Samantha was born at 34 weeks for no other reason that she wanted out! She was 5 pounds 6 oz (very large for a 34 weeker) and was 19" long. Her EB was present at birth- no skin on her ankles or wrists- and had no preemie issues from being born 6 weeks early. But because of her EB she was placed in the NICU for 13 days against our wishes.
After coming home she was back in the hospital for a staph infection on her hand and was there about a week. Two weeks after being released she got another infection but we were able to convince the doctors that she didn't need to be admitted and she was sent home with an IV and we went to the ER each day for almost a week for IV antibiotics. After that it wasn't until she was 2 that she was hospitalized for another infection- this time cellulites on her foot.
Sami's EB was very severe as an infant. By the time she was 4 months old she was missing about 75% of the skin on her body. Eating was a struggle and it wasn't because she couldn't eat, she didn't want to. My BIGGEST regret was NOT getting her a g-tube when she was a baby. By the time we decided to have one placed, we learned because of her stomach's shape and location a g-tube could not be safely placed.
By the time she was 1 we were able to reduce the amount of bandages we were using. In simplex it is better to not wrap all the time because wrapping slows the healing process. This is JUST in SIMPLEX and not other forms of EB. Between the ages of 1 and 2 we wrapped even less and by the time she was 2 all we wrapped daily was her torso and other spots when needed. Today she is almost 8. Her skin still scraps easily and she has to be careful when playing out side and riding her bike. She still gets some blisters and scraps and we spot wrap when needed. She prefers to have wounds covered than air dry, but she does notice they heal quicker when we don't wrap all the time.
Some common milestones:
crawled at 13 months
walking at 19 months
sleeping thought the night at 3 years
She also has acid reflux and is lactose intolerant.
In addition to EB, Sami also had torticollis (shorten neck muscles) and severe congenital scoliosis. But time she was diagnosed her curve was greater than 20 degrees which meant automatic brace but because of her skin it was not an option. So we decided to take the 'wait and see' approach. Unfortunately her curve progressed very rapidly. But the time she was 2 1/2 her curve was at about 60 degrees and it started to impact her lungs and was diagnosed with restrictive lung disease. We had no choice but to do surgery. Some plates and screws were placed into her spine to try and slow the progression of the curve.. She did very well in the hospital and was released about a week later.
That bought us about 2 years and shortly after she turned 4 her curve had progressed to 85 degrees and it was impacting her heart. It was now a matter of life and death. She could have surgery and have growing rods placed which would need to be expanded every 6 months for the next 8-10 years. The thought of her having major surgery and being in the hospital at least twice a year for many, many years was very upsetting. The risk of infection was great not to mention the emotional impact on Sami. We got a 2nd, 3rd and 4th opinion and made the decision to have permeant rods placed on her back. It would mean just one surgery but it also meant that part of her spine would forever remain the size of a 4 year old. It was a tough decision but it had to be made. So on Halloween 2006 she had her spinal fusion and it went well. The doctor only fused what he absolutely had to which was about 5-6 vertebrates. Which allows the other vertebrates to continue to grow. The surgery was about 10 hours and she spent a week in the ICU but did very well and doesn't even remember it!
Today her curve us stable- only moved a few degrees in the past 4 years. She will need another surgery when she stops growing (hopefully not till age 12 or so ) to fuse the rest of the spine. EB wise is is doing well. Issues here and there but nothing major.
I always get asked why her skin does as well as it does and I honestly don't know. I don't think we care for her any different than other parents who have EB kids. But I think there are two things that may influence her great looking skin- she eats very little sugary foods and she eats/drinks very little dairy products. Personally I think in her case is helps a lot. But I also have come to realize she and I are the EXCEPTION when it comes to EB simplex dowling meara, not the norm. I know many other kids her age and adults my age and people in between with the same form of EB and their EB still affects them a great deal and impacts their daily lives.
