fullhouse3b2g
07-22-2008, 05:49 PM
Okay, I guess it’s about time I wrote up Isaac’s story. I truly hope it can help someone.
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On August 26<SUP>th</SUP> in the wee hours of the morning, I awoke to my water breaking and painful contractions. Because of how close together my contractions were, along with a history of fast deliveries my husband called an ambulance which rushed me to the hospital. Isaac Matthew was born 10 minutes later at 35 weeks gestation (5 weeks early). Isaac was a whopping 9lbs 7oz at birth…a huge preemie! Isaac had a few minor problems at birth, but was mostly healthy. His face and head were very swollen and bruised from such a fast delivery. He looked like a sumo wrestler!!
In the hospital we noticed that Isaac made a sweet little sighing sound everytime he took a breath. It was very sweet and we commented that he was singing lullabyes to us. Over the next few weeks we noticed that the sweet sighing turned into a gasping sound. We also noticed that Isaac seemed to be struggling for each breath and would often stop breathing for several seconds while he was asleep.
We took Isaac to his pediatrician who then referred us to a pediatric pulmonologist at the children’s hospital. After testing and X-rays, and additional referrals to an ear, nose, and throat specialist; Isaac was diagnosed with laryngeomalacia, trachealmalacia, tachypnea, stridor, subcostal and intracostal retractions, obstructive apnea, and possible sleep apnea. Basically, Isaac’s airway was too floppy and collapsed on itself whenever he tried to take a breath in. He also breathed very rapidly at 80-100 breaths per minute (20-40 was the norm for his age). The doctors felt that there was a structural problem deeper in Isaac’s lungs that was causing all these breathing problems so they scheduled an exploratory scope surgery.
The day before Isaac’s scope surgery, he developed RSV and was hospitalized with severe breathing difficulties. His surgery was postponed. As soon as Isaac had recovered from the RSV (which took several weeks) he came down with croop. He went back and forth in a cycle of respiratory illnesses which he was more susceptible to due to his airway problems.
Isaac was a wonderful baby! He was full of smiles and giggles for anyone who looked at him. He had the kind of personality that made people stop and notice him. We were constantly getting comments about his wonderful personality. Isaac was always very content with just about anything. We couldn’t have asked for a better baby!
One day, my husband and I were looking at some pictures of Isaac when he was a few days old and we noticed that he had a strange line that went up his forehead. This didn’t bother us a bit, as all 4 of our older children had the same line. We joked that Isaac had joined the ranks of the “Klingon Kids” (a character from Star Trek who had weird ridges on his forehead). We never thought that it could be anything medical, until a few days later when I received a phone call from my sister in law.
I will never forget the words that were spoken that day. My sister in law in Texas (we live in Virginia) called and was upset because her doctor had told her that her son (who is 6 months older than Isaac) may have this condition called “Craniosynostosis”. I asked what his symptoms were and when she told me about this strange line that went up his forehead and showed up really well in pictures, I literally laughed at her. I told her that ALL of my kids had that line, even Isaac and she didn’t need to see a neurosurgeon for it! In hindsight I feel horrible for laughing and blowing her off like that. I sent her pictures of my kids in an email and she called me back and told me that they looked just like her son. I did a google search of the word “Craniosynostosis” and when I saw pictures of other kids with cranio I literally cried. The more I saw and read, the more I became convinced that my kids had cranio. My sister in law took my children’s pictures with her to see their neurosurgeon in Texas. He said that we needed to get them in to see a neurosurgeon here, because they all looked like they had it.
I marched into our pediatrician’s office here and told him that Isaac had Craniosynostosis and we needed a referral to see a neurosurgeon. It was my turn to be laughed at. The pediatrician looked at Isaac’s ridge on his forehead and then walked out of the office. He returned with a piece of paper with pictures of baby’s heads on it. The top of the paper was labeled in large, bold letters “Positional Plagiocephaly.” He then proceeded to show me the pictures on the page and said, “Isaac does NOT have Craniosynostosis, and if he did then his head would be grossly misshapen like these kids. Really all we would do anyways would be to change the way the baby lays, that is causing the head to be misshaped”. He refused to give me a referral for a neurosurgeon. I didn’t argue with him, because it was obvious to me that he had no idea what Craniosynostosis was.
After researching on the internet a little more, I found out that a lot of people have a hard time with their pediatricians not knowing about cranio. We also found out that we didn’t need a referral from our pediatrician to see a NS. I called a large hospital about 3 hours away from us and asked for an appointment with a NS. After a little bit of a runaround we got an appointment to see a NS. We went in totally expecting that the NS would take a look at Isaac (who we realized was not as severe looking as a lot of kids with metopic synostosis) and order a CT scan. We thought we had found someone who would give us all the answers. We were disappointed. This NS walked in and told us that Isaac had mild metopic Craniosynostosis and that he did not feel that a CT was necessary. He told us that ICP is extremely rare in metopics and the only metopics that he did surgery for were ones that were very severe shaped like a boat’s keel. He did seem slightly concerned that Isaac was having some possible vision problems, and told us that he wanted Isaac to see a neuro opthalmalogist if we didn’t see any improvement. He said he would be highly surprised if Isaac ever showed any signs of ICP, but he did give us some signs to watch for.
We left with mixed feelings. On the one hand we were glad that Isaac didn’t need this scary surgery. On the other hand, I just didn’t feel right about it. We waited a few months and watched as Isaac’s head-shape worsened a bit. Isaac’s cousin had surgery to correct his Craniosynostosis and did wonderfully! I just couldn’t let go of the feeling that the cranio was an issue for Isaac. I scheduled an appointment with a different NS for a second opinion. We took Isaac for what turned out to be one of the worst experiences we’ve ever had at a doctor’s office.
The neurosurgeon walked in and very accusingly asked, “First off, who told you that your son had Craniosynostosis?” Then he told us, “Your son does not have Craniosynostosis, and even IF he did it’s only the metopic suture and metopic isn’t even a TRUE synostosis”. The appointment went downhill from there. The NS was very derogatory towards us, accusing us of being horrible parents who “wanted” our kid to have surgery. He asked if we knew what they would do to our precious son in surgery and proceeded to tell every detail of the horrendous surgery. Several times he told us to “look at your son…he’s beautiful! Why would you want to put him through this?” When we asked about the closed soft spot, he stood up and put his thumb where Isaac’s soft spot should have been and started pressing down. He continued to press down as his knuckles turned white…pressing….and pressing…and pressing! I was sure that Isaac’s head was going to crack open right there!! Finally Isaac let out a loud shrieking scream and the NS let go, backing up with a huge smile on his face. He said, “He’s got a soft spot still. It’s palpable”. After berating us some more, the NS told us to get out of his office and he never wants to see us again. We left the office not having a clue of what to do. It seemed that the door had been slammed shut on the Craniosynostosis thing, no matter how we felt about it.
We tried to put Craniosynostosis out of our minds. Isaac was still a wonderful baby! No matter how hard I tried, I could not get past a feeling that something was wrong with Isaac. Isaac still met developmental milestones, but he was later in meeting them than his peers. I felt like I was going crazy in thinking that something was wrong with Isaac when he was such a good baby.
Isaac continued to lag behind in his development. He also continued to have severe breathing problems. In early November 2007, Isaac had a very confusing episode of illness. One day he began vomiting. He vomited over and over again throughout the whole day and night. He didn’t have a fever or any other signs of illness, just the vomiting. We took him in to his pediatrician who told us that if Isaac continued vomiting we would need to take him in to the ER for some IV fluids. Isaac stopped vomiting that afternoon and was able to hold down a little bit of pedialyte. That evening the vomiting started back up again. We took Isaac in to the ER thinking that they would give him a bag of fluids through an IV and then we’d be back on our way home. While in the waiting room Isaac became very lethargic and limp. By the time the ER doctor saw him, he was not responsive. Isaac had become so severely dehydrated that his blood sugars dropped dangerously low, in fact several of his “levels” were dangerously low. They immediately started injecting him with a “sugar gel” and started an IV with other fluids. Once they got him stabilized, they transferred him to the children’s floor and he was admitted to the hospital.
While in the hospital, Isaac’s body took a long time getting everything back to normal. They ran many tests to see if he had a virus or infection. None of those tests showed any indication of virus or infection. The doctors were stumped as to what had caused all of the vomiting. One doctor mentioned that she was suspicious of ICP from his metopic Craniosynostosis causing the vomiting. She told us that if Isaac did not stop vomiting then she wanted to order a CT scan to check for ICP. Isaac stopped vomiting and all of his blood levels returned to normal (a CT scan was not done). He was released from the hospital without any answers about what had caused the vomiting.
