CHICAGO (Ivanhoe Newswire) - Imagine taking your normal prescription medication or even just an ibuprofen and then watching your skin painfully peel off your body. That's what happens to people with a mysterious disorder, but the condition threatens more than their skin.
Patients look like burn victims, but reactions to prescription or over the counter medications is what causes this.
"Her skin peeled off. It was horrible. I was scared," Gina Parks, whose daughter had this disorder, told Ivanhoe.
It's called Stevens Johnson Syndrome.
"Which is characterized by sloughing of the skin and the mucus membranes," Amy Lin, M.D., Assistant Professor in the Department of Ophthalmology at the Loyola University Medical Cente, said.
Gina Parks' daughter Brianna got the potentially deadly condition after taking children's Tylenol.
"My mom told me I could go blind," Brianna Parks said.
"It can lead to permanent scarring in the eyes and the eyelids, and that can lead to severe visions deficits and even blindness," Dr. Lin told Ivanhoe.
But Loyola University Ophthalmologist Amy Lin said part of a human placenta called the amniotic membrane helped Brianna and others like her keep their sight. The fetal tissue is full of anti-inflammatory and wound healing properties. It's sutured on to the eyes and eyelids and within weeks the membrane is absorbed into the eye helping create a new surface.
Studies show when the syndrome affects people's eyes, those who don't have the procedure are eight times more likely to have vision problems than patients who do have the surgery.
As for Brianna, her eyes are just fine.
"You can barely tell anything even happened to her," Dr. Lin said.
Now, this natural shooter has her sights set on making her school's basketball team.
Dr. Lin said if the amniotic membrane transplant doesn't work the first time it can be repeated.
She said she has to repeat the surgery in about 25 percent of patients. The doctor said it is unknown why Stevens Johnson Syndrome affects some people but not others. She's currently conducting research to look at the condition at a cellular level to increase understanding of the disease and hopefully develop new state of the art treatments.
BACKGROUND: Stevens-Johnson syndrome is a rare, serious disorder in which your skin and mucous membranes react severely to a medication or infection. Often, Stevens-Johnson syndrome begins with flu-like symptoms, followed by a painful red or purplish rash that spreads and blisters, eventually causing the top layer of your skin to die and shed. Stevens-Johnson syndrome presents a medical emergency that usually requires hospitalization. (Source: Mayo Clinic)
SIGNS: Signs and symptoms of Stevens-Johnson syndrome include facial swelling, tongue swelling, hives, skin pain, a red or purple skin rash that spreads within hours to days, blisters on your skin and mucous membranes, especially in your mouth, nose and eyes, and shedding (sloughing) of your skin. If you have Stevens-Johnson syndrome, several days before the rash develops you may experience fever, sore throat, cough, or burning eyes. Stevens-Johnson syndrome requires immediate medical attention. Seek emergency medical care if you experience any of the following signs or symptoms: unexplained widespread skin pain, facial swelling, blisters on your skin and mucous membranes, hives, tongue swelling, a red or purple skin rash that spreads, or shedding of your skin. (Source: Mayo Clinic)
TREATMENT: Stevens-Johnson syndrome requires hospitalization, often in an intensive care unit or burn unit. The first and most important step in treating Stevens-Johnson syndrome is to discontinue any medications that may be causing it. Because it's difficult to determine exactly which drug may be causing the problem, your doctor may recommend that you stop taking all nonessential medications. Currently there are no standard recommendations for treating Stevens-Johnson syndrome. Supportive care you're likely to receive while hospitalized includes: fluid replacement and nutrition, wound care, and eye care. Medications commonly used in the treatment of Stevens-Johnson syndrome include: pain medication to reduce discomfort, antihistamines to relieve itching, antibiotics to control infection when needed, and topical steroids to reduce skin inflammation. In addition, you may receive one of the following types of medications that are currently being studied in the treatment of Stevens-Johnson syndrome: intravenous corticosteroids or immunoglobulin intravenous (IVIG). If a large area of your body is affected, skin grafting — removing skin from one area of your body and attaching it to another or using a synthetic skin substitute — may be necessary to help you heal. This treatment is only rarely required. If the underlying cause of Stevens-Johnson syndrome can be eliminated and the skin reaction stopped, your skin may begin to grow again within several days. In severe cases, full recovery may take several months. (Source: Mayo Clinic)
NEW TECHNOLOGY: Loyola researchers studied a relatively new eye treatment for SJS/TEN patients called amniotic membrane transplantation. Amniotic membrane is part of the fetal membranes that surround and protect the baby in the womb, and have natural therapeutic properties. When placed on the eye, amniotic membrane can help aid healing, decrease inflammation and minimize scarring. (Amniotic membrane is donated by a consenting mother following the birth of her baby.)
