We were called. We were called/invited for Nicholas to participate into the phase I gene therapy trial in NYC but were given a two week notice. When Chris told me the news my stomach burned and I filled with anxiety.  This could be Nicholas’s only chance to live.  This is what we were pushing Nicholas to work so hard to get into. It was a scramble at our house to make arrangements for this three week journey. We packed and Chris, Nicholas, Shane (our service dog) and I were out the door on Wednesday August 18, 2010. I had so many thoughts running through my head as we pulled out of the driveway. Positive and negative it was hard to think clearly but I did hold Nicholas little hand. He asked me “where are we going mom?” I told him “we were going into the city, to get you better”. Nicholas responds “are we going to the place with the balls?” Which he remembers Children’s hospital, Boston has a big ball display in the lobby. Nicholas knows he is going into the hospital and then says “mom…I am scared” I respond “We will be together at all times Nicholas… you are always safe with me or your dad by your side.” He just responded “ok”

Here is a link to the clinical trial that Nicholas was entered into: Gene Therapy

Our journey begins:

Day 1: (Thursday) We stayed over night in CT the night before we were due to arrive in NYC. I know I did not sleep. On April 19. We were due to report at the front lobby of the NY Presbyterian Hospital in NYC.  We were met promptly at 1PM and we were brought to admissions, then to the pediatric wing of the hospital. We were first met by one of the scientist who reviewed in detail the study and our rights. Followed by a exam by a neurologist. They asked Nicholas to walk. Which he did with assistance, drink with a straw, feed self, follow commands and a neurological examination. This was all videoed so the exam could also be reviewed and scored by two other neurologist subjectively. Chris always stays quite calm but I was sweating bullets. We would not officially find out if we got into the study until the following day.

We found out that we did get admission into the Ronald McDonald House in NYC. But because we brought our service dog we were not able to stay at this facility due to the immune compromised children. We then made arrangements to stay at the hemsley tower hotel which is a hotel next to the hospital.  I planned to stay with Nicholas in the hospital. I just could not leave him for a minute.

Once we were settled into the hospital room I took Shane out to do his business outside.  I found a grassy area in front of the hospital, a perfect spot for a country dog.  As I was cleaning up his poops a hospital security guard quickly came over and yelled “hey- get that dog out of here! He can not be doing that here!” I said he is a service dog and I am cleaning it up. He said you have to take him to central park (10 blocks away). I just said ok. I went to walk back into the hospital and again another hospital security guard stopped me on the way and said “you can not bring that dog in here!”. I reported we were already admitted to the pedi floor with the dog and it was already okayed. He reported he was not told about this situation. “No dogs allowed!” He called the charge nurse on Nicholas floor and she told the security officer we were already “okayed”. I finally got back to Nicholas’s room and my anxiety over everything got the best of me and I just cried. Chris just looked a me and said “seriously your crying about the dog!” Later the next day the head of the hospital security came to our room and appoligized about the dog situation. It was completly unexpected as we certaintly did not bring him to make any trouble or waves. I felt after this situation we were give extra special treatment. We brought Shane to help Nicholas cope and feel secure as well as detect his seizures and help him walk.

Day 2: (Friday) We found out we were excepted into the study. Nicholas passed with an acceptable score. We were relieved by the news, but I think I was just so numb from an endless anxiety.  Nicholas had a 24 hr EEG scheduled for this day. The leads were placed and we remained in the hospital room together playing with toys, watching movies and reading books. The time seemed to go by quickly. Shane stayed on Nicholas bed at all times comforting him.

Day 3: (Saturday)The EGG leads were taken off and Nicholas had no seizures to report. It was the weekend now but we were still not able to leave the hospital or off the pediatric floor. We had family come and visit. For the weekend we did a lot of walking, playing with toys we brought or borrowed from the children’s room, movies and books.

Day 4 (Sunday): Nothing scheduled on this day except to remain in the hospital.  Nicholas did have a small 20 second seizure around 9AM. An Iv was quickly started and Nicholas ended up relaxing the rest of the day after the seizure.  The study team reviewed again if we were still eligible for the study. It was agreed that Nicholas had overall good seizure control. So we still had a green light to go through with the study. Nicholas only ate fair on this day due to having a seizure.  He was NPO after midnight for a MRI scheduled for the next day. 

