Two days after we got home from hospital, Noah became very irritable. Jalen had reflux when he was a baby, so we thought we were unlucky to have a second baby with reflux and tried all the remedies we could think of. A GP even sent us off for a barium swallow, which confirmed that he had reflux so he went on different medications, but nothing helped. It seemed that whenever Noah was awake he was screaming uncontrollably. He would arch back, stiffen his whole body and nothing we would do would relax him. If he would wake in the night for a feed he would have a little feed and then scream solid for two hours before eventually wearing himself out and going back to sleep. Nothing could consol him.
When Noah was around 4 weeks I kept waiting for his first smile. This didn’t come and he continued to scream. I also started to show him toys, hoping that he would start to follow them with his eyes, but it seemed that he was so upset by the ‘reflux’ that he couldn’t do anything except scream. Finally after 5 or 6 weeks of trying different medications for reflux we were referred to a paediatrician, but was three weeks before we could get an appointment to see him. Noah was now 9 weeks old.
I took Noah into the paediatrician’s office and he asked me if that was Noah screaming in the waiting room. I told him it was and he said ‘it definitely sounds like we have a very unhappy boy’, and asked me a lot of questions. I told him how Noah would start to settle after screaming for hours and then he would ‘yawn’ and arch back and then scream again. I told him how he vomited all the time, and how he would hold his hands in fists all the time. I told him how he always had his body turned or twisted to the left side.
After examining Noah the paediatrician said that he wanted us to get some tests done – EEG, MRI, and a CT scan. He said that he thought that Noah was ‘fitting’ (having seizures). I was shocked as I thought I was taking my baby in with reflux, but was being told he may have epilepsy and asked when he wanted the tests done (thinking that he would say next week) and he said ‘today’. I couldn’t believe that he wanted them done so quickly and I asked if it was okay if we did them the next day as I wanted to go home and tell Aaron first and process what we were being told, and he said to come into hospital and he would arrange everything.
I went home and Aaron what he said and said that it sounded like he may have epilepsy and we were of course upset, but thought things would be okay. The next day I took Noah into hospital. One of the nurses took Noah from me to ‘give me a break’ and took him up the hall so I could fill out the paperwork with another nurse as we were being admitted overnight. I could hear him screaming up the hall and knew exactly how to settle him quickly but the nurses insisted that I have a break and ‘relax’, but I couldn’t as I knew that I was the only one who could settle him.
That afternoon we had a CT scan done. The man doing the scan kept asking me lots of questions about my pregnancy and birth asking me if I had a traumatic birth etc. I told him that everything was fine and he went and got a doctor to take a look at it. They both looked at the screen very concerned and I heard the word ‘haemorrhage’. They told me that the results would be given to my paediatrician who would talk to me about them later on and it was then that I really started to worry as I knew something was very wrong.
Later in the afternoon our paediatrician came to see me in the hospital and told me that they wont know anything for sure until the next day after they did an MRI, but it did look like there had ‘been a change in his brain’. I asked if it was serious and he seemed to avoid my question and just said that we would know more the next day.
The next day we had the EEG and MRI done. A resident doctor from the ward actually came with us when we had the MRI done, and I knew that it was pretty serious, and he wouldn’t look at us very much, and kept saying ‘you know there has been a change in his brain right?’. Finally our paediatrician came onto the ward in the afternoon and took Aaron and I into a room to tell us the news. He showed us some scans of a normal brain. He pointed out the white and grey patches and showed us that a normal brain has lots of white and grey showing the parts of the brain.
He then showed us the scans of Noah’s brain and said that when I was pregnant with Noah it looked like he had a stroke which caused him to have a lot of fluid in his brain. He showed us how dark Noah’s brain scans were compared to a normal brain, and pointed out that this was the fluid. He said that there was no way of knowing why it happened especially as I don’t smoke, and don’t drink alcohol and am healthy.
We sat in disbelief as we listened and I said ‘so he has brain damage’? He said that he did and that there are different degrees of brain damage – ranging from someone who is developmentally delayed to someone who is reliant on others for everything. He said that Noah was at ‘this end of the range’, and he motioned with his hands showing us the left side. I asked ‘you mean the worst end?’ and he said ‘yes’.
He then told us that Noah would never walk or talk, will never be able to go to the toilet on his own, is probably deaf and blind, won’t be very responsive to us and will rely on us for everything. He also said that his body would become stiffer as he got older.