Previous studies have found that amniotic membrane transplantation is effective in the chronic stage. The Loyola case-control study is one of the largest studies to examine the effect of amniotic membrane transplantation in the early, acute stage. (Source: Sciencedaily)
Dr. Amy Lin, Assistant Professor at Loyola University Medical Center, Department of Ophthalmology, discusses Stevens Johnsons Syndrome and a new treatment that may help patients keep their sight.
What is Steven Johnsons Syndrome?
Dr. Lin: As an ophthalmologist I treat patients with inflammatory conditions of the eye. One of these conditions is called Stevens Johnsons Syndrome and the most severe form of it is called toxics epidermal necrolysis syndrome. It is a rare disorder which is characterized by sloughing off the skin and the mucus membranes and it is usually caused by medications such as anti-seizure medications, antibiotics, even over the counter pain relievers. Occasionally in some cases it is caused by some infections. It starts off with fever and then it progresses to a rash and then that rash starts to blister and the skin actually sloughs off along with the mucous membranes. Along with the mucous membranes, the eyes are also involved as well and this inflammation in the eyes can be very devastating. It can lead to permanent scarring in the eyes and eyelids that can lead to severe vision deficits and even blindness.
Medication would raise parents' eyebrows I would think.
Dr. Lin: Yes, Stevens Johnson Syndrome is not really tied to any one particular medication. In all honesty, almost any medication can cause it, and the scary thing is that we've seen it in patients who've taken over the counter acetaminophen or ibuprofen, medications that we all take and obviously not all of us get Stevens Johnson Syndrome but a few patients do. The incidence is probably about three to seven people per million people, so it's quite rare. We really don't know why it affects certain people and not others, there's still a lot that needs to be learned about the disease.
What is the reaction, is it an allergic reaction?
Dr. Lin: I guess you could think of it as an allergic reaction. It's a hypersensitivity reaction, where basically a lot of cells start to receive messages to die off. So instead of the normal turnover of cells where a few cells are dying and some are regenerating, all the cells get the message to die and everything just sloughs off. It's very scary.
What does it feel like?
Dr. Lin: There is sloughing along all the mucous membranes' cells within the interior of the mouth down the windpipe. This burning sensation all over the skin occurs because it's all sloughing off and so are the mucus membranes. I don't know what it would feel like but I imagine it would feel pretty terrible, like you are burning up inside your mouth and skin.
Are they treated by the burn unit when they come in?
Dr. Lin: Exactly, so in the intensive care unit we see patients obviously with burn injuries, but because Stevens Johnson Syndrome has a sloughing of the skin and mucus membranes it actually resembles severe burns. So these patients are actually referred to burn centers for treatment. Loyola is one of the major burn centers in the Chicago area so we get a lot of referrals from other hospitals in the area for the Stevens Johnson patients.
The skin issues can be treated by the burn unit but when it comes to the eyes, what can you do?
Dr. Lin: When there is severe inflammation of the surface of the eyes there are new techniques which can be performed to try and prevent some of these scarring and blinding complications of Stevens Johnson Syndrome. There is a tissue called amniotic membrane, and it is a tissue that's actually harvested from the inner membrane of the human placenta. This tissue contains several biologic mediators and growth factors and they have anti-inflammatory as well as wound healing properties. We actually have found that by suturing this amniotic membrane on the surface of the eyes and also the inner lining of the eyelids, that we can substantially decrease the likelihood of developing these vision complications down the road. If that's done within the first two weeks of the onset of symptoms, we've found that the benefit is greatest at that time.
It is sewn right on to the eye?
Dr. Lin: It is sewn right on the surface of the eye with tiny little sutures and the sutures are removed about two to four weeks after the procedure is done. The amniotic membrane actually dissolves over time and it almost gets absorbed into the body. After a couple of weeks, when that process is done and it's all dissolved, we take a look at the eyes again and if it looks like there's still a lot of inflammation we'll actually repeat the surgery.
You can do it again?
Dr. Lin: Youcan do it again, yes. Most times I've found that after doing the surgery just once they do pretty well and we don't need to repeat the surgery again.
How new is this procedure and how was it discovered?
Dr. Lin: I'm not sure how it was discovered but the procedure has been around for a few years now. Ophthalmologists have been using amniotic membrane for a variety of other uses, not just for Stevens Johnson. We found that it's a good substitute for mucous membrane so for any other eye procedures where we need an extra layer of tissue to cover the surface of the eye, it's been used for that. I would imagine people started using it for Stevens Johnson because we do need another surface of the mucous membranes in the eye, and also to try and calm down all that inflammation on the surface.
Is it healing, is it protecting? Describe what its job is?
