Being 17 is an exciting time for most young people.
On the cusp of adulthood, they can learn how to drive, and the future is ripe with promise. But for Kyran Richmond, this is far from the reality.
Due to an incurable genetic disorder, the teenager suffers from tremors because of Parkinson’s and memory loss caused by Alzheimer’s – symptoms classically associated with people many, many decades older.
But with an abundance of love and courage, Kyran’s family do everything they can to fill his challenging life with joy.
For the first few years after he was born, Kyran – the middle of three boys growing up in Chilton, County Durham – seemed perfectly healthy.
‘He could run about with his friends, play football, and went to a mainstream school,’ explains mum Debbie, 40, who works in healthcare for NHS ophthalmology.
‘It was when he was six that I spotted him on the floor with his arms stretched out. He told me he’d dropped his pen, and when I realised he was feeling for it instead of looking for it, alarm bells rang.’
Kyran’s eyesight was getting progressively worse, and when he became frustrated at not being able to see properly, his behaviour soon deteriorated too. Worried Debbie took her son to the doctor, who in turn referred Kyran to the hospital for more tests.
Six weeks later, in March 2011, his blood results threw up a devastating discovery: Kyran had juvenile Batten disease, a neurodegenerative disorder.
Children with the disease are born seemingly healthy, but later develop epilepsy and lose their sight, speech, mental and motor abilities. The life expectancy for people with juvenile Batten disease is late teens to early twenties. As Debbie sat holding hands with her husband Carl, 47, a steel plater, they struggled to take in the doctor’s words.
‘It was like the air had been sucked out of the room as we were told our beautiful little boy would gradually see his life deteriorate. It was too much to take in.
‘The words “terminal illness” kept going round in my head, Carl and I sat staring in shock.
‘The doctors said because it was so rare they could not predict how long Kyran had left to live, but that eventually he’d develop dementia and would not know what was happening to him. They warned us not to Google it, but of course we did. And saw it was every parent’s worst nightmare.’
After Kyran’s diagnosis, Debbie and Carl both tested positive for the CLN3 gene (responsible for Batten disease), which lies on chromosome 16.
As Batten disease is an autosomal genetic disorder, both parents are unaffected carriers of the mutated gene, but have a one in four chance of a child inheriting both faulty copies, and developing Batten disease.
Their youngest son Hayden, now 14, was tested shortly after Kyran. He’d inherited one copy of the gene and is unaffected. Their oldest son Cameron, 21, who is in the Navy, is healthy and hasn’t been tested.
With no cure or treatment available, Debbie and Carl tried desperately to come to terms with Kyran’s future outlook. ‘Of course you get angry, and think why has he got it, what have we done?’ admits Debbie.
‘It’s utterly devastating. Learning to accept the condition is hard. We’ve struggled with depression at times. Couples deal with it differently, I am more of a talker than Carl, who copes differently.
‘There’s denial, too. We thought in the beginning, “What if we took him to America, would there be a cure?”
‘Carl said to me, “How do we tell our son he’s dying?” I told him we couldn’t, he was too young. Kyran was so innocent with not a care in the world. It is so mentally exhausting.
‘Even as he’s got older, how can you say that to someone? So we vowed to make his life as fulfilling and joyful as we possibly could.’
Within a year of his diagnosis, Kyran’s sight further deteriorated, he was consumed by night terrors, and balance issues took hold. On a family holiday to Tunisia he started having seizures.
Knowing rocky times lay ahead, the family began fundraising for things they knew would be needed in the future – and had an extension built on their home to make it wheelchair accessible.
Now, at the age of 17, Kyran is registered blind and uses a wheelchair 90% of the time, though he can manage with a stick for support when in the house.
‘He has tremors, which is due to childhood Parkinson’s and very tense muscles in his legs,’ explains Debbie. ‘He has childhood Alzheimer’s, so he can remember events years ago but can’t remember what happened today.
‘He has lowered immunity and our consultant warned us he could be quite poorly if he contracted coronavirus. So we had to shield for months. That was tough as we didn’t get any respite.
‘Usually Kyran attends a special needs school and stays for overnight respite care one night a week. That’s the only time we sleep well, as he suffers with anxiety and disturbed sleep. At the respite centre he goes on trips to the beach, to the cinema and enjoys the garden with a paddling pool.
‘He can still eat and drink but when he talks he repeats himself a lot of the time. He needs a lot of help to wash and dress and go to the toilet.
‘We have issues with his behaviour. It comes from frustration and pent up anger. He used to lash out at school, but specialist nurses have guided the staff on how best to manage Kyran’s frustration and calm situations down.’
Yet despite all these epic life hurdles, Debbie and Carl have shown superhuman strength and positivity.
‘You try to work and keep a normal life throughout. It’s hard for Cameron because if he hasn’t seen Kyran for a few weeks, he notices the difference in him.
‘We are lucky we have a good support network, we raise awareness and we have a fundraising group to make memories for Kyran. We have lots of friends helping in our area, and my friend Cheryl has just been amazing.’
Through the Make A Wish Foundation, football-mad Kyran got to visit Newcastle United FC, and even Barcelona’s football ground. One of his favourite things is listening to music, watching Star Wars, and going to see Newcastle play at home with his dad.
After his older brother Cameron went off to his school prom in a limo, Kyran announced that when it was his turn to attend a prom, he wanted to travel in a helicopter.
So Debbie and her friend Cheryl worked hard to make this wish come true. Last year, Kyran not only travelled to his prom in the desired chopper – he was escorted there by Miss England Alisha Cowie!
‘I had tears streaming down my face when I saw him wearing a suit and beaming from ear to ear,’ smiles Debbie. ‘I gave him a big hug and told him he looked like James Bond.’
When the teenager turns 18 next year, his parents and friends are planning a black-tie ball to mark the milestone birthday, if social distancing rules allow it, that is.
‘We cannot use magic to make our son better, all we can do is try to make his time with us magical, and hope that for other sufferers, in the future, there may be a cure.’
– Kyran is helping to raise awareness with the charity Genetic Disorders UK and their annual fundraising day, Jeans For Genes Day. This year’s campaign runs from September 14-20, asking us to wear jeans to work or school in return for making a donation HERE