Nicky's Story

The story of Nicky's birth started way before he was ever conceived. His big brother Alex died the day before he was due of what was probably a cord accident (we are still unsure of the cause at this time, and will probably never know, although it's apparent he also had EB and that could have played a major part in his demise), so his daddy and myself did not want to "wait" until the normal onset of labor started, we were just too afraid of a repeat of circumstances, so, after many trying talks with the Dr., let's just say we "forced" him to induce early... 2.5 weeks early. The Dr. was not happy at all about this, and he did not hesitate to let us know. He told us many times that it would be a very long labor because I was not dialated at all, and he was less than pleasant about the whole thing.
He did not realize how stressed both Nick and I were about this baby making it, we wanted him to be born alive, and we knew that he was alive at that moment. We did not want to take any chances and risk his life just to arrive at 40 weeks.
This was not a "normal" set of circumstances, we were scared to death, and felt that, since babies are no longer considered "preemies" at 37 weeks, that he would be "better off" out than in. Not to mention the fact that it would have been stressful for the baby and for us to continue a pregnancy when it could be over. In our minds, fogged with worries, we would have much rather have a baby in NICU for a few days than having it die inside of me again. Sure, the overall odds of that happening again (and to me!) were extremely low, but not unheard of. I read about a lady who had 3 cord accidents in a row, so we didn't want to take any chances. What gave us the feeling that everything would be OK were the countless friends and family members who had their babies at or around 38 weeks and their babies were just fine.

Nicky was actually due December 12, 1996, but one late evening on the 24th of November I left for the hospital ready to be induced. It had been a difficult pregnancy, but all tests showed that our baby was healthy and we could not wait to meet him.

We were told the baby was big for 37.5 weeks, but not too big, they induced my labor, and things started going real fast at first, the water broke almost right away, but then everything slowed down considerably, and it was then that they realized he was posterior presentation, or "facing up". Nicky was not descending in the birth canal; so all my pushing was worthless. They tried to turn me around like a salami for 5 hours to make the baby turn, but they only succeeded in making the baby's heartbeat fall, which scared both Nick and myself to death. We found ourselves crying, and the doctor, on the other hand, would continuously tell us "I told you so, the baby was not ready to come out".
It was now 7 pm, I had been in labor for almost 20 hours, and I was no closer to have this baby vaginally than I was 20 hours earlier, even if I was dilatated to 10 cm. Nick pleaded the Dr. to give me a c-section to end this torture, but he refused. He told us that he would not be able to sleep that night knowing he gave me a c-section before he tried everything in his power to make this baby come out vaginally. Nick and this Dr. were batting heads constantly; it was pure hell for everyone involved. It was almost like this Dr. was willing to leave me there indefinitely just so the baby could come out vaginally, out of spite for my husband.
Thankfully the end of Dr. L's shift finally arrived. A nurse took care of me until the Dr. on call came in. I will never forget this new Dr, he was an angel that came to save the day, I truly believe it. As he entered the room he made everyone at ease. Both my MIL and my mom were in the room, both Italian, and Dr. A. (also Italian) started conversing to them in Italian!!!

He was immediately concerned about the baby and me, and immediately suggested a c-section. Yes...it was HIS idea! The funny part about this is that, not knowing that this is what we wanted, he spent the next five minutes trying to convince us to do it!!! Among other things, he told us that, sure, the baby could come out vaginally, but not for who knows how long.... He also said that he was concerned because my water had broken almost 24 hours earlier and was afraid of an infection. We told him that since Alex had been due on the "26th" (of another month, February, but still the 26th), we did not want Nicky to be born on that "day", too many bad memories. Even though there was a little more than an hour left on the 25th, he promised that he would be born that day. He told us that he had also lost a son much like we had lost Alex, and that he understood our worries and concerns completely. He talked to Nick like a father, reassuring him and helping him. He told us that he would do his best.
His best he did indeed, and Nicky came into this world at 11:42 pm!! With almost 20 minutes to spare!!

