A Patients experience with Hughes Syndrome

At the age of 35 I started to become very ill. I had dizzy spells, horrendous migraines that could last for weeks, I had all of the early symptoms of Multiple sclerosis and I also had an over active thyroid. My memory was terrible, I ached all over, I was so tired I couldn’t function. I had 4 young children and had to have help in the house to cope. I couldn’t read as the words were blurred and jumped all over the page. I lost the sight in one eye whilst shopping for about 5 minutes. I couldn’t peel potatoes, my son Mike who often accompanies me to Hughes syndrome events, used to do that job for me, my arms were too painful.

The thyroid was treated but still I was ill and no answers seemed to be found whatever expert I saw.

I was told I was clinically depressed, that I might have MS, that I just had complicated migraines but I knew I was seriously ill and no one was understanding how bad this was. I had two years of feeling that people thought I was either crazy or a hypochondriac. I remember my husband saying to me “you are either in bed or in the bath you need to get this sorted out”, as if I had the power to do that! I thought about suicide but dismissed it because of my children. I wrote in my diary I AM BRAVE AND STRONG AND ONE DAY ALL OF MY DOUBTERS WILL KNOW THAT.

I saw a doctor about my eyes as I had blurred vision, visual disturbances and blurry dots all over my vision like raindrops on a window, it was impossible to read. He tested me extensively, including an MRI of my brain. The MRI showed nothing but some of the other tests showed my eyes weren’t working together properly. He had no explanation but knew there was a problem. He told me that if I found the answers he really wanted to know what it was.

Then when I was 37, I had a heart attack and suddenly all the doctors were interested. Incidentally while in hospital after my heart attack I was given heparin injections daily and felt better than I had in years and suddenly I could read again I was amazed and delighted.

6 months later I had such a terrible angina attack that I ended up in a heart hospital in Leicester. They finally tested me for Hughes syndrome as my arteries were completely clear and my heart attack was a mystery to them. I was not told about the blood test or the results but 6 months later I (a year after the heart attack) was asked to see a local expert who told me I had a blood clotting illness, and I should take aspirin and look out for clots.

I got home, having forced a name for my illness out of the doctor. Lupus anticoagulant. I read everything online, joined help and support groups and came across Professor Hughes and realised that Lupus anticoagulant was the name of a test not the illness. I begged my GP to send me to see Prof Hughes about what I now knew was Hughes syndrome. I knew warfarin was the answer if I was to survive this illness, my risk of another blood clot was 50/50 without it.

The rest is history, I keep my INR very high, between 4 and 5 as this keeps my angina away. This discovered by trial and error, using my home testing machine.  I am reasonably well most of the time now. I self-test and medicate and take full responsibility for keeping myself well. If my INR is high enough I have no migraines, no MS type symptoms and nothing like as much angina as I had when my INR was kept around 2.5 to 3. I only trust myself to test my INR once a week and decide on my dose of warfarin.

The only time I feel afraid is if I am in hospital and the doctor wants me to miss my warfarin because my INR is 4. I have threatened to discharge myself over that. Missing a dose causes my INR to crash and is very dangerous, I only ever reduce my dose unless my INR is outrageous like 8 which it was one time. Then I only miss one dose.

If anything, these days the other illnesses that have come along like Diabetes Sjogren’s and autoimmune thyroid problems have caused me more trouble than my Hughes syndrome.

In the almost 20 years since my heart attack I have had no clotting incidents and no bleeding incidents. Before diagnosis Hughes syndrome affected almost every organ, including my brain, every muscle in my body, my nervous system and my mental health. Now I am well enough to be managing director of an engineering company, look after my grandchildren and enjoy a full life.

I am not always well, I still have bad times, I still get angina now and then, my aches and pains are really depressing some days. However, as a dear old Auntie told me when I was first ill, “If you have these things, they have to come back to remind you now and then or else you would be cured”.

My normal is not other people’s normal but it is a great life, and I am very grateful that I have had the privilege of Prof Hughes being my doctor. Too many people still die because of this condition. Too many people are treated with an INR of 2 to 3 which really doesn’t do a lot for most of us. It breaks my heart when people I have known online die or are severely disabled because their doctors still don’t understand quite how sticky our blood can be. A dear friend Mark had 7 heart attacks over many years. He finally died over two years ago. His doctors had his INR set between 2 and 3 so he continued to clot over and over until his heart gave up. What a tragedy. I miss him so much as do his family.

A young reporter who interviewed me almost 20 years ago when I wrote my first book for patients. recently contacted me to say she now has Hughes syndrome and is being treated with an INR between 2 and 3 and still feels very ill. I told her to ask for a higher INR or better still get her own machine and self-test and medicate so she can try for herself where her INR gives her relief. I hope she takes my advice, but I also hope this wonderful charity can spread awareness within the medical world and save lives.

I help people new to the illness most days online and if I can save them the problems that I had, I will. Hughes syndrome is part of who I am now and helping others is my mission, I’m almost 60 now and I feel lucky that I have survived and survived well for 25 years since my symptoms began.

– Kay Pryszlak