Isaac’s breathing issues worsened again and we spent many days seeing doctors. Being admitted to the hospital was a constant threat, and we prayed that Isaac wouldn’t have to spend Christmas in the hospital. At one point, Isaac’s medications took up a whole counter in our kitchen! Isaac began showing other strange symptoms. He started being really “clumsy”. He would walk right into the table, walls, etc. He also started falling down. He fell over sideways and backwards without having tripped on anything. He was black and blue from falling so much. He started walking weird too. He was off balance and had a really wide waddle type walk. We ended up keeping him strapped in a stroller most of the time for his own safety. He also had many problems with sleep. He would wake up screaming in pain and start slamming his head on the crib rails. When he was able to sleep he would moan and whimper in his sleep. This was torture to us as parents to sit and helplessly watch.
We talked with Isaac’s pediatrician, who felt that something was going on neurologically and referred us to a pediatric neurologist. They couldn’t get us in for two and a half months! I kept asking if this could be caused by the Craniosynostosis and if I should call a NS. They kept saying, “Just wait and see what the neurologist says.” In the meantime, we sat and watched helplessly as more symptoms popped up. Isaac began having seizures several times a day as well as tremors. The vomiting was off and on… sometimes disappearing for a few days, or even a few weeks, and then returning again. One day Isaac had another serious episode of vomiting. He vomited over 30 times in 6 hours! His development stalled and even went backwards. Isaac began choking and gagging on his foods, drinks and his own saliva. He couldn’t even eat cheerios. We were very worried and we bugged his pediatrician to let us call the first NS that Isaac had seen and just ask for his opinion.
Finally, one day a nurse was talking to Isaac and she looked at me and asked, “How long have his pupils been unevenly dilated?” After taking a closer look I could see that his left pupil was larger than his right one. When they tested it, the left pupil was delayed in its response to light. Isaac’s pediatrician then became very concerned and told us to either get him in to see the NS that very same day or to take him straight to the ER. We were worried, but finally felt like we were getting somewhere! We tried calling the NS and drove Isaac in to the ER. As we pulled into the parking lot to the ER the NS called us back and told us that it definitely sounded like something neurological and some ICP symptoms. He told us to go into the ER and demand a CT scan, if nothing showed up then demand a MRI. He said that no matter what they found he wanted the scans sent to him and he would help us figure out what was wrong with Isaac.
When we were in the ER they treated Isaac really seriously at first. They noticed on their own his uneven pupil dilation. When we told them he needed scans done, they gave us a hard time. They finally gave in and said they would do one scan, but didn’t want to do both because of the amount of sedation that they would have to use. They explained to us that sedation is really risky in kids with breathing conditions like Isaac’s. They ended up doing an MRI because they felt it would give them more information than a CT scan. Isaac did really well with the sedation for the MRI and they were able to get really clear pictures. The ER doctor came in very briefly afterwards and told us that everything looked good. We got the feeling that the doctor was overwhelmed and didn’t know what to tell us. Finally he came in and said, “I’ve got good news. There is absolutely nothing wrong with your son’s brain.” He told us that they MRI didn’t show ANY problems AT ALL, except for a small lesion on the front part of the brain that he didn’t think was affecting anything. Most importantly, is that he didn’t find anything wrong with the cerebellum, which is what he had expected with all the balance issues. He seemed rather hasty to get rid of us, and handed us a printed copy of the MRI report and told us that at least we had that report to take to our NS and sent us out the door.
We left. I was in tears. I just knew that something was majorly wrong with Isaac, but I felt like nobody was listening to me. I felt like my son was suffering and wasting away and nobody cared! In the car on the way home from the ER, I got over my initial shock that nothing was found and started reading the MRI report. I couldn’t believe my eyes! The report started listing “findings” and there were many things listed. I didn’t understand what it was trying to say, but there were words and phrases like: Chiari malformation, prominent subarachnoid space, arachnoid cyst, trigonocephaly, low lying cerebellar tonsils, hyperintensity in the left temporoparietal matter, convex ependymal surface of the lateral ventricle suggesting volume loss, etc. I am not a doctor, but looking at that report made me think that my son’s brain was not “perfectly normal”.
We faxed the report to the NS and his office called us back that same day and told us they wanted to see Isaac very soon. They didn’t tell us if they were concerned about the Craniosynostosis, or ICP or something else altogether. I tried not to get my hopes up that they would be able to tell us what was wrong, but I wanted more than anything for them to be able to help my little Isaac! Isaac was such a trooper. Watching him suffer all day and all night, day in and day out, was heartbreaking, but then he would smile and you just knew he was going to be okay!
At the appointment, while the NS reviewed the MRI films from Isaac’s ER visit, the physician’s assistant (PA) came in and talked to us. She also observed Isaac a little bit. In the brief time that she was in the room with us, Isaac fell on the floor twice and also walked straight into the examining table. With a raised eyebrow, the PA told us she could definitely see what we were concerned about. When the NS came into the room, he sat down and asked a few questions about Isaac. We answered his questions and he sat silently for a few seconds. My heart was racing in fear and expectation. Then the NS leaned forward and said, “Your son has a cyst that has formed on his brain, and he needs surgery to remove it.” He went on to tell us more about the procedure that he would use to remove the cyst from Isaac’s brain. I asked if it had anything to do with Isaac’s Craniosynostosis, and he told me, “Yes, the metopic suture being fused, as well as his small narrow head has caused the brain to rub against the skull in the back which caused the cyst to form. The cyst has now grown so large that it has pressed in on the brain and caused the brain to herniate (chiari malformation) into the opening to the spinal column and is blocking the flow of cerebro-spinal fluid to the cerebellum.” He told us that Isaac has probably never known a single day without pain. He told us again about the procedure, and told us it was a somewhat risky surgery. I asked if it was about the same as a cranio surgery and he told me that this would be riskier because it is actual brain surgery; they would be cutting into the brain itself. He told us that Isaac would need blood during the surgery, but reassured us about the safety of the blood bank there. We asked if Isaac would need more surgery in the future for the Craniosynostosis, and he told us that it was a possibility and that Isaac would be watched very carefully and we would make that decision later. We were told to go home, think and pray about the surgery over the weekend, and call him back on Monday with our decision.
It’s amazing how easy, yet how hard that decision was. Clearly, Isaac needed the surgery, but I was so scared. We called the office on Monday and told them we had decided to go ahead with the surgery. They told us they would start the insurance approval process and call us back with a date. We were shocked when they called us back the very next day and told us the surgery was scheduled for April 1<SUP>st</SUP>…10 short days away!! It was a mad dash to get everything done before hand, and make arrangements for a place for us to stay (the hospital was 3-4 hours drive from our house). In hindsight, I’m so glad that things happened so quickly, as I had less time to sit around and worry.
We became very familiar with the surgical procedure the NS had talked to us about, as we had to explain it many times to friends and family. I tried not to think about the fact that this was going to be done to Isaac, as I described the surgery to those that asked. They would make an incision from almost the top of the back of Isaac’s head, down to the bottom of his neck. Then they would remove the back part of his skull, from that they would cut off the very bottom and that would never be replaced. They would also remove a portion of the back of his vertebrae to allow the cerebro-spinal fluid more room to flow. They would cut into the dura (the “skin” that protects the brain), and then drain the cyst. Then they would collapse the walls of the cyst and remove it from his brain. Then they would make a tissue graft to patch the dura, and put the skull back into place (minus a small portion) and close up the incision.
At the hospital, on April 1<SUP>st</SUP> Isaac was great. His surgery was slightly delayed, but he did very well even though he hadn’t eaten. Some clowns came to the pre-op playroom and blew bubbles with him and played instruments and sang. Isaac joined right in with them. He charmed all the nurses there with his cute personality. They told us that one of us could walk him down the hall. My husband was very gracious and let me be the “walker”. I had almost prepared myself for the moment when I had to hand my precious son over to the doctors and trust that he would be returned to me, the same little boy minus the horrible symptoms.
What happened next took me by complete surprise…they walked me all the way back to the surgical room! They had me sit and hold Isaac (he had worn himself out playing and was asleep) in a rocking chair that was wedged in between several huge stainless steel sinks and the back wall while several doctors and nurses prepared the equipment for Isaac’s surgery in an adjoining room. Then I heard a voice among the masks call out, “Bring him in.” They told me to come in and lay Isaac on the operating table. I was SO not prepared for that. My feet felt like lead as I carried my son to the table. Pictures of the Biblical story of Abraham laying his son Isaac on the altar to be sacrificed ran through my head. At the same time all the sights, sounds, and smells of the operating room were overpowering and I felt I was going to be sick right there. Somehow, I found the courage to lay Isaac on the table. They put a mask on his face to administer the gas to put him to sleep. He woke up flailing and kicking and trying to scream, while they held him down. He looked at me for help, and I’ll never forget feeling like I had just forsaken my sweet son. He finally drifted off into his drug induced sleep. The anesthesiologist told me to pull down my mask and kiss his cheek before I left. Walking out of that room and leaving my precious son was the hardest thing I’ve ever had to do.
