Doctors Warn Waiting Too Long For Help Can Be Disastrous

Dry eyes and a parched mouth signaled something terrible inside Julie Cook's body. The disease mostly targets women, yet doctors warn that waiting too long for help can be disastrous.

Sheeraz Henderson was on holiday in France when she first noticed her foot swollen. She had taken the train there, so she thought laziness might be to blame. 'I wondered if it was from not moving around enough,' she says. But the swelling refused to go away. She swapped her normal shoes for Crocs just to cope.

Two weeks later, Sheeraz returned home to the UK and visited a doctor. By then her foot was swollen with a constant dull ache. 'The doctor asked me if I'd done anything to it or if exercised a lot and had sprained it,' she says. 'I said no.' Blood tests showed raised levels of inflammatory markers. Yet nothing else was done immediately. 'I was referred to a rheumatologist but there was a year's waiting list,' Sheeraz explains.

Her foot stayed swollen for more than that entire year. Over time her skin turned dry and sensitive. Her hair thinned out too. Her mouth became so dry the skin peeled off. A hoarse voice emerged as she sipped water constantly. Aches racked her legs and jaw. Just before seeing the consultant, she was sent for physio due to terrible hip pain.

Sheeraz Henderson, 53, finally received a diagnosis of Sjogren's syndrome in October 2023. It is an autoimmune disease where the immune system attacks moisture-producing glands. 'I was also quite fatigued making usual activities more of a challenge,' she admits. 'I used to love walking but I felt too tired to go on long walks.'

Ben Fisher, a professor in rheumatology at the UK's University of Birmingham, explains what happens next for patients like Sheeraz. 'Patients very often get problematic dryness of the eyes and mouth,' he says. 'And the skin and vagina can also be affected.' Around 30 to 40 per cent of patients face inflammation in their joints, causing pain and stiffness. Their lungs may cough or struggle to breathe. Nerves can cause numbness too.

This condition affects mainly women. Professor Fisher notes that many autoimmune diseases favor females over males. Sjogren's is probably the most sex-biased of all. It is at least nine to ten times more common in women than men. 'But even then, the vast majority of Sjogren's patients don't have a family history of the disease,' he adds. 'We don't know what triggers the disease in most cases.'

Some genes linked to these conditions sit on the X chromosome. Women possess two such chromosomes while men do not. Sex hormones also influence immune cell function, creating differences between genders and across life phases. Research remains far lighter for Sjogren's compared to other autoimmune diseases like rheumatoid arthritis. We know fewer genetic risk factors for this specific illness.

Symptoms can be quite subtle or overlap with other health issues. This leads directly to delayed diagnoses. 'It's like a jigsaw of lots of different symptoms, all of which can be quite vague on their own,' says Professor Fisher. For example, patients experience a gradual onset of dry eyes and mouth alongside fatigue. Yet many other things cause these same signs. Eye conditions like blepharitis [inflammation of the eyelid] and other causes of tear loss exist too.

Fatigue isn't just a bad feeling; it is a common companion to many chronic illnesses. Sheeraz puts it simply, saying she feels like someone putting the pieces of a jigsaw together without seeing the final picture. Eventually, her doctor prescribed hydroxychloroquine, an anti-rheumatic drug that eased her symptoms within days. Now, Sheeraz manages this incurable condition with medication and support from a charity for those with Sjogren's.

Diagnosis often relies on what patients say rather than clear proof. A specific antibody found in the blood or a biopsy of the salivary glands can confirm it, but these tests only happen if a doctor suspects something is wrong. Antibodies are supposed to help our immune system fight bacteria and viruses, yet in some people, they bind to proteins inside the body instead. Several autoantibodies show up in Sjogren's cases for this reason.

He explains that even awareness of Sjogren's itself may be low because it is less common than other autoimmune diseases. The pressures on primary care teams often mean doctors miss the signs until complications arise. Delayed diagnosis can cause long-term problems. Over time, untreated Sjogren's leads to gland damage and a progressive loss of tears and saliva. This dryness invites dental decay, for example. One in 20 patients may go on to develop lymphoma, a type of blood cell cancer, due to uncontrolled inflammation.

Research conducted by the Sjogren's Foundation in the US found the average time it used to take people to be diagnosed was around six years. This has gone down to just under three years now, but there are still many who wait a long time for an answer. The disease could impact up to four million Americans, making it one of the most prevalent autoimmune diseases according to the foundation.

Once Sheeraz got her diagnosis, she received eye drops for dry eyes and a saliva spray for a parched mouth. Each symptom gets treated separately, explains Professor Fisher. There aren't any therapies that can control how Sjogren's affects the whole body at once. So in the majority of people it really boils down to using symptomatic treatments. Artificial saliva often isn't very effective, and artificial tears do not work for everyone. Some need them every hour just to try and obtain relief, which is neither convenient nor pleasant.

Immunosuppressants and drugs like hydroxychloroquine are used when the condition attacks other organs such as the joints or lungs. Hydroxychloroquine regulates rather than suppresses the immune system in a specific way. Sheeraz says she felt amazing within days because she could walk faster and for longer after taking the medication.

Professor Fisher notes there is hope of new drugs on the horizon. A lot of clinical trials are going on right now, so we are in a very different place than we were even ten years ago. Four or five drugs globally are currently in late-stage clinical trials. The results of these may be available in the next one to three years. These drugs target parts of the immune system that seem overactive in Sjogren's cases. Although they focus mainly on treating organ involvement outside moisture-producing glands, there is hope they will also improve symptoms like dryness and fatigue.

While no cure exists, Sheeraz manages her life thanks to her medication and support from the charity Sjogren's UK. Through this group she has met others with the condition. I am relieved to have a diagnosis but do wish more in the medical profession were aware of it, she says. Hopefully my story will help someone else find answers sooner. Visit The British Sjögren's Syndrome Association for more information at sjogrensuk.org or check out The Sjogren's Foundation if you are based in the US at https://sjogrens.org.