Day 4 (Monday) Nicholas was scheduled for this MRI at 8AM on this day. He was sedated for this procedure which was supposed to be around 1 1/2 hours but with about 5 more minutes left in the MRI Nicholas started to twitch. This is something that Nicholas does once in a while when sleeping. The anesthesiologist ended the MRI early in fear it was a seizure. But when it was described to me I was able to display to the doctors what Nicholas twitching and seizures look like. It was determined it was only twitches. Last minute the study team decided  to keep Nicholas sedated to complete the eye exam part of the study. So Nicholas was sedated for about 3 hours this day. It took him most of the day to wake up. He was not able to wake up enough to eat a full meal and I was only able to get a small container of apple sauce into him. 

Day 5 (Tuesday) I was woke at 1AM by the neurosurgeon fellow who told me that Nicholas was going to have to get a CT scan of his head before 7:30AM.  The reason is because his MRI was ended early the day prior due to the twitches.  Information needed was missed and could be seen on a CT scan.   The fellow placed markers in spots mostly on Nicholas fore head area and traced the markers with purple ink. These markers would show up in the CT scan. Blood was also drawn at 1AM by a resident. We were woke again at 4AM that the CT department was ready for us to come down. Luckily Nicholas slept through most of this ordeal and Shane stayed right at the end of Nicholas bed. 

The morning came quickly and a volunteer transported us down to the operating department. Chris gowned up and went into the room with Nicholas to be sedated. I gave Nicholas a hug and a kiss and stayed outside the operating room (OR) with Shane. I just said a prayer and tears poured out of my eyes as I waited for Chris to come back.  Shane was comforting for me this time. 

The surgery was about 5 hours.

The study-coordinators and one of the scientist came and got us in the waiting room. Reporting that everything went extremely smooth for Nicholas. We could go into the PICU to see him. We went to the PICU but were told that Shane was not allowed so we put him into his create in the hotel. Chris and I went back to the PICU and I only remember that there were a lot of people (doctors, study people, fellow, resident, intern, students) in our room. I looked at Nicholas and bursted out into tears. I am a mom and to see Nicholas after surgery was scary to me. The top of Nicholas’s head was only shaved on the top. He had three one and a half inch incisions on the left and right top sides of his head with about 8-10 stitches in each. They were slightly bloody but they looked clean and neat. He did have a roughly 2 inch hematoma on the left side, last incision of his head. Chris assured me medically he looked great his oxygen and heart rate were at a good range. He said he did not appear to be in any distress or pain.  I did have a very difficult time lookng at Nicholas like this and shed a lot of tears.

Even though medically Nicholas did not look like he was in pain I was surprised he was not on any pain medications. We discussed this with the nurse and fellow and decided that he should be given one dose of morphine. Chris did not think this was a good idea. A nasogastric tube was placed for suction of secretions after surgery. There was only room for one parent to stay in the ICU. Chris and I decided that it would be best if he stayed the night. If anything was to happen to Nicholas I wanted Chris to be there  to make any medical decisions. I stayed in the hotel with the dog and did not sleep a wink! 

Nicholas ended up vomiting all night. Chris and Nicholas’ nurse suctioned him after each episode to prevent any aspiration which could lead to infection. Chris had the nurse call in the nerosurgery team to reassess Nicholas due to the vomiting. He was afraid that the vomiting may be due to swelling of his brain due to the surgery. Nicholas was re-evaluated and they determined it was from the morphine. He was given zofran to settle his stomach. Finally the vomiting subsided by morning when I arrived. It had been a rough night for Nicholas. Both he and his dad look exhausted. 

Day 6 (Wednesday) Nicholas was scheduled for another MRI this morning under sedation. It was postponed until the afternoon because of all the vomiting the night before. The team wanted to make sure his vomiting had enough time to stop. He did have the MRI completed in the afternoon. Nicholas slept the rest of the day due to all the sedation’s in the past three days. His body was wiped out! 