Once I got out into the hallway and was able to compose myself, everything became very surreal. I hadn’t thought that I would be able to eat while Isaac was in surgery, but I found myself very hungry. After checking in with the waiting room attendant, we went down to the cafeteria to get something to eat. Melissa (a friend from CK) met us there and stayed with us throughout the whole surgery. She was a wonderful support! She brought snacks and activities to help keep us entertained. As the hours passed we received messages from the operating room on their surgery progress. My mind continually went back to the day that the NS explained the procedure to us, and I would wonder which part they were on at that moment. I sent up many silent prayers; most of them were not complete thoughts, but instead, fragments of thoughts in my racing mind.
At one point during the surgery, we were walking down the halls trying to find a place more comfortable to sit and eat a snack when a doctor in a white coat walked up to us and asked if we were Isaac Lucky’s parents. My heart stood still as I answered. She then told us she needed to talk to us in private away from other people. Fear gripped me and I looked to Melissa, who also appeared unnerved, but tried to look reassuring. Melissa kept an eye on our bags as we walked down an empty hallway to speak with the doctor. Finally, the doctor explained that she had nothing to do with the surgery and she just needed to get our permission to use a new kind of baby wipe to bathe him with during his hospital stay after surgery. We signed her “permission slip”. This was NOT a good thing to do to a parent when their child is in brain surgery!
Finally, we got the word that they were closing up and that the NS would be out to talk to us in just a few minutes. When I saw the NS walk into the room, I could barely stay in my chair! He walked to us with a huge smile on his face. It instantly calmed me. He told us that the surgery had gone very well. He said he was surprised at the amount of pressure the cyst was putting on the brain. He told us he had found the brain was under a “tremendous, TREMENDOUS amount of pressure”, he went on to explain how when he had cut into the cyst and relieved the pressure from all the fluid, the brain (which had been extremely compressed) literally popped out. He told us that Isaac had definitely needed the surgery, and that he expected great things. We were later told that the cyst was much worse than what they could see on the MRI and that they had left a portion of the cyst wall in. The cyst had been pressing against the skull in the back so hard for so long, that it had worn the skull down. The skull was so thin that they didn’t even have to cut it to remove it – they just broke and chipped it out. Isaac had been suffering for a very long time.
We got a small glimpse of Isaac as they wheeled his bed from the OR to the PICU. Waiting to see him while they got him settled in the PICU was very hard. When we did see him, he was still sedated. He looked so peaceful laying there. I couldn’t believe how good he looked…he was still my same little boy. I wanted so badly to scoop him up and kiss him! I took several pictures. Melissa got to come back and see him for a few minutes before she left. The nurses explained the PICU rules to us and allowed us a few extra minutes with Isaac before we had to leave. I didn’t want to leave him, but they assured us that they would call us at the Children’s House if he had any problems. When we got out into the hallway, I was hit hard with an intense feeling of joy mixed with pride. My precious little boy had made it through brain surgery! Nothing can parallel that feeling. I was so proud of Isaac. I wanted to stop every stranger I passed and tell them about my son and how proud I was of him! Sam, my husband, told me I was glowing. I made a bunch of phone calls from our room at the Children’s House telling everyone how well Isaac had done. Everyone told me they could hear the joy and relief in my voice. I couldn’t wait to see Isaac again the next morning.
The day after Isaac’s surgery, time seemed to drag. I was rushing to get dressed and get a bag ready to take to the hospital with us. I was ready way ahead of the time that they had told us we could go in to see Isaac. We found a little café that served breakfast and ate there. When it was finally time to go see him, I was excited. I felt like a giddy school girl as we took the elevator to the 7<SUP>th</SUP> floor and down the hallways. Finally, we pressed the button and the big door into the PICU opened up. The hallway was pretty much empty, except for a large group of doctors who were walking in and out of a doorway. Then I realized that they were standing at Isaac’s door! Many thoughts raced through my mind as fear and panic overtook me. I tried to tell myself that nothing was wrong; that it was probably normal. Sam actually sped up his pace and made it into Isaac’s room well before I did. As I approached the room, a doctor blocked my path and asked me if I was Isaac’s mother. I could see other doctors and nurses talking to Sam inside the room and I longed to be able to push past the doctors and see Isaac, to make sure he was okay. My attention focused back on the doctor who had stopped me. He said that Isaac had a really rough night, and was up all night vomiting and in pain. I felt horrible for not being there for him when he needed me the most. Inside I was screaming, “Why didn’t they call me?” The doctor then told me that Isaac’s heart was doing some weird stuff and they were trying to figure out what was wrong. He said they’d be bringing a large machine in to do an ECG (an ultrasound) to look at his heart. He wouldn’t give me any clear details about what they had seen that concerned them. All we could do was wait. The doctors also told us that they had called for a “genetics consult”. They explained that whenever more than one system of the body had a defect, testing was needed to rule out the possibility of a genetic syndrome.
When I saw Isaac, he was very restless. Even when he slept, he did not look peaceful like he had the night before. They brought in some machines and a technician introduced himself to us. He told us that we could stay for the ECG, but we couldn’t ask any questions about what he was seeing. The ECG took over an hour. Isaac screamed through the whole thing. It was so very hard to sit and hold his little arms down after all he had been through. Finally it was over and the technician told us that we would hear back from a cardiologist once they had reviewed the results. Isaac finally slept for about 15 minutes…he was so exhausted.
Isaac’s nurse told us that Isaac needed to wake up and eat and drink something, and she felt that the pain meds that he was receiving through the IV’s were going to keep him too sedated to wake up. She refused to give him any more pain medicine. Isaac cycled through waking up screaming in pain and sleeping for a few minutes, throughout the day. His nurse was not successful in getting him to drink anything because every time he was awake, he was in massive amounts of pain and he just screamed. Watching him suffer in that much pain was very, very hard. When he opened his eyes they were very dull, and unfocused. We couldn’t tell if he even knew we were there. The doctors came to talk to us again. Isaac’s blood was tested hourly, and they had noticed a steady decline in his sodium level. They told us that the sodium had to be stable before he could be transferred out of the PICU to the regular floor. That evening his levels finally stabilized enough to be transferred. I was worried about how the nurses on the regular floor were going to handle the amount of pain he was having.
On the regular floor that night, Isaac struggled with the pain. He screamed and screamed. We tried to comfort him, but could not. He didn’t respond to us in any way. We didn’t even know if he knew we were there. At shift change, we got a new nurse who was immediately alarmed at how much pain Isaac was in. She went personally to find the pain management team. She came back into the room and gave him some medicine. I was in tears at this point and asked her if it was normal for Isaac to be in this much pain. She told me that in the PICU they had decreased Isaac’s pain medicines so much that he was receiving a smaller dosage than regular Tylenol…basically nothing. She said he hadn’t had good pain control since the surgery. When the pain management team found out how small of a dose he was getting not even a full day after his surgery, they were very upset. They immediately tripled the amount he was getting in the IV and added a few more pain meds as well. It was amazing how fast the medicines helped. Isaac was able to really sleep for the first time since his surgery.
The next day was a busy one! Isaac was more aware of his surroundings, and just quietly watched everyone coming and going. Whenever the nurses or doctors would come in, he would watch them until they started talking to him, then he would shut his eyes as if that would make them go away. Physical therapy came to work with him. They were concerned because he kept his head turned to the left and would not let anyone move it. The left side of his face was pretty swollen (swelling is normal after brain surgery.) His lips were swollen and cracked as well. The therapist used a moistened swab to wipe across his lips. He seemed to like it and even held one in his own hand. The geneticist came to talk with us and get some basic family medical history. He told us that he believes there is definitely a syndrome and that we needed to have some testing done when Isaac had fully recovered from this surgery. Everything seemed to be going better for Isaac… until night time.
Isaac’s IV went bad (from previous hospital stays, we knew that Isaac’s veins don’t like IV’s for very long) and he didn’t receive his pain medicine. By the time they noticed it he was already screaming, flailing, and kicking. It took a while before the IV team came to give him a new IV and the pain was already ahead of him. Even after they got the IV hooked back up to the pain pump we had a really hard time getting control of the pain for him. He was screaming and throwing his body around, he was ripping at his own hair and the bandage that covered the incision site. They had to brace his arms and legs and tie them down to the bed for his own safety. Even then he still tried to throw his body around. The nurse and I were both in tears! It was heart-wrenching to watch. I have never seen anyone in that much pain before, and it killed me to see my sweet son’s body wracked with that much pain. The pain management team doubled the amount of meds they were giving through the IV and added Valium by mouth. Isaac finally settled down a bit. He woke up in pain about once every hour and cried until someone bolstered (gave an additional boost) his pain meds.
Isaac continued to do better the next day, but still had a lot of issues with pain that night. Once again his IV went bad (for the 4<SUP>th</SUP> time), and we got behind on the pain meds. More thrashing and screaming and ripping at his hair and bandage. He had to be braced and tied down again. Sam and I helplessly endured many more heart-wrenching hours. Isaac finally got to sleep around 2am. Words can’t even begin to express how it feels to see your child like this.
