Aidan's Story
(as told by Daddy)

 

We knew our baby Aidan was special from the day he was born- October 12, 2005. On October 12, 2004, my wife and I lost our first ever baby boy Ethan prematurely (at 6 months) to a renal failure, so we knew Aidan was a gift from heaven being born on exactly the same day a year later. He brought such joy to us from the moment we saw him, and I had this strong feeling that he was different from the other babies around him and that he was destined for greatness.  He had the most amazing eyes- so huge and alert, and this was paired with one of the most beautiful baby cries I have ever heard.  I loved him so much.

Just before Christmas, my wife (a doctor herself) took him to her local general practioner because Aidan had some unusual creases or folds on his bottom and was very floppy (which I thought looked adorable-especially when taking him out of his baby bath). The doctor said all looked well but asked if he moved his legs and arms equally. It was only upon leaving that my wife realised that he really never moved his legs that much at all. So acting on her motherís instinct, she took him to her pediatrician Dr McCreanor. The tests revealed that Aidan had no reflexive movements in his arms and legs, so he referred us to a prominent neurologist, Dr Appleton in Brisbane. He was so courteous that he came in, in the middle of his holiday on a weekend especially to see us. He looked Aidan over, and while acknowledging that something was wrong, couldnít detect any classic symptoms of SMA such as breathing difficulties, tongue fasciculationís, excess secretions, problems with feeding/sucking, etc.  Up until this point, my wife and I hadn't realised that his lack of reflexes could be life threatening- we simply believed it could result in a disability of some kind-something we would certainly be able and willing to handle.  

We were referred to our local hospital to participate in some physiotherapy to encourage Aidan to use his legs and arms, and I must say that Aidan developed a special bond with his physio Angie straight away (the flirt). We went once a week, although his mum and I put him through a grueling workout 5 or 6 times a day involving pushing with his legs, rolling, neck strengthening exercises and the like.  I even made up ramps and small tables out of foam so we could try new exercises on equipment. We noticed immediate improvement in his arms and legs, but his head and body was still floppy. On a follow up visit to Dr Appleton he was impressed with Aidanís progress and was happy that he still had none of the usual symptoms of SMA other than muscular weakness. He decided to run an MRI on his spine and brain and also took some blood to fully discount SMA. His MRI came back clear after a scary hospital visit where Aidan was anaesthetized, and we were hopeful. Aidan continued to make progress.

Several weeks later on February 13, my wife called me at work to say the results had come back positive for SMA.  It was the longest drive home I have ever had, and it was as though my life had just fallen away.  Once I got home the only thing I could think of through the endless flow of tears was to go and buy a video camera straight away to capture the endless moments and images that I had fallen in love with- the smiles, the sounds, the movements, everything had to be recorded.

We revisited Dr Appleton the next day who confirmed the worst. I feel so sorry to hear parents talk about how cold their doctors are when delivering the bad news, because he was so gentle and kind, while at the same time not building us up with false hope when telling us what the final outcome would be.  I remember crying all over him, all the while looking at my beautiful boy as he smiled up at me, completely unaware of his fate.

I immediately left my job to join my wife at home as we focused fully on Aidan, because we were given only months.  His diagnosis was a non-typical type 1 or type 2.  Aidan did not typically fit either category, with some symptoms such as fasciculationís of the tongue, excess saliva secretions, feeding and breathing being quite normal.  However almost immediately upon receiving the diagnosis we noticed signs and symptoms appearing that up to that point had been missing or less pronounced- his breathing began to labour, his strength waned, and his feeding deteriorated to a point where we had to put in a naso-gastric tube through his nose. The problem with this tube was that not only did it exacerbate his coughing and secretions, but also practically stopped his gibbering or "talking" which we loved so much. We decided soon after to have him fitted with a mic-key button in his tummy so we could feed him with less intrusiveness. Once the tube was removed from his nose his personality and talkativeness returned better than ever.  After a few weeks we were also loaned a Kangaroo feeding pump for night time feeds which not only helped his secretions but also gave him a much better sleep.  We still fed him via syringe during the day and when we went out but noticed that his ability to take large feeds was diminishing each week.

Soon after we noticed the wallowing or cavity appearing on the left side of his chest as his breathing became more difficult.   His condition gradually worsened to a point where he could not be held upright or even partially elevated, so we sought permission from the police to remove his car seat because his secretions were unmanageable.  We lay him on the back seat with soft doonas and pillows all around with his mum right beside him on every trip.  We had also begun to use glycopyrrolate medication combined with a suction pump to help control his secretions as well. 

