the episode referring people to the Kawasaki Disease Foundation. Sometimes Kawasaki disease affects the coronary arteries, which carry oxygen-rich blood to the heart.
One of the main symptoms during the early part of Kawasaki disease, called the acute phase, is fever. During the acute phase, your child also may be irritable and have a sore throat, joint pain, diarrhea, vomiting, and stomach pain. Within 2 to 3 weeks of the start of symptoms, the skin on your child’s fingers and toes may peel, sometimes in large sheets.
Without treatment, about 25% of children develop heart disease involving the coronary arteries.
To make the diagnosis a child should have a a fever that lasts longer than 5 days plus four other classic signs or symptoms of the disease.
The standard treatment during the disease’s acute phase is high-dose aspirin and immune globulin. Most children treated for Kawasaki disease fully recover from the acute phase and don’t need any further treatment. Children who have had immune globulin should wait 11 months before having the measles and chicken pox vaccines. If Kawasaki disease has affected a child’s coronary arteries, he or she will need ongoing care and treatment with a pediatric cardiologist (heart specialist for children). Once again, a personality is using their celebrity to educate the public about an important health issue.
AboutWe are a wife & husband team of physicians who have trained and taught at some of the top medical schools in the country including Harvard, Johns Hopkins and Washington University in St. Our mission is both a journalistic and educational one: by reporting on common diseases affecting uncommon people, and including the medical facts behind the headlines, we provide a dynamic collection of Teachable Moments in Medicine™ to increase health awareness and medical knowledge. We give you the medical facts behind Hollywood's health headlines plus information that empowers you to achieve your health and wellness goals.
Recently a breakthrough therapy for this rare skin disorder from Scioderm has shown promising results. The impact of this product has received a breakthrough designation form the US Food and Drug Administration and this would indeed expedite the development of this product, as the cream has demonstrated the substantial improvement of this condition. It does not treat the underlying genetic cause of the disorder, which prevents the skin layers from binding property and resulting in a fragile skin that causes excruciating pain, eating complications and unbearable itching. Many of the victims are bandaged to cover their wounds in public to prevent exposure and further tearing. The third and final test planned for nearly 100 patients is expected to be concluded next year.
But none of them is magical enough now that his EB has worsened: In the past four months, his wounds have become bigger, deeper and slower to heal. He takes half a dozen pills three times a day along with two doses of methadone just to make the pain tolerable.
The Grade 9 student at Embrun’s Catholic secondary school is trying to find a dosage that will reduce his pain but still allow him to concentrate. Gibson, the family’s Boston Terrier, gets in on the action as well, waking up his pal with a lick. The fan is going in his room because, even though he’s just wearing panama shorts, being wrapped from head to toe in bandages is hot.
Then, two years ago, he went to a gathering of EB patients in Toronto sponsored by DEBRA Canada, a charity devoted to supporting those with the disease.
They were the only young people he’s met who could understand what he faces each hour of every day. He arrives to school early and gets to classes a couple of minutes late to avoid all the traffic in the hallways.


It is a refuge of sorts where he can relax safely, but, unavoidably, it also separates him from teenage life in the hallways of the school.
It is the endless cycle of blistering and scarring that often leads to a fusing of the fingers. Boileau worried about bringing Gibson, a Boston Terrier into the house, but the puppy somehow understands that Jonathan is unique.
When Jonathan is in pain and crying, Gibson will often put on the slapstick: slamming his head into the wall or falling off a chair. Bath time: Washing Jonathan in a tub filled with warm water, pool salt and some bleach, stripping and re-dressing all of his wounds can take up to four hours and must be done every other day to fend off infection and keep the blistering at bay as much as possible. We are committed to maintaining a lively but civil forum for discussion, so we ask you to avoid personal attacks, and please keep your comments relevant and respectful.
Elliot Reid on the hit comedy Scrubs, played Casey, a mother desperate to find out what medical illness her son has. It’s a form of a condition called vasculitis, meaning an inflammation of the blood vessels. The disease can affect any type of blood vessel in the body, including the arteries, veins, and capillaries. The body’s response to a virus or infection combined with genetic factors may cause the disease. Inflammation in the walls of these arteries (which supply oxygen to the muscle of the heart) cause them to weaken and balloon out.
These diseases include Rocky Mountain spotted fever, scarlet fever, and juvenile rheumatoid arthritis. Tests and procedures, including an echocardiogram (sound wave test of the heart), blood tests, chest x-ray and EKG may help confirm whether a child has the disease.
Rarely, children whose coronary (heart) arteries are affected may need medical procedures or surgery. As soon as his or her fever goes away, a lower dose of aspirin is given to prevents blood clots which can form in the inflamed small arteries. Ryan said the average insurance payment for medications to treat this rare disease is $60,000 a year per patient.
Severe cases of this widespread blistering can lead to infections and other medical problems EB may be life threatening in infancy.
EB remains a reclusive disease and not widely reported on thus few people know about the horrors of the disorder. Jonathan will often nod with sleep as his mother, Tina Boileau, wraps his shoulders and arms. His skin is so sensitive, often the slightest of touches in the wrong place can hit a wound and cause excruciating pain.
Jonathan has had a number of surgeries to literally pry the fingers apart and his mom is careful to wrap them separately in bandages to keep them working as best as possible. He’ll pant and whine as Jonathan does his homework, waiting impatiently for attention. If you encounter a comment that is abusive, click the "X" in the upper right corner of the comment box to report spam or abuse. She came to believe that her son had a rare and difficult to diagnose disease called Kawasaki Disease (KD), but had a hard time getting doctors to listen to her.
However, no specific virus or infection has been found, and the role of genetics isn’t known. The plasma, processed from donated human blood, is rich in antibodies that protect the body against diseases..
He justified the cost by saying it is cheaper than the alternative, numerous bandaging, pain killers and potential hospital visits that are needed to deal with the skin wounds that proved to be resistant to healing. Treatment is most effective in preventing coronary aneurysms if given within the first 10 days of the disease. She was a medical internet pioneer having established one of the first medical practice websites in 1997.



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