At some point the next day, Isaac “turned the corner”. His blank expression with pain dulled eyes was replaced with a more alert and interactive look. He started sitting up and wanting to play with toys. He actually started eating a few bites of food. He wanted to be held a lot, which was very difficult due to all the wires on him and the stiffness he had from pain. The pain management team made a plan ahead of time to keep him from having the same kind of night as the last few had been. From there on out, he did better and better.
The last day that Isaac was in the hospital, I noticed an area of his incision site that stuck out a bit farther than the rest. I asked the doctors about it and they told me it was normal swelling. I noticed that when Isaac cried the area grew and started bulging out a bit, but they reassured me that it was normal. We got home in the middle of the night. Isaac seemed glad to be able to sleep in his own bed, but still had a lot of pain. The next morning Isaac vomited. He was very stiff still and would turn his whole body instead of turning his head. The next few days were very tough. Isaac didn’t play, he was very fussy and couldn’t sleep very well. As a mom, I could only take so much and began to really wonder if we had done the right thing for Isaac in having the surgery. I felt like I had lost my little boy, and Isaac would never be happy again. We called the NS to ask if Isaac should still be having this hard of a time. The NS ordered a higher dose of pain medicine for him. That night Isaac was able to sleep a little bit better. The next day he was more playful and even gave us a glimpse of his mischievous grin! He tired very easily, but it was good to see him play again.
We started getting a little concerned about the swollen area of his incision site. Each day it seemed a slight bit larger. One day, when we woke up and saw the back of Isaac’s head, I gasped. The swollen area had doubled overnight and Isaac looked like he had a golfball sticking out of the back of his head! I sent Sam pictures of it at work and tried to contact the NS again. When the NS called back they said they needed to see Isaac in the E.R. there. Even though it’s a 3-4 hour drive, they felt that it was too complex for our local hospital’s ER. We found babysitters for our other children, threw some clothes in an overnight bag, and headed for the hospital.
At the ER, when they found out that he had just had brain surgery and saw the big bump sticking out of the back of his head, they took him straight back to the examining room. After three failed attempts at getting a CT scan done, they finally got it on the 4<SUP>th </SUP>time. The doctors determined that the dura graft (the skin like tissue surrounding the brain) had a hole in it and was leaking cerebro-spinal fluid (CSF). The CSF was pooling right behind the skin at the incision site and that’s what had created the bulge. They admitted Isaac to the hospital again. All of the nurses recognized Isaac and were glad to see him a bit more playful than the last time.
They were all impressed at the big bulge sticking out of his head. Each of them wanted to feel it. It was very gross. It was squishy and soft like a water balloon. His incision seemed to be holding well, but was definitely stretched out. They kept Isaac there a few days for observation. Isaac still had a lot of pain and was having a really rough time sleeping as well. On the last night there, a wonderful nurse saw how utterly exhausted I was, and when Isaac woke up screaming she came to his crib-side to care for him and told me to go back to sleep. Off and on throughout the night this wonderful nurse stayed at Isaac’s crib and tried to calm and care for him for hours at a time. It was so nice to have that break. When Isaac screamed and cried it wasn’t just a matter of calming him… he threw his body and tried to slam his head and rip out his IV’s. Calming Isaac was also a matter of keeping him from hurting himself. It really wears you out! I made sure I told that nurse’s supervisor how wonderful she was.
After a few days of observation we were sent home to continue watching the bulge. The NS said that the leak should heal on its own in a few weeks and the excess pool of CSF would be reabsorbed. If it didn’t heal on its own in a few weeks then it would have to be surgically fixed.
While at the hospital we were able to find out a little bit more about Isaac’s heart defect. The results of his ECG during his previous hospital came back showing an Aortic Root Aneurysm. Isaac’s aortic root (part of the main blood vessel to the heart which contains the aortic valve) is enlarged. He will have to be watched very closely and have regular ECG’s to monitor the amount of dilation. If the aortic root grows too large, it could cause the heart to rupture (this typically causes immediate death). At this time, Isaac’s aortic root is only mildly enlarged, but if it continues to enlarge to the point where the doctors think he is in danger of his heart rupturing then he will need major heart surgery to replace the aorta and the aortic valve. The doctors made it very clear to us that Isaac needs to be followed very closely by a cardiologist.
At home, the bulge went away completely within a few weeks. Isaac did well, and we saw a little bit more of his personality returning day by day. Therapists came to our house to work with Isaac to help him with the developmental delays that had been caused by the pressure on his brain. Isaac’s incision site looked really good, except for one spot that started looking infected. We were sent back to the ER to have it checked out.
Isaac did not enjoy seeing the inside of a hospital again! He was so upset that the doctors and nurses felt sorry for him and let him play in the toy room. We have been to this ER many times yet I never knew that the ER had a toy room in it. After a long day in the ER they determined that Isaac had a localized infection and didn’t need to be hospitalized for it. They told us that Isaac’s heart defect causes them to be extra careful anytime he has an infection in his body, because infection could travel to the already weakened heart valve and that would be very bad. They gave us instructions on how to care for him and what to watch for and sent us home again.
At home Isaac continued with his therapy and we started seeing a huge difference in him. All of his horrible symptoms from before the surgery had disappeared and he was able to sleep well, for the first time in his life. We saw great strides in his development and he started speaking real words. We noticed that he had the cutest personality. He started being mischievous and teasing us; it was a complete turnaround! We went back up to Johns Hopkins for his post-op check and his NS was very pleased with his progress. We were told that Isaac would need to come back for regular checks every 6 months, but that he was doing great.
We decided that since Isaac was doing so well, we should take a break from all things medical and go camping for Memorial Day. The kid’s were thrilled, and we headed up to a friend’s house in the Shenandoah Mountains. We camped in their front yard, and roasted hot dogs and marshmallows on the fire. Isaac had a blast! As I watched him running and playing with the other kids, I marveled at his strength. Who would’ve ever thought that he was the same kid that had just 2 short months before spent his days and nights suffering through so many medical problems?
Sometime that evening, we noticed that a mosquito had bitten Isaac’s forehead. The reaction seemed to be a slight bit worse than most people have, but nothing to be concerned about. When we woke up the next day, Isaac’s whole forehead was swollen and bright red and it was traveling down the left side of his face. I gave him some Benedryl for the allergic reaction and some Motrin to help with the swelling. A few hours later, Isaac took a nap and when he woke up we knew we needed to take him to the ER. His whole face was swollen and the left side was purple. His left eye was almost completely swollen shut! (He looked worse at that point than he had right after his brain surgery!) The local ER there treated Isaac for what was deemed to be a severe allergic reaction to a mosquito bite. They also prescribed a round of antibiotics as a precaution against infection due to Isaac’s heart defect. So much for our break away from the medical world!
We took Isaac for a follow-up appointment with his pediatrician. The pediatrician was concerned about the severity of Isaac’s reaction to the mosquito bite and what would happen when he got bit in the future. We were sent to see an allergist. The allergist told me that to have a severe reaction to a mosquito is very rare, but it can happen. Isaac just likes to be unique, I guess! The allergist was also concerned about the possibility of an anaphylactic reaction (severe reaction that causes the victim to stop breathing) in the event of another mosquito bite. Isaac was prescribed an epi-pen for an anaphylactic reaction and also a prescription for a steroid to keep the swelling down. Isaac will now need to be followed by the allergist for regular checks.
The genetics clinic called, and after reviewing the information from Isaac’s medical records they feel that our family carries a genetic syndrome. They set an appointment for our whole family to go in together. All the children (including Isaac) will have ECG’s done to check for heart defects and then they will go over the results and order any additional testing to help them figure out which syndrome we have. That will be one very long day for us, but it will be a day of hope. Hope that we will finally know what has caused so much pain and hardship for little Isaac (and our other children).
Isaac was doing really well and we were almost 3 months out from his surgery, when he began having a few setbacks. His head-shape began changing (even his therapist noticed it) and his lower jaw seems to be recessing. He has started showing some of the same symptoms that he had before the surgery (vomiting, seizures, head-banging, sleep disturbances, gagging, fussiness, etc.). It was extremely hard to see him go through all of that the first time, but to see him like this again is unbearable. Isaac could not even make it through his speech therapy session this week. He laid on the floor, cried, and hit his head. The therapist could not believe the change in him. After unsuccessfully trying to calm Isaac down, she looked at me with tears streaming down her face and said, “I can’t even imagine how you must feel to see him like this.”
We have contacted some cranio-facial surgeons who think that Isaac would benefit from having further surgery for the Craniosynostosis. His own NS is not as concerned, but wants to see him back in his office in August. We are hoping that his own NS has some answers for us in August, but we are aware that most likely we may be having to make a really tough choice about which doctor’s opinion we should trust, and if Isaac will need more surgery. Please keep our family in your prayers as we try to figure out what is wrong with our precious Isaac, and how to fix it.
