A mother from Kent has spoken of her double tragedy after her husband and son both died in their sleep from a rare genetic disorder, having gone to bed with what seemed like mild cold symptoms.
A Family's Devastating Loss
Amber Selvey, a paediatric nurse from Seasalter in Whitstable, lost her husband Jason in 2017. He was 47 years old. Six years later, in 2023, her 24-year-old son Daniel died in an identical manner. Both father and son had gone to bed with a runny nose and a tickly throat before suffering fatal cardiac arrests during the night.
"When my husband passed away, it was a massive shock – really unexpected," Amber said. "When you essentially have somebody who’s fit and well and not experiencing any problems, it’s not what you expect in life." The mother-of-five explained that caring for her children gave her the strength to return to work after Jason's death.
Understanding Alagille Syndrome
The cause of both deaths was Alagille syndrome, a rare and incurable genetic condition. It affects the bile ducts, which are crucial for digesting fatty foods. When these ducts are missing or underdeveloped, caustic acid builds up in the liver, leading to organ damage and vitamin deficiencies.
Over time, the syndrome also causes blood vessels in the heart, lungs, brain, and spinal cord to narrow. Symptoms can include dark urine, pale stools, and yellowish, deep-set eyes. The condition can lead to liver failure, kidney disease, and strokes. It is exceptionally rare, impacting only one in every 30,000 to 70,000 births.
Jason, a locksmith, only discovered he had been born with Alagille syndrome after his son Daniel was born. Doctors noticed Daniel had breathing difficulties, a trait also seen in the couple's next child, prompting genetic testing. Amber had to push for this testing, as health professionals initially dismissed the issues as "bad luck".
A post-mortem revealed Jason had underdeveloped ventricles in his heart. Daniel's autopsy showed he had a unicuspid aortic valve, meaning his heart had one valve instead of the normal three. Amber expressed shock at these findings, as doctors had previously insisted the syndrome primarily affected the kidneys.
Building a Community of Support
Now, Amber is campaigning to raise national awareness of Alagille syndrome. Of the estimated 180 people in the UK with the condition, four are in her own family. With help from the US-based Alagille Syndrome Association, she has established a support group in Whitstable for grieving parents and families.
The group meets on the third Wednesday of every month at the Revival Food & Mood café on the High Street. "People who’ve had a loss, you can often feel isolated," Amber said. "It’s hard some days to get up and find a reason and a motivation, and we all need that in life. We all need connections."
She came up with the idea while studying for a counselling, coaching, and mentoring degree at Canterbury Christ Church University. Howard Dove, a trustee, praised her efforts: "Amber’s compassion and expertise have a profound impact on children and their families."
Reflecting on the loss of her son, Amber added: "With child loss, it’s a very difficult one. It’s not the natural order of the world. We shouldn’t lose children before ourselves. It’s not just the child you’ve lost, but it’s their life moving forward."