Nicky was just fine, even if early, big, lungs going great, and as cute as a button. The Dr. kept Nick and everyone's mood up by laughing and joking the whole time. He wanted the baby to be named Salvatore like him, and he even gave my husband an anatomy lesson at my expense. (!) We did not know it at the time, but being born c-section, hence being induced early, was the very best way for Nicky to be born, and the best thing that could have happened to him. If he would have been born vaginally they would have torn his skin off on his head and who knows where else. He was certainly spared a lot of pain. Unfortunately, many other EB babies were not spared this pain, I have heard of babies being born without skin on many places of their little bodies because of the friction of passing through the birth canal.
Soon after he was born they showed him to me and he was absolutely perfect, he looked a lot like his big brother, but he had a much smaller mouth, had a lot less hair, he was blonde instead of dark haired... and, most important of all, he was breathing!!! They took him to the nursery to run all the newborn tests, and everything "looked" normal at first except for his mouth. After they aspirated all the liquids from his mouth it started bleeding and losing its skin.
At 4 am they finally brought him to me to nurse, and this was the first and last time I ever remember thinking of him as a normal baby, even with his mouth problems. I was cradling his little sleeping body in my arms and I was in heaven. The only thing that ruined the moment was my tiredness, I could not keep my eyes open, even with all the adrenaline of seeing and holding my baby! I felt like such an terrible mother. I had gone 2 complete nights without any sleep, I was hungry, tired and I felt horrible. There were tubes attached to me all over the place, but I was at peace seeing my little Nicky sleeping. He refused to nurse, but who could blame him? That was the one and only time that they ever brought him to me.

Around 8 am the pediatrician came into my room to tell me that they had to put Nicky in NICU in an incubator because of the problem with his mouth. They thought it was either a "Staph Infection", "Herpes" or some rare skin disorder called "Epidermolysis Bullosa". By then, in fact, he had started blistering all over the place.
They told me that they had contacted a dermatologist and that he would have come there that same morning on his way to the Children's Hospital in Phoenix from his office in Tucson. When he arrived it was around noon, he told me that he had seen Nicky and did some sort of test with the eraser from a pencil and he wrote down the name of the skin disorder. He mumbled something about us having a 25% chance of this reoccurring in another pregnancy and then he wanted my signature on a piece of paper to let them take a skin sample for a biopsy.
I was in a fog, I was drugged, I couldn't get out of bed on my own, I would throw up everything I tried to eat, and I still had not had a few good hours of sleep, so my mind was not clear, this is all I remember of Dr. H's visit unfortunately, aside the fact that he was very interested in seeing pictures of Alex.
The nurse took me down to NICU in a wheelchair and then left me. I was stunned to see that my baby had been put in isolation. When I got there the nurse was busy, she told me she did not want to make me hold him because she had just calmed him down. Soon thereafter though Nicky started crying and I wanted to hold him to comfort him, but the nurse was busy taking care of another baby, and I, because of my condition, was unable to get Nicky myself. They had to put an IV on the baby because he could not eat since his mouth was in such a poor shape, and he was attached to a heart monitor and other things. He was in an incubator to keep him warm, had no clothes on him aside some bandages and the diaper. They were keeping the tubes attached to his tiny body with gauze. They could not use tape. The tape had already damaged his tummy, and the hospital bracelets that they had put on his ankles had done some serious damage as well. When the nurse finally put him in my arms he was crying hysterically, and she was giving me an attitude thinking that I woke him up. I held him for what I thought was an eternity, the blisters on his little body were horrible, I had never seen anything like it. On the right side of his head there were a lot of little red marks where the skin had torn off. The nurse told me he got those spots when I was trying to push him out, he kept banging his head onto my bone. The heart monitor they had put onto his head when his heartbeat had fallen caused other spots on his head.

