Nicky's EB Info World ~ Feaured EB patient

EB entered our lives on October 8, 2002 when our son, Harrison, was born. He was three weeks early and perfect! Dark hair, big brown eyes opened and eager to take in all his surroundings had to offer, 10 fingers and toes and a great set of lungs!

          My pregnancy up to then had been uneventful, so we didn’t anticipate any problems for Harrison’s arrival. On a Tuesday morning, three weeks before my due date, I started feeling some slight discomfort and just knew Harrison was ready to come out and join our family. However, because he had his right hand on top of his head my OB warned me that a C-section may have to be performed. I had delivered two prior babies vaginally, but wasn’t concerned about having a C-section. We had to do what we had to to deliver Harrison safely. By late that afternoon, Harrison’s hand had not reduced and he was still ‘hand presentation’ so by 10pm that night when his hand was still there, off to surgery we went.

          A few minutes later, we had our beautiful baby boy! 6lbs, 13oz and only 18.5 inches long! He was so alert and didn’t seem bothered by all the commotion of being born via C-section. The doctors soon discovered something a little unusual with Harrison’s thumbs—they were denuded, free of any skin. The doctors didn’t seem too concerned and said it was most likely due to vigorous thumb sucking in utero. Sounded about right to me so we didn’t make a big issue of it. By morning, when his pediatrician came by to see him, he looked at his thumbs and said those words that still ring in my memory ‘this looks like it could be Epidermolysis Bullosa.” He consulted a pedi-dermatologist to see Harrison, but he didn’t think it was EB because he said ‘EB doesn’t usually present bilaterally like this.” Boy was he ever wrong. Leave it to Harrison to be the first. As we found out later, Harrison wasn’t your usual baby or EBer, by far.

          My husband and I had discussed circumcising Harrison prior to his birth and because we didn’t have a definite EB diagnosis, went ahead with the procedure. Needless to say it was a horrific 5 months on the healing road. In the interim, he had two (2) cauterizations to try and stop the bleeding-ouch!-and hated diaper changes… can you blame him? Looking back on it, we would have done a few things differently, namely the cauterization, but hind sight is 20/20, right!

          By the end of the first week, Harrison began to experience a hoarse cry. We didn’t think much about it and figured he was just hoarse from crying or would have a raspy voice like one of my nephews. After a couple of weeks, our pedi-derm was still stumped as to what Harrison’s denuded thumbs were being caused by and he decided to consult his former partner at Texas Children’s Hospital to see if he could help diagnose Harrison.

          By the end of the week, we had an appointment with Dr. Levy, what luck, huh? He saw Harrison the next day and because he only had the two denuded thumbs, he said he most likely

had Epidermolysis Bullosa Simplex subtype Kobner (the mildest form of Simplex). He went on to explain EB and what it entailed but because Harrison wasn’t showing any blistering he thought it was most likely a very mild case of EB Simplex…well, that didn’t last long.

          By seven weeks, the first blister appeared on his upper left thigh. We didn’t know what to do, so I called his pedi-derm who didn’t have any suggestions but to wait until it ruptures on its own…not a good idea! By late that afternoon, the small pin size blister was the size of a pea pod. By day three, he had over 15 blisters around his groin area.

          We got an immediate appointment through our pedi-derm with Dr. Levy who biopsied a blister and a couple of weeks later, it came back EB consistent with Simplex.

          We were still concerned with his hoarseness and Dr. Levy referred us to an ENT he knew who worked with EB patients. We contacted her and soon she became an integral part in Harrison’s fight for life, along with several other health care professionals.

          After a couple of visits, our ENT scheduled a bronchoscope in late January and we got some bad news. Harrison’s airway was the size of a pin and after extubating him, his airway closed off and he had to be reintubated. He spent a week in a drug-induced coma and discussion of a tracheotomy commenced. By the end of the week, his airway had not improved and a trach was imminent. We spent three months in and out of the hospital with various complications and infections…his skin held up pretty well but I was constantly by his side to ensure the nurses didn’t cause any further damage.

I did learn that the inner, leak-guard, liner of the diapers were causing the blistering in his groin area so I would diligently go through a pack of diapers and tear those liners out. The nurses didn’t interfere with my changing him or feeds because they were very concerned about his skin and I’m sure, felt relieved that I was willing to do all his hygiene for them. By this time, Harrison had lost all his fingernails and toenails and had developed a large wound on the back of his left calf that, unfortunately, never healed.

          On Harrison’s last admission in March of ’03, for a pulmonary infection, we were told by his pulmonologist that she could not and would not release him home without either a feeding tube or a gastric tube. Harrison was 12lbs at 5months and was drinking minimally because of the oral blistering which would leave his palate raw and bleeding even with a special feeding bottle used on cleft palate children, the Haberman feeder. It was a difficult decision to make because I was apprehensive about another invasive procedure and the problems that it may and could cause. After a few days of pondering and discussion with my husband, we decided Harrison would benefit from a g-button. We stayed a couple of days after his procedure to become familiar and comfortable with feeding him and then it was off home again.

          Third time was the charm, since Harrison never had to spend another night in the hospital but his experiences thus far left him deathly afraid of doctors-the white-coat syndrome- as they call it and very attached to me. It became so severe; he wouldn’t even go to his daddy or his sisters.

