Huntington’s has blighted Peter Allen’s family for generations. He watched his mother, Stephanie, slowly die from the disease and before that his grandmother, Olive, fell victim to the same illness. At 51 years old, Peter is the first of his generation to show signs of the illness, but his sister, Sandy, and brother, Frank, know they are also carrying the gene.
The onset of Huntington’s is insidious. Psychological changes typically come first – tiredness, mood swings, apathy and anger. Four years ago, Peter was formally diagnosed as symptomatic when he began suffering anxiety and panic attacks so severe he would become convinced that he couldn’t swallow. In retrospect, the depression he suffered in his thirties may have been an earlier manifestation of changes happening his brain.
Huntington’s disease is a congenital degenerative condition caused by a single defective gene. Most patients are diagnosed in middle age, with symptoms including mood swings, irritability and depression. As the disease progresses, more serious symptoms can include involuntary jerky movements, cognitive difficulties and issues with speech and swallowing.
Currently there is no cure for Huntington's, although drugs exist which help manage some of the symptoms. It is thought that about 12 people in 100,000 are affected by Huntington's, and if a parent carries the faulty gene there is a 50% chance they will pass it on to their offspring.
In person, Peter is articulate, funny and exudes affection for his wife and siblings, but there are small signs of the changes that are underway. Every now and then he pauses to search for the right word. A loss of dexterity means he can no longer write or sign his name, his balance is unsteady and, when tired, his speech becomes slurred.
“You know that you’re gradually lessening,” he says.
A lack of awareness about the disease and its symptoms means people sometimes assume he is drunk. “I’ve been asked to leave pubs before I’ve even had a drink,” he says. “I don’t go to those pubs any more.”
Peter took redundancy from his marketing job at Network Rail in 2015 and has not returned to full-time work, although he is retraining to become a garden designer.
Anti-depressant drugs have helped bring the psychological symptoms under control. In future, he will be offered other drugs to stiffen his muscles, which helps reduce involuntary movements. But no current treatments can slow the relentless progression of the disease, the loss of memory, motor control and eventually the ability to think.
“I don’t have a vision of what we’re going to be doing in ten years,” Peter says.
Only Peter was eligible for the UCL trial, but his siblings and their spouses all donated spinal fluid samples to help with the research. They are motivated not just for their own future, but for their children and grandchildren.
The disease exerts cruel pressures on family relationships. Peter’s sister, Sandy, was the first to take the genetic test, and describes learning the result as “a bereavement” that she had to keep secret for several years. “The hard thing was I didn’t want them to be affected by my decision,” she says.
Even those who are not biologically affected can be deeply traumatised. The Allens’ oldest sibling, David, who does not carry the Huntington’s gene, has struggled most with depression and “feelings of guilt”, Peter says.
The family talk of the stigma faced by earlier generations, although this is less of an issue today. “It was almost a shameful disease to have,” Sandy says.
In the case of the Allens, the three affected siblings and their families are very close and all live in the same area in Chelmsford, Essex, and say they have chosen to live positively, have fun and refuse to be defined by the illness.
The results have given the family “a glimmer of hope” that the spectre of Huntington’s could finally be lifted. Peter jokes that he and his wife have never invested, because until now there didn’t seem to be any point.
“It’s a lovely problem to have,” he said. “We really have got hope.”