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On August 26<SUP>th</SUP> in the wee hours of the morning, I awoke to my water breaking and painful contractions. Because of how close together my contractions were, along with a history of fast deliveries my husband called an ambulance which rushed me to the hospital. Isaac Matthew was born 10 minutes later at 35 weeks gestation (5 weeks early). Isaac was a whopping 9lbs 7oz at birth…a huge preemie! Isaac had a few minor problems at birth, but was mostly healthy. His face and head were very swollen and bruised from such a fast delivery. He looked like a sumo wrestler!!
In the hospital we noticed that Isaac made a sweet little sighing sound everytime he took a breath. It was very sweet and we commented that he was singing lullabyes to us. Over the next few weeks we noticed that the sweet sighing turned into a gasping sound. We also noticed that Isaac seemed to be struggling for each breath and would often stop breathing for several seconds while he was asleep.
We took Isaac to his pediatrician who then referred us to a pediatric pulmonologist at the children’s hospital. After testing and X-rays, and additional referrals to an ear, nose, and throat specialist; Isaac was diagnosed with laryngeomalacia, trachealmalacia, tachypnea, stridor, subcostal and intracostal retractions, obstructive apnea, and possible sleep apnea. Basically, Isaac’s airway was too floppy and collapsed on itself whenever he tried to take a breath in. He also breathed very rapidly at 80-100 breaths per minute (20-40 was the norm for his age). The doctors felt that there was a structural problem deeper in Isaac’s lungs that was causing all these breathing problems so they scheduled an exploratory scope surgery.
The day before Isaac’s scope surgery, he developed RSV and was hospitalized with severe breathing difficulties. His surgery was postponed. As soon as Isaac had recovered from the RSV (which took several weeks) he came down with croop. He went back and forth in a cycle of respiratory illnesses which he was more susceptible to due to his airway problems.
Isaac was a wonderful baby! He was full of smiles and giggles for anyone who looked at him. He had the kind of personality that made people stop and notice him. We were constantly getting comments about his wonderful personality. Isaac was always very content with just about anything. We couldn’t have asked for a better baby!
One day, my husband and I were looking at some pictures of Isaac when he was a few days old and we noticed that he had a strange line that went up his forehead. This didn’t bother us a bit, as all 4 of our older children had the same line. We joked that Isaac had joined the ranks of the “Klingon Kids” (a character from Star Trek who had weird ridges on his forehead). We never thought that it could be anything medical, until a few days later when I received a phone call from my sister in law.
I will never forget the words that were spoken that day. My sister in law in Texas (we live in Virginia) called and was upset because her doctor had told her that her son (who is 6 months older than Isaac) may have this condition called “Craniosynostosis”. I asked what his symptoms were and when she told me about this strange line that went up his forehead and showed up really well in pictures, I literally laughed at her. I told her that ALL of my kids had that line, even Isaac and she didn’t need to see a neurosurgeon for it! In hindsight I feel horrible for laughing and blowing her off like that. I sent her pictures of my kids in an email and she called me back and told me that they looked just like her son. I did a google search of the word “Craniosynostosis” and when I saw pictures of other kids with cranio I literally cried. The more I saw and read, the more I became convinced that my kids had cranio. My sister in law took my children’s pictures with her to see their neurosurgeon in Texas. He said that we needed to get them in to see a neurosurgeon here, because they all looked like they had it.
I marched into our pediatrician’s office here and told him that Isaac had Craniosynostosis and we needed a referral to see a neurosurgeon. It was my turn to be laughed at. The pediatrician looked at Isaac’s ridge on his forehead and then walked out of the office. He returned with a piece of paper with pictures of baby’s heads on it. The top of the paper was labeled in large, bold letters “Positional Plagiocephaly.” He then proceeded to show me the pictures on the page and said, “Isaac does NOT have Craniosynostosis, and if he did then his head would be grossly misshapen like these kids. Really all we would do anyways would be to change the way the baby lays, that is causing the head to be misshaped”. He refused to give me a referral for a neurosurgeon. I didn’t argue with him, because it was obvious to me that he had no idea what Craniosynostosis was.
After researching on the internet a little more, I found out that a lot of people have a hard time with their pediatricians not knowing about cranio. We also found out that we didn’t need a referral from our pediatrician to see a NS. I called a large hospital about 3 hours away from us and asked for an appointment with a NS. After a little bit of a runaround we got an appointment to see a NS. We went in totally expecting that the NS would take a look at Isaac (who we realized was not as severe looking as a lot of kids with metopic synostosis) and order a CT scan. We thought we had found someone who would give us all the answers. We were disappointed. This NS walked in and told us that Isaac had mild metopic Craniosynostosis and that he did not feel that a CT was necessary. He told us that ICP is extremely rare in metopics and the only metopics that he did surgery for were ones that were very severe shaped like a boat’s keel. He did seem slightly concerned that Isaac was having some possible vision problems, and told us that he wanted Isaac to see a neuro opthalmalogist if we didn’t see any improvement. He said he would be highly surprised if Isaac ever showed any signs of ICP, but he did give us some signs to watch for.
We left with mixed feelings. On the one hand we were glad that Isaac didn’t need this scary surgery. On the other hand, I just didn’t feel right about it. We waited a few months and watched as Isaac’s head-shape worsened a bit. Isaac’s cousin had surgery to correct his Craniosynostosis and did wonderfully! I just couldn’t let go of the feeling that the cranio was an issue for Isaac. I scheduled an appointment with a different NS for a second opinion. We took Isaac for what turned out to be one of the worst experiences we’ve ever had at a doctor’s office.
The neurosurgeon walked in and very accusingly asked, “First off, who told you that your son had Craniosynostosis?” Then he told us, “Your son does not have Craniosynostosis, and even IF he did it’s only the metopic suture and metopic isn’t even a TRUE synostosis”. The appointment went downhill from there. The NS was very derogatory towards us, accusing us of being horrible parents who “wanted” our kid to have surgery. He asked if we knew what they would do to our precious son in surgery and proceeded to tell every detail of the horrendous surgery. Several times he told us to “look at your son…he’s beautiful! Why would you want to put him through this?” When we asked about the closed soft spot, he stood up and put his thumb where Isaac’s soft spot should have been and started pressing down. He continued to press down as his knuckles turned white…pressing….and pressing…and pressing! I was sure that Isaac’s head was going to crack open right there!! Finally Isaac let out a loud shrieking scream and the NS let go, backing up with a huge smile on his face. He said, “He’s got a soft spot still. It’s palpable”. After berating us some more, the NS told us to get out of his office and he never wants to see us again. We left the office not having a clue of what to do. It seemed that the door had been slammed shut on the Craniosynostosis thing, no matter how we felt about it.
We tried to put Craniosynostosis out of our minds. Isaac was still a wonderful baby! No matter how hard I tried, I could not get past a feeling that something was wrong with Isaac. Isaac still met developmental milestones, but he was later in meeting them than his peers. I felt like I was going crazy in thinking that something was wrong with Isaac when he was such a good baby.
Isaac continued to lag behind in his development. He also continued to have severe breathing problems. In early November 2007, Isaac had a very confusing episode of illness. One day he began vomiting. He vomited over and over again throughout the whole day and night. He didn’t have a fever or any other signs of illness, just the vomiting. We took him in to his pediatrician who told us that if Isaac continued vomiting we would need to take him in to the ER for some IV fluids. Isaac stopped vomiting that afternoon and was able to hold down a little bit of pedialyte. That evening the vomiting started back up again. We took Isaac in to the ER thinking that they would give him a bag of fluids through an IV and then we’d be back on our way home. While in the waiting room Isaac became very lethargic and limp. By the time the ER doctor saw him, he was not responsive. Isaac had become so severely dehydrated that his blood sugars dropped dangerously low, in fact several of his “levels” were dangerously low. They immediately started injecting him with a “sugar gel” and started an IV with other fluids. Once they got him stabilized, they transferred him to the children’s floor and he was admitted to the hospital.
While in the hospital, Isaac’s body took a long time getting everything back to normal. They ran many tests to see if he had a virus or infection. None of those tests showed any indication of virus or infection. The doctors were stumped as to what had caused all of the vomiting. One doctor mentioned that she was suspicious of ICP from his metopic Craniosynostosis causing the vomiting. She told us that if Isaac did not stop vomiting then she wanted to order a CT scan to check for ICP. Isaac stopped vomiting and all of his blood levels returned to normal (a CT scan was not done). He was released from the hospital without any answers about what had caused the vomiting.
