Waiting for Decompression Surgery

We were home again but still with the same problems as when we arrived in Brisbane for a hopeful answer. The spina bifida clinic was only a short time away and we were going to have to do something. Faith couldn't live like this and nor could we. There was no escape for Faith from the pain, vomiting and suffering, day after day it went on. It went on into every night and we reached a point when we needed a game plan to educate these doctor's on what life is like for Faith and how she is symptomatic for both the syrinx and the arnold chiari. But how? We decided to get the social worker involved. We sent her an email outlining what Faith goes through every day and every night. We explained how life was and how we were feeling about the situation and the lack respect for the parents opinion. When we arrived at the spina bifida clinic, the social worker sat in with us on the consultation with the neurologist. She had already spoken to her and at last we were being listened too. Faith had changed so much since our last visit and she saw first hand how sick she was. The neurologist saw the hot and cold sweating on different sides of the body, the sweating on one side of the body, the pain and the distress she was in. She saw it at last and she acted.

The neurologist got a second opinion from another consultant and then hastily called the neurosurgeons. We also saw a consultant paediatrician who specialized in spina bifida and he called the neurosurgeon too. The doctors at last understood. It went on for a few hours, it felt like a whirlwind. We saw doctor after doctor and explained what was going on. The neurosurgeons decided that posterior fossa decompression surgery and shunt the syrinx was the way to go. It was explained how this operation would work and what it would do. Frightening, but what other way forward was there, there was only one. The surgery was scheduled for a couple weeks into February 2011. At first the doctors wanted Faith to stay in hospital until surgery but they thought we have been living with this for a long time now and it might not be best for the family. Faith might catch some other bug in the hospital so decided to discharge her.

A week before the surgery we had to bring Faith in for another MRI so the neurosurgeons could see where the blood vessels were and be as prepared as possible for the surgery. Faith was already scheduled for a CT scan as the plastic surgeons were looking at the possibility of whether it would be beneficial for Faith to have a helmet to reshape her head. We were very unsure about this as we had to be careful of pressure spots and her shunt. In consultation with the neurosurgeons we later decided that her head shape had formed nicely and it was a waste of time but at this point we were still seeing where the road led. Back to the MRI/CT scan, we had to report to the day surgery unit and sit in a crowded waiting room until they were ready for Faith. She had to have another general anesthetic and because an MRI had been added we were bumped to the bottom of the list. She was so hungry and we waited and waited. This time there was no power outages and the MRI and CT were completed as quickly as possible. We were taken back to the day surgery unit where we had to spend a few hours waiting to see if Faith had any reaction to the anesthetic. She was fine.

With the surgery the following week we started to plan, we would have to be in Brisbane at least a week. We had to organise food and accommodation. We had to be at the hospital the day before and we were desperately hoping that this surgery would give Faith a better life. It was hard waiting for another week and we really wished it wasn't so far away. A week felt such a long way off. But the days did pass and the day to go back to Brisbane came. We were feeling so anxious and stressed, we were sharp with each other and struggling. The trip there took longer than usual as it was raining again. But we eventually arrived.

We lined up at admissions and booked in.  The staff were friendly and talked to Faith. We headed back to the well walked corridors of the babies ward and to our surprise they had no beds. Yes, we were expected but no where for us to go. This threw us and we were asked to wait in the parents lounge. It was hard not to jump to conclusions thinking what does this mean, no surgery, no bed, what are we going to do. The neurosurgeons had not forgotten us and we had a visit from the registrar and fellow who we had never met before. We were very impressed by this new registrar and fellow. They explained the procedure. Faith was to have a posterior fossa decompression. The goal of this procedure is to create more room around the malformation and restore the flow of spinal fluid. They said that this procedure was rarely done done at this hospital on someone so tiny. The neurosurgeons had to order a special instrument from the Melbourne children's hospital to assist them. We also went through the risks of the surgery. Faith would have to go to the Paediatric Intensive Care (PICU) afterwards and would have to keep her head very still and this was a very dedicate time after the operation. She might be there for a few days but would take it a step at a time. Brain overload! Then the nurse came in. Faith's bed was ready. Here we go!

Faith had a lovely position next to the window and I had a window bed to sleep on. There were two other babies in the room. A new born near the door and a little baby boy opposite us, who was alone. Faith had to have bloods taken and get a drip put in her hand. She is such a challenge to get a drip into as she has had so many. This happened later that night. This was really going to happen. They were checking everything including blood type, white cells all the usual tests. The surgery was scheduled for pretty much first thing in the morning. The surgery schedule had be cleared as they were not sure how long it would take. It all just sounds like a bad dream, I am now meant to wake up!

MRI

We drove to Brisbane for the MRI and it wasn't a fun trip, it was pouring rain and the rivers and creeks were raising but we had to get through and luckily we made it (it also happened to be the worst floods Brisbane had experienced). We rang to confirm our appointment and I think they were about to cancel it until we told them we drove all this way. We later found out we were the only appointment that day, the remainder got rescheduled due to the floods. We got there early as usual. Faith had to be put under a general anesthetic and the scan was going to take a couple of hours at least. She was going to be taken to the babies ward afterwards to ensure she recovered ok. If everything went ok we were free to take her back to our hotel. We had to stay nearby for one night as a precaution. Half way through the MRI the power went out as the flood waters were rising. We panicked as Faith was inside the MRI machine. A nurse came out and said that she is fine, they have a generator but the MRI machine did go off line and they would have to reboot it so we had to wait even longer. Faith was under an anesthetic for a lot longer than should have been. She came out and was so hungry. We took her upstairs to the babies ward and gave her a big feed. With no problems from the anesthetic we returned to our hotel.