Day 7 (Thursday) Nicholas woke at 6AM and listened to his movies. He was able to talk a little and respond with one word sentences to questions.  He took a small nap  in the afternoon. A tube feeding was started to get his stomach churning and meds were being given to the NG tube. The tube feeding was stared at 30cc/hr and Nicholas vomited it up immediately. The rate was started too fast and that is the reason it was not tolerated. Chris insisted that it only be re-started at 10cc/hr and continued over night if tolerated.  We planned for the TF to only be used to get his stomach churning  and ready for food.

A good jump start hopefully to getting home.

Day 8 (Friday)I slept in the ICU with Nicholas on Thursday night. He had a good night sleep despite all the beeping and poking he had from the hospital staff that night. In the morning he woke around 6AM and watched snow white. His color looked good and he seemed to be in good spirits. We read books and listened to many movies during the day. Nicholas was sitting up, talking 1-3 word sentences but tired easily. IV and NG Tube were both turned off. Meds were given by mouth. Staff wanted Nicholas to eat by mouth but apple sauce and jello did not sound appetizing to Nicholas.  In the mean time Nicholas decided to take out his own IV himself. And Chris took his NG tube out as well… these boys were ready to bolt! Nicholas wanted PIZZA! Chris ran out and got him a slice of pizza. Nicholas ate the whole slice except for the crust. Moments later a smiley Nicholas got out of bed and with assistance he walked wobbly down the hall with Chris. We were thrilled. The hospital staff decided there was no reason to keep us any longer. We were discharged at 8PM to the hotel next to the hospital. I think this was the best night sleep the three of us had in a week.

Day 9 (Saturday) I was able to carefully give Nicholas a bath in the morning. Being very careful not to get his head wet but just to wash his stinky body. It made him feel SO good. He ate a full breakfast of eggs, bacon and sausage. We rested most of the day at the hotel and later went out for lunch. Nicholas was in good spirits. Talking in 1-3 word sentences, walking with assistance only in the hotel room and we did not push his physical activity. We went to the park close to the hotel, Nicholas stayed in his buggy and listened to one of his shows and he children playing in the park. Nicholas and Chris both got hair cuts at the barber.  

Day 10 (Sunday) Nicholas remained no change on this day. We had family come visit us from Connecticut and we all went out to a local restaurant. Nicholas enjoyed being surrounded by familiar voices. He spoke in 1-3 word sentences, played with cousins for about 30 minutes in our hotel room, walked with full assistance 20 feet a few times on this day. Again Nicholas fatigued easily and listened to many movies and books through out the day. 

Day 11 (Monday) The weather in NYC was SO hot and humid but we had to get out of the small hotel room because we were all going stir crazy! Late in the afternoon we decided to try to find Bloomingdales because that would be the closest shopping like a “mall” and inside with cool air. On the way we found Dylan’s Candy shoppe (which is owned by designer Ralph Lauren’s daughter ) which is a candy heaven of two floors of candy and the top floor a ice cream parlor. Nicholas ate a home-made s more dipped in chocolate from the chocolate fountain. He is not a sweets guy but I told him remember eating smores at the camp in VT…and he wanted one.  Again walking around the very loud streets of NY with Nicholas in his buggy was too much on his ears and he looked like he “checked out” due to the sensory overload. We got back to the hotel and he was back to himself wanting to play with his toys and listen to books and movies. 

Day 12 (Tuesday) Today Nicholas had a evaluation and blood drawn by the study doctor and study co-ordinators. Nicholas remained the same in the past few days. Talking in 1-3 sentences, walking with full assistance about 10-15 feet, eating fair/small quanity amount of food. Tires easily. We did take Nicholas into the theater district of NYC to see the Broadway musical Mary Poppins. This is a movie that Nicholas knows every song and  line to the movie, he was so excited. He is all smiles for most of the show but he does get tired at intermission and reported to me after the show “to loud”. We are still glad we brought him to listen to the music. 

Day 13 (Wednesday)Today Nicholas has had not change. We took a walk to the park down the street from the hotel. Nicholas got out of his buggy and with assistance walked through the park sprinklers a few times. He also was placed on the slide and went down. I could hear him yell “WEEEE” in excitement. Otherwise we took it easy today in the hotel room watching movies, reading books and relaxing.  Our younger son William (age 3) came into the city with his grandparents (my parents) and my sister Amy. We were all excited to see them.

