Living with hATTR-CM (hereditary transthyretin amyloid cardiomyopathy) — a serious genetic disorder that gets worse over time — can be challenging.
Treatment goes beyond medication: how you move, what you eat, and where you’re at mentally all play a role. Taking small, consistent steps can help control symptoms and protect your heart over time.
Follow these tips to feel better and boost your quality of life.
Stick to Your Medication
Medication is a must when it comes to treating hATTR-CM. There are two types: transthyretin (TTR) stabilizers and TTR RNA-targeted therapies.
TTR stabilizers help keep the TTR protein in your body stable by attaching itself to the protein to keep it strong, so it's less likely to fall apart and form dangerous clumps. These include:
TTR RNA-targeted therapies turn off the instructions that tell your body to make the TTR protein. As a result, your liver makes less TTR protein, which ideally means fewer clumps. These include:
Patisiran is specifically designed to treat polyneuropathy (nerve damage) in people with hATTR-CM. The nonsteroidal anti-inflammatory drug (NSAID) diflunisal is sometimes used "off-label" to treat hATTR-CM. That’s when a doctor prescribes a medication for a condition it’s not FDA-approved to treat.
Taking your medication as prescribed eases symptoms and helps avoid unnecessary hospital stays, diagnostic tests, and doctor visits. Research shows that many people who take hATTR-CM medication stick with it. But some struggle for lots of different reasons, such as forgetting to or not understanding how to take their medication and uncomfortable side effects.
You’re more likely to follow your treatment plan and feel confident about your care when there’s clear, open communication between you and your doctor and pharmacist. You should understand why a drug is needed, how a drug works, and what to expect.
Practical support, like written instructions, pill organizers, or automatic medication dispensers, can also make it easier to stay on track with treatment.
Some people find it hard to start or continue treatment because they don’t trust the health care system. This can be especially true for people of west African descent, who are also more likely to carry the genetic mutation.
Keyur Shah, MD, interim chief of cardiology at VCU Health Pauley Heart Center in Virginia, says building trust often starts with education and outreach through community programs. He also suggests improving awareness of and treatment for hATTR-CM outside specialty clinics.
“If people are recognizing and treating this disease just like they treat hypertension or diabetes, [patients] will be engaging with a provider they trust and have known for many years,” Shah says.
Track Vital Signs and Stay on Top of Doctor’s Appointments
Living with a lifelong condition means seeing doctors regularly so they can adjust your care if needed. hATTR-CM can lead to high blood pressure, so you’ll need to monitor yours and keep a record to share during appointments. You’ll also want to monitor your heart rate and oxygen levels. Write down the date and time after each reading in a notebook or in your smartphone.
Prepare for emergencies
Emergencies can happen at any time with a heart condition. It’s critical to have a simple plan for when to get emergency care, such as serious shortness of breath, chest pain, or fainting. Keep a list of emergency contacts in an easy-to-find place you can access quickly if needed.
Lifestyle Changes
Everyday habits, like what you eat and drink, play a big role in how you feel with hATTR-CM.
Be mindful of what you eat and drink. hATTR-CM can lead to fluid buildup, so your doctor may suggest limiting the amount of salt you eat and how much fluid you drink. Focus on fresh, whole foods that aren’t heavily processed. Include plenty of fruits and vegetables, along with healthy protein sources like beans, fish, skinless chicken, tofu, chickpeas, and lentils. It’s also a good idea to cut back on added sugar and high-fat foods.
Exercise regularly. It’s important to stay active. Low-impact movement like walking, swimming, and biking can boost strength and energy levels. If you start to feel tired while exercising, take a break. Ask your doctor which exercises are safe for you and how often you can do them.
Avoid smoking and excessive drinking. Smoking affects the lungs and blood vessels, which can add extra stress to the heart. Alcohol, in large amounts, can weaken the heart muscle over time.
“Any additional stress around the lungs or heart is going to make bad symptoms feel worse,” Shah says.
Get quality sleep. People with hATTR-CM often face sleep issues, including insomnia. Small habits can make a big difference. Try to go to bed and wake up at the same time every day. Avoid heavy meals, caffeine, nicotine, and alcohol close to bedtime. Make sure your sleep environment is calm, dark, and quiet.
Take care of your mental health. It’s just as important as caring for your body. Use relaxation techniques, breathing exercises, or yoga to relieve stress. Consider seeing a therapist or talking to a trusted family member or friend. Joining an online or in-person support group can also make hATTR-CM easier to manage. Mackenzie’s Mission, Amyloidosis Support Groups (ASG), The Amyloidosis Foundation, and The Amyloidosis Research Consortium (ARC) offer trusted information and can connect you to people facing the same challenges.
Show Sources
Photo Credit: iStock/Getty Images
SOURCES:
Keyur Shah, MD, interim chief of cardiology, VCU Health Pauley Heart Center, Virginia.
Cleveland Clinic: “ATTR-CM (Transthyretin Amyloid Cardiomyopathy),” “Living With ATTR-CM: Your Questions Answered.”
American Heart Association: “Transthyretin Amyloid Cardiomyopathy (ATTR-CM).”
Patient Preference and Adherence: “Baseline Characteristics and Secondary Medication Adherence Patterns Among Patients Receiving Tafamidis Prescriptions: A Retrospective Analysis Using a National Specialty Pharmacy Dispensing Database.”
American Journal of Cardiovascular Drugs: “Contemporary Oral Medication Use and Frequency in Patients with Transthyretin Amyloid Cardiomyopathy.”
JACC Journals: “26-A-8296-Acc Adherence And Persistence With Vutrisiran, An Rnai Therapeutic For The Treatment Of Transthyretin Amyloidosis.”
MSD Manual: “Adherence to Medication.”
Orphanet Journal of Rare Diseases: “Patient and family experience with transthyretin amyloid cardiomyopathy (ATTR-CM) and polyneuropathy (ATTR-PN) amyloidosis: results of two focus groups.”
Mayo Clinic: “Sleep tips: 6 steps to better sleep.”
World Heart Federation: “Living With Transthyretin Amyloid Cardiomyopathy: A Toolkit For Patients.”
