My FH Story
(Originally written to be published on FH Australia's website)
Sadly, my self-conscious FH journey began late. It was only in my late twenties that my psychiatrist wanted my cholesterol tested for the first time. He was concerned about my general health. When my results came back, my Total Cholesterol was over 10 and my LDL Cholesterol was over 7. I was told to see my GP about it. My local GP looked at my results and gave me a fact sheet about a low cholesterol diet. He did not mention medication or genetics, nor did he do further tests. This was about the mid-2010s, and it showed a dangerous gap in the knowledge of FH amongst Australian general practitioners.
I tried to become vegetarian, and though my cholesterol remained unchanged, I was not overweight, and so I promptly forgot about my high cholesterol.
My first GP retired, and years passed. It wasn’t until I was in my early thirties that I went to a new GP. He tested my cholesterol as a routine health check. I only then remembered my previous discovery of having abnormally high cholesterol. My new GP referred me to a cardiologist immediately after receiving the results, and he said, using the Dutch Lipid Clinic Network Score, I appeared to have FH. Then this GP moved on, and I got yet another new GP. My new GP put me on a moderate dose of atorvastatin, telling me if I did not medicate, I would develop heart disease in ten years’ time. My cardiologist gave me a stress test and a CT Coronary Angiogram. The only hint of how life-threatening cholesterol damage could become, was when he said he hoped my arteries were not “furred up”. He then told me I had FH. Thankfully, my stress test result was perfectly healthy, and my calcium score was zero. Because my heart was in such good health, my cardiologist told me to see him again in 5 years’ time. This shows a lack of ongoing support in the Australian healthcare system for FH sufferers, as I was to be left without specialist care for 5 years. Ideally, I think there should be a registry of FH cases that is accessible by every individual in a FH patient’s healthcare team, so that there is continuity of care between GPs and other healthcare professionals. But there wasn’t, and I was not about to accept that, as you will see later. Meanwhile, the cardiologist, like the GP, agreed that I was to take 20mg of atorvastatin, though I was also told to start taking 10mg of ezetimibe.
I was initially worried about the side effects of these long-term drugs, and my GP said my liver test results showed elevated readings. I read up on the negatives of statin therapy, and I was reluctant to go on permanent medication for the first time in my life. My GP and I argued about the pros and cons of higher and lower doses of statins, and I swung between 20mg and 40mg of atorvastatin. Thankfully, my GP always urged me to increase my statin dosage, saying the liver side effects were a small price to pay for the benefit of reducing my heart disease risk. Finally, her words got through to me, and after almost a year of disagreements with my GP and my own persistent research into FH, using online resources like the Family Heart Foundation, I told my GP to prescribe to me the maximum dose of 80 mg of atorvastatin. At this point in time, I was suddenly directing my own treatment plan by requesting to have my statin dosage increased and self-educating myself through reputable medical resources online, from scientific journals to heart associations’ websites. Increasingly concerned about my FH status, and simultaneously horrified to know of how it could shorten my lifespan, I decided to take control of my own health. I empowered myself through knowledge, then protected myself through self-advocating.
I lost weight, dieted, exercised and asked for help in cholesterol management. I shifted towards a plant-based diet, then finally aspired to be a vegan. I saw the Family Heart Foundation as a life-saving representative of people like me. The Family Heart Foundation also advised people to get their cholesterol as low as humanly possible, and to take the maximally tolerated or highest dose of lipid-lowering therapy available. It was through this foundation that I first encountered the advice that every FH sufferer should get a FH specialist or lipidologist on their personal healthcare team. And so was born my desire to be managed by someone other than my GP or cardiologist. I wrote a draft letter about my health and asked my GP to use it to write a referral for me to the RPA’s Vascular Health Clinic, which is a specialist in FH care, and I was accepted, after a long waiting period, as one of their patients.
This is an edited version of that referral letter:
I am referring my patient to your clinic. My 36 years old patient was diagnosed four years ago with HeFH using the Dutch Lipid Clinic Network Score. Her untreated total cholesterol was 10.3 mmol/L and her LDL-cholesterol was 7.5 mmol/L. She has a family history of premature cardiovascular disease. She has been on lipid-lowering therapy for only four years. She is interested in PCSK9 inhibitors as she has a liver condition. She is interested in a FH genetic test and in a Lp(a) test. She is currently between cardiologists. Recently, she had a calcium score of zero in a heart scan and a good stress test result. On 80mg of atorvastatin, her Total Cholesterol and LDL Cholesterol are both usually reduced by over 50%. Thank you.
I had three main questions for the RPA FH specialist:
1. If I have a liver condition (multiple hemangiomas), do I go on PCSK9 inhibitors to avoid possible liver damage from statin therapy?
2. Is my Lp(a) high (another thing I learnt from the Family Heart Foundation are the dangers of high Lp(a))
3. Can I get a FH genetic test?
My questions were quickly answered, and I was tested for FH and my Lp(a) level.
I had to pay for my own Lp(a) test, though I think this should be covered by Medicare for FH patients. I didn’t need to use PCSK9 inhibitors.
On 80mg of atorvastatin and 10mg of ezetimibe, I have had my cholesterol reduced to a minimum of: Total Cholesterol 3.3 and LDL Cholesterol 1.5. But I do so wish my cumulative exposure to cholesterol since birth and increased disease burden of being untreated until when I was in my thirties were prevented with mandatory childhood FH screening.
In the last ten years, I have tried to set up blogs about my experience of FH, inspired in part by https://livingwithfh.blogspot.com/ and I have even wanted to publish a book about it. Finally, this month, I set up www.he-fh.com, as I couldn’t find anything else more important to do than to take care of my health. Also, with my maternal grandmother having been a surgeon, and my mother having trained as a doctor, I have a particular sensitivity towards health matters.
I am excited to be involved with FH Australia. It is the first patient-centred FH organisation in Australia. I imagine it will become indispensable, like the Family Heart Foundation in the USA. And to be working with my primary school friend, Jing Pang, current secretary of the organisation, and lipidology extraordinaire, is amazingly lucky. One day, I hope that FH will become a harmless condition through improvements in healthcare. I am honoured to be invited to write about my FH story, and it is a dream come true to help the FH cause. This is called living life to the fullest.
Last year, my calcium score was zero again, and this is in part due to my ongoing adherence to FH treatment. With my story, I hope to inspire everyone to take care of themselves. Thank you.
Xi Xiao
6/7/2024
This story is now published at this link:
