I honestly do not know where to start. Finley has totally enriched our lives but inevitably when a diagnosis like this is made, with it there comes pain and mortifying revelation. Our world changed dramatically when he was diagnosed with Spinal Muscular Atrophy Type 2 aged 13 months old in June 2014. This is a very potted account of our journey and the emotional rollercoaster that is now our every day normal life. I have kept my promise to write this as I hope it will help gather evidence of how important hope for a better future is but it is desperately painful to recall the events of repeated intensive care admissions not knowing if my baby would survive… ..through no ones fault but because of genetics. SMA, the crazy odds of it, the adaptations that we as a family have to undertake for the all encompassing love of Finley who means so much to us all as a son, a little brother, a grandchild, a nephew, a cousin and a friend.
I gave up my position of Adult Community Nurse, the career that I had studied hard for whilst I was a single parent to my older son aged 13 at that time. It was the right decision as the emotional pressure of learning my child was severely disabled with a potentially life limiting condition was a devastating blow rendering me a blubbering mess for several months. My partner Joel (Finley’s dad) was now the ‘bread earner’ but the stretch on his income was and always will be, cause for anxiety with extensive house renovations, suitable vehicles and essential equipment needing to be sought. We tried to remain positive about things after the initial shock, throwing ourselves into living and hoped that although life would be different to how we had ever anticipated it would all still be manageable.
Almost a year later on the 5th June 2015 Finley became very unwell. To be honest I cannot remember the ins and out now, I think that my mind has blanked it out to try and protect my sanity. I remember it was horrific though. A respiratory virus lead to him gasping for air like a fish on the river bank….a memory burnt in to my brain now. He spent 3 weeks in the paediatric intensive care unit (PICU) intubated for 10 days and a hospital stay until the 14th of July 2015. He came home with a ward full of equipment. A Nippy ventilator and humidifier for use at night, a cough assist and a monitoring (SATS machine) and the worst of all a nasogastric (NG) tube. His swallow had weakened and we were now only able to feed him small amounts of pureed food. My chubby little boy had loved his food, blueberries in particular and the pain that is associated with this is still exceedingly raw (the flood gates have now opened as I write).
Our next PICU admission was on the 12th September 2015, intubated once more and discharged on 29th September. I will never for as long as I live forget the Speech and Language Therapist telling us that Finley now had an unsafe swallow and that they were limiting to either 2 teaspoons of food per day or ideally none at all. Joel and I sobbed and clung to one another realising that Finley’s health had deteriorated despite the fact that he was so incredibly full of life. We decided that it was cruel to give Finley this tiny amount as he cried for more and decided to go nil by mouth….a decision that was completely right at the time but now I am worried about the ‘use it or lose it’ scenario related to muscles. I have reoccuring dreams that I had forgotten to feed my child and wake in a blind panic, then coming to and realising the awful truth, I am not able to fulfull one of the most basic functions of keeping him alive and he was now fully reliant upon an Abbotts pump and manmade milk to keep him nourished. For me it was and still is one of my most painful symptoms of SMA to date.
On the 28th November 2015 we were back in PICU once more intubated and feeling like life was just unbearable but managed to console ourselves as we were discharged a few days before Christmas on the 23rd December. Christmas used to be a lovely big family affair but it was downscaled significantly due to our fear of Finley coming into contact with any other germs. This, as adults was a small adjustment but for my older son Kyle must have been another big disappointment. We were also in hospital for his 15th birthday so meant that we had not had a proper celebration . Each time we were admitted it meant that my mum came to live in our house to care for Kyle who was a young teenager and Joel and I lived on the hospital site at the fabulous Ronald McDonald house. Our family was ripped apart with every admission and it was at this time that I realised Kyle had started to suffer from mental health issues. He had become withdrawn and his school work was suffering. I had been far too busy caring for Finley to fully appreciate the impact that this was all having upon him and in turn made me feel terrible for neglecting him. I was (and still am) torn between the two of them, loving them equally but Finley’s severe physical health needs always seemed to take priority over Kyle’s mental health, the unseen. My mental health has been fragile since Finley’s diagnosis and still can be but like many of us SMA parents we plod on as there is no other option. I have seen a psychologist when Finley was in PICU and it just became this massive sobbing ordeal which if I was feeling stronger and more positive I did not want to have to face. When I did though I did feel its benefits offloading as I was protective of burdening family with all my angst. Kyle is now seeing a counsellor and is working through his anxiety and self worth issues and it also seems to be helping. Joel throws himself into his work and is an all round super hero but suffers from sleep deprivation which is almost certainly down to our home issues.
