Tips for Managing ATTR - CM: What Works for Me

For seven long years, the symptoms piled up: bilateral carpal tunnel, “trigger finger” in three fingers, peripheral neuropathy advancing up my arms and legs, continued gastrointestinal problems causing the eventual loss of more than 60 pounds, repeated bouts of severe orthostatic hypotension, and even an enlarged heart misdiagnosed as an “athlete’s heart.”

Nobody was able to connect the dots; my primary care physician, hand surgeon, gastroenterologist, endocrinologist, oncologist, neurologist, and cardiologist... no one had answers.

Finally, in February of 2019, at last, the amyloidosis team at Brigham and Women’s Hospital provided an answer. The diagnosis was hereditary amyloidosis with cardiomyopathy and polyneuropathy.

By fortunate timing, a newly trialed RNA-interference therapy had just become available, offering the possibility of drastically slowing, or even halting, the disease progression.

My approach to managing my diagnosis

Now, six and a half years later, here I sit, happy to share that all indications point to my disease progression being halted. The journey isn’t without its challenges— I continue to manage the lasting effects: peripheral and autonomic neuropathy, ongoing gastrointestinal complications, and an enlarged, stiff left ventricle of the heart, but life is moving along nicely.

At first, managing the disease was challenging, both physically and emotionally. Over time, however, I developed a three-part strategy that works for me: a mental health component, a physical exercise component, and a medicinal therapy component.

Component 1: Mental health

For me, addressing the mental health component had to come first. As my physical condition declined, I was forced to retire from a 30-plus-year career in nuclear reactor operations, and many of the activities I once loved, playing ice hockey, lifting weights, tackling heavy projects around the house, were no longer within my physical capabilities. Robed of both my work and my passions, I felt I had lost my identity and sank into depression.

With the support of my wife and siblings, I was able to reframe my situation and see it as an opportunity rather than a loss. Out of necessity, I reinvented myself.

In time, I discovered an aptitude for art, developed a passion for cooking, started tending to a small but rewarding vegetable garden. Most importantly, I found a way to give back— serving as a patient educator with the Amyloidosis Speakers Bureau, a nonprofit dedicated to raising awareness and educating the medical community.

Component 2: Physical health

I also came to realize that I needed to care for my physical health by focusing on exercises that my body could handle.

Peripheral neuropathy made walking difficult, though I manage well with walking sticks or a cane. With the guidance of a physical therapist, I developed an upper-body strength training routine that wasn’t overly taxing on my heart, which has been weakened by thickening and stiffening that limits my endurance.

These adjustments also opened new opportunities: my wife and I now enjoy daily walks with our dogs, mostly along a flat rail trail that I find manageable, and I’ve been able to continued strength training— though at a gentler pace than before.

Component 3: Symptom management

Lastly, I came to understand that my health depended not only on halting disease progression but also on carefully managing each of my symptoms. This required a strict routine with multiple prescription medications - treatments for fluid buildup in my lower extremities, orthostatic hypotension, gastrointestinal issues, and neuropathy-induced muscle cramps.

Over time, my doctors and I worked together to fine-tune the dosages so that I could gain the greatest benefit. These medications, combined with the RNA-blocking therapy, have helped maximize my physical well-being.

This three-part strategy has truly worked for me- focusing on mental health, physical exercise, and medical therapy. If you’re searching for a way to manage your disease, I encourage you to give it a try.