Jane Madden shares her story living with HHT and talks to us about the challenges of Liver Vascular Malformations (VMs).

What age where you first diagnosed with HHT?

I was diagnosed in 2017 while I was living in Hong Kong. I was 39 and had been experiencing HHT related symptoms on and off for approximately ten years when the Dr spotted the liver function abnormality and requested further blood tests and scans and a follow up with a gastroenterologist. This resulted in me having MRI’s and CT scans. The timing coincided with my own mother’s HHT diagnosis and the Dr informed himself of the condition (thanks to literature from Dr Adrian Brady) and made the diagnosis of having HHT with Liver VMs. Alongside the liver symptoms I was also displaying other common HHT symptoms such as occasional nose and tongue bleeds and the presence of small telangiectasia on my skin.

Your diagnosis of HHT was it due to symptoms related to the liver, or did these symptoms appear later? 

I had nosebleeds on and off for years. I always related them to my dust mite allergy although I became a bit more concerned when they really ramped up during pregnancy.

The first liver related symptoms I had appeared approximately twenty years ago when I was 25 during a routine medical exam. My liver function showed abnormalities and at the time the Dr attempted to convince me that it was alcohol related despite me having no other indications of an alcohol related illness. In hindsight I wish I had not accepted the Drs diagnosis. Had I challenged the findings and requested follow up testing, members of my family would have been diagnosed decades earlier.

How did the presence of Liver VMs (vascular malformations) manifest? Did you have any of the following symptoms- high heart rate upon minor physical effortheavy feeling of the right armheavy stomach pain after dinnervomitingdepressionpain in the back/neck; tingling of limbs?

I have very fortunately had none of the above symptoms until very recently. In the last 12 months I have experienced an occasional dull ache when I lie on my right side, particularly if my lifestyle has not been at its best. For me a lifestyle is impacted by alcohol intake, lack of exercise, excess weight and eating heavily processed foods. If my lifestyle is good I generally feel little to no discomfort.

I also have a number of very large nodules on top of my liver that have grown as a result of VM’s. There are three in and in total cover an area of 15cm in diameter.

Upon discover of these vm’s I was very fortunate to be referred to one of the most senior and experienced liver Drs in Asia. Professor Henry Chan conducted some further blood tests along with MRI (with contrast) and diagnosed the nodules as Focal Nodule Hyperplasia (FNH). These growths can occur for a number of reasons and do not always coincide with HHT. Between my liaisons with him and Dr Brady I very quickly became an expert on my liver condition.

Did you get the impression that the affected Liver made nosebleeds (or digestive bleeding) worse?

This is not my experience in general. However as indicated above lifestyle factors impact my liver and also my nosebleeds. But generally speaking if I manage these factors my bleeds reduce

What support have you received from experienced HHT physicians (diagnosis, treatment indications, surveillance, support in major decisions, other)?

I have been extremely fortunate with the support I have received from Professor Chan, Dr Brady, my local gastroenterologist (I now live in the Philippines), my local ENT and the HHT websites. I have also purchased and read the HHT book and also read other liver related books specifically those issued by medical institutions. These have all armed me with support and knowledge.

In the early days of diagnosis I was in very frequent contact with Dr Brady and he always made time to see me when I visited him in Cork. 

In latter years I have come to rely heavily on Professor Chan who has made himself available to me whenever I need him. I video call with him every six months where we review my routine ultrasound and bloods, and beyond that, he is always on hand to answer any questions I may have in the interim.

A number of years ago one of my nodules grew exponentially within a very short time frame. Both Prof Chan and his radiology counterpart were concerned and felt that they needed to rule out liver cancer. This meant that I had to undergo a risky and stressful ultrasound guided liver biopsy. This was a very difficult decision to make as all of the literature and guidance up until that point was to “never stick a needle in it!”. However, through many long phone calls and emails with several experts I made the decision to go through with it. Thankfully it was all clear and I was very grateful for the support of the team around me who helped me reached the decision.

Were you ever offered supplementation of Vitamin B12, Vitamin D and/or Calcium in the treatment of your Liver HHT?

The only supplementation specified by my Dr is vitamin D and occasionally Vitamin C (with calcium). However he cautions against over supplementation in both cases as it can be damaging to the liver. I have these levels checked regularly.

I also choose to supplement with silymarin (milk thistle) which has had some scientific and research based evidence that it can support liver function. I also take the best probiotics that I can afford to maintain a healthy gut which in turn supports my immune system and liver function. The science is becoming more evidential that a good gut health plays a significant role in our overall health including liver health

What impact has living with HHT of the liver had on your personal life: family, relationships, work, physical activity, psychological effect?

In many ways it is harder on my parents and husband as they understandably worry about me. The psychological impact has been hardest on us all especially during the period of initial diagnosis and when I had to go through the biopsy. For my part I have talked about it with a counsellor who has been a wonderful support to me through the years and has guided me when times have been tough.

From a physical perspective the diagnosis has made me become more aware of my body and how I need to look after it the best way I know how. This has required a long period of adjustment and education and I don’t always get it right. But knowing that I am trying is always a move forward.

Based on your experience, what advice do you think is most important to give to other people (usually women!) with your condition?

I have lots of thoughts on this!!

Firstly, the hardest lesson I have had to learn during this process is that we must all become our own best physician and work hard to find the best medical professionals that we can. Just because someone wears a white coat does not mean they are an authority on your body. If you don’t gel with a specialist, find another one. I had a very disappointing interaction with a specialist in Ireland who only wanted to paint the bleakest picture for me and was not at all open to how I can help myself. I dread to think who else he advises.

Alongside liver specialists it is very important that you find an excellent radiographer to conduct scans. I frequently lie on the hospital table with the head of radiography and their team around me all craning to have a look at the scans! Our condition is not for junior radiographers, we need the best there is!  When I had my liver biopsy it was conducted by the head of radiography and in hindsight I really appreciated his  expertise. It is essential for anyone who needs to go through such a procedure to really carefully consider who is doing it and their level of expertise and capability.

It is very important that we continue to educate ourselves and become experts in our field. I have found the following books to be very helpful understanding HHT with Liver VMs; Living with HHT by Sara Palmer and Your Complete Guide to Liver Health by Paul J. Thuluvath.

Become aware of how any medications or supplements you are taking impacts your body. The vast majority of pain killers are processed through the liver and anything that puts pressure on the liver should only be taken in consultation with your team of experts.

The biggest thing that I can do however is eliminate alcohol and sugar from my diet. Sugar, present in all refined carbohydrates (including my beloved gin!), can contribute to fatty liver disease and puts the liver under undue pressure. I have some small fatty deposits on my liver that I am working hard to eliminate and cutting out refined carbohydrate is essential for me. In addition the medical literature is overwhelming in advising that those with liver conditions should ingest zero alcohol. Easier said than done especially in Irish culture.

Women in particular should make sure that their hormone levels are accurate as this in turn can have an impact on how the body processes and stores sugar and as advised above this can have a significant impact on liver function if it is out of whack.

And lastly I am a big advocate for therapy. Therapy made an enormous difference to how I viewed myself and my condition and it has provided me with a metaphorical coat of armour that I can wear when things are tough. It is hard to always be positive so it is really great to have somebody there to pick you up when you are down.

Thank you Jane for taking the time today to talk to us about your experience of living with HHT in the Liver.

Facts about Liver AVMs









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