Migraines? Cold hands? You may have ‘sticky’ blood
It could raise your risk of stroke — or stop you having children
WRITING a document in her barracks one day, 23year- old military clerk Kelly Hart suddenly felt pins and needles in her left hand. She went to stand up, but collapsed. An officer called for help and though Kelly could see and hear the paramedics when they arrived, she couldn’t grasp what was going on. She was rushed to hospital, where she ended up staying for several months while doctors tried to find out what was wrong with her.
Scans revealed that Kelly had suffered a stroke brought on by a blood clot on the brain. But the question doctors could not answer is why this would happen to someone so young and fit.
Kelly played five-a-side football and went to the gym every week on top of daily workouts as part of her job in the Army — these could involve a ten-mile run and a gruelling assault course.
Immediately after the stroke, Kelly had no movement in her left hand and leg, her sight had deteriorated until she was almost blind and she struggled to speak. She was also experiencing epileptic seizures, poor circulation and memory loss.
It would be months before she could walk or even hold a pencil and, though her sight gradually improved, she has permanently lost her peripheral vision.
Eventually, nearly two years later, the cause was identified. Kelly, 34, is one of the 600,000 people in this country with Hughes syndrome — and like many other sufferers, she had no idea that she had it. The condition had not only caused the stroke, but was to blame for the ongoing problems with her circulation and seizures.
Also called Antiphospholipid syndrome (APS), or sticky blood syndrome, it is an autoimmune disease that leads to the production of abnormal antibodies called antiphospholipid antibodies.
Antibodies are normally made by the immune system to identify and destroy bacteria or viruses, but antiphospholipid antibodies target fat molecules in the blood, making it more likely to clot. This can affect any organ and lead to a host of problems.
Hughes syndrome is the cause of one in six strokes, heart attacks and DVTs in people under the age of 50 and is the most common treatable cause of recurrent miscarriage. It also increases the risk of stillbirth up to five times.
The sticky nature of the blood can also impair circulation and mean not enough oxygen is transported around the body. This can lead to more subtle symptoms, such as migraine, memory loss, dizziness, sight problems, loss of balance, joint pain and a purplish discoloration of the skin.
People who have suffered migraines since adolescence are often found to have Hughes syndrome, linked to the lack of oxygen it causes in the brain.
Similarly, Hughes can lead to problems with short-term memory, sometimes to the point where it is mistaken for dementia.
‘If a patient is not treated, one of the things they notice is that their memory is poor, they can’t remember words and often think they have Alzheimer’s, but it’s not, it’s totally different,’ says Professor Graham Hughes.
A CONSULTANT rheumatologist at london Bridge Hospital, in 1983 he was the first to describe the disorder. It is named after him. He adds that if Hughes syndrome patients are diagnosed and treated with medication to keep the blood thinner, ‘their memory often improves dramatically’.
The condition is also frequently misdiagnosed as multiple sclerosis (MS) because both conditions can lead to pins and needles.
other symptoms include angina, chest pain (if blood flow to the heart is affected), stomach pains after a big meal because of the extra oxygen and blood needed for digestion, and sometimes skin can look blotchy.
Yet even though Hughes is as common as Alzheimer’s and more common than Parkinson’s and MS, few people have heard of it and doctors may be slow to spot it.
‘Many doctors do not know about Hughes syndrome. It’s frustrating, but it is a fact,’ says Professor Hughes. ‘Some doctors just think of it in relation to miscarriage and DVT, but the most common symptoms are migraine and memory loss.’
Why people develop it is not fully understood. There is a strong genetic link — one study found 60 per cent of those affected had a relative with the condition.
Women are also three times more likely to suffer from it than men, possibly because of the effect their hormones have on the immune system.
Some people are born with symptoms, but the majority of those affected, such as Kelly, are aged 20 to 50 when it sets in.
Hughes syndrome is diagnosed by three simple blood tests, which may be done at a GP surgery, though some doctors will refer patients to specialists.
The tests measure the levels of abnormal antibodies in different ways.