Later that day we received a telephone call from a rather concerned neurosurgeon. He explained that he was the on call registrar and that he had been asked to review the MRI report as they had found an extremely large syrinx. A syrinx? We didn't even know what that was. It is a abnormal fluid-filled space found within the spinal cord. It is often found in children with spina bifida myelomeningocele. This is what Faith has.  We were not sure what this meant. He said that our neurosurgeon wasn't on call so he would talk to the one that was and would call back early the next morning. He said that if an adult had a syrinx that big then they would operate in the not to distance future. Was this the answer that we needed? It this why Faith was having so much trouble. We didn't know. We just had to wait.

The next morning dragged on until we got a call from the neurosurgeon registrar and he said that we would need to come back and see our own consultant neurosurgeon. He said that a syrinx can either symptomatic or asymptomatic. We were thinking, well it's obvious that it is symptomatic. Faith was starting to go numb in her legs and didn't respond anymore to touch. With the floods continuing to rise we couldn't stay in Brisbane any longer and with the appointment with our own neurosurgeon a little way away, we went home. Life at home wasn't easy, the mornings dragged out with Faith so chronically sick and not much being done. We pushed for appointments to be brought forward but didn't have much success.

 After some research we believed the syrinx to be the cause of this pain and it appeared the arnold chiari ii malformation was also symptomatic and it needed to be addressed. We just couldn't make the doctors believe it. We were told we just had to wait for our appointments. We'd spend every day with a very sick baby and we struggled with knowing what to do. Our hearts were heavy and anxious with worry. The days all felt dark. There was no rest for any of us. Faith had been up most of the night in distress and with focal seizures continuing we gave her some midazolam. We had never tried this before and her breathing slowed and she was asleep. We raced her as fast as we could to the local hospital and we were taken straight in. Of course, this woke her up and after being monitored for several hours she was fine. Faith has a strange temperature control as well, so half her body would feel ice cold and the other would be hot. Because of that taking her temperature was a challenge. When they did her temperature it was high and so on that they admitted her. We protested and explained this does happen with her. At last, our own paediatrician agreed to let us take her home but leave her admitted and come back if there are any problems.

That night she seemed ok but the next morning again she had a temperature on top of the morning seizures, sweating, pulling up legs and jerking them. Our local hospital promised to talk to the neurologists but never did so we left very early in the morning and presented to the Brisbane hospital. Faith was admitted straight away and it turned out that in the previous 24hours she had picked up a gastro bug. So we were isolated as well. We spoke to the neurologists and they said the best thing is for them to film her so they could see what is happening with the seizures. This also gave us a chance to get the neurosurgeons to come and talk to us about the MRI report as our appointment was still a couple of weeks off. We spoke with the consultant neurosurgeon and he said, yes it was a syrinx and it was one of the largest he has seen and there was a lot of built up pressure in the brain from the arnold chiari ii malformation but unless symptomatic there was nothing they would do at this stage. We couldn't believe he was serious in what he was saying, can't you see our little baby and how she is suffering but he was putting that down to the neurologists to deal with. He said that if the neurologist said that Faith was symptomatic with the syrinx and arnold chiari ii then they would act but not until then.

The seizures were filmed and they did see what we were talking about and they were not seizures, wow, we couldn't believe it, what were we going to? Astonished. They said of course it wasn't normal but it wasn't seizures. If this wasn't seizure activity what is causing Faith's foot to twitch and the sweating, the hot and cold temperature, the vomiting, the pulling up of the legs and the jerking movement. What was it then?

A Plan

Our trip to the neurosurgeon took us to a different hospital again and I wasn't really sure what to expect. We were just following this road laid before us because we didn't know how else to proceed. As usual we arrived way to early and sat in the corridor and I read my book The Promise by Naomi Reed and I think my other half was reading a train magazine. We watched the lifts go up and down and I know I thought that is how I feel, emotions up and down. I tried not to think of what this appointment held.

We eventually went into Dr RC's office and the waiting room was full of a variety of people. I saw a few younger people in wheelchairs and wondered what there life was like or how there parents coped. Finally we got called in and we were faced with a man of medium height in a blue/black suit with a stern face, his office had a huge desk and we sat opposite him. I suddenly felt very protective of my unborn baby as he talked of surgery and what would happen after the birth. He spoke of spina bifida, hydrocephalus and arnold chiari malformation. He also talked of what would happen after the birth, how the baby would be wrapped in a cling wrap material to protect the baby from infection, I started to tune out and just took in very basic information. My husband absorbed more I think but I was just concerned with now and getting through to the birth.

The neurosurgeon then asked our plan and we said what plan? The plan for antenatal care and the birth, and he added that our obstetrician didn't do births so what were we going to do? I didn't know, I hadn't thought about it. Our own home hospital had basically disowned us so I really had no idea. I think that we must have looked confused as he said well, let me call the hospital here and sort something out. A consultant neurosurgeon took the time to call the antenatal clinic and spoke to a doctor and arranged for us to go over and talk to them. We were so overwhelmed by this specialist doctor taking the time to help us. He then said there are some useful associations and websites to learn more about spina bifida, hydrocephalus and arnold chiari malformation. There was a local group nearby and he looked them up and printed out directions on how to get there so we could get more information and support. We were astonished by his kindness! So we thanked him and headed over to the Mother's Hospital.

It wasn't like any other hospital we had been to, it was nicely furnished, extremely clean, light, airy and life seemed to be in the air. We went to the antenatal area reception and were warmly welcomed and within minutes two ladies were fussing over us. They were organising antenatal appointments, ultrasounds and appointments with doctors and of course this is where the baby was going to be born. These ladies gave us encouragement and such care that we felt we could tackle this challenge even though it was so unknown. We were to come back a few days later to start the process. Now we had a plan!