Cure hope for patients suffering same genetic illness that blighted life of Keir Starmer’s mother

Patients suffering the same genetic illness that blighted the life of Sir Keir Starmer’s mother now have hope of a cure, thanks to new breakthrough drugs.

The Labour Party leader spoke movingly on BBC Radio 4’s Desert Island Discs last week of the illness she suffered, Still’s disease.

As a young teenager, he was once told she might die during one bout of severe symptoms and faced the prospect of telling his siblings the awful news.

In fact, Sir Keir’s mother Josephine survived. Although ultimately wheelchair-bound and unable to talk, she lived long enough to see him knighted in 2014 and passed away the following year.

In an earlier interview, the 58-year-old former lawyer – who once led a successful retrial of Stephen Lawrence’s killers – said: ‘I remember as a child spending many nights by her bedside in hospital worrying about what might happen to her. In the end, things were very difficult. 

Sir Keir Starmer, pictured during Prime Minister’s Questions on November 11, spoke movingly on BBC Radio 4’s Desert Island Discs last week of the illness his mother suffered, Still’s disease

‘For the last ten years of her life, mum could not move or talk. One of the great sadnesses for me was this meant she was never able to talk to my children, who were born after she lost her voice.’

What makes Josephine’s story even more poignant is that, in the five years since her death, treatment of Still’s disease has advanced beyond all recognition.

‘Newer treatments have transformed the outlook for most patients,’ says Rob Moots, professor of rheumatology at Edge Hill University in Lancashire.

‘One patient is in her early 40s with two young children and could not walk or get out of bed when she first came to see me.

‘Now that she is on the right treatment, she’s back at work and able to pick up her children from school.’

Today, an army of new drugs can curb the immune system reaction that causes the agonising symptoms of Still’s. They include excruciating pain in the limbs, skin rashes, fevers and fatigue.

It means 80 per cent of patients are now able to go back to work and take an active role caring for their children or grandchildren.

Still’s disease is a rare genetic condition, affecting about 800 Britons. It is a form of arthritis triggered when the immune system goes haywire, often as an overreaction to a mild infection, and causes widespread inflammation – attacking joints and tissue.

Because the inflammation affects the body’s energy-producing mechanisms, a high temperature of up to 40C and severe exhaustion are common.

Previously, the main treatment was high doses of steroids – drugs that dampen down the inflammation. But the long-term effects of this treatment can be worse than the disease itself, causing extreme weight gain, thinning bones and increased risk of infections in the throat, affecting speech.

As the inflammation affects the body¿s energy-producing mechanisms, a high temperature of up to 40C and severe exhaustion are common (file photo)

As the inflammation affects the body’s energy-producing mechanisms, a high temperature of up to 40C and severe exhaustion are common (file photo)

One patient, Clare Asher, 47, from Peterborough, who was prescribed the drugs to treat Still’s disease, suffered double vision and facial weight gain so severe that it left her disfigured.

‘My face was so stretched I could no longer smile,’ says the teacher, who was diagnosed with the condition in 2018 following a period of violent vomiting, extreme exhaustion and swelling in her finger joints.

Other standard treatments for tackling inflammation, such as a drug called methotrexate, also triggered side effects, including severe vertigo.

Earlier this year, Clare decided to come off all medication, and says her symptoms are at bay.

But doctors say that if her condition worsens, they can now offer new, safer and more effective medicines instead.

Two of them, anakinra and tocilizumab, are injected fortnightly and target the two proteins in the body responsible for the immune system malfunctioning.

Additional new research suggests that a class of arthritis drugs called JAK inhibitors, which block enzymes that tip the immune system into overdrive, are also highly effective.

Half of patients who take the inhibitor pills every day for a year will see complete remission of their condition. The remainder experience a significant reduction in symptoms, according to a recent study published in the Annals Of Rheumatic Diseases. Although the pills are not licensed in the UK, doctors say they can still be prescribed by GPs as they’re already being used to treat other illnesses such as rheumatoid arthritis and ulcerative colitis.

Overall, eight in ten Still’s patients are effectively cured.

Dr Sinisa Savic, consultant in clinical immunology and allergy at St James’s University Hospital in Leeds, and one of Britain’s leading experts on Still’s disease, says: ‘New drugs have made an enormous difference to many patients. Some go into complete remission after a few treatments.

‘In the past, it’s likely that many would have been in and out of hospital, just like Sir Keir Starmer’s mother.’