Isaac’s breathing issues worsened again and we spent many days seeing doctors. Being admitted to the hospital was a constant threat, and we prayed that Isaac wouldn’t have to spend Christmas in the hospital. At one point, Isaac’s medications took up a whole counter in our kitchen! Isaac began showing other strange symptoms. He started being really “clumsy”. He would walk right into the table, walls, etc. He also started falling down. He fell over sideways and backwards without having tripped on anything. He was black and blue from falling so much. He started walking weird too. He was off balance and had a really wide waddle type walk. We ended up keeping him strapped in a stroller most of the time for his own safety. He also had many problems with sleep. He would wake up screaming in pain and start slamming his head on the crib rails. When he was able to sleep he would moan and whimper in his sleep. This was torture to us as parents to sit and helplessly watch.
We talked with Isaac’s pediatrician, who felt that something was going on neurologically and referred us to a pediatric neurologist. They couldn’t get us in for two and a half months! I kept asking if this could be caused by the Craniosynostosis and if I should call a NS. They kept saying, “Just wait and see what the neurologist says.” In the meantime, we sat and watched helplessly as more symptoms popped up. Isaac began having seizures several times a day as well as tremors. The vomiting was off and on… sometimes disappearing for a few days, or even a few weeks, and then returning again. One day Isaac had another serious episode of vomiting. He vomited over 30 times in 6 hours! His development stalled and even went backwards. Isaac began choking and gagging on his foods, drinks and his own saliva. He couldn’t even eat cheerios. We were very worried and we bugged his pediatrician to let us call the first NS that Isaac had seen and just ask for his opinion.
Finally, one day a nurse was talking to Isaac and she looked at me and asked, “How long have his pupils been unevenly dilated?” After taking a closer look I could see that his left pupil was larger than his right one. When they tested it, the left pupil was delayed in its response to light. Isaac’s pediatrician then became very concerned and told us to either get him in to see the NS that very same day or to take him straight to the ER. We were worried, but finally felt like we were getting somewhere! We tried calling the NS and drove Isaac in to the ER. As we pulled into the parking lot to the ER the NS called us back and told us that it definitely sounded like something neurological and some ICP symptoms. He told us to go into the ER and demand a CT scan, if nothing showed up then demand a MRI. He said that no matter what they found he wanted the scans sent to him and he would help us figure out what was wrong with Isaac.
When we were in the ER they treated Isaac really seriously at first. They noticed on their own his uneven pupil dilation. When we told them he needed scans done, they gave us a hard time. They finally gave in and said they would do one scan, but didn’t want to do both because of the amount of sedation that they would have to use. They explained to us that sedation is really risky in kids with breathing conditions like Isaac’s. They ended up doing an MRI because they felt it would give them more information than a CT scan. Isaac did really well with the sedation for the MRI and they were able to get really clear pictures. The ER doctor came in very briefly afterwards and told us that everything looked good. We got the feeling that the doctor was overwhelmed and didn’t know what to tell us. Finally he came in and said, “I’ve got good news. There is absolutely nothing wrong with your son’s brain.” He told us that they MRI didn’t show ANY problems AT ALL, except for a small lesion on the front part of the brain that he didn’t think was affecting anything. Most importantly, is that he didn’t find anything wrong with the cerebellum, which is what he had expected with all the balance issues. He seemed rather hasty to get rid of us, and handed us a printed copy of the MRI report and told us that at least we had that report to take to our NS and sent us out the door.
We left. I was in tears. I just knew that something was majorly wrong with Isaac, but I felt like nobody was listening to me. I felt like my son was suffering and wasting away and nobody cared! In the car on the way home from the ER, I got over my initial shock that nothing was found and started reading the MRI report. I couldn’t believe my eyes! The report started listing “findings” and there were many things listed. I didn’t understand what it was trying to say, but there were words and phrases like: Chiari malformation, prominent subarachnoid space, arachnoid cyst, trigonocephaly, low lying cerebellar tonsils, hyperintensity in the left temporoparietal matter, convex ependymal surface of the lateral ventricle suggesting volume loss, etc. I am not a doctor, but looking at that report made me think that my son’s brain was not “perfectly normal”.
We faxed the report to the NS and his office called us back that same day and told us they wanted to see Isaac very soon. They didn’t tell us if they were concerned about the Craniosynostosis, or ICP or something else altogether. I tried not to get my hopes up that they would be able to tell us what was wrong, but I wanted more than anything for them to be able to help my little Isaac! Isaac was such a trooper. Watching him suffer all day and all night, day in and day out, was heartbreaking, but then he would smile and you just knew he was going to be okay!
At the appointment, while the NS reviewed the MRI films from Isaac’s ER visit, the physician’s assistant (PA) came in and talked to us. She also observed Isaac a little bit. In the brief time that she was in the room with us, Isaac fell on the floor twice and also walked straight into the examining table. With a raised eyebrow, the PA told us she could definitely see what we were concerned about. When the NS came into the room, he sat down and asked a few questions about Isaac. We answered his questions and he sat silently for a few seconds. My heart was racing in fear and expectation. Then the NS leaned forward and said, “Your son has a cyst that has formed on his brain, and he needs surgery to remove it.” He went on to tell us more about the procedure that he would use to remove the cyst from Isaac’s brain. I asked if it had anything to do with Isaac’s Craniosynostosis, and he told me, “Yes, the metopic suture being fused, as well as his small narrow head has caused the brain to rub against the skull in the back which caused the cyst to form. The cyst has now grown so large that it has pressed in on the brain and caused the brain to herniate (chiari malformation) into the opening to the spinal column and is blocking the flow of cerebro-spinal fluid to the cerebellum.” He told us that Isaac has probably never known a single day without pain. He told us again about the procedure, and told us it was a somewhat risky surgery. I asked if it was about the same as a cranio surgery and he told me that this would be riskier because it is actual brain surgery; they would be cutting into the brain itself. He told us that Isaac would need blood during the surgery, but reassured us about the safety of the blood bank there. We asked if Isaac would need more surgery in the future for the Craniosynostosis, and he told us that it was a possibility and that Isaac would be watched very carefully and we would make that decision later. We were told to go home, think and pray about the surgery over the weekend, and call him back on Monday with our decision.
It’s amazing how easy, yet how hard that decision was. Clearly, Isaac needed the surgery, but I was so scared. We called the office on Monday and told them we had decided to go ahead with the surgery. They told us they would start the insurance approval process and call us back with a date. We were shocked when they called us back the very next day and told us the surgery was scheduled for April 1<SUP>st</SUP>…10 short days away!! It was a mad dash to get everything done before hand, and make arrangements for a place for us to stay (the hospital was 3-4 hours drive from our house). In hindsight, I’m so glad that things happened so quickly, as I had less time to sit around and worry.
We became very familiar with the surgical procedure the NS had talked to us about, as we had to explain it many times to friends and family. I tried not to think about the fact that this was going to be done to Isaac, as I described the surgery to those that asked. They would make an incision from almost the top of the back of Isaac’s head, down to the bottom of his neck. Then they would remove the back part of his skull, from that they would cut off the very bottom and that would never be replaced. They would also remove a portion of the back of his vertebrae to allow the cerebro-spinal fluid more room to flow. They would cut into the dura (the “skin” that protects the brain), and then drain the cyst. Then they would collapse the walls of the cyst and remove it from his brain. Then they would make a tissue graft to patch the dura, and put the skull back into place (minus a small portion) and close up the incision.
At the hospital, on April 1<SUP>st</SUP> Isaac was great. His surgery was slightly delayed, but he did very well even though he hadn’t eaten. Some clowns came to the pre-op playroom and blew bubbles with him and played instruments and sang. Isaac joined right in with them. He charmed all the nurses there with his cute personality. They told us that one of us could walk him down the hall. My husband was very gracious and let me be the “walker”. I had almost prepared myself for the moment when I had to hand my precious son over to the doctors and trust that he would be returned to me, the same little boy minus the horrible symptoms.
What happened next took me by complete surprise…they walked me all the way back to the surgical room! They had me sit and hold Isaac (he had worn himself out playing and was asleep) in a rocking chair that was wedged in between several huge stainless steel sinks and the back wall while several doctors and nurses prepared the equipment for Isaac’s surgery in an adjoining room. Then I heard a voice among the masks call out, “Bring him in.” They told me to come in and lay Isaac on the operating table. I was SO not prepared for that. My feet felt like lead as I carried my son to the table. Pictures of the Biblical story of Abraham laying his son Isaac on the altar to be sacrificed ran through my head. At the same time all the sights, sounds, and smells of the operating room were overpowering and I felt I was going to be sick right there. Somehow, I found the courage to lay Isaac on the table. They put a mask on his face to administer the gas to put him to sleep. He woke up flailing and kicking and trying to scream, while they held him down. He looked at me for help, and I’ll never forget feeling like I had just forsaken my sweet son. He finally drifted off into his drug induced sleep. The anesthesiologist told me to pull down my mask and kiss his cheek before I left. Walking out of that room and leaving my precious son was the hardest thing I’ve ever had to do.
