A Year of Oxygen

When I woke this morning I must admit that my mind floated back to 12months ago when Faith, my husband and I were in Brisbane thinking we could be here for weeks all because of cross border political red tape in acquiring an oxygen concentrator. I had mixed feelings and thoughts. I could picture not just the room with a cross hanging above the door that Faith was in but the other people that were there. It was like my mind was replaying a story in my head all the while I went on my daily activities. I felt like I was in two worlds, trapped in the present and the past. This seems to happen more when I can easily identify a date related to this time. It was a couple of days before Mother's Day 2011 and now it is around the same time but in 2012. We came to the hospital free of cords and the freedom to move around and we left with a supply of nasal prongs, tape and oxygen bottles. The little face I love now obscured by plastic nasal prongs and a heavy bottle on my shoulder. She is still just as perfect though :) I know this is a positive step in its on own way. Two days after we started the oxygen Faith started talking and saying words, she no longer breathed so deeply and there was no huge dent in her chest when she breathed. All this change over .25 of a litre, such a small amount of oxygen to make such a huge difference. I did feel safer with Faith on oxygen as now her oxygen saturation didn't drop so she was more stable. Faith has no respiratory problems it is all related to the Arnold Chiari ii malformation.

I don't know if other families struggle with doctor's not fully understanding Arnold Chiari ii, but we do. I can see why Faith doesn't progress in some areas and it is because of the Arnold Chiari ii. I don't understand why the doctor's cannot see this, one doctor said to me the other day that Arnold Chiari is just part of spina bifida, true in some cases, but Faith's is symptomatic. For us, if Faith didn't have symptomatic Arnold Chiari she most likely wouldn't have developed syringomyelia and Faith wouldn't have had so many challenges. It just frustrates me sometimes when doctor's fail to see or just can't. Our neurologist and neurosurgeons understand it because not only have they physically seen it in Faith they have seen it in the multiple MRI scans. But I digress, I think my brain needs a print off function so that when I have lots to say I can print off the emotions, frustrations and feelings. It usually happens late at night and then if I could print it off I could then refer to it later or recount ideas I had during the dark hours of the night. I am always thinking of ways to help Faith progress further to grow and prosper.

Joys of our garden

With a year of oxygen we have learnt many things about patience, perseverance and appreciation of the little things. Life is more fragile that I had imagined but has more beauty to be found when I know where to look. Life is precious. Faith loves going outside and watching the breeze touch the leaves and the birds sing a song as they feed in the trees and talk to each other. She is so intrigued with the world outside. She watches from our deck but hopefully soon the time will come and we will have a low set house so we can go outside and touch the soft grass, watch the ants march by, smell the flowers and watch nature in action close up. I just have to be patient while we wait for our house to sell. But when it does the world will open up a little more for my husband, Faith and I. Again patience and trust in God is a must. (James 1:1-8) Time will pass and hopefully sooner rather later Faith will be off the oxygen and she can be free from one less tube.

Spina Bifida Clinic

Time passes as a blur sometimes and I find it hard to define time and stages as I just keep going and going. My brain is consumed by helping Faith move forward, to gain weight, to have feeds on time, to exercise muscles, practice speech and time passes. But, I must remember my husband too and make time for him as he is my best friend and without him I couldn't do this. It is so exciting Faith hasn't been in hospital since November (3months) and that is a big achieve for us. I hope it continues. It is so nice to just have a routine. To plan and follow through. Faith is sleeping pretty well at the moment but we are still having the challenge of UTI's. Will it not end? I only know two other families whose children have spina bifida and they live a great distance away. It would be so good to communicate with others who have been through similar experiences and know the day to day struggles and be able to share life with ideas on coping and moving forward more.

We made a phone call to the urology nurse and she spoke to the urologist and he said we should catheterize more frequently. I can't see the reason in that considering when we do catheterize there is barely 10mls of wee, in my opinion I think we should be stopping catheterizing as Faith's nappies are always heavy now. We have a spina bifida clinic in a week, so we can talk to him about it then.