But they are far from perfect — 20 per cent of people with Hughes will have a negative result in one or another of them — so researchers are looking into more effective alternatives.
Professor Hughes believes that if people are showing symptoms, they should receive treatment even if they have tested negative i n one test. He says bloodthinning medication, such as warfarin, heparin or aspirin, can be ‘life-changing’.
Professor Hughes urges anyone who has experienced a combination of miscarriages, migraines from the age of 15 and a suspected DVT, or who had a suspected DVT when on the Pill, to ask to be tested, particularly women who are thinking of getting pregnant.
Yet it is more likely a woman will be tested for syphilis than Hughes syndrome at a pregnancy clinic, according to Professor Hughes. ‘The official line is you don’t need to test for Hughes syndrome until you’ve had three miscarriages, which is a great pity,’ he says.
He believes the number of stillbirths, strokes and heart attacks in women under 45 could be ‘greatly reduced’ with more testing. ‘ There are three simple questions a doctor should ask a patient when they first go to a pregnancy clinic,’ he says.
‘Do you suffer from migraines? Have you had a clot or thrombosis? And do you have a family history of auto-immune diseases, such as rheumatoid arthritis, lupus or thyroid disease?
A positive to all three would make you a strong candidate for Hughes syndrome.’
If a woman is found to have Hughes, she can be given junior aspirin during pregnancy or injections of heparin to thin the blood. This can i ncrease the chance of having a successful pregnancy from 20 per cent to 80 per cent.
Someone with Hughes syndrome not hoping to get pregnant but with symptoms may be t r eated with si milar bloodthinning medication.
For Kelly, diagnosis came too late. losing her peripheral vision meant losing her independence, including being able to drive.
‘I had a brand new car, a citroen Saxo, and it only had three miles on the clock,’ she says.
Kelly had no family history of the condition and had never heard of Hughes syndrome. She says there were no s y mpt o ms she can pinpoint.
‘It’s been soul- destroying,’ she says, 11 years after her stroke. ‘I have tears in my eyes as I say it. The stroke took away my sight, it ruined my career, it ruined my life. ‘I had to move out of my home and it took away any prospects I had of a successful career in the Army. It has altered my life in such a huge way, but I have had to learn to adjust and it has made me stronger.’
KELLY had no choice but to move back home to Plymouth to live with her mother and brothers. Though she regained the movement in her arm and leg, her sight did not improve.
Adjusting to life as a blind person was a huge challenge. Kelly was given a white cane, but even then people would ignore it and barge past her in the street. ‘I felt completely isolated,’ she says.
Joining a disabled football club and local running club helped change her mindset. ‘I thought OK, I’m disabled, but I’m still capable of doing things — what else can I do?’
She has run four half marathons with her running partner, caroline. ‘I listen for her footsteps,’ she says. ‘Running has made me feel as if I am not just existing, but living again.’
Kelly has to inject the anticlotting medication fondaparinux into her stomach and take 20 tablets every day for epilepsy, caused by Hughes syndrome.
Her dog, Edi, can sense if she is about to have a seizure and alerts her. She still suffers from memory loss and has Raynaud’s phenomenon — poor circulation in her fingers and toes — again caused by Hughes syndrome.
Professor Hughes urges anyone who suspects they have Hughes syndrome to press for a test.
‘It doesn’t cost the doctor or the country much, but could save a fortune in medical care in the future,’ he says.
Kelly has gone back to college and hopes to become a fitness instructor helping other people with disabilities.
She spent the past year training for the london marathon and despite injuries from tripping over bins, kerbs and potholes — and bruising more easily due to her blood disorder — she was determined to cross the finish line.
Kelly completed the marathon in four hours, 21 minutes and 53 seconds — a personal best.
‘It was amazing. I am so happy to have completed it,’ she says. ‘I was nervous about the crowds, which I found intimidating, but I was really excited. It was a huge thing for me. I trained all year for that moment.’
CONTACT hughes-syndrome. org and blindveterans.org.uk (freephone 0800 389 7979).