I talked to Nicky and sang to him, then he calmed down and the nurse came in all apologetic to put him back into the incubator.
Several hours later she came into my room to let me know that I had worked wonders on him and that he was still resting peacefully. I went back to see him later on with my mom and my MIL, and it was there that Nick called me in tears. Earlier he had come to see us, and I had given him the name of that strange skin disorder. Now he had had the time to research the Internet about it, and he was completely devastated. He told me that he would most likely die from this. The neonatologists comforted us a lot, and told us that EB is something that could be lived with. Another nurse told us that he had seen a newborn with EB before that did not survive, but that was in much worse shape than Nicky and that, unlike him, this baby's wounds never healed. We did not know what to think.
The following day I walked on my own to see Nicky in NICU and I spent lots of time with him. I got discharged the morning after, Thanksgiving Day. I really didn't think I was ready because I was still in a lot of pain, and I didn't like going home without Nicky. Some people told me that they did not know how I could bear going home without Nicky, but I told them that last time I went home without my baby, Alex was in the morgue, and this time it was a lot easier knowing he was simply in NICU.
I went to the hospital every day even if I had to be put into a wheelchair from the pain once I entered the hospital. Every day I would spend hours holding him, talking to him, rocking him, feeding him... I could have never imagined to love a child as completely as I loved Nicky... yet I was so devastated both that I did not get to enjoy Alex this much, and that Nicky was in this much pain.
Since I was pumping the milk for Nicky I could not take too much medication for the pain, but I felt Nicky needed the breast milk more than I needed the pain to go away. The neonatologists told me that they could not let me take him home until they figured out a way to make him eat on his own.

Using a little creativity, the nurses and the neonatologists were successful in making him eat from a bottle. He could not suck much because his mouth was so sore, so they used a "Haberman feeder" to squeeze the milk into his mouth. They used some lydocaine for his mouth so the bottle would not cause any pain if he were to suck. This worked so well that within a couple of days Nicky was off the IV.

Now they wanted me to learn how to medicate his wounds and care for him in every way, so the following Monday night I spent it at the hospital in a room. The nurse instructed me how to care for him and I stayed in a regular hospital room with Nicky and my MIL for the entire night and the next morning. Nicky had a lot of gas during the night; hence a lot of stomach ache, so he cried a lot. At one point I took him out of the room with me and I started walking around the hospital while I talked to him about his daddy, his big brother, the cats that were anxious to meet him and everything else that came into my mind. Looked like my voice soothed him, so that is what I did to calm him down.
I will never forget one nurse's comment on "how well we were dealing with this". But after the experience with Alex, we were just happy we could hold a living baby, blisters or not.
The dermatologist came back the following morning; he talked to us for a while and managed to really depress us. Over and over again he did not want to give us any hope for his survival. He told us that we would be in week after week to treat infections, if not worse, and totally painted an extremely bleak picture for us. Afterwards he took us step by step on how to care for his wounds taking extreme care in doing so.
Thinking back, there is a lot to be said of all the wrong things that this Dr. did. For starters, when he came to see Nicky the very first time, (when he was only a few hours old), he never told any of the nurses to puncture the blisters or how is the proper care for these types of wounds. Because blisters usually should go unpunctured (on a normal person), the nurses did not touch them, but with EB it is of extreme importance to drain and tear these blisters otherwise they can grow and grow and became really big.

Because he failed to tell us this, Nicky was in real bad shape. Both elbows were completely skinless, and so were his ankles, the top of one hand, several fingers, one of his knees, the big toe on his right foot, several spots on his legs, and his chest.
This Dr. could have painted a less bleak picture for us too. Knowing what we know now, unless a child is born with Junctional EB (the Herlitz type only, where all the internal organs are effected, I will refer to this from now on as HJEB), as long as he's taken care of properly, he can be OK! Sure, he will be limited and in a lot of pain, but he can live with this.
When we went back to see Dr. H. at Phoenix Children's Hospital a couple of weeks later, he brought in a lady who had lost a son from HJEB a few months earlier to help us and teach us proper wound care. But when this lady saw our son, she immediately knew he could not possibly have HJEB, and that he was going to be OK. She showed us photos of her son and we could definitely see a difference.