Once I got out into the hallway and was able to compose myself, everything became very surreal. I hadn’t thought that I would be able to eat while Isaac was in surgery, but I found myself very hungry. After checking in with the waiting room attendant, we went down to the cafeteria to get something to eat. Melissa (a friend from CK) met us there and stayed with us throughout the whole surgery. She was a wonderful support! She brought snacks and activities to help keep us entertained. As the hours passed we received messages from the operating room on their surgery progress. My mind continually went back to the day that the NS explained the procedure to us, and I would wonder which part they were on at that moment. I sent up many silent prayers; most of them were not complete thoughts, but instead, fragments of thoughts in my racing mind.
At one point during the surgery, we were walking down the halls trying to find a place more comfortable to sit and eat a snack when a doctor in a white coat walked up to us and asked if we were Isaac Lucky’s parents. My heart stood still as I answered. She then told us she needed to talk to us in private away from other people. Fear gripped me and I looked to Melissa, who also appeared unnerved, but tried to look reassuring. Melissa kept an eye on our bags as we walked down an empty hallway to speak with the doctor. Finally, the doctor explained that she had nothing to do with the surgery and she just needed to get our permission to use a new kind of baby wipe to bathe him with during his hospital stay after surgery. We signed her “permission slip”. This was NOT a good thing to do to a parent when their child is in brain surgery!
Finally, we got the word that they were closing up and that the NS would be out to talk to us in just a few minutes. When I saw the NS walk into the room, I could barely stay in my chair! He walked to us with a huge smile on his face. It instantly calmed me. He told us that the surgery had gone very well. He said he was surprised at the amount of pressure the cyst was putting on the brain. He told us he had found the brain was under a “tremendous, TREMENDOUS amount of pressure”, he went on to explain how when he had cut into the cyst and relieved the pressure from all the fluid, the brain (which had been extremely compressed) literally popped out. He told us that Isaac had definitely needed the surgery, and that he expected great things. We were later told that the cyst was much worse than what they could see on the MRI and that they had left a portion of the cyst wall in. The cyst had been pressing against the skull in the back so hard for so long, that it had worn the skull down. The skull was so thin that they didn’t even have to cut it to remove it – they just broke and chipped it out. Isaac had been suffering for a very long time.
We got a small glimpse of Isaac as they wheeled his bed from the OR to the PICU. Waiting to see him while they got him settled in the PICU was very hard. When we did see him, he was still sedated. He looked so peaceful laying there. I couldn’t believe how good he looked…he was still my same little boy. I wanted so badly to scoop him up and kiss him! I took several pictures. Melissa got to come back and see him for a few minutes before she left. The nurses explained the PICU rules to us and allowed us a few extra minutes with Isaac before we had to leave. I didn’t want to leave him, but they assured us that they would call us at the Children’s House if he had any problems. When we got out into the hallway, I was hit hard with an intense feeling of joy mixed with pride. My precious little boy had made it through brain surgery! Nothing can parallel that feeling. I was so proud of Isaac. I wanted to stop every stranger I passed and tell them about my son and how proud I was of him! Sam, my husband, told me I was glowing. I made a bunch of phone calls from our room at the Children’s House telling everyone how well Isaac had done. Everyone told me they could hear the joy and relief in my voice. I couldn’t wait to see Isaac again the next morning.
The day after Isaac’s surgery, time seemed to drag. I was rushing to get dressed and get a bag ready to take to the hospital with us. I was ready way ahead of the time that they had told us we could go in to see Isaac. We found a little café that served breakfast and ate there. When it was finally time to go see him, I was excited. I felt like a giddy school girl as we took the elevator to the 7<SUP>th</SUP> floor and down the hallways. Finally, we pressed the button and the big door into the PICU opened up. The hallway was pretty much empty, except for a large group of doctors who were walking in and out of a doorway. Then I realized that they were standing at Isaac’s door! Many thoughts raced through my mind as fear and panic overtook me. I tried to tell myself that nothing was wrong; that it was probably normal. Sam actually sped up his pace and made it into Isaac’s room well before I did. As I approached the room, a doctor blocked my path and asked me if I was Isaac’s mother. I could see other doctors and nurses talking to Sam inside the room and I longed to be able to push past the doctors and see Isaac, to make sure he was okay. My attention focused back on the doctor who had stopped me. He said that Isaac had a really rough night, and was up all night vomiting and in pain. I felt horrible for not being there for him when he needed me the most. Inside I was screaming, “Why didn’t they call me?” The doctor then told me that Isaac’s heart was doing some weird stuff and they were trying to figure out what was wrong. He said they’d be bringing a large machine in to do an ECG (an ultrasound) to look at his heart. He wouldn’t give me any clear details about what they had seen that concerned them. All we could do was wait. The doctors also told us that they had called for a “genetics consult”. They explained that whenever more than one system of the body had a defect, testing was needed to rule out the possibility of a genetic syndrome.
When I saw Isaac, he was very restless. Even when he slept, he did not look peaceful like he had the night before. They brought in some machines and a technician introduced himself to us. He told us that we could stay for the ECG, but we couldn’t ask any questions about what he was seeing. The ECG took over an hour. Isaac screamed through the whole thing. It was so very hard to sit and hold his little arms down after all he had been through. Finally it was over and the technician told us that we would hear back from a cardiologist once they had reviewed the results. Isaac finally slept for about 15 minutes…he was so exhausted.
Isaac’s nurse told us that Isaac needed to wake up and eat and drink something, and she felt that the pain meds that he was receiving through the IV’s were going to keep him too sedated to wake up. She refused to give him any more pain medicine. Isaac cycled through waking up screaming in pain and sleeping for a few minutes, throughout the day. His nurse was not successful in getting him to drink anything because every time he was awake, he was in massive amounts of pain and he just screamed. Watching him suffer in that much pain was very, very hard. When he opened his eyes they were very dull, and unfocused. We couldn’t tell if he even knew we were there. The doctors came to talk to us again. Isaac’s blood was tested hourly, and they had noticed a steady decline in his sodium level. They told us that the sodium had to be stable before he could be transferred out of the PICU to the regular floor. That evening his levels finally stabilized enough to be transferred. I was worried about how the nurses on the regular floor were going to handle the amount of pain he was having.
On the regular floor that night, Isaac struggled with the pain. He screamed and screamed. We tried to comfort him, but could not. He didn’t respond to us in any way. We didn’t even know if he knew we were there. At shift change, we got a new nurse who was immediately alarmed at how much pain Isaac was in. She went personally to find the pain management team. She came back into the room and gave him some medicine. I was in tears at this point and asked her if it was normal for Isaac to be in this much pain. She told me that in the PICU they had decreased Isaac’s pain medicines so much that he was receiving a smaller dosage than regular Tylenol…basically nothing. She said he hadn’t had good pain control since the surgery. When the pain management team found out how small of a dose he was getting not even a full day after his surgery, they were very upset. They immediately tripled the amount he was getting in the IV and added a few more pain meds as well. It was amazing how fast the medicines helped. Isaac was able to really sleep for the first time since his surgery.
The next day was a busy one! Isaac was more aware of his surroundings, and just quietly watched everyone coming and going. Whenever the nurses or doctors would come in, he would watch them until they started talking to him, then he would shut his eyes as if that would make them go away. Physical therapy came to work with him. They were concerned because he kept his head turned to the left and would not let anyone move it. The left side of his face was pretty swollen (swelling is normal after brain surgery.) His lips were swollen and cracked as well. The therapist used a moistened swab to wipe across his lips. He seemed to like it and even held one in his own hand. The geneticist came to talk with us and get some basic family medical history. He told us that he believes there is definitely a syndrome and that we needed to have some testing done when Isaac had fully recovered from this surgery. Everything seemed to be going better for Isaac… until night time.
Isaac’s IV went bad (from previous hospital stays, we knew that Isaac’s veins don’t like IV’s for very long) and he didn’t receive his pain medicine. By the time they noticed it he was already screaming, flailing, and kicking. It took a while before the IV team came to give him a new IV and the pain was already ahead of him. Even after they got the IV hooked back up to the pain pump we had a really hard time getting control of the pain for him. He was screaming and throwing his body around, he was ripping at his own hair and the bandage that covered the incision site. They had to brace his arms and legs and tie them down to the bed for his own safety. Even then he still tried to throw his body around. The nurse and I were both in tears! It was heart-wrenching to watch. I have never seen anyone in that much pain before, and it killed me to see my sweet son’s body wracked with that much pain. The pain management team doubled the amount of meds they were giving through the IV and added Valium by mouth. Isaac finally settled down a bit. He woke up in pain about once every hour and cried until someone bolstered (gave an additional boost) his pain meds.
Isaac continued to do better the next day, but still had a lot of issues with pain that night. Once again his IV went bad (for the 4<SUP>th</SUP> time), and we got behind on the pain meds. More thrashing and screaming and ripping at his hair and bandage. He had to be braced and tied down again. Sam and I helplessly endured many more heart-wrenching hours. Isaac finally got to sleep around 2am. Words can’t even begin to express how it feels to see your child like this.
