Life with EB

EB

In this section, I will tell you about the steps that I went through to learn about the proper care for EB. Share my experience, if you will. At the end, I will provide a list of things to do and things not to do as Useful Advice on Care and Management of EB.
The story of how I discovered best management steps can best be told perhaps by going through the growing stages of Yasmin.
After I discovered Yasmin had EB (see the story of birth and discovery), it was a very difficult process to find someone who could tell me what to do to care for my baby girl. So many things I needed to know; so very few people who knew and so very few places to go to.
The trouble with EB is that it is rare. Most dermatologists will have seen only a handful of EB cases at the most. They all know it has no cure, and the conservative ones will offer you advice that gets you and your child almost incapacitated from the very many precautions they demand, and the more careless ones will tell you no more than what the condition is and leave you to take care of finding out the rest yourself and fending for your child.
The Internet was heaven-sent to me. I was able to learn about other people’s experiences. I saw my Yasmin’s case and future in some, and I also got good advice. I now have added quite a few more tricks, especially that I live in a developing country, where one sometimes has to make do with what’s available.
This is why we developed this website. I am hoping to contribute my experience, hoping people in my country, similar countries, and the EB community can find it useful and benefit from it to make their own experience with EB easier.

Yasmin as a Baby

Let’s start with when Yasmin was still a baby, after we had confirmed that she did have EB. At first we would carry her as we would a regular baby, astride to our side for instance. But we soon discovered that this hurt the skin behind her knee badly. We decided to handle her as more delicate than the rest.
The first thing that came to my mind was to find a way to reduce her skin’s contact with things. I first started using cotton. That was a mistake. Cotton clung to her skin.
I then started to try regular gauze. It worked to a certain degree up to the time she started walking. Then the bandages I put on her wounds would start loosening as she walked and either fell or really bothered her.
At this time, I was a regular reader of anything about EB on the Internet. At the same time, I contacted someone I knew of who had a baby with this condition a few years earlier. She was very helpful and offered to provide me with special wound dressing. It was amazing and stayed on Yasmin without clinging to the wound.

The Hand Operations

As Yasmin’s hands were growing, we found that her bones weren’t having room to grow properly because the skin wasn’t stretching as much as it should, instead the skin around he fingers was causing the fingers to fuse together. After medical consultation in Egypt and in England with a specialized surgeon, it turned out that an operation was necessary to help the skin fit the growing bones.
Accordingly, the expert came and the operation was performed on one hand when Yasmin was almost seven. To get a grasp of what the operation entails, I have included a few pictures of Yasmin’s operation. I took them myself as I attended the sugery!
Please be warned that the pictures are rather graphic and are not for the squeamish or faint-hearted.
The operation went very well on the hand. But what the doctors omitted to mention was that physiotherapy was necessary after the operation. Because we didn’t know, she didn’t receive physiotherapy at first. Thankfully, when we started the physiotherapy sessions, she has improved a lot.

Yasmin and EB

The Story of How Yasmin was Born and How EB was discovered
I am Hanaa, Yasmin’s mother. In this section, I would like to share with you how Yasmin came into my life and the sequence of events that led to us discovering that she had EB.
My aim from this is to share with you the process of discovery, hopefully to help some of you make a faster discovery or help their friends or relatives with the knowledge, because time counts here, and also to share my experience with other mothers who have had the same path of discovering EB in their baby. And finally, to share the joy of having my Yasmin come into my life.
Another section closely related to this is the process to enlightenment, how I gained the experience (the hard way) of proper management of EB. That section, Yasmin and EB, will give advice to people who have just discovered their babies have EB and want desperately to give them the best possible care.

Yasmin’s Birth
Yasmin was destiny’s child, no pun intended. She was not planned, as we already had three children, three healthy boys. My younger twin boys, were all ready to go to school and I had thought I had said goodbye to diapers, and after taking care of twins I was beat!
So much was I not expecting this pregnancy that I didn’t realize I was pregnant till I was three months on the way. When my husband and I found out, it was earthshaking for us to think we would go through the routine of baby care again. But as I was going through my worries, something happened that I could only say it was one of many proofs that Yasmin was destined to be. A religious friend of mine who I hadn’t seen for some time called me and when she heard I was pregnant talked a lot about how much of a blessing it is to have a child and how God planned for us every baby that is created. She quoted citations from the Quran, the Muslim holy book, and it just all went right through and settled into my heart and I was quite peaceful with the idea of having another baby, my fourth child.

Then, when I went to the obstetrician, I found it was a girl and my excitement with the pregnancy was complete.
At this point, another proof of destiny came, except no one realized what it really was at that time. The doctor believed he discovered an odd medical condition in me that meant a C-section delivery would be necessary, and so from that early period in my pregnancy I was to have a C-section. Now I know that this was the best way to deliver Yasmin, since a normal birth would have meant that her skin would have been bruised very badly. But at that time neither the doctor nor I had a clue, we thought the C-section was meant for my condition not the baby’s.
And she came to the world by C-section. The strange thing is, the condition that the doctor thought necessitated a C-section just went away, it was just the Lord’s way of directing us to have Yasmin delivered in a way that would protect her weak skin. She was meant to be.
The First Hint of EB
Shortly after Yasmin was born, while we were still at the hospital, I noticed the first hint something was not so perfect. At that time I had no real knowledge of EB; my only encounter with it was that I had heard that an acquaintance of my husband’s had a grandchild with a serious problem with her skin. At that time I had never heard of EB, and never thought twice about it.

