Last week was PH Awareness Week. The ‘PH’ stands for pulmonary hypertension. It’s a rare, incurable, life-shortening disease and not the easiest to diagnose.
I spoke to Angela Cran, whose son Keir died of PH aged eight and a half after being diagnosed at five and a half. One of the cruellest aspects of Keir’s condition was that it crept up on them disguised as something less sinister.
They thought it was asthma because the symptoms Keir suffered were similar and Keir’s dad was asthmatic. The reason Angela asked me to write this piece was to put families on the alert so they have a chance of getting the correct diagnosis early.
Despite the severity of his symptoms and his diagnosis, Keir was a happy wee boy. He loved art. He liked to draw superheroes, adding his own imaginative twists to familiar super-powered characters like Optimus Prime.
He was into cooking too and he loved to eat. He had a sophisticated palate for a wee boy – he’d eat fillet steak, even olives. But when he was out playingwith his brother, his folks began to notice he had become, in their words, self-limiting.
“He must have been experiencing discomfort on exercise, so instead of kicking the football out the back with Finn and his pals, he would be the referee on the side, holding up his squares of yellow and red card for bad fouls or sending offs.”
When his mum and dad became increasingly alarmed at his breathlessness and the fact he wasn’t growing properly, Keir was sent for an ECG and echocardiograph. A few days later they were told Keir had pulmonary hypertension.
Their relief at finally getting a diagnosis has to be set against the grimness of the bad news; that Keir’s life would be cut short. His name was added to the lung transplant list which put the family on permanent red alert, hoping for a donor.
This is a difficult piece to write and it’s too heart-breaking to go into any more detail about Keir’s decline. But his mum has written movingly about it in blog pieces and poetry and with her permission, I’d like to share a few verses here:
In Coram’s Fields, you asked her to paint you Batman, your face a pale canvas for the Caped Crusader, flicked and dabbed out in exquisite minutes from her tiny paints, circular sponges and miniature brushes, a work of art that demanded an immediate close-up photo.
Back at the hospital, your consultant marvelled and took the same. But the most striking features she did not paint: the sorrow of your opaque blue eyes, stained below in angry pink, the thin downturn of your mouth, the indelible knowledge that her palette could not touch.
Six and a quarter years old and we had just made you swallow half a ‘vile’ tablet, crushed with water, that might (or might not) help you.
A tablet so toxic we were only allowed to handle it with gloves.
We helped you clamber up the chute, played at cafes underneath it, watched floppy-eared brown goats jump on and off benches and wondered at our wee Batman stalking rabbits and chickens.
But it turned out nothing could catch what was destroying you inside, no superhero doctor or antidotal elixir would save you. And though in hope we would wait, no transplanted lungs would come to your rescue.
Keir’s mum, dad and brother Finn are only just beginning to embrace life again four years after their son’s death. They want other families to be aware that pulmonary hypertension exists, although it’s rare, and if they notice their child’s breathlessness becoming more severe and their height and weight failing to increase, they should see their GP, if only to alleviate their worries.
You can find out more about pulmonary hypertension on the PHA website at www.phauk.org or read Keir’s story and Angela’s blogs and poems at angelacran.wordpress.com