At some point the next day, Isaac “turned the corner”. His blank expression with pain dulled eyes was replaced with a more alert and interactive look. He started sitting up and wanting to play with toys. He actually started eating a few bites of food. He wanted to be held a lot, which was very difficult due to all the wires on him and the stiffness he had from pain. The pain management team made a plan ahead of time to keep him from having the same kind of night as the last few had been. From there on out, he did better and better.
The last day that Isaac was in the hospital, I noticed an area of his incision site that stuck out a bit farther than the rest. I asked the doctors about it and they told me it was normal swelling. I noticed that when Isaac cried the area grew and started bulging out a bit, but they reassured me that it was normal. We got home in the middle of the night. Isaac seemed glad to be able to sleep in his own bed, but still had a lot of pain. The next morning Isaac vomited. He was very stiff still and would turn his whole body instead of turning his head. The next few days were very tough. Isaac didn’t play, he was very fussy and couldn’t sleep very well. As a mom, I could only take so much and began to really wonder if we had done the right thing for Isaac in having the surgery. I felt like I had lost my little boy, and Isaac would never be happy again. We called the NS to ask if Isaac should still be having this hard of a time. The NS ordered a higher dose of pain medicine for him. That night Isaac was able to sleep a little bit better. The next day he was more playful and even gave us a glimpse of his mischievous grin! He tired very easily, but it was good to see him play again.
We started getting a little concerned about the swollen area of his incision site. Each day it seemed a slight bit larger. One day, when we woke up and saw the back of Isaac’s head, I gasped. The swollen area had doubled overnight and Isaac looked like he had a golfball sticking out of the back of his head! I sent Sam pictures of it at work and tried to contact the NS again. When the NS called back they said they needed to see Isaac in the E.R. there. Even though it’s a 3-4 hour drive, they felt that it was too complex for our local hospital’s ER. We found babysitters for our other children, threw some clothes in an overnight bag, and headed for the hospital.
At the ER, when they found out that he had just had brain surgery and saw the big bump sticking out of the back of his head, they took him straight back to the examining room. After three failed attempts at getting a CT scan done, they finally got it on the 4<SUP>th </SUP>time. The doctors determined that the dura graft (the skin like tissue surrounding the brain) had a hole in it and was leaking cerebro-spinal fluid (CSF). The CSF was pooling right behind the skin at the incision site and that’s what had created the bulge. They admitted Isaac to the hospital again. All of the nurses recognized Isaac and were glad to see him a bit more playful than the last time.
They were all impressed at the big bulge sticking out of his head. Each of them wanted to feel it. It was very gross. It was squishy and soft like a water balloon. His incision seemed to be holding well, but was definitely stretched out. They kept Isaac there a few days for observation. Isaac still had a lot of pain and was having a really rough time sleeping as well. On the last night there, a wonderful nurse saw how utterly exhausted I was, and when Isaac woke up screaming she came to his crib-side to care for him and told me to go back to sleep. Off and on throughout the night this wonderful nurse stayed at Isaac’s crib and tried to calm and care for him for hours at a time. It was so nice to have that break. When Isaac screamed and cried it wasn’t just a matter of calming him… he threw his body and tried to slam his head and rip out his IV’s. Calming Isaac was also a matter of keeping him from hurting himself. It really wears you out! I made sure I told that nurse’s supervisor how wonderful she was.
After a few days of observation we were sent home to continue watching the bulge. The NS said that the leak should heal on its own in a few weeks and the excess pool of CSF would be reabsorbed. If it didn’t heal on its own in a few weeks then it would have to be surgically fixed.
While at the hospital we were able to find out a little bit more about Isaac’s heart defect. The results of his ECG during his previous hospital came back showing an Aortic Root Aneurysm. Isaac’s aortic root (part of the main blood vessel to the heart which contains the aortic valve) is enlarged. He will have to be watched very closely and have regular ECG’s to monitor the amount of dilation. If the aortic root grows too large, it could cause the heart to rupture (this typically causes immediate death). At this time, Isaac’s aortic root is only mildly enlarged, but if it continues to enlarge to the point where the doctors think he is in danger of his heart rupturing then he will need major heart surgery to replace the aorta and the aortic valve. The doctors made it very clear to us that Isaac needs to be followed very closely by a cardiologist.
At home, the bulge went away completely within a few weeks. Isaac did well, and we saw a little bit more of his personality returning day by day. Therapists came to our house to work with Isaac to help him with the developmental delays that had been caused by the pressure on his brain. Isaac’s incision site looked really good, except for one spot that started looking infected. We were sent back to the ER to have it checked out.
Isaac did not enjoy seeing the inside of a hospital again! He was so upset that the doctors and nurses felt sorry for him and let him play in the toy room. We have been to this ER many times yet I never knew that the ER had a toy room in it. After a long day in the ER they determined that Isaac had a localized infection and didn’t need to be hospitalized for it. They told us that Isaac’s heart defect causes them to be extra careful anytime he has an infection in his body, because infection could travel to the already weakened heart valve and that would be very bad. They gave us instructions on how to care for him and what to watch for and sent us home again.
At home Isaac continued with his therapy and we started seeing a huge difference in him. All of his horrible symptoms from before the surgery had disappeared and he was able to sleep well, for the first time in his life. We saw great strides in his development and he started speaking real words. We noticed that he had the cutest personality. He started being mischievous and teasing us; it was a complete turnaround! We went back up to Johns Hopkins for his post-op check and his NS was very pleased with his progress. We were told that Isaac would need to come back for regular checks every 6 months, but that he was doing great.
We decided that since Isaac was doing so well, we should take a break from all things medical and go camping for Memorial Day. The kid’s were thrilled, and we headed up to a friend’s house in the Shenandoah Mountains. We camped in their front yard, and roasted hot dogs and marshmallows on the fire. Isaac had a blast! As I watched him running and playing with the other kids, I marveled at his strength. Who would’ve ever thought that he was the same kid that had just 2 short months before spent his days and nights suffering through so many medical problems?
Sometime that evening, we noticed that a mosquito had bitten Isaac’s forehead. The reaction seemed to be a slight bit worse than most people have, but nothing to be concerned about. When we woke up the next day, Isaac’s whole forehead was swollen and bright red and it was traveling down the left side of his face. I gave him some Benedryl for the allergic reaction and some Motrin to help with the swelling. A few hours later, Isaac took a nap and when he woke up we knew we needed to take him to the ER. His whole face was swollen and the left side was purple. His left eye was almost completely swollen shut! (He looked worse at that point than he had right after his brain surgery!) The local ER there treated Isaac for what was deemed to be a severe allergic reaction to a mosquito bite. They also prescribed a round of antibiotics as a precaution against infection due to Isaac’s heart defect. So much for our break away from the medical world!
We took Isaac for a follow-up appointment with his pediatrician. The pediatrician was concerned about the severity of Isaac’s reaction to the mosquito bite and what would happen when he got bit in the future. We were sent to see an allergist. The allergist told me that to have a severe reaction to a mosquito is very rare, but it can happen. Isaac just likes to be unique, I guess! The allergist was also concerned about the possibility of an anaphylactic reaction (severe reaction that causes the victim to stop breathing) in the event of another mosquito bite. Isaac was prescribed an epi-pen for an anaphylactic reaction and also a prescription for a steroid to keep the swelling down. Isaac will now need to be followed by the allergist for regular checks.
The genetics clinic called, and after reviewing the information from Isaac’s medical records they feel that our family carries a genetic syndrome. They set an appointment for our whole family to go in together. All the children (including Isaac) will have ECG’s done to check for heart defects and then they will go over the results and order any additional testing to help them figure out which syndrome we have. That will be one very long day for us, but it will be a day of hope. Hope that we will finally know what has caused so much pain and hardship for little Isaac (and our other children).
Isaac was doing really well and we were almost 3 months out from his surgery, when he began having a few setbacks. His head-shape began changing (even his therapist noticed it) and his lower jaw seems to be recessing. He has started showing some of the same symptoms that he had before the surgery (vomiting, seizures, head-banging, sleep disturbances, gagging, fussiness, etc.). It was extremely hard to see him go through all of that the first time, but to see him like this again is unbearable. Isaac could not even make it through his speech therapy session this week. He laid on the floor, cried, and hit his head. The therapist could not believe the change in him. After unsuccessfully trying to calm Isaac down, she looked at me with tears streaming down her face and said, “I can’t even imagine how you must feel to see him like this.”
We have contacted some cranio-facial surgeons who think that Isaac would benefit from having further surgery for the Craniosynostosis. His own NS is not as concerned, but wants to see him back in his office in August. We are hoping that his own NS has some answers for us in August, but we are aware that most likely we may be having to make a really tough choice about which doctor’s opinion we should trust, and if Isaac will need more surgery. Please keep our family in your prayers as we try to figure out what is wrong with our precious Isaac, and how to fix it.