I am not looking forward to going to Brisbane as not only do we have spina bifida clinic, Faith is having a sleep study, neurosurgery appointments and other ones too. So it will be 3 days of appointments. And we have just discovered another UTI so we have put her on antibiotics but the vomiting has increased and when we usually go to Brisbane and have such big days Faith's vomiting increases due to stress and lack of sleep. I think sometimes the doctors and nurses forget the stress it causes on the patient and then on the families. This is an ongoing concern we struggle to communicate across to the Brisbane hospital. It is wonderful they want to keep such a close eye on her but she is well now and since we don't live locally that needs to be considered. Since Faith is still on oxygen we have to take 5 bottles of oxygen with us so we are prepared. I am finding all the extra planning stressful. I am so grateful my husband is taking time off work to come with us.

We have booked hospital accommodation and we arrive after lunch, as Faith's first appointment is the sleep study that night so we don't have to be there until 5pm. Last time Faith spent the first hours there screaming from all the cords, wires and prodding so I am feeling quite apprehensive about this one. Faith might be 19th months now but since she has a UTI she is more clingy and wanting to be left alone. She has a temperature and increased vomiting too. We had a very helpful nurse who let us give Faith a bath first but then came the preparation of all the cords and wires. She cried and cried, there was nothing that could calm her. It took quite a while for her to settle, hours actually. Around 1am an alarm went and woke her up and that was it, we spent the next two hours with Faith happily chatting, it was beautiful to hear but it is a sleep study not a talking study. Around 3-4am Faith went back to sleep until 5:30am at which point they decided to finish the study and send us back to our accommodation; hopefully to get a few hours sleep before the days appointments started. Sadly Faith still needs the oxygen and will be reviewed again in 6 months. I knew it was a possibility as Faith hadn't gained much weight but I had hoped and I will continue to hope.

When we were just about to leave to start the days appointments Faith pulled out her NG tube which of course meant we had to put it back down and run late for all our appointments. The exciting news is that the spina bifida clinic went really well and all the doctors were happy that she was starting to progress now. She did spend the entire 3 days vomiting off and on.  A couple of the highlights were no more catheterizing. We were to catheterize 1 a day for a week and if the wee continues to be in small volumes then, ready for it, no more catheterizing. Yay!!! The neurologist said that we can start really slowly weaning Faith off some more medication. She has been on so much so we are very pleased. The other exciting news was that Faith is to get AFO's, at last her feet are bigger enough. And when that all goes well we can look at a standing frame. Love the positiveness! Usually spina bifida clinic doesn't give us good news, so this is wonderful! What exciting days to come!

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?

Home!

With such excitement we are home! Our house had been decorated with welcome home signs and flowers. It was 9 weeks since I have been home and it feels so good, though strange. I brought Faith straight upstairs and showed her the house and her room, took her onto the deck pointing at this and that. So good to be home. Our Faith was with us at last. Over the next few days and nights we felt Faith wasn't feeding well and she is such an unsettled baby. She sweats a lot and pulls her legs up. We just assume this is the way it is. My husband had to go back to work the next day so it was just Faith and I. It was nice though I was exhausted. Being out of the hospital environment was fantastic. Friends and family visited and brought presents for us and we also got yummy meals and we felt loved and cared for. We managed to get a baby health nurse to come and visit and weigh Faith and talk to the nurse about feeding issues. She said that there is a feeding and sleep clinic we could come too and managed to get in the following week. The first few days at home were busy, we had the wound nurse coming most days. I would change the dressing but she would just check it and provide the supplies we needed. It was looking so much better. The spina bifida site was healed completely now, it was just where the flap of skin had been moved that needed to heal.