I listened to this and although I was shocked and sad I actually felt relieved as I knew that there was something wrong with him, and was glad to finally have some answers. Our paediatrician then said that because of the amount of damage to Noah’s brain, that Noah’s life expectancy would be greatly shortened.
I asked him how long he expected he would live for and he said that you couldn’t really say. I kept pushing for an answer and asked if he would live to be a teenager and he said that he wouldn’t. I then asked him again how long he would live for and he still didn’t want to give me a figure. I said ‘well, will he live to be one or live to be twelve!?’, and he then said ‘probably two to four years’.
Aaron and I sat and cried together for a while as we tried to take it all in. The doctor told us that his condition was called ‘hydranencephaly’ – ‘hydran’ meaning water or fluid and ‘cephaly’ meaning brain which meant that the brain was replaced with fluid. So instead of him having hydrocephalus where there is a build up of fluid inside the skull, most of his brain was actually gone and replaced with the fluid. Some kids with hydran suffer from hydrocephalus as well, but Noah has been lucky and hasn’t needed a shunt, but has microcephaly instead (a small head).
The same day the doctor started him on phenobarb for his seizures and we took him home the next day. About two days later Noah was a completely different boy – he no longer screamed all day, but actually made baby sounds and even smiled now and then. We realized that most of the screaming was because of the seizures he was having and the phenobarb definitely helped him.
Because of his condition Noah had many health issues. He suffered from spastic quadriparesis and used a wheelchair full time. He ccould not sit up at all or control any of his movements. He had been assessed as to having the physical and mental development of a three month old baby. Because he ccould not move, he relied on us to move his body for him, both day and night to not only keep his muscles and limbs moving as much as possible to keep his muscles loose and for comfort, and also to relieve any pressure on his body to avoid pressure sores. In the past he had needed treatments such as Botox injections to relax his muscles, followed by serial casting for six weeks at a time.
He was fed continuously using a pump via a PEG tube day and night. When he was four months old he just stopped breast feeding one day and could no longer feed orally. The doctors said that he was just worn out from feeding and after trying to train him to feed with a bottle, a ng tube was inserted. A month later a PEG tube was inserted and since then he has been fed via the tube as he started to aspirate whenever he was fed orally.
Noah suffered from GERD (reflux) and has had an operation called a Nissen Fundoplication to prevent him from aspirating (which he often does), which results in aspiration pneumonia and hospitilisation. He needed to have medication five times a day including up to eight different medications for seizures, muscle spasticity, secretions and constipation. Because he doesn’t feed orally he requires oral care numerous times a day, and needs to have his mouth swabbed and cleaned frequently.
He had a lot of lung issues and has acquired bronchiectasis which is destruction and widening of the large airways. Because of this he required four treatments every day via a nebuliser which also includes physio sessions twice a day of postural drainage to remove bronchial secretions, which was done by us, or his carers or aides when he is at school. These sessions take up to twenty minutes morning and night. He required suctioning around the clock and we use a portable suction machine that needed to be taken everywhere with Noah. He also needed three different puffers, twice a day for his lung condition.
Noah had been admitted to hospital over fifty times, and many of these times have been for pneumonia and other chest infections.
Noah had severe scoliosis and wasn't a candidate for surgery to correct it, because the doctors do not feel he could cope with such a big operation, and probably wouldn’t pull through it. The curve in his spine was so severe that it is pressing on his internal organs, and compromising his lung function even more. The only option to treat his scoliosis has been to have a custom made body splint made for him by a company called ‘Second Skin’. The suit cost almost $4000 and we have had to rely on funding from charities to cover the cost of having a new one made every 12 months or so.
Noah had gone through precocious (early onset of) puberty which has meant he has had massive growth spurts. This has also meant that the curve in his spine has increased very quickly as his body had not coped with the growth spurts well.
Noah's body was often very stiff and needed physio therapy and massage every single day to keep his limbs moving and to keep his muscles as loose as possible, to avoid getting contractures. This took a lot of time and was done at home every day, after were shown by his physio what to do. 
Noah used nappies full time and had neurogenic bladder, which meant that his brain wasn't sending signals to his bladder when to empty. This meant he needed assistance to use his bowels and bladder including giving enemas, laxatives, bowel massage, and also doing bladder massage to get him to empty his bladder properly or he gets frequent urinary tract infections. Without the assistance of us doing a bladder massage, he was unable to urinate and has bladder spasms making it even harder for him to go.
