Harley, a “cheeky chops” seven-year-old from Llanelli, is definitely one of a kind. He had his first operation on a cystic lung when he was still inside his mum Becca’s tummy and at two days old, he had open surgery to remove most of his left lung.
A week later his other lung collapsed and doctors found a hole in his heart. He has lived his whole life with multiple health issues from having to be tube fed to severe seizures and developmental delay.
Harley has a number of genetic abnormalities and has been diagnosed with adenylosuccinase deficiency (ADSL), with symptoms including autistic behaviours, epilepsy, feeding difficulties, hyper mobility and hypotonia (low muscle tone).
ADSL is a progressive disorder and Harley’s condition has developed over time. But while he is at high risk of sudden death through epilepsy, Becca was never sure that they were eligible for support from Tŷ Hafan. However, it was another Tŷ Hafan parent she knew who suggested that they come to see us, especially as symptom management is provided at the hospice.
Becca said: “I never thought Tŷ Hafan was right for us because I thought it was for end of life only, and that wasn’t us. But when we went to the hospice to stay, it gave the doctors a chance to see what was going on. Harley didn’t eat or drink for a week, but it meant that they knew what they were dealing with and it helped us get the referral we needed.
Becca and Harley came for their first visit in summer 2020 amid all the chaos of the Covid-19 pandemic. They had been on the waiting list for local respite and were at a crossroads with carers as they needed to shield. With nobody able to babysit, the whole family was at breaking point.