One Of This Month's Featured Families!
March 2003
Nicholle
Crouzon Syndrome
Pennsylvania
"Kevin, I just don't feel right today," I said to my husband. I left early to go to work and stopped at my doctor's office because I had an idea of what was wrong. "Chris, I know you are just opening up, but I wanted to know if you could send me for a pregnancy test."
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I left the office with a lab script in my hand and stopped at the hospital before going to work. Later in the afternoon, I called Chris and she said, "You are expecting." I was really excited and could not wait to tell my husband. We were both excited and wanted nothing more than a normal pregnancy and a helathy child. My first pregnancy was normal until I went into preterm labor around seven months and it was discovered that my son had a kidney blockage.
This pregnancy started out normal but I noticed that I had to wear maternity clothes at around seven weeks. This was very unusual because I was five months along with my fist child before wearing maternity clothes.
Around eight weeks along, I woke up one morning with severe cramping in my stomach and back. It felt like labor pains and I scared that I was going to lose my child, so I called the doctor. He was very willing to fit me in and I went to his office about two hours later.
I remember his very straightforward questions. Are you sure about your last cycle? Are you absolutely positive? Could you have gotten your dates mixed up? He decided to send me for a sonogram immediately, and little did I know that my life was about to change forever.
I remember lying on the table and looking at the monitor. "What's going on," I asked the technician. "Did your doctor tell you that we were permitted to give you the results?" the technician asked. "Well, he never said you couldn't," I replied. "The reason you are having cramping in your stomach is because your uterus is expanding to hold your babies," said the technician.
"What did you say? Babies?" I was speechless.
"Yes, you are expecting twins." The room suddenly got quiet and I let the technician finish her exam. A few minutes later, she could hear me crying. "Are you o.k? Is there anyone in the waiting room that you would like me to get?" "No," I said between tears, "I am fine and I came by myself, because I didn't think anything was wrong. I can not believe I am expecting twins! How am I going to handle two babies?"
She smiled and handed me a sonogram picture of my babies and said, "Don't worry honey, you will be o.k."
Of course, I now became a high risk pregnancy with lots of extra monitoring. At around 32 weeks, I went into preterm labor and was able to hold on for another 4 ½ weeks before delivering. But this bout of preterm labor and subsequent sonogram brought bad news. One of the babies had 3 ovarian cysts that were consuming her ovaries. Since this was something that was newly discovered on sonograms, the unknown was scary. Delivery day finally arrived; I had gone to work feeling some
contractions, but nothing out of the ordinary. Finally, I called my doctor and he said, "Why don't you come over and let's check it out." I left work at 11:00 a.m. and delivered Haylee at 1:43PM and Nicholle at 1:46PM.
It was standing room only during the c-section. There were 2 of everyone because of the twins, what a circus. The next morning our pediatrician visited me and said that everything looked o.k. with the girls and we could discuss more at our 1 week check-up. Haylee was sent for a sonogram before we went home which confirmed the ovarian cysts that were detected during my pregnancy. We went home 3 days later and went to our first check-up 1 week after delivery.
At our 1 week check-up I remember the words so clearly. "We need to send you to see a specialist" our doctor said. "What kind of specialist do you see for a baby with ovarian cysts?" I asked. "No, Cheryl, it's Nicholle, she does not have a soft spot, she needs to see a neurosurgeon at Children's Hospital. My staff has already made the appointment for you." "I noticed that her head is rather large, what do they do for children without a soft spot?, What causes this?" I asked.
"I think Nicholle has Crouzon syndrome and generally children only need surgery for cosmetic reasons." replied my pediatrician. I went home and cried and asked God, why me? I am a good person and all I ever wanted was a healthy child. Kordell was born with a kidney blockage and had surgery at 6 weeks of age, Haylee was born with ovarian cysts and Nicholle may have something called Crouzon syndrome. WHY ME? WHY MY CHILDREN? Maybe it was something I did wrong during my pregnancy. Maybe I did not eat the right things; maybe I did not take every pre-natal vitamin? What did I do to my child? This just is not fair I thought. I did not smoke; drink alcohol or caffeine during my pregnancy. Why can someone do everything wrong during their pregnancy and have normal children and I did everything right and my children have problems? I knew nothing about Crouzon syndrome and in fact did not know how to even spell c-r-o-u-z-o-n.
