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My Life
Miranda's Story
Miranda was born on December 14, 1998 in Rolla, Missouri at Phelps County Regional Medical Center. She was a planned C-Section because my doctors and I thought she was gonna be at least 9 pounds. She was born at 6:57 a.m. She weighed 7# 4.7 oz. Her right leg was missing skin from her knee to her toes with the exception of her calf and four little toes. There were also three small blisters on her forehead that had already popped. They immediatly called in a dermatologist who came to the hospital and looked at her. There stood three doctors and many nurses who had never seen anything like it. By this time, not knowing any better, the handling and wrapping of her leg to prevent infection, had caused several more blisters. Miranda' doc told my husband (Matt) that Miranda would need to be transferred to another hospital and since I was asleep he would have to decide which hospital she would go to. Matt decided to send her to St. Louis Children's Hospital. When the doctor called the hospital, they told him they thought they knew what it was from the description he had given them. My doctor came in and told me that there was a defect on Miranda's leg and some blisters on her head??!! They still had no idea that the blisters were anything to be concerned about. This was very upsetting (to say the least). I laid there guilt stricken and cried. They sent Matt in to be with me and calm me down. It was Monday and I would have to stay behind for two days until the doctor could release me. Doctors from St. Louis called and started to explain this and that and that she had Epidermolysis Bullosa and Cutis Aplasia. Huh!!!???; The doctor from Saint Louis said her main concern was the blisters in her mouth. They also said that she could get blisters in her throat. I'd have to say those were the longest two days of my life. In the meantime they called and got our permission to do a biopsy to confirm the diagnosis. On Wednesday, when we all (our 3 year old also) got to St. Louis, the doctors started pouring in. She was in the NICU. The first matter I wanted to take care of was nursing her. I had already lost two days with her, I wasn't gonna wait any longer. It was crazy and extremely overwhelming for all of us. Miranda was crying. It was the first time I'd held her. Makayla was very anxious, hungry, tired etc. I had one doctor standing over me explaining what eb was while two nurses are standing over me while I'm trying to nurse telling me to give her a bottle. My three year old is "swinging from the vines" while my pour husband was trying to keep up with all of it.We went to St. Louis with this impression. We would be there for awhile and the cutis aplasia was the main concern. When we got there we learned that the leg was a matter of "procedure" and the blisters were a disease called EPIDERMOLYSIS BULLOSA, EB for short. This is a very rare (1-50,000)genetic skin disease. There is a 25% chance (with recessive type) with each pregnancy. The total opposite of what we expected. She received her skin graph on Friday and we were home on Sunday afternoon. Now we really are overwhelmed. Her derm gave us a (owners manual) tape explaining some things and the rest we've learned as we go.
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Miranda's right leg at birth
Miranda is a beautiful child. She is very happy and full of life. She has many limitations though. Epidermolysis Bullosa has many hidden concerns that people would not imagine when told it is a skin disease. It can cause malnutrition, contractures, deformities and diabilitation. The constant blistering of the skin causes a build up of scar tissue. If this happens in their throat the child will need a dialation to open the passage to allow food to pass through; if it happens on the joints, it causes contractures which will lead to diabilitation. These are only some of the fears that we face every day. Miranda at 17 months old weighs 20# 8 oz. She is thriving well for a child with EB. She can only eat soft foods and this can become very challenging espeacilly if the child is finicky. Her clothes cannot have zippers or snaps because they will damage her skin. Through all this (and more) Miranda's is a ray of light. Smiling, laughing and overflowing our hearts with joy. She, and many other EB children have been through more pain and anguish in their tiny little lives than most adults will go through in a lifetime. |
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Our life has changed dramatically to say the least. Matt works and I am lucky to get to stay home with my children. We made the decision when I was pregnant with Makayla (our 4 year old daughter) that no matter how poor we would be I would stay home with our kids. THANK GOODNESS!!!!!!!!!!!!! There is no way I could imagine having to leaving them with someone else everyday. When Matt gets home, we eat supper. At 7:00 I give Miranda her itch medicine. Itching is a big problem with EB and without medication the child will tear theirselves apart. We give the medicine about half an hour to work then the fun begins. We're lucky, Miranda likes her bath. In fact, she will set in the tub any where from an hour and a half to three hours. I use vapor bath in Miranda's bath water because, for some unexplainable reason, it seems to help her itching. While I'm giving Miranda her bath, Matt is preparing her dressings. In the 17 months that we have been doing this we have probably cut our time in half. I take Miranda out of the tub, put her on a changing table and away we go. Matt has become quite the professional wrapper. He wraps her legs and then I wrap her hands and arms. Miranda is a troper. Most of the time she is the most patient little angel you could imagine. We've hung stuffed animals and posters from her ceiling for her entertainment and when that doesn't work we entertain her however we can. Makayla is the best big sister in the world. Many times she's right there with us talking to Miranda, singing to her, whatever it takes. Miranda loves to hear Makayla sing and so do I. This has all been a big challenge. Taking care of Miranda takes up so much time that sometimes I feel like I don't get to spend near enough time with Makayla. Matt & I don't get much time to ourselves either but I know as she get's bigger it will get easier and she's only little once. Both of my kids have changed my life dramatically and Miranda having EB has made me a stronger and wiser person. I have no problem being assertive now. This whole experience has made my family much closer than I ever thought we could be.
Miranda taking a bath. Playing with her soft puzzle. September 1999
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