By Sheri Coil
A bit of History
Shortly after Christmas one year, we received a phone call asking us if we were interested in caring for two little boys with a very rare disease that affected their skin. We are caregivers to medically fragile children and like to care for those with ‘challenging’ conditions. The oldest, Corey was 14 months and the younger brother, Alex, was only five weeks old and not yet five pounds. They had Recessive Dystrophic Epidermolysis Bullosa: Hallopeau-Seimens subtype, a rare double recessive genetic disease that affected the integrity of the collagen VII area’s in the skin. This meant that any shear pressure on their skin would cause it to lift causing blisters to form or for the skin just to peel off at the lamina densa level or in other words at full depth of skin. The fibrils that hold the skin to the flesh are made of Collagen VII are greatly compromised if not non existent. When the blisters form in an area of damage, they fill with more and more liquid and keep growing larger and larger since the anchoring fibrils were not there to hold the surrounding tissue together.
We said that we would take them but had no idea of the ‘challenge’ we had accepted. They were both hospitalized in Packard Children’s Hospital at Stanford for ”wound management”. Both children were on morphine for pain and in very poor condition. Corey had an absence of skin on about 75% of his body, his toes were webbed over completely and his fingers were encroaching his palms they were so badly mittened and contracted. What everyone failed to tell us was that no one expected them to live long enough to make it back to the next clinic appointment. Which turned out to be a good thing in the long run. We got them and only saw future. We did not concentrate on death, but rather on life! It was a year before we were told this expectation. When their little sister, Brandi, was born the next year, she also joined the family.
Care instructions were few and supplies were hard to come by at first. We were told to soak Corey every day in a whirlpool bath that had bleach and salt added. We were told not to try to wrap too much. Not to try to wrap to protect them, that they would get blisters anyhow. That the fewer bandages would give them more room to move as the wrapping would get in their way.
For some reason, we just could not get connected with the insurance and suppliers. The insurance that covers them will and does pay for all their supplies, but putting the three parts together was a frustrating challenge. But we persevered and prevailed! In the meantime we learned to stretch a bandage many ways.
Alex was released from the hospital a few days before Corey as his wounds were not as infected and serious. We had to stop at another hospital for our daughter to have some tests before we went home. I was holding this tiny scrap of humanity and noticed he had what looked like a chunk of raw liver in his mouth. Alex had formed a huge blood blister in his mouth and when it broke, it was impressive. This was our first blister and it was a good one! This was not what babies are supposed to be all about.
On the way home from the hospital the day we picked up Corey, he threw up in the car. We thought that it was car-sickness. We should have been so lucky! He had a bad case of flu with all the things that are inherent with it. It was impossible to keep him clean on either end. It is bad enough to have any baby get the flu, but one who is wrapped up like a mummy is really impossible to keep clean. No just stripping off the soiled clothes and washing the baby here. All the bandages had to be changed too. A two-hour job. Only he was not lasting even that long between bouts of nausea and diarrhea. He finally dehydrated so badly that the hospital near us could not find a vein to put the IV in. And this was made worse by the fact that every nurse they called in was frightened by his EB, they offered us a free vein screening afterwards, but I was kind of scared. Finally, in desperation, the Dr. put it in his jugular and they taped it to his neck in spite of my protests. They then transported him down to Stanford for another weeks stay.
The same week, Alex, who was almost big enough to see came down with pneumonia. And of course the technicians in the radiology lab, who I warned against handling him wrong, took all the skin off his feet and a goodly part of his upper body. I was very upset and vowed that this would never happen again as I would be a very involved and vocal, even physical, guardian angel from then on. And I have been.
This was our first week’s introduction to EB. We were impressed by our experiences and worried about the boys. As challenges go, this was a first rate one. It would take everything we could give it. And then some!
Things we observed and the evolution of how we care for them:
The boys were in extreme pain during bath time. They constantly developed new blisters during the process. Especially on areas like the heels and bottom. Picking them up, Corey with his mass of open wounds and Alex, so tiny and easily damaged, was very, very difficult when they were completely naked and that warm, wet skin seemed to be even more fragile. So, rather than completely stripping them and doing a full bath daily, we began to wash and rewrap smaller areas like the upper body, using a small tub (one or two gallons) of water with bleach (Dakin solution) and salt added. Using pieces of Kerlix as a washcloth and running the water over the wounds that didn’t fit in the tub with a turkey baster or the gauze squeezed out over them. Also, we keep a spray bottle with a gentle spray full of bleach water to spray on small areas quickly. After we wash, medicate and rewrap the area, say the arms and torso, we then go on to the next, lower body and feet, and repeat the procedure.