Noah had intractable seizures (seizures that are difficult to control), and had them since birth, and possibly had Lennox Gastaut Syndrome. He trialled many different medications to try to stop the seizures, as well as going to the Royal Children’s Hospital in Melbourne numerous times to try alternative treatments such as the ketogenic diet. Unfortunately he still had seizures and had probably only had five days in total in his whole life, when he didn't have a seizure. On average he had around five seizures per day, and on a bad day can have up to ten or even more when he was sick.
Noah also suffered from osteoporosis and had three fractures in three years, just from slight knocks. He had one treatment of bisphosphonate therapy to increase his bone density, but had a lot of bad side effects from the treatment, so he didn't have a second dose.
Noah was cortically visually impaired, which meant that his eyes are okay, but his brain didn't register what his eyes were seeing. We did a lot of vision work with him to assist him in tracking and seeing objects and he also did vison work at school. He is lucky enough to be able to go to two different schools - part time at a special school and part time at a mainstream school. When he goes to his special school he catches the bus to school.
Noah communicated to us in his own way. We knew when he is happy, upset, in pain or relaxed, by the ‘baby’ sounds he made and his body language and eyes. Even though we were told he wouldn’t recognise us or respond to us he knows his family and familiar people and did respond with a smile or a look when you talk to him, or touch him.
We have been told numerous times that the reason that Noah lived so long with his condition is because of the love and care that we gave him. We were proactive in seeking treatments for him including alternative treatments such as a seeing a bioresonance therapist and chiropractor regularly. 
He had regular oxygen energy therapy. 
As Noah got older he became more and more tired, and would sleep more often. His health was stable, but he needed a lot more suctionining and help with his bowel and bladder. During the 12 months before he passed away I knew that Noah wouldn't be with us much longer as everything just seemed so much harder for him.
The month before he passed away I felt that his body was starting to shut down. He was getting puffier and puffier and even after starting new medications to get rid of the extra fluid he was still puffy and was having a lot of trouble breathing although he didn't have a chest infection. His body temperature also started dropping a lot more than normal. After a month of him being unwell, but not really sick he woke up one Saturday morning having trouble breathing. We knew straight away that he had a chest infection and it looked like he needed to go to hospital, but after we got him out of bed and put him in a certain position his breathing would settle down. By lunch time we knew that his breathing was getting worse, so I took him into Emergency at the hospital where they straight away took him through, did an X-ray and saw that one lung was completely collapsed and said he had aspiration pneumonia.
They rang the ICU and anethetist straight away and decided that he needed to be ventilated and transferred by plane to the Royal Hobart Hospital paediatric Intensive Care Unit. After a stressful few hours they said that the procedure went well and that he was sedated and that the venilator was now breathing for him. We saw him before he was transferred to Hobart and he looked so relaxed and peaceful after having trouble breathing earlier on.
Aaron and I came home and packed our bags and drove straight to Hobart while my Mum stayed with our other boys. We actually beat Noah to Hobart by about half an hour. He then spent the next week in the NPICU at the Royal Hobart Hospital where he was so well cared for by the doctors, nurses and staff there.
It was his first admission to ICU and we were so grateful that the doctors there treated Noah just like they would any other child. They gave him every chance of getting well, so that he could get off the vent and breath on his own, but as the week went on it became more and more obvious that Noah's body was shutting down and his fight to live was coming to an end.
On Saturday the 8th of October, 2011 we had to spend the day saying goodbye to Noah which was the hardest day of our life. All five of us were with him when he passed away and it was very peaceful. His funeral service was held on Thursday 13th October, 2011. It was a beautiful day which we tried to make a celebration of Noah's life because we know how blessed we are to have him in our lives.
Life for Noah was definitely very hard at times, but we wouldn't have changed the past 10 years and 3 months for anything. We are so grateful for every single day that we got to spend with him on earth We try not to count the days that we will have with Noah on this earth, but tried not to count how many days we would have together, but to make each day count. We are so glad that we know we will be with him again one day.
It was his first admission to ICU and we were so grateful that the doctors there treated Noah just like they would any other child. They gave him every chance of getting well, so that he could get off the vent and breath on his own, but as the week went on it became more and more obvious that Noah's body was shutting down and his fight to live was coming to an end. 
Life for Noah was definitely very hard at times, but we wouldn't have changed the past 10 years and 3 months for anything. We are so grateful for every single day that we got to spend with him on earth We try not to count the days that we will have with Noah on this earth, but tried not to count how many days we would have together, but to make each day count. We are so glad that we know we will be with him again one day.