About human skin being a source of life-threatening problems similar to bad burns

I just took skin for granted, as most of us do until we meet EB. When they brought Yasmin to me at the hospital, Yasmin’s wrists were bruised where the hospital nametag was placed (you know, that little bracelet with the child’s name tag). What came to my mind was that it might have been too scratchy or that one of the nurses might have tightened it by mistake. I just insisted that we remove the name tag immediately, not caring a bit while they were warning me that without these bracelets, babies could get mixed up. I will not mistake my baby, so off it went.
But the wrist bruises didn’t go away, surprisingly. My next thought was the sleeves of the baby-suit she wore might be too tight.
But then I noticed something else. The skin on one of her ears looked strange, sort of like it had been burnt and the skin hadn’t come back yet.
At this point, my husband being a doctor, we started suspecting something was wrong. A pediatrician and a dermatologist were consulted. The diagnosis was, some skin condition known as “blistering skin” and is scientifically called “epidermolysis bullosa”. We said “what?” They went on to explain that it is very rare, the dermatologist had only seen a couple of cases in his entire career.
What I needed badly to know was what the cure was. But when we were told medicine and science had not yet come up with an answer, my next question was “does it go away as my baby grows older?” Some things do: the baby is born with a condition or another and as they grow up they grow out of it. The doctor instead told me that what we need to know is the severity of the disease, as it comes in different forms or types.
They took a sample from Yasmin’s skin to test it. The result was it was a simplex type. The doctor told us it was the least severe of types and we needn’t worry much. It is not going to interfere much with the child.

We checked with other doctors. None of them advised us to take any further action. Given that it has no cure, it kind of made sense. But they omitted to tell us that special care is a must, that it doesn’t have a cure but it takes very special management. We handled Yasmin just like any baby. We would carry her from under her arms and hold her across our sides. She was breast-fed and when she had trouble sleeping, which she had more often than normal, I would place her over my chest, just like a normal baby.
Bruises kept appearing, and I kept trying to help them heal, with antibiotic creams and just regular gauze.
When severe blisters started to appear at the back of her knees, we realized that she needs special care. It was probably from the way we carried her that her knees bruised like this. From that point forward, we started taking more care about how we handled her. Her brothers were not allowed to carry her, only my husband and I.
What Yasmin had was not simplex EB as we had been told, but rather it was the much more severe type. I do not know if she was born with this form and the test was wrong, or if it developed from simplex to complex.

School or No School, That is the Question

A very tough question I had to face was whether to send Yasmin to school, regular school of course. It was a lot of exposure and contact, and a lot of chances for blisters, accidents, and contamination. On the other hand, it was necessary for Yasmin to get a healthy social life, important for her psychological upbringing and for her mental growth as well. It was also important for me that she gets her best learning opportunity.
Weighing things on both sides, I came to my conclusion, my philosophy actually. EB should not stop us from enjoying life and living it well, we just have to be careful. To school she would go. My husband was reluctant because of the risks but he also believed that our daughter Yasmin deserved the best chances in life.
Then came the choice of school. After looking through other people’s experiences and reasoning it out with my husband, I came up with the following criteria:
1. The school needs to be close enough to minimize the distance traveled, both on a daily basis and in, God forbid, cases of emergency.
2. Understandably, I need to be accessible for the school to contact me and be available to travel the short distance if I am needed at school.
3. The school administration needs to be informed of the special precautions needed for people with EB and has to accept to take her on.
4. Teachers and people working at the school need to be mature enough and willing to learn about the condition of EB and how to handle Yasmin. God is Most Gracious. The school that two of my sons go to, which is one of the best schools in Cairo, fits the profile and was willing to take her as a student. Yasmin went through the interviews and they really liked her and now she has been there three years. Two of the female workers even acted, literally, as her body guards as she went up or down the stairs.
5. Yasmin blossomed as she started going to school. It’s true my heart stopped beating from the moment she left to the moment she came back home, but it was worth it. She made friends, she participated in external and internal school activities, and she had birthday parties not just for herself but for her dolls and she invited her friends over. She was accepted and loved by her school mates.

Tests for EB Patients

Due to a difficulty in absorption of iron in some cases of EB, an EB patient has to be tested for anemia. The laboratory test to evaluate anemia is CBC count. This is especially important for RDEB form of EB.
Because of the wounds, infection has to be measured by using bacterial cultures from poorly healing wounds or wounds that appear infected.
Because esophagus strictures are commonly associated with the complex types of EB, this needs to be measured using GI dysfunction.
Nutrition is another issue with EB. This is evaluated by monitoring serum albumin, height and weight curves, diet diaries.
In some cases, muscles are affected, they contract in an abnormal way. Contractures are tested by establishing the range of motion of limbs. Physical therapy is very important.