One of the most devastating aspects of Aidanís condition was that we were gradually beginning to lose the ability to pick him up for hugs because of his problems with secretions.  One of the great joys in my life was watching my wife pick him up and hug him- this happened with such regularity- in the shops, during walks, around the house.  She was even able to carry him while we kicked a football to each other as he watched from her arms.  It was a testament to her strength that she overcame losing something that meant so much to her- a hug.

It was during this time that we were forced to change Aidanís medication from childrensí Panadol to Painstop in order to control not so much the pain, but the distress his breathing problems were creating.  We were also forced to restrict our movements with him.  Gone were the long visits to the shops, or the long walks.  Aidan was gradually losing his fight.

We noticed that Aidanís tolerance for even small bolus feeds was decreasing.  We began to see regular episodes of small explosive vomiting, through the nose and mouth which was distressing for him and us.  We noticed that these episodes often coincided with him trying to poop.  We had always found it necessary to give him some liquid laxative (due to his formula giving him constipation) as well as holding his legs up to force the poops out, as he often had no strength for this.  Each evening my wife would hold him up, legs bent while I played catcher-sometimes with ugly results!

My wife told me that he would soon need to be on his feeding pump all the time to control the secretions and vomiting.  We made the most of those final few weeks, spending many hours walking near the beach, or around trees which he loved. 

In late May he moved to the pump permanently.  We spent hours watching colourful movies on my laptop, reading endless books and watching the trees in our front yard.  But we also noticed his mood changing.  Almost overnight he stopped talking, he stopped loving his baths, sometimes a day went by with only a handful of smiles.  We knew that his time was close. 

Our last visits to both Dr McCreanor and Dr Appleton were positive.  His lungs were working well and he still had enough fight to convince both doctors that he had a few weeks left yet. 

Around early June, we noticed that he had lost most of the movement in his arms.  However he made great use of his hands performing what my wife and I called ĎAngel Wingsí where he would just flap his little hands with his arms stuck by his sides. 

We also noticed each breath coincided with an audible grunt or moan as the difficulty with his breathing worsened, particularly at night.  My wife and I to that point had been alternating shifts watching over him at night.  But feeling that the end was close, decided to both sleep with him on the floor.  We even moved him onto his own mattress beside ours.   We were forced to administer morphine throughout the day to control his distress as Painstop became less effective.

Aidans last few days were spent sleeping, although he had worked himself into a pattern where he woke each night around 12:00am and stayed awake until 3:00am or 4:00am.  We read to him throughout the day which he loved and usually fell asleep to.

On the night of June 26, Aidan stirred at 11:30am.  I held his hand and read him some children's bible stories.  His mouth was very dry so I gave him a wet mouth swab which he loved and sucked on.

After being unable to settle him, my wife tried around 1:00am on June 27.  I awoke at 3:00am to find her still trying to settle him.  At around 4:00am both my wife and I were still up patting him trying to get him asleep when she commented on how shallow his breathing was.  I noticed his eyes losing focus and we both knew instinctively what was happening.

We brought him to our mattress and each held his hands and sung his favourite song ĎYou are my sunshineí.  I was crying so hard trying to tell him how much I loved him and managed to say a prayer with him.  As his eyes closed for the last time both my wife and I got a very gentle squeeze from each hand and we watched as his chest raised and lowered for the last few times. 

I prayed for so long that I would be there to hold his hand as he left us behind and I got to experience that.  Now two days later I just beg to see those eyes, or touch his face or hair like I used to.  I spent my first morning without him gathering everything of Aidans to collect- his old medicines, unwashed clothes and sheets, even his last nappy.  I just want to preserve my memories of the most cherished person I have ever known.  My first night away from him was spent in his room clutching his old satin and fleece sheet.  I found peace just sniffing it every now and then remembering his smell.  The next morning was our most painful as we awoke realizing that our days are empty and our house is unnaturally quiet without our little guy in it.

At the moment I feel so very lost, defeated and empty.  My wife and I worry that in time his smell will disappear, his voice will be forgotten and we lose those precious memories that we couldnít manage to capture on video.  I worry that I never said how much I loved him enough or that I never showed him how much I loved him.  I keep on praying that he is happy and at peace and that he can somehow see how much we long for him.  I find peace myself in the belief that perhaps one day Iíll get to hold him again.