Noah's Story

Noah was born almost two weeks past his due date (26^th June 2001) after a normal pregnancy and birth.  Everything seemed fine after he was born, except for a little trouble with his breathing, so he was taken to the special care nursery for a couple of hours.  On the third day in hospital, we took him home. 

  Two days after we got home from hospital, Noah became very irritable. Our oldest son 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.  Our doctor 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.

  At around 4 weeks I kept waiting for Noah’s 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 pediatrician, but it took three weeks before we could get an appointment to see him.  Noah was now 9 weeks old. 

  I took Noah into the pediatricians 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 pediatrician 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’.  I 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 and he said to come into hospital and he would arrange everything.

  I went home and told my husband what he said and said that it sounded like he may have Epilepsy and we were upset, but knew it 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’. 

  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 ‘hemorrhage’.  They told me that the results would be given to my pediatrician who would talk to me about them later on. 

  Later in the afternoon our pediatrician 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 and finally our pediatrician took my husband 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.  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.  I said ‘you mean the bad end’? and he said ‘yes’. 

  He then told us that Noah would never walk, 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 actually felt relieved as I knew that there was something wrong with him, and was glad to finally have some answers.  Our pediatrician then said that because of the amount of damage 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 and he said ‘probably two to four years’. 

  My husband and I sat and cried 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.  This didn’t really sink in until afterwards – after we found the Rays of Sunshine website and email group. 

  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. 

  His seizures have  kept on changing and since then we have tried many different medications to help these, including phenobarb, epilim, vigabatrin, valium, clonazepam, clobazam,  neurontin, keppra, and lamictal.  Noah has a lot of myoclonic seizures everyday which are often set off by a loud noise, or something startling him.  When he has one of these seizures his whole body will just jump and his head will turn to the right side (it is usually turned to the left).    At one stage he had over 100 a day and we attempted the ketogenic diet to see if it would help in reducing his seizures, but he could not tolerate the high fat and kept on vomiting.  We have found that Noah’s seizures are best controlled with a mix of three different seizure medications and now and then we have to change the doses. 

  Noah was breast fed until he was 4 months old, when suddenly it seemed that it was too hard for him to keep feeding.   We persisted with a bottle for a week or so, until we decided with the doctors that a feeding tube would be best for both Noah and us as it was very difficult to feed him and also to give him medications without him coughing and going blue.  At 5 months old Noah got a PEG tube which was changed to a button a year later.  We LOVE being able to feed him this way and think it is the best thing we could have done for him as it has made life so much easier both for feeding, giving medications and also when he is sick as we can still feed him and know that he is getting enough of the things he needs to get well.

  Noah is very severely disabled, but things aren’t quite as bad as the Drs first predicted.  He has had his hearing tested and it is normal except for a slight loss with high pitches.   He sees light and dark and sometimes visually tracks objects and seems to look at things now and then.  He always turns to you when you talk to him.  When he is well we get a lot of smiles every day and he also laughs a lot.  Every morning when he wakes up he ‘calls out’ to us and we go in and say good morning and every morning he gives us a big smile as well as whenever he wakes up from a nap.

  He has a real personality and will laugh at the silliest things.  At the time of writing this he has a younger brother Harrison, who is now 6 months old.  Whenever Harrison cries, Noah thinks it is very funny and will laugh!  He ‘talks’ back to us by making an ‘aaah’ sound whenever we talk to him.  When he doesn’t like something he will always arch back.  If he is on the floor and he arches you know he wants to be picked up, if he is being held and he arches he wants to be put down and he ALWAYS arches when he is in his car seat as he always wants to get out.  Noah only ever cries now when he isn’t feeling well. 

  He loves music – especially rock music with lots of drums and guitars and will kick his legs and get so excited when he hears it. He also loves to just lay on the floor and kick and move his arms.  We have some toys which hang over him, and when he lays under them he kicks so much that he moves himself across the floor!  These ‘hanging toys’ used to be his favourite thing, but now the thing which he loves the most is his ‘Little Room’.  We have been very lucky to have Noah’s school purchase a Little Room which we are able to use at home and Noah will literally stay in there for hours if you let him.  We have put a lot of different objects in there including bells, wind chimes, and different things for him to touch and feel as well as coloured lights.  Often when we pull him out to do something different he will ‘yell’ at us to let us know that he wants to go back in there!

  Another thing that Noah loves is water – he loves to go swimming and also loves the bath.  He splashes the water by kicking his legs and always smiles and makes sounds when he is in the bath. 

  Noah goes to school (Early Learning) one afternoon a week which he loves.  He especially loves listening to the music, and being bounced on the trampoline. 

  We are so lucky to have found a wonderful chiropractor who sees him twice a week and has helped him so much. She uses a technique known as Sacro Occipital Technique.  This has helped Noah to be a lot healthier and we notice that he gets well a lot quicker.

  He does have high muscle tone but we do a lot of physiotherapy with him at home which is mainly just a lot of stretches as well as massage.  So far we have been very lucky and he has no contractures. 

  Noah has an Invacare Action Orbit wheelchair which we love.    He sits in it so well and seems to love being able to sit up and be a part of everything.  We have a lot of different equipment which we use at home for him including a prone wedge which allows him to lay on his stomach, a stander, a symmetrisleep system (which helps to place him in a good position for sleeping),  a hopsadress (like a jolly jumper for children with disabilities) and a hospital bed.

Itt has been hard work to get him to be so happy, but it has been worth it.  He is on a lot of different medications now for his seizures, reflux, secretions, constipation and muscle tone.  All of these meds have made Noah’s life a lot easier and helped him to be happy.  We have learnt how to use a nebuliser and suction machine and they are brought out when Noah isn’t well, but most of the time they are away in the cupboard. 

  He has had a Nissen Fundoplication which has stopped him refluxing all the time.  Since having this done he has had a lot less chest infections and has been a lot healthier and happier. 

  We are so grateful to have found the Rays of Sunshine website which has helped us to know how to care for Noah as best we can. 

  We love having Noah in our family and feel very lucky that he was sent to us.   It can be very hard to see Noah sick at times, but we know that the good times definitely make up for the harder times.  He has taught us a lot - especially what is important in life and no matter how long or short his life is here on earth, we know that Noah will be a part of our family forever.