The next days until the appointment at Children's Hospital were very antagonizing. I didn't know what to expect. I was so overwhelmed with having 2 babies let alone 2 newborns with problems. There was nothing we could do with Haylee and the doctor said that nature would take its course. Nicholle was a big challenge as a newborn; she cried a lot and was very difficult to feed. I would often look at her and just stare. What is so unusual about her? Yes, she has a large head and her forehead seems pushed out, but all babies are different and unique in their appearance. What did I do to cause her to have problems? Why her, why can't I have the problems instead. It's just not fair!!!
The neurosurgeon was very nice and asked a lot of questions. During his examination of Nicholle, I could not understand why he was looking at the spacing and width of her toes and fingers and taking measurements of her head. "Your daughter has crainosynothosis and will need surgery" he said. He went onto to say a lot more but honestly all I heard was "she will need surgery". Then he said the word "Crouzon syndrome", a word I had heard before. The doctor left the room and his assistant remained. She gave me the names and phone
numbers of 3 more doctors we needed to see (a plastic surgeon, opthalmologist and a genetics specialist) along with a script for x-rays and a ct scan. She asked me if I had ever seen anyone have a seizure before and I said no. "Why is that important?" I asked. She replied "Nicholle has a lot of pressure inside of her skull and she may have seizures before the operation." Within a 2 ½ week time period I went from having a normal child to one with crainosynothis possibly caused by a condition called Crouzon syndrome, to seeing 4 specialists (a craniofacial team) to receiving instructions on what to do if my child has a seizure. When we got to the parking garage, I took Nicholle out of her carrier and just held her. Why Nicholle? Why my family? What did I ever do to let you down God? Please God punish me, but please don't punish my child.
The next 4 ½ months flew by with multiple doctor's appointments, scans and x-rays. Nicholle was scheduled for surgery on September 29, 1998. I felt like there was nothing I could do for Nicholle before her surgery. But we found out that she would need a blood transfusion during the surgery and I contacted the local Red Cross and myself and other family members went to donate blood to be used for Nicholle during the surgery. Any unused blood was then given to the blood bank for others to use. It may sound insignificant but it made me feel like I was doing something instead of sitting around waiting for her surgery date.
The day before surgery, I took the day off from work and remained at home preparing our luggage and waiting for the hospital to call with instructions. The hospital called and gave us the surgery instructions. We were not allowed to feed Nicholle after midnight. "How am I supposed to do that?" I asked the nurse. "She is used to eating every 5-6 hours." "Don't worry, Nicholle's surgery is scheduled 2nd and the first surgery is a short one." replied the nurse.
We arrived at the hospital around 5:30 a.m. and began going through the registration process. During each step or phase of the registration process, we were moved from room to room. In each room were toys for the kids, video games and TV's to occupy the children. Of course at 6 months of age, she wasn't to interested and very hungry because the 1st surgery took more time then expected. Nicholle was crying horribly because she was hungry and I could not feed her. I wanted to make her stop crying, but I couldn't give her what she wanted and it was very difficult to deal with. The last step or phrase before her surgery was a room outside the operating room. In this room only 1 or 2 families were permitted at a time and the neurosurgeon, plastic surgeon and other necessary medical professionals come out to speak with us. There were rocking chairs for the moms to hold their kids and rock them. Nicholle was given a sedative and I was rocking her to help settle her because she was so hungry. When they called her name, the surgeon was waiting outside the operating room and I literally handed her over at the door. Of course by this time, which was
approximately 3 hours later than originally scheduled, I was crying and emotionally drained because Nicholle was crying so much. The nurse came over and said it was time to take us to the waiting room. We started to head down to the original registration area and the nurse said no there is a special waiting room for in depth surgeries. Her statement scared me to death, but our waiting room was on the same floor as the operating rooms. In this room I met other parents whose children were having organ transplants. This is when I realized the seriousness of her surgery. There was a nurse liaison that came in immediately and introduced herself and her only job was to check with the surgeons every hour and report to us with an update. She just didn't speak with the surgeons over a microphone, she actually dressed in scrubs, went in the operating room, saw Nicholle, asked questions and reported back to us directly. If we had any questions, she made sure they got asked and answered for us. Nicholle was in surgery for 5 1/2 hours.