Nicky thrived at home. When we brought him back to the hospital to see his nurses on Christmas Eve, (he was 1 month old) they were in awe on how well he was doing, this was a few days before the results of the biopsy came back confirming that Nicky had EB and classifying it as "Recessive Dystrophic".
When we went back to see the dermatologist when Nicky was 4.5 months old he took a pair of scissors and literally cut Nicky's skin from the bottom of the toe that was fusing without any anesthetic. Nicky screamed for hours. Needless to say we never saw this Dr. again, Nick was furious. We are more knowledgeable about EB than he is, and to get this kind of care from someone who is supposed to be an expert... I rather go without, thank you!! The real experts were the Drs at the EB clinic at Stanford University in San Francisco.
We took Nicky there twice, once when Nicky was 8 months old, and once when he was 14 months old. The first time they classified him as having "mild" dystrophic, but the second time, because of all the problems of vomiting blood, they unfortunately had to "upgrade" his condition as "medium", which has since been upgraded again to "medium-severe".
Nicky is and always was on the smaller size, and this is a concern. If he were a healthy baby I could just say he's taking after mommy, as I was also a little girl growing up, but since EB children have a tendency of becoming malnourished even if they eat OK because all the calories go to wound healing first, his weight/height is and always will be a concern, even if he hereditated his smaller size from me.
These Drs at the EB Clinic are wonderful because they are the only people that can truly answer all of our questions regarding EB itself. At Stanford we had the opportunity to meet other children with EB and some adults too. We were also given the hope for a cure, as the EBMRF (Epidermolysis Bullosa Medical Research Foundation) resides there, and we also got to meet Lynn Anderson, who is the president and founder. The Andersons have lost two children to Recessive Dystrophic EB, Chuck at 27 from skin cancer, and Christine at 14 from heart failure. Both children suffered deformities of the hands and feet, chronic anemia, malnutrition, and growth retardation. The people at Stanford were very helpful but nobody really knows how to deal with this disorder day by day better than people that actually live with it. Other parents met personally or through the Internet better answered questions dealing with everyday problems.

Life with Nicky and EB has not been easy, especially for him. At first we were transformed into nurses, and knowing nothing about medicine there was much to learn, about medications and other occupational hazards... However, 'til this day, Nicky has never been in to treat any infections, as the dermatologist told us would happen, not to say he has never been on antibiotics, but always for "prevention" only.
The parent of an EB baby cannot wear clothes that have buttons or anything that might cause a blister if rubbed against, cannot wear jewelry, including watches, and the nails have to be extremely short. The baby itself cannot wear anything that has too many buttons, or is harsh (like jeans), it needs to be soft and easy to put on with a large hole for his head to pass through without needing to do any pulling. Everything has to be padded, from the stroller to the bed, from the swing to the playpen.
The first six weeks at home I was lucky to have my mom's help. I would sleep cradling Nicky, I was too afraid to let him sleep alone in his bassinet, even if it was just next to my bed, then, in the morning, I would give him to my mom so I could catch a few zzz.

I took advantage of it until she left, then I went practically without any sleep for the following 2 months. Nick, who went to work, slept in the other bedroom for that time. Afterwards Nicky seemed to get the hang of sleeping, but even when he was 3.5 years old he still woke up at least twice a night, and only slept a total of about 7 hrs. His afternoon nap could be between 1-4 hrs, there was no telling.
Until Nicky was 4 months old he had to wear mittens to protect his face from scratches, and then wore gloves that were made of lycra and were elastic, so that the tips of his fingers could be free.
One by one, I watched all his fingernails fall off, and taking care of his hands is a big job.
His fingers have a tendency of contracting and webbing, and if this problem is not taken care of he will not have hands when he grows up.

Having the Dystrophic form of EB means also having the "disfiguring" kind. If an area gets too many blisters it will never regain its normal look, it will be reddish and squam constantly (his hands are like that). Nicky has many of these spots unfortunately, and these spots might become cancerous in his 20s if they are not replaced completely by new cultured skin (which is not available yet). Disfiguring also means that his hands and feet can web and contract, and his mouth and esophagus are severely involved. Because of this he can only eat mushy or liquid foods, and needs to be drinking constantly to keep the moisture. He has thrown up blood every now and then from a popped blister in his throat, and this is a very bad thing for him to happen for two reasons. For one, when he does this he will refuse to eat for a week... I believe it's from the pain of swallowing. I was lucky to have him take a couple of bottles a day, and he will throw up phlegm for that whole time. For two, and most worrisome, he built scar tissue in his esophagus and has needed dilatations every few months to enlarge the passage of food. It got so severe we had to put a g-tube to supply to him enough nutrition. It is hard to brush his teeth, and his tongue always has blisters. The tongue has somewhat healed attached to the bottom of his mouth, so he has a lot less use of it as a normal person.

