At the 2018 AGM (my first), I had the chance to meet so many other parents who care for children with HSP, as well as adults who contend with the condition themselves. It was a very helpful experience as I have found myself feeling very isolated at times. I am very fortunate to have many great friends, but even the medics are unfamiliar with HSP, and most specifically the unpredictability of SPG11, which my son has.
I am a Church of Scotland Minister and so I deal with the joys and suffering of life every day of my life. I also serve as a Relief Chaplain in The Prince and Princess of Wales Hospice in Glasgow. The challenges which suffering brings to body, mind, spirit, and faith (if relevant) are familiar to me professionally and personally. I struggle as most do. I have few answers, but my faith remains – most of the time.
It is true to say that life has been tough for a long while. Reader beware, as my story isn’t all roses and light. To give some background, my Dad died of cancer in 1999. In June 2010 I was diagnosed with testicular cancer and had surgery along with chemotherapy. In June 2011 my wife of 19 years left. We had been unhappy for years, but it was especially tough as I was still recovering from my illness. She took our youngest two with her, our eldest stayed with me. My son, David jnr, came home to me when he turned 16. In June 2012 my Mum died in the Hospice. 2013 proved lucky with no big disasters, but then my son started to walk with a peculiar dip.
He had never had any mobility issues. From his early years he had challenges and was diagnosed as having Speech and Language delay. He attended a specialist SLT Nursery and just after he started Primary 1, we moved to our present home and he changed school. He had a terrible six months at his new school – a high achieving school which had reassured us they were able to cope with our child – who had a formal ‘record of needs.’ In fact they were uncaring and ill equipped. With deep sadness I recall the mornings he would beg us not to send him to school, tears streaming down his face, refusing to dress.
Thankfully we managed to secure a place at a specialist Primary unit for SLT children. I had never allowed my son to go on a transport provision as he would have been unable to tell us if something had gone wrong or he had been harmed. However, I realised that I would need to let him be transported at some stage, and believing he was more able to inform us if he was distressed, I agreed to him being collected by ‘the bus’. We saw him onto that bus and worried the whole day about how he was getting on. I can still replay the video in my mind – as the bus arrived to drop him off – and our happy wee boy bounced out, full of fun and excitement. My son was back. In every sense.
He enjoyed his Primary years and the SLT team were great in encouraging him and developing his language and vocabulary. We used less and less sign language and coupled with his slowly increasing language skills, meant he was less restricted and therefore less often frustrated.
He still forgot things. His short-term memory was stunningly poor. His friend would come to the door to ask him to play football in the street or garden and he would go upstairs to change out of school uniform. We would need to go up and check as more often than not by the time he got to his bedroom he would forget his friend was there and sit down in front of the tv or start to colour in. One other element, which was significant in his childhood, was his hatred of balloons and party poppers. It wasn’t the fright – he didn’t fear the fright – it was the noise… it caused him pain. It took us a long time to discern this one, but his hearing receives slightly differently and there are some noises which simply cause him pain – akin to shouting in your ear. Cutlery on a plate has the same impact – it could make him cry.
At 16 and in 5th year of Specialist Secondary Education for Moderate Learning Difficulty pupils, his walking started to show a dip. A boy whose number one passion was playing football seemed to have a strange thing going on with his walking.
He attended the GP and then Orthopaedics for a year. I asked at the first appointment if it could be Neurological and was told it could be, but they needed to rule everything else out first. A year of tests and appointments resulted in us being told he had a slightly abnormal pelvic bone growth but it didn’t explain the presenting issues. We then had 18 months or so attending Neurology and more tests etc. Towards the end of that experience we were advised that it was Becker’s Muscular Dystrophy (BMD) which was a devastating diagnosis.
At the next appointment three months later the next Consultant dismissed the BMD diagnosis and said she wanted to refer him to the Professor of Genetics. More waiting (why is it always three or more months between appointments?) and we went to see the Professor. She was wonderful. She listened carefully, examined him and then told us she was certain it was something called HSP and would be the ‘Complicated’ type – but she would have to prove it and it would take a while. In the end it was 18 months before we had confirmation of SPG11. She also told us it was so rare, I would probably find more information and support from other families rather than medics. She was correct.
The local Physiotherapist has been great, as has the OT from the local Disability team, but they had never encountered HSP. Nor had his GP, who has also been wonderfully caring.
I have been his sole carer throughout these years, although his sisters have been great. I try not to lean on them, but there are nights when I go to meetings and it is reassuring to know the eldest is at home if there are any issues. David is wonderful. I am really, really lucky he has a sunny and positive nature. His learning difficulties mean he is immature – a 10 year old boy in a 22 year old man’s body. He is bigger than me now, which causes challenges when he falls or needs to be moved. I’m glad I did weight training in my earlier years as I learned how to lift!
I have coped with the pressures of single parenting, his decline, a demanding job and personal challenges, but in 2017, I finally had a collapse in my ability to cope and was off work for several weeks. It was at this stage I was advised by social services that by summer 2018 David would need to move into supported accommodation – his needs had, as predicted, reached a stage where I couldn’t cope for much longer. His fall risk was very high as he used his walker in the house and sometimes still ‘furniture surfs’. In the previous months he had needed more and more support. Simple tasks had become impossible. He couldn’t get to his feet, couldn’t put on socks or shoes, couldn’t dry himself properly after a shower. He was almost at a stage where he needed the wheelchair permanently.
He has also developed a stammer. At times almost non-existent, yet at other times he can get stuck (think “Kings Speech”) and say the same two words up to 30 times. He finds this very frustrating but generally copes well with it. We will sometimes guess the word and if correct he will thanks us, but most of the time we just reassure him he can take his time and we are ok with waiting while he tries to get the words out.
I am very fortunate that he was positive about moving to supported accommodation. I miss him like crazy. His laughter is an every present sound in the house – he laughs at cartoons, at Michael McIntyre and other comedians, at anything really. And he sings! Loud, enthusiastically, and terribly – but he is so happy I wouldn’t ask him to stop. I was also fortunate that his sister left home at the same time – so in his mind he wasn’t the only one who was leaving.
The move didn’t go smoothly. He was meant to move in the November, but the care home put their offer on hold at the end of October and then kept us waiting until the week before Christmas, when they withdrew the offer. They said they had not done their pre assessment properly, but in truth I think they were motivated by costs of his future care and the fact that they could find an easier resident. I remain bitter about the way they treated him (and me).
In the following February we identified a Supported Living situation and all parties agreed. However the Housing Association’s lawyers stated I could not sign the tenancy. I was appointed his Welfare Guardian in 2014 (NB Scottish Legal system). It lasts 5 years. It states I can decide where he lives and who looks after him. But it doesn’t explicitly state I can sign a tenancy agreement for him. This has been standard in Guardianship agreements since 2016. It took four months to sort it out.
His home is only 20 minutes from me and I am a frequent visitor. The Quarriers care team are wonderful.
I hope that reading my story has been of interest, and maybe even of help. I have done my best and continue to do so. It has been a lonely journey. Single parenting is tough enough without a child in decline. I am proud to have ensured the best care for him and his sisters, and that includes getting him into a Supported situation where his needs are met better than I would be able to meet them at home. I have the reassurance that I always do the right thing for him.