The legacy of a Co Armagh youngster who died after living with “one of the most cruel and degenerative” diseases could be ensuring other families do not endure similar heartbreak if demands over testing are met.
Teddy Johnson, who was born with Metachromatic Leukodystrophy (MLD), a rare metabolic disorder that impacts the brain and causes severe physical and mental degeneration, died on Thursday aged 7.
MLD can be treated if detected early enough in infants, but at the start of this year the UK’s National Screening Committee recommended against adding it to the list of 10 rare conditions that newborns are screened for through the ‘heel prick’ test.
This followed a public consultation launched last May into including MLD in the test, and the committee’s decision not to add it was based on concerns including test accuracy.
Upper Bann MP Carla Lockhart criticised that decision, saying at the time that although it was “too late” for Teddy to have had treatment, “no parent should ever be left knowing that a simple heel-prick test could have changed the course of their child’s life”.
The DUP MP raised Teddy’s case in the House of Commons last July. She told fellow MPs she spoke on behalf “of a little boy from Upper Bann who can’t speak for himself, who can’t walk and struggles to even smile”.
Ms Lockhart also praised Teddy’s mother Jemma “for being Teddy’s voice and a voice for the many children whose lives could be saved by this simple check”.
In 2023, a toddler from England, Teddi Shaw, became the first child with MLD ever treated by the NHS with a new one-off life-saving gene therapy costing £2.875m.
She was diagnosed in time due to her older sister – for whom it was too late to receive the treatment – also having the condition.
Only Norway currently screens newborns for MLD, but pilot studies have taken in place in other countries including Germany, France and the US.
Following the tragic news of Teddy’s death, Ms Lockhart told the Irish News that his story had “touched the hearts of many across Northern Ireland and beyond”.
“Despite facing one of the most cruel and degenerative conditions, he showed incredible courage, and his family demonstrated remarkable strength and dignity throughout,” the MP said.
“My thoughts and prayers are with Teddy’s family at this deeply difficult time. I know them well and their loss is unimaginable, and I hope they find comfort in the love and support that surrounds them.
“Teddy’s case highlighted the urgent need for newborn screening for conditions such as MLD, where early diagnosis can make the difference between life and death. There can be no greater legacy than ensuring other families are spared this heartbreak.”
She added: “I will continue to campaign with his mum Jemma so that no other child is denied the chance of early treatment because screening was not in place.”
A death notice said Teddy “fell asleep for the last time peacefully in his mummy and daddy’s arms”.
It said he is the “deeply loved son of Marvin and Jemma, amazing little brother of Jean, best friend of Evie,” adding he was a “precious grandson”.
The notice adds: “The family wish to express their heartfelt gratitude for the most amazing and loving care Teddy received from all the staff of Blossom Children’s Ward, the Metabolic Team in Belfast Royal Belfast Hospital for Sick Children and all those professionals who have contributed to Teddy’s care”.