Day 14 (Thursday) Today we took a taxi to the museum of Natural History. Nicholas especially enjoyed getting out of his buggy and feeling the different textures in on the walls. We visited the “snakes” exhibit and he also enjoyed listening to the different types of sounds these animals made by pushing buttons near the cages. He also liked riding through the different areas of the museum and hearing his echo from his voice yelling… which gave him some good smiles and laughs. Nicholas did walk about 20-30 feet at different parts of the museum. 

Day 15 (Friday)  Today we went to Central Park. The boys, Chris and my mom (nana) rode the carousel. We also found a amusement park called “Victoria Gardens” in central park. It had all sorts of carnival rides. Nicholas did ride on a few kiddie rides. He request to go on the roller coaster but we did not think that would be a good idea. He rode on the slowest rides.. which were the boat rode and train. He LOVED it and wanted more and more. William and Chris rode on all the the other rides and had a blast. We had lunch at Micky Mantel’s Restaurant. Nicholas ate well.

Day 16 (Saturday) 

Day 17 (Sunday) Today Chris took William to see the musical the Lion King. Nicholas, my mom and I went to the museum of Art. It was good because it was quite but today Nicholas was very quiet. He did not talk much and had difficulty getting out familiar words. He did not eat well. He seemed very fatigued so we did not stay long at the museum. We went back to the hotel and rested with movies and books. 

Day 18 (monday) We planned to visit the statue of liberty today. Nicholas again seemed very fatigued and tired. He did have a good night sleep. He continued not to eat very much at meals. We decided to just stay in the hotel room with Nicholas while he rested for the day. He did not sleep, he did communicate but seemed zoned out. No seizures.

Day 19 (Tuesday) Nicholas was admitted back to the hospital for a schedule 24 hour EEG, for his stitches to be taken out and a blood draw. Nicholas continued to be fatigued and tired, he spoke in 1-2 word sentences and often nodded his head yes and no. He slept well that night (despite being in a double room), I slept with him and I did not notice any twitching throughout the night. He did have a 1 minute seizure in the AM at 5:50AM before his 6AM dose of meds. A IV was started to give him his meds by IV and fluids to hydrate him for a better recovery from the seizure. We were afraid we were not going to be discharged due to the seizure but after being in NYC for 3 weeks they understood we needed to go home to New Hampshire. We were discharged after Nicholas ate some lunch at noon.

Sunday September 25, 2010We have now been home for about 3 weeks. Nicholas continues to fatigue easily through out the day. When we first came home he would play for 20 minute intervals throughout the day and then seem to need to relax listening to his movies or books. The amount of play time has increased but by the afternoon/evening he continues to be very tire. He is not willing to take naps which he clearly needs. The fatigueness has affected his talking. When he is active or motivated he will speak more but only 1-3 word sentences. There have been day that he hardly speaks at all but nods his head in “yes or no”. This does break my heart. As I do feel he was speaking more before surgery. I hope that with time over the next few months the fatigue will subside and Nicholas will be filled with more energy. He is not a lazy boy and wants to go go go so I know this frustrates him as well. He want to play with his toys, go outside and play on the playground but he gets so tired and just lays on the ground and cries. 

Nicholas just completed his first full week of 2nd grade. He goes to school from 8-12:30 followed by therapies after school. He is starting to eat more but by the evening he is very tired and eats less.  Nicholas continues to report his feet hurt and recently his knees. We were worried he may have bone fractures in his legs. They were x rayed and thankfully there were no fractures, but he does have some generalized aches which improve with motrin.

Today we took Nicholas apple picking. He really perked up while picking apples and had a lot of energy. See the pictures below.

Lastly thank you for family and friends support through this whole journey. We hope our journal will help the kids behind Nicholas prepare for this surgery. Time will tell if it will help him or not but we are proud that Nicholas is still fighting each day to get better. We are proud he got though the surgery with so much strength and determination. All we can do now is love him, hug him and wait. Our promise to Nicholas is to continue to fight toward a cure and we have not given up on our son and other children with batten disease.

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