He was 7 months the first time I was able to give him a real bath (meaning... immerse him in water), and his baths do take a few hours because all his bandages need to be changed afterwards.
He watches Barney or Blue's clues while mommy medicates and bandages all his wounds, then pads areas such as his knees, ankles, elbows, armpits, chest etceteras.

All of this is extremely time consuming, and thankfully, as time went by, Nicky has become more and more patient and cooperative. In Italy grandma helped, but when we got back to the U.S. the insurance would not pay for a nurse to come by to help us out because they claimed I did not need help because "I knew" how to do it. They just don't get the point.
In February 1998, when Nicky was almost 15 months old, I had to go to Italy to get my parent's help. I truly felt like I was nearing a nervous breakdown. Nicky is an extremely bright and careful little guy, and he's also extremely attached to his mommy. He knows he can get hurt, so he takes his time in learning skills that might hurt him. He practiced crawling for almost 4 months (from when he was about 8 months old) before he was actually able to do it. And he did it extremely slowly at first... and backwards!! He was 20 months old before he mustered up enough courage to walk on his own, but it wasn’t until after his second birthday that he was able to truly walk on his own without help and without falling.
One particularly precious thing about Nicky is that he is very affectionate, and likes to cuddle with mommy. After we got back from Italy we scheduled surgery to undo the webbing and contracting on his right hand and it was an awful experience. The problem was not the surgery itself, but the recovery and the making sure that the fingers healed with no webbing and straight and keeping them that way. In November 99 Nicky went through another hand surgery, this time on his left hand. The surgery itself was a little easier, but the recovery was a nightmare, and the hand did not heal right. In the summer 2002 we tried again to fix the left hand, and this time the surgery and recovery went so well, I am certain this is the very last hand surgery in a very, very long time.
When mommy and daddy got divorced and mommy had to go back to work, Nicky become quite accustomed at being with just mommy or his respite care provider, which he loved. He learned to be more independent. Nicky is also becoming a little social butterfly, which is amazing, since he does not spend much time with his peers. When he meets strangers for the first time he will go up to them and tell them... I am Nicky! He does have a preference for women, and gets closer to them faster than men, but overall, he is learning...
Thankfully mommy did not have to work full time outside the home for too long. After one year and a half mommy was finally able to work from home, and now that Nicky goes to school, just works while he's in school. He talks to daddy daily on the phone and they have a very close relationship.
Mommy remarried and now Nicky inherited a step sister and her half brother aside from a loving step-dad.
Nicky is extremely bright and finished on top of his kindergarten class even though he missed a lot of school. He's in third grade now and the teacher is very impressed on how hard he works and how fast he learns. We are all very proud of him.

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For more on these surgeries, please check the Surgery Tales page. For more about his life with EB, please check his Nicky and EB page.

To be continued...

Some may own castles on the banks of the Rhine,
and hire an orchestra each morning at nine.
But richer than I they will never be...
I had a mom who made Web pages for me!

~Nicky's Realm on the Web~
[Nicky's Journal] [Nicky's Story] [Nicky and Epidermolysis Bullosa] [Surgery Tales] [Picture Gallery] [Halloween Gallery] [Recent Photos] [1st Birthday] [2nd Birthday] [3rd Birthday] [4th Birthday] [5th Birthday] [6th Birthday] [7th Birthday] [8th Birthday] [9th Birthday] [DisneyWorld 2000] [Nicky's Wish] [Nicky's Toy Zone] [Nicky's Web Rings and Awards]

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Last Updated: July 02, 2006
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