Your Curse is My Miracle: June 2013

Early morning, August 26, 2009 I was awakened and knew it was time to go

to the hospital. We made arrangements to get the girls off to school and

we packed and headed off to St. Vincents Hospital to meet our son, Noah.

He was originally due September 22 but I knew he was at a decent weight because I had been to the doctor the previous day. I was a little worried, but not overly concerned.

Noah arrived in the world the morning of August 26, 2009. He did not make one

sound whatsoever when he was delivered, which worried me. I continually

asked the doctor if he was ok and she assured me that he was fine. There

was a small nagging doubt that kept telling me something was wrong. After awhile, Chris left to check the girls out of school and I settled in to hold

Noah and rest. The nurse brought him to me fresh from his bath. As I

was holding him, I noticed a small blue tinge around his lips. The

nurse came to check on us and I showed her. She immediately took him

and checked his vitals, reporting that his oxygen was a little low. She

gave him some o2 and worked with him for awhile. After about 15

minutes, she took him to the nurse’s station so she could keep an eye on

him. It wasn't long before she returned (without Noah) and informed me

that he had to go to the NICU because his oxygen kept dropping and there

was no way for him to be monitored in my room. She asked if the girls

were close to coming and stated that if they were 5 minutes out they

would wait for them, otherwise he was going on to the NICU. In a panic,

I called Chris to find out that they were in the parking deck. So the

nurses wheeled Noah in the room in an incubator. I held him while the

girls talked to him, touched him and kissed him. They only let them see

him for 5 minutes then he was wheeled to the NICU. It all happened so

fast, we had no time to process it. The girls kept asking where he went

and why couldn't they see him. I was extremely upset and it was so hard

to keep composed in front of them.

There were no first family pictures, no pictures of Noah with his grandparents, nothing but tears and fear. This was the year of the swine flu and the NICU did not allow anyone except parents to visit. Because of this, nobody except Chris and me got to see Noah until he was 4 months old.

We were first told that he would be able to go home with me. But the day came and they said, he would stay another week so I packed up and walked out of the hospital without my baby. It was the hardest thing I have ever had to do. We visited daily. It was extremely difficult having a child in the NICU and 2 children at home. After the girls left for school, I would drive

to the hospital and stay with Noah until it was time to leave to meet

the girls at the bus stop. We would do the regular after school things

of homework and dinner. When the girls had gone to bed, Chris would go

to the hospital to see Noah. On the weekends, we would get a sitter and

stay with him a few hours on Saturday and Sunday.

Noah had many different tests (MRIs, Blood Tests, Genetic Tests, etc). Everything came back normal. He would desat down to the 80s and then before the nurses could even come check on him, he was back up to the upper 90s. This was constant. He would do this all day and all night and they couldn't figure out why. He was eating fairly well. He was fine other than the many desats.

Then, a week after he was born he hit rock bottom. Chris had

gone to see him that evening and he called me when he got to the

hospital. His voice was shaking and he said they had closed the NICU.

I asked if it was Noah and he said he didn't know because they weren't

letting anyone back there. I hung up the phone with him promising to

call me soon. I immediately knew it was Noah. I just had this horrible

feeling and a deep blackness overcame me. I began crying hysterically,

trying to keep quiet so as not to frighten the girls. Not too much

later, Chris called me and I have never heard his voice sound so sad and

scared. He informed me that Noah had a seizure and they had to intubate

him. We were terrified. They said his CO2 was too high and they were

giving him phenobarbital to prevent more seizures (in retrospect this

was not good because it was depressing his breathing even more but at

the time we didn't know his diagnosis). The next day, they asked

permission to give him a blood transfusion. They had taken so much

blood from him for tests that he needed more. Seeing him laying there

with a tube down his throat, tape all over his face, iv in his head,

wires all over and blood on the bed was the most terrifying thing I had

ever seen. The depression hit full force and I feared for his life.

This was our life for another week. The doctors would come talk to us

in hushed tones. The nurses would give us sympathetic looks and pat our

backs. We watched many babies come and go from the NICU but there we

sat with no end in site. I began to dread going to the hospital but I

felt so guilty when I wasn't there. It was killing me to see him like

that. He was never awake and we did not get to hold him. They stopped

feeding him by mouth and gave him an IV. The lowest day was when the

doctor told us they could not care for him anymore. She informed us

that he was being transferred to our local children's hospital, which in

retrospect was the best thing that could have happened. With deep pity

in her eyes, she informed us that they suspected that Noah had Ondine's

Curse or Congenital Central Hypoventilation Syndrome, a very rare disease. She said that he would be on a ventilator the rest of his life and would most

likely have to be institutionalized. She said that she had only seen

this extremely rare disease in a child one other time. She handed us a

short 5 paragraph medical paper and told us she would let us "process the information" and then she walked off. We were devastated. How is a parent suppose to process that information? They gave us no hope, no information and it seemed as if they had just washed their hands of him.

Shortly thereafter, Noah was transported by ambulance to children's hospital. I did not get to go because I was at the doctor with Hannah, who had the flu. Chris made sure he got settled in and took some pictures for me. Unfortunately, I got the flu also and could not visit Noah for 2 weeks. It was truly the worst nightmare.

Once I finally got to visit, the whole atmosphere was different. The nurses were encouraging. Noah was awake and the nurse asked if we wanted to hold him. In shock, we asked if we really could and were told that they encouraged it. I finally got to hold him after 2 weeks. The pulmonologist did a sleep study, which Noah failed miserably.

I remember the day we finally got a diagnosis. We were visiting Noah together (Chris was on his lunch break) and all of the doctors and nurses gathered around Noah's bed. I immediately became nervous. The pulmonologist informed us that the test had came back positive for Congenital Central Hypoventilation Syndrome (CCHS). I immediately broke down. The doctor kept talking but for a minute there all I heard was a roaring in my ears. The fear was overwhelming. I had visions of having to send him out of state to some institution and never being able to see or care for him myself. I slowly began listening to the doctor. He told us that the diagnosis was scary but that Noah would be up in no time running around like every other child. He told us that he would be able to go to school, play ball and do everything else (except maybe scuba dive). We were told that we would have to have extensive training before bringing him home and it was still going to be awhile before he was released from the hospital, months not days. I was horrified and elated at the same time. We went from being told that he

would live in an institution to being told that he would not only be going home but that he would live life like any other child. It was an extraordinary amount of information to process. We went home and got on the computer, which was a mistake. There wasn't a lot of information out there and we really felt so alone and scared.

That evening, we got a call telling us that they wanted to do trach surgery the next day. We consented and went to the hospital after getting the girls off to school. The surgery did not last long and we were able to see him soon after. That first glimpse of him was amazing. For the first time since he had been born, we saw his little face. It was a little red from the tape, but he was sleeping so peacefully. The trach looked scary but he looked so

much more comfortable. The thing that scared us was that his breath

rate on the vent was up to 55 bpm and he was not taking any breaths on

his own. The ventilator was taking every breath for him. They informed

us this was because of the sedation.

The next day, he was awake and alert. His breath rate was down. After about a week, they told us that he would be moved to a floor as soon as they had a bed for him. We were so excited. This was one more step closer to coming home and the girls could finally come visit him. The day finally arrived and he went to the pulmonary floor. What an amazing day. The girls got to hold and snuggle with him and we finally got some pictures of them together. He

was 2 1/2 months old. We still could not stay there with him because the room he was in had no bed for us but at least we could let the girls visit whenever we wanted and stay as long as we wanted.

The nurses slowly began showing us how to suction and we began asking a lot of questions. The home ventilator team met with us and explained exactly what would happen. We decided to allow him to have a g-tube so that we would get to take him home quicker. He was eating, but not enough to sustain him. They felt that this was because he had been IV fed for so long.

After the g-tube and trach site had healed some, we would begin our training and train for 3 days a week for a few hours. Our training included doing

everything 3 times each (bathing, cleaning the trach, changing the

trach, suctioning, changing the circuits, doing a bolus feed, etc). We

decided that I would train while Chris worked, since he was our only

source of income. My mother and mother in law agreed to be trained as

well.

We began our training on my birthday, which was the best present

ever. For the next month, we learned as much as we could. We had an

excellent home vent nurse who made sure were were thoroughly trained.

We learned in one month what it took professionals years to learn. We

took lots of notes and knew that we were not eligible for nursing care

at home so we paid close attention. We learned quickly and they told

us they would try their best to have us home before Christmas.

December 15, 2009 was Noah's homecoming. It was a day we never thought we would see. The homecoming was a little crazy because Hannah had a stomach

virus and the DME had to meet us ASAP because Beth had her first band

concert. My mother in law stayed with Noah while we went to the

concert. The first night home, I didn't sleep at all. Our dining room

looked like a mini ICU. We had him hooked to the vent, pulse ox, apnea

monitor, and feeding pump. He looked like a little science experiment

laying there but we didn't care. He was finally home.

Slowly, we began to get in to a routine. I was able to wean him off the vent. I realized that I could do this. Then, he got sick. Never had he had

oxygen in the hospital but now he needed oxygen. I was so extremely

stressed for the week he was sick. He was ventilated 24/7 and I was

constantly suctioning and dealing with alarms. Christmas day, he slept

a lot. We really didn't care because we were all together. Eventually,

he got better and our routine returned to normal.

3 months after coming home, we took our first trip to the beach. It was the most amazing trip we have ever had. He loved it and we were elated to

realize that we could do this and live a relatively normal life. The

darkness was over.

Noah is now a happy, healthy 3 year old. He is off the vent during the

day and we trach his cap. His speech has improved greatly after 3 years of speech therapy and he has proven to be so smart with a great sense of humor. He is our miracle and our blessing. We have learned so much from him. I never tire of hearing him run around the house aggravating his sisters and being a real boy. I never thought we would get this far. We do not know what the future holds. Hopefully we will be able to have him decannulated and just use a bipap. We may even do the diaphragm pacer surgery. Attending school is our next big hurdle.

Life is never boring with our little miracle man. We are always doing research on CCHS, constantly having struggles with the equipment company, insurance, and doctors. Slowly, we have learned how to live with a special needs child. It has been rough at times but worth every minute.