World Antiphospholipid Syndrome Day falls on 9 June. Part of the Antiphospholipid Syndrome (APS) Awareness Month of June, the aim is to educate people about this little known autoimmune disease. I was diagnosed with APS about 30 years ago. APS sits alongside my primary Sjogren’s syndrome, Fibromyalgia and osteoarthritis. June is recognised as APS Awareness Month.
Please note that each person has their own journey with APS. I am not a medical doctor or making recommendations, this blog just summarises my own experience. Anyone who thinks they may have APS should approach their doctor.
What is Antiphospholipid Syndrome?
APS is an immune system, an autoimmune condition where the body attacks healthy tissue by mistake. It causes ‘sticky blood’ and an increased risk of blood clots. With this condition, the immune system produces abnormal antibodies called antiphospholipid antibodies. These target proteins attached to fat molecules with makes the blood more likely to clot. It can affect people of all ages including children. There is no cure but it can be managed with treatment.
The prevalence of APS in the population is estimated to be 1 in every 2000 which means it is quite rare. However, there are people who have APS who have no symptoms so the true numbers are not known. Most people are diagnosed between the age of 20 and 50 years of age. For example, I was 31 at the time of my diagnosis. APS affects more women then men.
The syndrome was discovered in 1983 by a team led by Professor Graham Hughes. To honour him, APS is also known as Hughes Syndrome.
Still with me? If you have never heard of this condition it is a bit mind boggling.
What are the Symptoms of APS?
This is just a quick overview of symptoms, there are many more but these are the main ones!
Antiphospholipid Syndrome causes clots which can occur in the legs (deep vein thrombosis) and in the lungs (pulmonary embolism). It can cause a clot in an artery (arterial thrombosis) which can cause a stroke or a heart attack. Blood clots can also occur in the brain which lead to problems with mobility, balance, vision, speech and memory. It can also cause pregnancy problems with miscarriage, sometimes multiple, and premature birth. It is one of the most significant, treatable, causes of multiple miscarriage.
Another symptom is a skin condition called livedo reticularis caused by small blood clots that develop inside the blood vessels of the skin. The skin has a blotchy red or blue appearance, often more severe in cold weather.
If that isn’t enough, APS sufferers can develop superficial thrombophlebitis which is inflammation of the veins just under the skin, usually in the leg.
Some people don’t suffer with too many symptoms, for others the symptoms can be similar to those related to multiple sclerosis.
Getting a Diagnosis
Finding A Doctor who Will Listen
Hah! Well, given how rare this syndrome is, the average family doctor has probably not even got it on his or her radar when you first start to list all the weird and wonderful symptoms you have. Particularly if, like me, the Antiphospholipid Syndrome sits alongside other autoimmune conditions and diseases. It is a common story with those suffering from autoimmune diseases, particularly APS because it is so rare, that it can take years to get diagnosed.
To get a diagnosis of APS you need a specific set of blood tests and a medical assessment by a specialist. The blood tests look for the abnormal antiphospholipid antibodies that increase the risk of blood clots. Usually, a diagnosis is made after 2 abnormal blood test results, with at least a 12-week gap between them.
A comprehensive medical assessment is conducted looking closely at your medical history and symptoms.
My Own Experience
I had a lot of issues as a child with what the doctor said were ‘growing pains’ etc. By the time I reached my early 20s headaches were becoming the norm. As time went on I got more symptoms, some of which could be put down to allergies but others were harder to pinpoint. As I got older I went to various doctors trying to get help. I had doctors accuse me of hypochondria, panic attacks and even lying about my symptoms at one point. Of course all the general blood tests that a family doctor usually orders came back normal. I remember this being said to me distinctly: “Listen to me carefully, you can’t possibly be ill as you look so well. This is all in your mind.”
Another doctor told me I was suffering from stress and overeating which was causing my symptoms. He very carefully drew a picture of a can of baked beans with beans tumbling over the side. He said I had to work out how many baked beans I could eat before I gained weight and stop just before I reached that number. I sat watching and listening incredulously. I can’t tell you what I would have liked to have done with that can of beans if it had been sitting on the table and not just a drawing!
After six years, I finally found a family doctor who listened carefully to my thoughts on what I thought might be wrong and acted on it. I was a researcher (labour market not medical) and I had spent a long time carefully researching my symptoms and possible causes. I had come up with the idea that I might be suffering from Lupus which is another autoimmune disease. The doctor agreed it was plausible and agreed to refer me to a specialist. At that point I was spending three days per week in bed and suffered dreadfully with headaches. Sometimes my speech would slur and I struggled to find words. It was like I had a gap in my brain at times – so hard to describe! I had a lot of tingling in my extremities. Other times I was absolutely fine.
Finally A Diagnosis
It was suggested I should see Professor Graham Hughes, who specialised in Lupus. At that point I had never heard of antiphospholipid syndrome. Sure enough, the Professor took a lot of time to talk through my medical history and symptoms and conduct various tests.
I cannot tell you the relief of having someone actually believe the strange symptoms I needed to talk about. He accepted everything I said as completely normal and understandable. I burst into tears and he gave me a box of tissues and said he saw this so many times, people who have their symptoms dismissed incorrectly. He pointed out the livedo reticularis on my legs as a clear sign of APS. An MRI of my brain showed I had suffered a couple of previous TIAs too.
What is the Treatment for APS?
I was diagnosed with primary Sjogrens syndrome and secondary antiphospholipid syndrome. Treatment for the APS depends on whether the APS is primary or secondary and the severity of symptoms. It usually involves an anticoagulant such as warfarin or an antiplatelet such as low-dose aspirin. I was prescribed aspirin. The treatment has to be taken daily for life.
Things can change of course, so regular check ups are required to ensure you are on the right treatment for the current phase of your condition. Alongside treatment there are lifestyle changes such as giving up smoking, eating healthily, taking regular exercise and losing weight. Again, this is just a brief overview.
For me, taking the daily aspirin made such a difference to my life it was a complete shock. It had an effect very quickly. I don’t know if this will make sense to anyone, but my eyes felt bigger, I felt I could see better, the headaches largely disappeared. I stopped slurring my speech at times and I felt clearer in my head generally.
For women who have been diagnosed with APS, careful planning is required for any pregnancy. I have no personal experience of this but you can find out more here.
A Note About Complications
This is a very rare complication of APS which occurs in less than one per cent of people with APS. It is called catastrophic antiphospholipid syndrome (CAPS) and it is where the body forms multiple blood clots causing multiple organ failure. Needless to say, this is a medical emergency. You can read more about CAPS here.
Living with APS
It is difficult for me to talk about APS separately from my other conditions, all of which I am being treated for. The biggest hurdle for me was getting diagnosed and from that point on I have not looked back. The treatment has made a huge difference to me. There are some lifestyle changes I made including no more long haul flights because of the risk of clotting!
There are times I have to take more precautions, for example if I am particularly tired, stressed or ill. Physical injury has to be managed carefully. I fell and hurt my knee which caused clots requiring treatment with heparin. Any surgery I have had has had to be carefully planned, not least because of the need to suspend the aspirin I take beforehand. I get some bad days but many more good days.
If you would like to find out more about APS, there are many national support organisations and you can Google the ones for your country. As an example, the main one I follow in the UK is APS Support UK.
Before you go
My name is Dorothy Berry-Lound an artist and writer. You can find out more about my art and writing at https://dorothyberryloundart.com.
Thank you for reading!