“Doing What it Takes”: Effective Approaches for Navigating Life with ATTR

Nearly seven years have passed since my diagnosis and the beginning of treatment for hereditary amyloidosis. Happily, I am convinced that the progression of the disease appears to have been halted, and life is going well. That said, I still need to stay on top of the symptoms that remain and make adjustments as needed.

Whether it’s staying disciplined with my medication, relying on a cane when needed, or adapting the scope of what I can and cannot do for work, I’ve learned to "do what it takes" to keep moving forward.

Staying on top of my medications

In my case, the disease has manifested through cardiomyopathy, peripheral neuropathy, autonomic neuropathy, and gastrointestinal complications. While the RNA-silencing therapy I receive has kept the disease progression in check, I’ve come to realize the importance of strict discipline with my prescribed medications. Maintaining a consistent routine has been very beneficial in managing these symptoms and preserving my physical wellbeing. All of my prescribed medications, midodrine for hypotension, gabapentin for nerve pain, furosemide for fluid retention, vitamin A for retinol replacement, and omeprazole for acid reflux, have proven very effective, provided I remain disciplined about taking them.

Managing hypotension

For me, the most persistent and troublesome symptom has been orthostatic
hypotension. Over the past 7 years, I’ve fainted on 6 separate occasions due to sudden drops in blood pressure. The first 3 episodes occurred before I was prescribed midodrine to help manage the condition. A fourth episode happened shortly after starting the medication, while we were still working to fine‑tune the dosage. The most recent event took place last year, and that one was my fault, as I had not been as mindful as I should have been about following the strategies I rely on to keep the problem in check.

I learned to manage my hypotension by first identifying situational triggers. I’m most vulnerable after long periods of sitting or sleeping, especially following extended car rides or travel flights. Meals can also leave me susceptible. To counter these risks, I’ve developed a set of strategies:

• When rising from a chair or bed, I transition slowly and remain close by until I feel steady.
• After driving, I step out of the car and lean against it for a few minutes before walking away.
• And because larger meals tend to worsen the problem, I try to eat smaller, more frequent, meals throughout the day. This adjustment not only reduces hypotension episodes but also eases gastrointestinal symptoms that I experience.

Navigating gastrointestinal complications

Regarding gastrointestinal symptoms, my most persistent challenges include occasional nausea while eating, episodes of vomiting, acid reflux, bloating, and feeling full too quickly. My gastroenterologist has explained that these issues are likely the result of amyloid deposits affecting multiple parts of the digestive system— the mucosal lining, the muscles of the GI tract, the nerves that regulate its function, and even the blood vessels that supply it.

Fortunately, my gastrointestinal symptoms have not worsened since beginning treatment for amyloidosis. At this stage, it is primarily a matter of managing them day to day. I have found omeprazole effective in reducing acid reflux, smaller meals helpful in easing bouts of nausea, and a small dose of Gaviscon beneficial when taken before eating. In addition, using Pepto-Bismol proactively has been very helpful in keeping diarrhea under control.

Coping with fatigue

Another frustrating symptom is the sudden onset of fatigue, which often seems to strike without warning or pattern. Over time, I have learned to “listen to my body.” Some days I have plenty of energy, while other days I struggle just to get through. To cope, I keep a “to‑do” list and tackle tasks as my energy allows, while also giving myself permission to slow down and rest when needed. On days when I have less energy, I lighten the mood by joking to myself, “Never do today what you can put off until tomorrow.” That mindset helps ease the frustration and makes the ups and downs easier to accept.

My strategies for air travel

Lastly, I have developed specific strategies for navigating certain situations, such as with air travel. Managing airports and flights requires careful planning, the use of a cane for support, and proactive steps to reduce my susceptibility to orthostatic hypotension. Before heading to the airport, I always take midodrine, which helps prevent drops in blood pressure after long periods of sitting, especially during flights. I also build in extra time, arriving nearly 3 hours before departure to account for my slower pace. Once on board, I make a point of standing and walking the aisle every 45 minutes or so, another simple but effective way to keep hypotension under control.

My approach to navigating life with ATTR-CM

As I mentioned up front, I’ve learned to “do what it takes” to keep moving forward. Over time, I’ve refined these strategies through trial and error. Each of us living with amyloidosis faces a unique mix of symptoms to manage, and it takes patience to discover what works best.

My hope is that you’ll give yourself the time to fine‑tune your own approach — and if your symptoms happen to resemble mine, perhaps some of the strategies I’ve shared may be of use to you.