Dr. Lin: Well, the job is once the amniotic membrane is in there it is covering all those open areas in the mucous membrane, it's also feeding these anti-inflammatory growth factors and tries to get the surface of the eye healed properly without scarring. It's been working really well. We just recently published an article in the Journal Cornea and we found out that patients who did not receive the surgery were eight times more likely to have poor vision outcomes compared to patients who did receive the amniotic membrane transplant surgery.
Dr. Lin: Eight times. This is patients who have severe inflammation on the surface of the eyes.
Does everyone need some kind of eye treatment when they have this?
Dr. Lin: Not everyone needs it. If some people come in and they have very mild symptoms and their eyes just have a little bit of inflammation, we actually will just treat them with lubrication, sometimes with other anti-inflammatory eye drops, and they don't need the surgery and often times those patients do well later on. If we see that they have anything more severe, so any moderate to severe inflammation, we don't wait. We go ahead and do the surgery because we've seen the consequences of waiting and of not doing anything where patients do really poorly. Once they start developing the scarring it's really too late, there's really not a lot you can do. You can try and do some reparative measures, some other surgeries, but they really don't do as well as the patients who have the initial surgery from the outset.
What is the time limit?
Dr. Lin: Within two weeks of the onset of symptoms.
How long is the healing process, you said at about four weeks you get the sutures out but are there any more?
Dr. Lin: Yes, it's dependent from patient to patient. Some patients do really well and they look good in that first month after surgery. Some patients, they have a lot more inflammation for whatever reason and we need to repeat the surgery and it becomes an ongoing chronic problem where they need to be seen by the ophthalmologist quite often.
The membrane itself is just clear?
Dr. Lin: Yes, the membrane itself, it's a bloodless tissue, is a little bit opaque. It's a very thin membrane. It's actually quite elastic, it's a little bit stretchy, and it's quite strong even though it is very thin.
Have you had to repeat it before and if so how often?
Dr. Lin: Right, I would say probably in about twenty five percent of cases I've had to repeat the surgery again. Again, we want to do it quickly, we don't want to wait several months later to repeat a surgery; you would rather do it while the patient is having a lot of inflammation because that's the critical time period to calm down the inflammation.
Kind of reduce the risk at times.
Dr. Lin: At times, yes.
It might not work in everybody?
Dr. Lin: It might not work in everybody. We know that it does work in many patients; there have been a few patients where it hasn't been as effective, but none of those patients have severe vision problems. They more have problems with some scarring; a lot of dry eyes. What we think is that patients who did receive the surgery and still weren't doing quite as well, that if they didn't have the surgery they'd probably be doing a whole lot worse and they may even be blind because of it.
Are there any side effects besides dry eyes?
Dr. Lin: Well dry eye is a side effect of the Stevens Johnson Syndrome. The side effects of the amniotic transplant surgery; there's a theoretical risk of infection just as with any eye surgeries, so we have them on antibiotic drops. There are no cases of rejection of the amniotic membrane transplant, patients tolerate it pretty well. During the time the membrane is put in it hasn't dissolved yet, so for that first couple week period the surface of the eyes is covered so they can kind of see through the membrane and things are very cloudy. It's kind of like looking through a frosted glass door. That is one thing that I tell patients is that your vision is going to be blurrier for these two weeks after the surgery but later on, once that dissolves, everything clears up and the vision goes right back to what it was before.
Do you see a lot of results where they just bounce back after the membrane are out?
Dr. Lin: Yeah we do. Briana was here over three years ago when she was just nine years old and she did have inflammation on the surface of her eyes. She had the amniotic membrane transplant pretty soon after she was admitted to our burn unit and she did remarkably well afterwards. Today we can barely tell that anything even happened to her, and if she was just a little girl running around you wouldn't have known she had Stevens Johnson Syndrome. We do have success cases like Briana.
Anything else you would like to add?
Dr. Lin: There's still a lot that we don't know about Stevens Johnson Syndrome and we don't know why it affects some people and not others. We're actually currently undergoing research here at Loyola looking at the disease process from a cellular level. We're hoping that with our research we can increase our understanding of the disease and it can help us develop state of the art treatments that our patients deserve.
What kind of research is that?
Dr. Lin: We're looking at patients once they get admitted to the burn unit with new diagnosis of Stevens Johnson Syndrome and we're taking swabbings of their eyes, swabbings of their oral mucosa, swabbings of their skin, and we're also taking a little piece of the skin to look at it under the microscope to see what kind of inflammatory mediators there are, what's going to separate a patient who has really bad eye disease versus one whom really doesn't. We're hoping we can find some more information. Later on down the road if we can find out what it is that really makes a difference, maybe we can develop a treatment like a medication, an eye drop, to really fight that inflammatory marker that's causing it.
FOR MORE INFORMATION, PLEASE CONTACT:
Maggie Reeb Loyola University Medical Center email@example.com
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