Antiphospholipid syndrome

The jigsaw pieces

Imagine a box of jigsaw pieces tipped out on the floor, all the bits are there, but to have a clear picture, I first had to put them all together to make sense. This was a journey that I was to take.

I had a normal childhood. One delay that stands out was that I did not begin menstruating until the age of 18, and my periods were irregular. Around this time I experienced stabbing stomach pains that left me short of breath. My two pregnancies in 1990 and 1997 were complicated because I experienced severe unexplained abdominal pain, vomiting, and fatigue during and after the birth, and I put my forgetfulness (not remembering friends’ names) down to becoming a mum.

After the birth of my second child I started vomiting every day. After two weeks I was admitted to hospital and after another few weeks of investigations I was taken to theatre as an emergency. A computed tomography scan showed a “swelling” in my abdomen. I was diagnosed with an unexplained blood clot in my large bowel that had burst. The incident was explained to me as “a mystery” as I had had a normal second birth.

After discharge following my bowel operation I was promptly readmitted with a suspected pulmonary embolism. I was treated with heparin injections, and a computed tomography scan was inconclusive. In 2001, I returned from a holiday abroad with a throbbing pain in my left leg. I had several blood tests, but was not contacted about the results by the hospital staff. The pounding pain subsided and I forgot all about the discomfort. When I finally got a diagnosis of antiphospholipid syndrome I requested access to my GP records to look for clues or things that had been missed; looking back through my notes I saw that a lupus anticoagulant blood test was positive.

In the following years I underwent several operations—for example, removal of my gall bladder and a thrombus in the portal vein. Both were accompanied by continuous extreme fatigue and “brain fog.” I overheard a consultant commenting after one of the operations that my blood had been “acting weirdly,” but I did not take any notice or ask why.

Putting the pieces of the jigsaw together

In 2008 it seemed to me that a guardian angel pointed me in the right direction. My left leg was constantly hurting, so my general practitioner referred me to a vascular consultant. He arranged a battery of blood tests and a Doppler examination of my left leg, which identified a clotting abnormality and showed that I had developed a blood clot in my popliteal artery and long saphenous vein. In addition, my kidney function was severely compromised so he urgently transferred me to a haematologist. Apart from the ongoing fatigue and pain I now had a continuous headache. The haematologist took a detailed history, advised me not to travel by plane, but instead ordered specific blood tests, a magnetic resonance imaging scan, and an angiogram. I think now that this consultant saved my life. I felt physically and emotionally exhausted. To my surprise the scan and angiogram showed that my right kidney and coeliac and superior mesenteric arteries were occluded. A subsequent brain scan showed high signal lesions. A week later I was referred to the lupus clinic where I was diagnosed with a rare autoimmune condition called primary vascular antiphospholipid syndrome, which causes an increased risk of blood clots (and miscarriages). My medical history now suddenly all made sense to me and I finally had a name to explain my strange, unexplained symptoms over so many years.

Life after diagnosis

The first few months after diagnosis were hectic. I had to get my drug regimen stabilised. The treatment was lifelong anticoagulation that required close regular blood monitoring. I was also diagnosed as having stage 3 kidney failure and therefore my hypertension required frequent check-ups (I had lost one kidney already). I was prescribed hydroxychloroquine for muscle aches in my arms and legs, and fatigue.

I continue to work full time in “planning and performance” for a government department. My employers have been sympathetic and understanding about my illness and have made reasonable adjustments for me. I describe my body like an old car—I cannot sit in it and drive off. Instead, when I wake up in the morning I have to gently start myself up before I am able to function physically and mentally—sometimes I cannot function at all. Every day I experience antiphospholipid syndrome in a different way.

Personal reflection and self management

Unfortunately, it took 17 years to receive a diagnosis, which meant a lot of damage was already done to my body. I was introduced to the manager of the Hughes Syndrome Foundation, who provided me with leaflets and important information about antiphospholipid syndrome. Going forward is the only way for me and I have to make the best of my situation. I have therefore decided to raise awareness and educate people about antiphospholipid syndrome, including family, friends, and colleagues, and sometimes even doctors. Many healthcare professionals that I meet have not come across patients with antiphospholipid syndrome and therefore lack information about this rare autoimmune condition. I have learnt not to ignore symptoms. I have decided that it is better to consult a doctor to avoid further negative consequences to my health or miss out on vital treatment.

I manage my pain through painkillers and heat therapy—hot water bottles, hot baths, and saunas. Some of the pain, I have discovered, seems to be triggered by specific foods—for example, wheat, red meat, and certain fruits and vegetables. I therefore follow a diet that facilitates easy digestion such as fish, chicken, rice, and bananas. I still have stomach pains on a daily basis. I use a walking stick most of the time because of leg pain and joint stiffness. This visual aid lets people know that I am unwell as antiphospholipid syndrome is mostly invisible to others. I had to learn to pace myself—if I do not rest during the day, I experience unnecessary pain and fatigue and I am mentally unfocused. I have come to accept that I have a lifelong condition and that it is not going away, and to be honest it has contributed to who I am now.

Lessons learnt over the years

Since my diagnosis I no longer ignore any symptoms that I experience and make sure that I bring them to the attention of my GP or consultant. I also ask more questions as I believe my own understanding of my condition plays a vital part in my wellbeing and helps me to stay in control. I prefer to be treated as a whole person, instead of attention being paid to my coagulation time (international normalised ratio) only. I also like to ask medical and nursing staff questions so that I can untangle in my mind whether new symptoms are pieces of the complex antiphospholipid syndrome jigsaw puzzle or something else.