The story of Gemma
It’s hard for me to explain, difficult for me to describe. It’s impossible for me to tell a story from Paul’s point of view, he’s a pretty closed book, just the same as any 20 something year old guy.
I can say, nothing will stop your world spinning like a diagnosis of NPC. And I guess this story is
solely from a big sisters point of view.
I was stepping up and trying to help out by taking the phone call we would soon learn would change our worlds forever. I guess being a nurse myself, it made sense I was involved with discussions around Paul’s investigations and diagnosis.
If I’m honest, I find it very difficult to cast my mind back, I guess they do say that our brains are clever enough to block out painful memories in order to protect us.
What I do remember perfectly well is growing up with my little brother. Only 18 months between us we went from being best friends to being worst enemies and back again. All of my childhood memories include me and Paul, happy, healthy and blissfully unaware of the cruel destiny that lay ahead. It’s true, sometimes you don’t know a special moment until it becomes a memory.
Growing up Paul was a normal brother, he struggled at school often, particularly with reading, but this was always put down to more common childhood problems like dyslexia. As young teenagers Paul and I spent a lot of time together, swinging in trees, running through rivers, playing in the park. Paul was like any other teenage boy, rode a BMX bike, played XBOX, watched WWF and had a scooter. As he got older he joined the Army Cadets and later trained exceptionally hard and was accepted into the Army. At 18 years old Paul’s hard work paid off and he moved out of home and into the Army Barracks to start his new life as a soldier. I knew how hard Paul had worked for this and we were all so proud of him.
Little did we know that this would mark the start of Paul’s deterioration. Paul’s hard working, loving and kind soul would begin to struggle under the strain.
Probably triggered by the physical and mental pressure of being a soldier, Paul began to have problems. Firstly with his balance and tremors in his hands, eventually he suffered an acute mental episode which landed him admitted into the Military Hospital. This was an extremely worrying and upsetting time for everyone involved. Not so long after, when Doctors couldn’t understand what was wrong, Paul was discharged from the Army on medical grounds.
This day would be the start of many years of hospital appointments, admissions, scans, blood tests and biopsies before finally being offered an explanation for the deterioration that was now becoming more obvious and increasingly more extensive. This would be the moment that would change our lives forever.
Now, I can’t put into words how it feels to hear that your little brother, someone that’s been by your side through thick and thin as long as you remember, to hear that they would, without a doubt deteriorate both physically and mentally, a terminal illness to which there is no cure……
Even now I can’t explain how that feels, or what kind of an impact it has on your life. They are words you never expect to hear, cannot find the strength to comprehend, cannot ever fully accept.
As reality set in , fear and lack of understanding would engulf us, confusion over how to protect Paul and how to protect each other. Inevitably no amount of protection could defer from the bleak reality we all faced. My brothers young life was being stolen and ravaged by such a cruel disease. In that split second, everything I had imagined for the future, everything I imagined that Paul would become and the man I admired that he was growing into, all of a sudden became so uncertain.
During the grieving that would follow, my main concerns became trying to make sure Paul was happy, and just as importantly that mum was coping. I felt guilty that I couldn’t stop Paul suffering, that it was him who had inherited this cruel disease. I felt helpless that I couldn’t tell my mum it was going to be ok. It became increasingly hard to deal with my own emotions and the thought and fear of losing my little brother, not only physically, but mentally absolutely terrified me. I felt selfish, upset at the prospect that this awful disease could take his mind as well as his physical being. I wanted more than anything for Paul to remember everything we had experienced together when he was well..the highs, the lows, the secrets we shared, I needed him here. Loneliness filled my days and nights, not a physical loneliness. I found it hard to talk about Paul, about the future. I was trying so hard to stay strong for my whole family. Everyone was struggling.
My outlook on life very quickly changed. I had an immense urge to live life to the absolute full, for Paul as well as myself. He’s always 100% supportive, if not persuasive over my decisions in life and whatever I’m doing. He says ‘you only live once Jim’. I’m so lucky to have Paul as my brother, he understands me and my view on life, he encourages me to follow my dreams. I feel blessed, to have my health and my life ahead of me. The small things have become so significant, just going to work and going out for dinner with friends. In so many ways I felt so lucky this hadn’t been me, but so painfully sad that it had happened to Paul, the chances were so so tiny, any smaller, it would be impossible.
Over the months, maybe even years. I became more level minded. My thoughts moved towards acceptance, making the best of things and trying to be positive…for Paul and my mum. I was reading a lot about NPC. It’s so rare, Paul could live a long time, anything could happen. The Doctors hadn’t expected Paul to still be here now. I took great comfort from the rarity of NPC and that Paul hadn’t been diagnosed until later in life than most (NPC is predominantly a childhood disease).
During this time, Paul started taking Migulstat. A drug which aims to slow down the progression of the disease. Despite unpleasant side effects, Paul doesn’t complain. This is my brother through and through. Never complains about feeling unwell. Just gets on with it. Sometimes he’s very matter of fact about the NPC…other times he talks about the future like the NPC doesn’t exist. I guess that’s a reflection on how he’s feeling.
I wish more than anything I could say things haven’t changed since then. Watching so helplessly as Paul finds it increasingly hard to complete simple day to day tasks breaks my heart into a million pieces. Paul manages to identify his own weaknesses and works well with them, he often adapts himself without even noticing. Like with his food and drink and how he manages it. About 7 years since Paul’s diagnosis, his main struggles are the coordination of his hands and feet. This makes things difficult for Paul, such as eating dinner or walking long distances, he falls frequently. Sometimes they call NPC childhood dementia, not a nice name. Sometimes I notice Paul’s personality changing, the shift upsets me, but mainly the NPC goes in a separate box. One that’s taken years for me to accept is now part of my little brother.
When I see Paul living happily and independently I wonder, ‘has the NPC slowed down? Has it stopped progressing?’. I know deep down it hasn’t, and it never will, but I’ve learnt that it’s ok to take some comfort and positivity from these dreams. I don’t know how much longer Paul will be here, how much longer I’ll watch him playing happily with dogs, or drinking a coffee from Costa…but at the moment, he’s really fighting all the odds and all we can do is hope everyday for a cure, be grateful he’s such a fighter and stay strong, together as a family. Everyday he makes me proud, my little brother, my best friend (most of the time).