Updated: Dec 20, 2019
At my first CHD appointment of 2019 my doctor said they are beginning to automatically add all liver blood work to the routine labs for Fontan Patients since we have different versions of Liver Disease, Fibrosis, or other concerns-depending on our individual anatomy. Some CHD Adults are diagnosed with Cirrhosis of the Liver, and some have what they now call FALD (Fontan Associated Liver Disease).
CHD Doctors are now seeing Liver Cancer more and more in Fontan patients - spread the word. Get your tests. Make sure your CHD team has a new routine of including all liver labs in your routine blood work.
I have been going to a Liver doctor on and off since I was diagnosed with Cirrhosis at 19 years old. Thankfully, and accidentally, my Gynecologist found seven spots on my liver while doing my tubal ligation. She said, "because of your serious heart condition, I thought it wouldn't hurt to look around at surrounding organs while I was in there." She couldn't believe what she had found. She recommended I see a Liver specialist immediately after healing from my procedure. This was terrifying as there was no information on anything related to CHD and the liver...yet. So, I thought it was from too much partying. After many tests, my mom and I went to my first Liver appointment and that is where I was diagnosed with Cirrhosis of the liver. I found out ten years later they were finding more and more Fontans with the same or similar liver. I can do a follow up post on how this affected me mentally and emotionally later.
Recently after waiting for 4 months to find out if one of the three small tumors on my liver was cancerous, I finally got to hear the magical words, "no it's not cancer, but we need to keep an eye on it and test it every three months."
If they catch Liver cancer that is only in the liver, they can kill it in a quick procedure where chemo will go straight into the liver instead of throughout the body, following with a one to two day hospital stay. There are other options as well, but that's what my doctor said he'd want to do with me if my next appointment came back as a yes. So, thankfully it's a no.
I have not seen any information in the CHD Community about Post Fontan Liver Cancer and wanted to share it with the community in hopes of helping and educating people as well as hopefully saving lives. There are often no symptoms of Liver Cancer until it's too late, but with MRI's they can catch it early. Please make sure to ask your CHD Doctor if he or she has a recommendation or relationship with any Liver Doctors who are familiar with Fontan patients - this is critical.
My advice to parents with CHD children in 2019 would be to ask your child's CHD doctor when he/she thinks it is best for your child to begin liver tests, as each of us are different. And please keep in mind-we have technology and lots of research on our side. There's nothing we can do about it except monitor it as we grow...Yet! Never doubt what our amazing advancements in medicine can do, we have already come so far in such a short amount of time. I am excited and hopeful of the future for CHD in every aspect.
Below is a quote from an article I found:
"This study demonstrates high mortality following a diagnosis of HCC in patients undergoing a Fontan operation that is comparable to the <50% 1-year survival among patients with HCC secondary to other etiology.3 Patients undergoing the Fontan operation are at risk of developing HCC from a young age (early second decade), confirming observations from previous case reports. In this cohort of patients with HCC undergoing the Fontan operation, 50% had a diagnosis of cirrhosis, which is in contrast to only 20% of patients who develop HCC in the context of other etiologies in noncirrhotic livers.4 This suggests that either there is an increased risk of HCC development in patients with noncirrhotic livers after the Fontan operation or perhaps that cirrhosis is being underdiagnosed. It is interesting to note that a previous study reported 5 cases of HCC among 113 patients after Fontan palliation (4%) followed in a single institution who undergo routine abdominal imaging surveillance.5 In their study, the authors report that 4 of the 5 patients had no evidence of chronic liver disease on the initial liver imaging performed <2 years before the HCC diagnosis, suggesting that HCC can occur rapidly in the Fontan population. Along with the results from our study, this suggests that liver imaging alone may be insufficiently sensitive to risk stratify those at risk of HCC development and has important implications for screening strategies."
Please get your Livers checked regularly, especially if you are a CHD Adult with the Fontan and share this article to help save lives! I hope this article and blog helped you. I am a living, breathing, walking, "guinea pig" in this alone as one of the oldest living HLHS Survivors and complex Fontan patients. Please support my work by giving credit for where you found your resources-wherever that is. If I have helped you, you can tag and/or share on Facebook or Instagram. If I have inspired you, please leave a review. There is no payment with money for Advocates (yet) and this is how you can show your appreciation. Thank you!
Pic 1: Trying to stay positive for cancer results
Pic 2: MRI #2 in 3 months...complete
Pic 3: Liver Detox Tea post workout