The 24th January 2016 and my birthday, blue lighted once more into Intensive Care and intubated. Happy sodding new year. I think it was this admission that I met a mother of a Type 1 baby who had just received a diagnosis, he passed away and I took this almost personally feeling desperately sorry for their loss and almost guilty that we still had our child. Unfortunately when in this environment you become acutely aware of other children and their parents battles to keep their children and we witnessed the stuff of nightmares. It all takes a massive toll and you cry for not only your child but all the other children that are not lucky enough to survive. When your child has had a good day you want to be smiley and happy and tell people but you are also conscious of the fact that the family in the next bed may not have had such uplifting news. The emotion in PICU is exhausting and after just a day down there witnessing all the heartache, worrying about both my children and Joel juggling work and being a present father to Finny we both became shadows of our former selves.
The 25th April same again, intubated once more and Finley celebrated his 3rd birthday downgraded to the paediatric high dependency unit (PHDU). I had planned a trip to the zoo for his birthday which for us is and was a big plan…..the night before his birthday I was so sad but we managed to escape (with lots of new toys!) on the 27th May.
Happy to say that the following admission was actually scheduled! We had PEG surgery on 18th August 2016 and have never looked back. The change from NG to something more permanent was long overdue and meant no more sticky plasters on his face, traumatic monthly tube changes and the sometimes unnecessary comments or looks from Jo Public. A trip into hospital was met with my usual pit of the stomach anxiety, What if he did not react well to the general anaesthetic? Would I cope seeing him with the horrific ventilation tube in his nose or his mouth? Would he pick up a bug whilst in hospital and would surgery affect him after having such a good run of health? Luckily I did cope and he did marvellously and was discharged after only 2 nights.
The next admission was 16th December until 20th December…..4 nights just in PHDU with a more powerful ventilator and the first time we had ever avoided PICU. Kyle turned 16 on the 19th December so again we were not there for his birthday but atleast all together again for our second very downscaled Christmas.
Our most recent admission was our hairiest yet. Being old hands in PICU never makes it easier. Finley was admitted in the middle of the night on the 19th May 2017 after a very rapid deterioration. I was at home with the two boys as Joel was working away and I had to rouse Kyle to tell him that an ambulance was on its way for Finley. I had been dreading another admission as Kyle was doing much better. The timing was dire once again with Kyle’s GCSE exams starting on the 22nd May, Finley’s 4th birthday on the 25th May and our building work being started in June. Finley spent 10 days in HDU, ‘celebrated’ yet another birthday there and we thought we may avoid PICU but he became oxygen dependant and an x ray showed the whole of his left lung had collapsed. The sinking dread came back and he spent 3 weeks intubated in PICU, also experiencing a chest drain due to a punctured lung. I and some staff were unsure that we would even get him back home……. thankfully we did and after a 6 week admission we took our precious baby boy back to Joel’s mums as our house was inhabitable as the accessibility renovations were underway.
Joel and I are once more feeling the horror of Finley’s SMA. Spinraza could improve his respiratory function and knowing this is truly agonizing. He is unable to access the drug as he was able to sit independently therefore categorised as a Type 2 yet he displays far more Type 1 symptoms. I was relieved when he was not a Type 1 yet I find myself in the very uncomfortable place of wanting him to be a Type 1 child now as his chance of getting on the Extended Access Programme (EAP) is greater. His, hands, arms, legs and neck are all very weak, he cannot eat, needs help breathing and I want him to live a life where we do not all live in fear….every day of our lives. I am brave and I will continue to be but I am terrified.
Finley will be starting school next month in September 2017. He will be very part time, we would love him to go more but we are walking that tightrope between education and social development and precarious health. I hope that he can increase his immune system whilst there but am always anxious about how ill he could get whilst building it up! I worry about social isolation, we do not see our friends very often for fear of coughs and colds but I am overtly aware of not keeping him in a box.
Finley is ridiculously happy to be alive and is full of vitality…….I marvel at all he has gone through and feel that I need to take a leaf out of his book. Anyone that meets him adores him, he never stops smiling and has an incredibly positive, sunny dispostion. Finley has accepted hospital life far better than we have, the nurses are now so well acquainted with him they are his friends so he will be more than happy to go and get Spinraza administered!!!! As always it’ll be us the parents and family who are grey with worry by their bedsides but I would do it all again in a heartbeat for him. Our children are our world and deserve a brighter future. The adults with SMA deserve to be able to experience or to re experience more mobility and health. The results we are seeing are life changing and I am genuinely thrilled every time I see SMA people achieving the ‘unachievable’. Here’s to 2017 and more hope for the SMA community who are all exceedingly worthy.