We noticed that whenever an area started breaking down, it continued to break down. It was fragile and very susceptible to damage. Those fibrils were broken and did not reform well. As the children got older, the skin areas that had never broken down, were stronger and not near as easily damaged as those areas previously blistered. And that when a blister was growing, it would stop dead where ever there was a bandage. So the wheels started turning. If the bandages could stop blisters from growing bigger where they would stop them, could they prevent the blisters from forming at all. Could this preventative, or prophylactic, bandaging keep that fragile and oh so tender baby skin in tact for a while and maybe the skin and fibrils, what few there were, might become stronger with aging. Thus began our journey of discovery, to find every possible way to wrap all the body successfully to protect it from the fast lane of breakdown.
The OT’s and PT’s were determined to find splints and gloves that would prevent some of the contractures from happening in their hands. We used everything we could think of to help. Plastic splints that were heat formed. Foam ones. All sorts of them. They were less than worthless and some even caused more pain and damage than they would have prevented.
We were assured that the burn glove web-spacers would prevent any more web space lost. In theory it sounded great. Reality for us was they were a no- go! They were hard to fit properly on these tiny little hands. Remember that my kids were losing web space from birth. And if they didn’t fit right and keep those web spaces very snug, they couldn’t do any good. They were stiff and the Velcro closures were bigger than the gloves! The burn garments are made from a very stiff latex. In trying to put it on fragile EB skin, it was causing a lot of shear pressure that was doing damage. A true case of the cure was worse than the illness. Any glove type product was a problem as it would do damage if it was snug enough to do a good job. I finally decided to find a way to wrap the gloves on them instead of pulling them on. So the hand wrapping technique was developed.
When ‘designing’ our wrap-on glove, we had two areas of concern or criteria that had to be met. One was that it must protect the hand and fingers from damage as much a possible and the other was that it keep the web spaces intact. We needed about a one inch bandage to wrap around those tiny fingers. The suppliers we were using assured us that no-one made a one inch roller gauze bandage. So we cut 2″ soft-cling in half to make 1″. The next year we found 1″ Conform at the hospital where Brandi was taken after her birth and changed suppliers!
The rest of our bandaging techniques just grew with the kids. We shared ideas with other parents and played with different ideas and products. We are ever-changing as the kids grow and as the blistered areas of concern move around, but all our wrapping is basically as we show you in the Wrapping the ABC Way pages. We encourage you to be creative to suit your own ideas and needs. This is just what works for us and is somewhere to start.
The kids after five+ years of being bandaged this way are doing well. Corey still has ten fingers with very little lost web space. They are a bit crooked and are going to be straightened fall of ’99, but are still doing well, in spite of the fact that they are very fragile and the skin in tissue paper thin. I have no doubts that they would have been completely webbed in again within months had they not been wrapped. The wrapping also gives him a lot of protection for that so fragile skin and enables him to do so much with them that he could not do if they were not wrapped. He has gone from being 75% with out skin to 15%to 20%. His feet, shins, hands and elbows are almost always broken down, but the area reduces every year.
Alex maintained well with almost no breakdown, once we got him healed up, for some time until he got the itchies. Then, the bane of EB reared it’s ugly head and he scratched himself from maybe 5% of breakdown to over 75% of his body without skin. He was a mess. A regimen of special creams (first our own concoction and then the Alwyn cream), and oral drugs (Claritin in the am and Atarax in the pm) has cleared almost all of this up, but has taken a year and has left him scarred. We had to do some very heavy wrapping to try and keep those persistent little fingers from doing constant damage. He was far more tenacious than we were. I cannot imagine what he would have looked like if he wasn’t wrapped at all. Any area he could reach was constantly blistered. But he is doing well now. His hands are exactly where they were when we started wrapping them fully at about six months, slightly crooked with a very small amount of lost web space.
And “Baby Princess Brandi” (as she calls herself) is amazing. She was a mess when we got her home from the hospital. A friend, who happened to be the attending neonatal nurse at her birth, told me that one could just stand and watch the blisters coming out all over her. This nurse was the home care nurse that came to our home when we first got the boys and gave us so much support and help, so she had an excellent handle on EB. She was able to tell the doctors right away all about Brandi. They sent her to a children’s hospital for care that knew nothing about caring for an EB child. I have never understood why she was not transferred to Stanford, just a few miles further. We did not get the final word that Brandi was ours for two weeks and could not care for her until then. As soon as we got the ok, we went to the hospital and started wrapping her. None too soon, either. As soon as her pediatrician there watched us wrap her and tell all about EB, she released her to us. Thank goodness! We had her healed up in about two weeks and has been wrapped ever since. Her feet are fragile, as they keep getting stepped on and find their way under bike tires frequently. And she got the dreaded itchies on one arm, which is hard to heal. She has had blisters on her elbows after falls and small ones on her hands as she persists in pulling off the finger wraps. (She looks like she has about fifteen fingers all the time as the wrapping stays in the shape of a finger) She takes out her knees pretty often from falling. Other than that, she has had virtually no breakdown. And is a very active child. She is even taking ballet lessons. We credit all to her being wrapped from the start.