A few days later we went back to Brisbane to see the plastic surgeons and Faith was upset most of the way and cried a lot. The plastic surgeons were happy that the wound on her back was healing slowly but well. We were told just to keep on going with what we were doing. It was a long day but we successfully made it through and were very happy to get home. Faith is our first baby so lack of sleep is something we both expected but she was always restless and cried so much. We got go to the sleep and feed clinic and discovered that Faith had a uncoordinated suck and swallow so even though she looked like she was feeding well, she wasn't getting much milk. We tried bottles and holding her cheeks together to give her some support and she sucked like crazy. Our baby was hungry! I felt terrible, I was trying so hard. Breast feeding was over and expressing again begun and bottle feeding was the way to go. Whatever is best for Faith. Expressing, bottle feeding and caring for a baby. Crazy times.

One day when we were on a play date and I was holding Faith, the little boy we were visiting said, Faith has a lemon shaped head doesn't she and I said, we all have different shaped heads, yours is like an apple. We are all unique. We celebrated Faith's dedication with our church family in December and had a lovely BBQ lunch afterwards. It was really special for our friends and family to see Faith and how she was doing. Faith wore the same little white dress with the intricate details as I wore at my dedication, that was special. It was nice to have these little bright spots in our calender with something good. As most days were not good at this point. Faith clings to me and doesn't like to be held by many others but she loves to watch what is going on. She was also getting quite picky on who was to give her her bottle, it was only my husband and I.

Faith seemed like she was in pain as she pulled her legs up a lot, it was hard to figure out what was going on. We were also a bit concerned as Faith took so long to feed, we would sit there for sometimes an hour feeding her. The uncoordinated suck and swallow seem to cause her problems. At other times she would drink the milk really fast and sometimes she would vomit it up. I wish that the hospital had picked this up so we could have figured it out before we left. Time passed and we had horrid nights and the occasional good nights. We didn't go out much as Faith never seemed settled. By December, life still hadn't improved and we started noticing a twitching in her hand and particularly her thumb and the sweating was still profuse, all over her body. Infant's friend syrup seemed to help a little with any wind pain but not much. We saw our other friends with the same age babies not having so much trouble and wandered what was going on with Faith.

On this particular day that we noticed the twitching, we contacted our local pediatrician and he suggested that we should do some tests to see what was going on. A EEG was conducted and it came back clear. An ultrasound of Faith's head was done to check if the shunt was working and it was fine. But still no success. Faith was admitted to the local hospital for observation. The doctor decided to put her on Tegretol and see if that helped. Amazingly it did and we saw a marked improvement in Faith's health. But what was causing these focal seizures and what other investigations were going to be done. We ended up back in hospital a short time later with seizure activity. It generally happened in the morning, Faith would get so distressed and scream and pull her legs up in such intense pain and then her foot would start twitching and she was covered in sweat. All we could do was hold her close and do our best to comfort her. The local hospital had no idea what was going on and was not much help. I remember one nurse saying, just give her a bottle and it will better, but a bottle wouldn't help with a focal seizure. The paediatrician called the neurologist in Brisbane and they put her on more medication, Keppra this time. It didn't seem to help and we thought we need to get to the source of this problem not keep giving her more medication.

We had a trip to the spina bifida clinic in Brisbane and saw a urologist who said that Faith was retaining some wee in her bladder but didn't think we should catheterize just yet. We were keen to see the neurologist as Faith still had these terrible focal seizures in the early morning. It was decided a MRI would be booked for January to look and see if the Arnold Chiari ii malformation was causing this problem. In the mean time we just have to make do. We were not sure what was going on but Faith was not enjoying life she was either vomiting or in such distress nothing would help. The orthopaedic surgeon saw movement in her legs so that was encouraging.

Christmas week was the best Faith had ever been and maybe we were turning a corner. We just wanted a quiet time as we wanted to enjoy the peace but it wasn't to last. New Year's Eve she got worse, this foot twitching and pain or whatever it was got worse in a different way with a new intensity and we knew it had to be something serious.