In the 60s, my cousin died at age 12 from an ‘enlarged heart’. We also lost two uncles at age 50 from sudden cardiac death. In the 70s, my brother died at age 37 from the same cause. Since 2010, we unexpectedly lost three nieces. One of their brothers suffered from a massive stroke and another niece reported palpitations. Despite the major occurrence of these issues in our family, I am not able to convince any of them to get tested for HCM. I chose to attack the problem head-on and chose Dr. Barry Maron to be my HCM specialist and he saved my life. Though he said that I had to take a break from my active lifestyle and could not bike or hike and had to avoid stairs, I knew it was only to help me. He also said that I should consider moving out of Alaska but when I refused, he said that I had to learn about HCM and find a doctor here that was willing to learn as well. Because of my family history as well as recent evidence of sustained rhythm issues, it was recommended that I receive an implantable cardioverter-defibrillator (ICD). I’ve had 5 ICDs and have been hospitalized for a blood clot. I still walk a tightrope daily but my sparkies (ICD) give me a safety net.
In 2015, I reached the lowest point in my emotional health and was dealing with an onrush of aggressive symptoms like fluid retention and memory issues. I was also dealing with a lack of appetite and weight and muscle loss. In 2016, I had an ablation surgery, but I knew that I was running out of time. With the help of the HCMA, I found a doctor at OHSU who recommended that I start seeing a transplant team because at my current rate of progression, it was a possibility that I might need a transplant sooner than later. Around the same time, I was given the opportunity to sign up for the Mavacamten drug trial. Because of it, my mental health, as well as my quality of life, have greatly improved. Though I still have many of the same issues, they are far more manageable. The biggest improvement is that I am not as fatigued and my memory is no longer in decline. I am happy to be living a full life again. I had stopped gardening, volunteering, cooking, raising poultry, music, and walking, but now I am glad to be able to return to all those activities. I have my life back and even have energy to spare! I have even started biking again with the help of an electric bike. I am doing so well that no one will bike with me because they can’t keep up!
As for my children’s congenital disabilities and in-utero brain deformities due to low oxygen, no one has answers for that. Was it because of my heart’s inability to keep up during pregnancy and delivery or was it for other reasons? No one knows for sure. We have a lot to learn and I am unsatisfied with still not having an answer. I worry for my kids a lot and they both need to be tested for HCM but neither has made it a priority. How will they manage with yet another diagnosis thrown in the mix?