          Over the next few months, he became more alert and when bandaging his hands was introduced, he absolutely hated it. He would fight me on the wraps and pull his hands away, cry, and make a big fuss. However, after a couple of weeks, he realized that he could use his hands and hold objects without hurting them. After that, he freely let me wrap them and didn’t fuss.

          Now, having an EB baby

is hard enough with all the dressing changes, wound care, baths, feeding and clothing them, but added to this was the fact that he was trached, which involved trach care, twice a day, trach tube changes once a month, and g-button care three to four times a day. It was exhausting! Baths were probably the worst because Harrison hated being naked without his bandages, and being in the water. So, I would prepare everything ahead of time and do his baths as quickly and efficiently as possible. His trach care wasn’t too strenuous, since Harrison kept pretty still and didn’t fuss much so I learned to do trach care solo early on, a task I was quite proud of.

          Harrison’s first year went by with several ups and downs, and countless happy and joyous memories and experiences. Through it all his face was the one area that remained uninvolved. His body took most of the hit, but his face was perfect! But for some reason, after his first birthday his face suddenly became one of our most difficult areas to heal. Over the next few months, we tried many different healing suggestions, and looked like we were finally on the road to recovery.

          Of course, as he began to grow and gain weight, we encountered new challenges. Because he was not ambulatory nor could he crawl, we carried him to and fro causing severe breakdown to his skin, especially his upper back thighs which were in constant fluctuating stages of healing.

          His muscle tone was weak and though he had several therapists working with him, he never developed the ability to ambulate, crawl or pull up on his own. One of his favorite activities that made him grin from ear to ear and entertained him for several minutes was his bouncer. We would strap him in and he would ‘fly’ in his bouncer. He loved it and it brought us great joy seeing him so happy. It is one of our fondest memories of him.

          Another challenge we encountered as Harrison grew and developed was the narrowing of his airway. We had much difficulty reducing the inflammation and were forced to use his nebulizer more and more. His secretions had reduced as he grew, but unfortunately, this tended to dry out his respiratory track causing blistering and bleeding. We did manage to keep the bleeding to a minimum and somewhat under control with his nebulizer and humidifier. His airway remained an area of concern and anxiety throughout his remaining days, though.

          When we heard of the upcoming NPCC in Palo Alto, CA we were hesitant about going seeing as how Harrison had been lethargic, due to severe anemia, for a couple of months and we didn’t feel he was well enough to travel; never mind, the fact that he had never flown or that I had never been away with him without my husband. I was unsure and anxious as to how he and I would tolerate being away from home for three days on our own.

          A couple of weeks before the conference however, Harrison had improved tremendously and he was sitting up and smiling spontaneously at us and his surroundings. He was such a happy baby boy and ready to take on the world. Thus, we decided that the benefits of traveling to California outweighed risks and anxiety we faced by traveling alone. So off we went, just Harrison and mommy and tons of luggage. Fortunately, the airline was very accommodating to our needs and they were able to reserve us a row to ourselves, which was just perfect. Aside from a few stares, the flight to Palo Alto was uneventful and Harrison slept through both the take-offs and landings.

          In Palo Alto, we had the opportunity to meet many of the EB moms and families who supported our journey with EB from the beginning. Though

Harrison had a severe case of stranger anxiety, he became quite comfortable around the EB families and even let some touch him, which was a huge breakthrough. It was quite a challenge doing all of Harrison’s wound care, baths, and trach care alone, but one that I really enjoyed, since I didn’t have to focus on anything or anyone else but him. I treasure those three days alone with him and remember how content he was to be alone with ‘mommy’ and not have to share my attention with anyone else.

          We made many breakthroughs at the NPCC, and were very optimistic about his treatment, healing, and future. We made some wonderful friends and met some amazing families, care-takers, physicians, and professionals dedicated to bringing awareness and working diligently towards an eventual cure for EB.

          We had a wonderful experience in Palo Alto, but returning home was just as rewarding. Harrison’s eyes lit up when he saw his daddy at the airport, then again seeing his awaiting sisters as we walked in the door to our home, and I could see the excitement in his eyes when he saw his crib again after three days. He was up playing late that Sunday night of our return and finally fell asleep content and happy to be home.

          The night went by uneventful and by morning Harrison was still sleeping quietly and peacefully. I ran errands and decided to shower before disrupting his slumber to begin our daily routine of bandage changes and trach care. A little after 12 noon, my oldest daughter came to get me from the shower saying that “Beto” (Harrison’s nickname) couldn’t breathe. I told her to get her daddy, thinking he had pulled out his trach, as he had done before, but when she said ‘daddy is already with him’, I felt a surge of panic. Entering Harrison’s room, I saw my husband diligently administrating CPR on him. He told me to call 911 because he didn’t want to stop working on Harrison. Harry was truly calm and efficient, while I was a mess wondering where the paramedics were. It was only 3 minutes from the time I called 911 to the time they arrived, but it seemed an eternity. My husband never stopped working on Harrison, and I could see Harrison’s little face between chest compressions and he looked so peaceful like he was still sleeping. When the paramedics finally arrived they worked on Harrison for another 20 minutes before transporting him to the hospital. We of course informed them of his skin condition because they were taken aback when they cut his clothe off him and could clearly see all of his wounds.

          At the hospital they continued working on Harrison, and when the attending physician came in, he asked how long they had been working on him and after informing the physician of the time, he looked at me and told the paramedics to stop. Our beautiful baby boy had become an angel!

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