After Nicholle was done, the nurse liaison took us to the ICU unit and we were told to wait for Nicholle's arrival. No one warned us about the swelling. I remember the ICU doors opening and a small child was wheeled by and taken to another station. I remember thinking that child had been through something horrible. There was some commotion and that same child was wheeled back to where we were sitting. I honestly did not recognize my own child. Nicholle had been within 2 feet of me and I did not recognize her, I just cried. Once I got things together emotionally, the nurse explained about the swelling. There was a rocking chair beside the bed/crib and the nurse suggested that I just hold her so that when she woke up, I would be the first thing she saw. As I held her for the next 45 minutes, I was able to remove some of the dried blood from her tiny face and watch as little patches of blood developed on her bandaged head. Nicholle's eyes fluttered open and she looked so scared. You could tell she was trying to focus and finally you could see the instant recognition in her eyes.
At this time, the nurse sedated her a lot and we decided to leave for a while at the urging of the nurses. We had already booked a room at a motel approximately 2 blocks away and were encouraged to leave by the nurses. As they put it, we needed our rest because the next day Nicholle would be more alert and would need us. We gave the nurses the name and room number of the motel we were staying at and left around 9:00 p.m. Around 10:00 p.m. the phone rang at the motel and the nurses were just giving us a courtesy call to let us know she was resting and told us to call anytime we wanted. With that wonderful piece of information, we tried to sleep as much as possible, which was very little. Finally about 4:00 a.m. we left the motel and went back to the hospital and stayed by her bed in the ICU unit.
Nicholle was moved into a regular room approximately 24 hours after her surgery ended and things got better. We were in the hospital for 4 days. My husband returned to work and I stayed with Nicholle non-stop.
Nicholle left the hospital with a pink helmet. It looked sort of like a bike helmet, but she wore it for 8 weeks, non stop. This prevented any injury to her head and prevented her head from becoming flat in some areas. She had 6 plates and 12 wires placed in her forehead. Her hair was shaved in a straight line across the top of her skull from ear to ear. During that 8 week period, we hardly left the house with her for fear that she would become ill and I did not want to deal with the inevitable stares and questions from strangers.
Nicholle needed a follow-up surgery 6 months after her original surgery because she broke one of the wires in her skull when she was trying to walk. We have spent the last 4 years making many trips to Children's Hospital. It is unknown if Nicholle will need additional surgery but her plastic surgeon has mentioned that a mid-face advancement may be in her future. Nicholle has turned into a beautiful little girl and to look at her, you would have difficulty realizing she has gone though a lot. In fact, you can barely notice her incision and must actually look for it.
Nicholle has been recently diagnosed with bilateral hearing loss. The amount of loss is minimal at this time and hearing loss was never anything that I associated with Crouzon syndrome, but have since found out is common in children with Crouzon syndrome. Her older brother Kordell was diagnosed 1 year ago with moderate bilateral hearing loss and it was discovered that he also has Crouzon syndrome, but is affected differently than Nicholle.
We are very fortunate that Nicholle's illness was caught early and preventive measures could be taken. It has been a tough journey with many ups and downs, but I would never trade Nicholle for the world. She is a very sensitive, loving, caring and devoted child and I cherish everyday I have with her and my other children.
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This cleftAdvocate page was last updated March 25, 2014
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