Coordinating care for ATTR cardiomyopathy (ATTR-CM)

As their name implies, transport proteins are proteins that help move substances throughout the body. One example is transthyretin (TTR), a transport protein produced in the liver. TTR is a transport protein for vitamin A and thyroxine, a hormone produced by the thyroid gland.

Transthyretin amyloidosis (ATTR) is a rare disorder that occurs when the body produces unstable TTR protein. The unstable TTR breaks apart, and the pieces (called subunits) misfold to form abnormal shapes. These misfolded subunits clump together to form deposits called amyloid deposits, which can build up in various organs and tissues throughout the body.

ATTR cardiomyopathy (ATTR-CM) occurs when these amyloid deposits accumulate in the muscles of the heart, causing these muscles to thicken and stiffen, and interfering with the normal functioning of the heart. ATTR-CM is a progressive condition that can lead to heart failure, arrythmias (abnormal heartbeat), and other serious complications.

Treatment options for ATTR-CM have improved significantly since 2019, with the approval of multiple medications that can help slow or stop amyloid deposit formation and buildup. While these therapies cannot reverse ATTR-CM, they can slow the progression of the disease, and it’s recommended that a person begin treatment with these therapies as early as possible.

The healthcare providers on your ATTR-CM care team

Treatment for ATTR-CM is typically overseen by a cardiologist, ideally a cardiologist that specializes in treating ATTR or has experience treating it. However, ATTR can involve multiple organs and affect many areas of a person’s health, and a person will need to work with a multidisciplinary team of healthcare providers with different specialties. This team can include:

• Primary care provider
• Genetic counselor
• Cardiac surgeon
• Radiologists and imaging technicians
• Nurses
• Pharmacists
• Physical and occupational specialists
• Social workers and mental health professionals

A person may need to work with other specialists if ATTR is affecting other organs, such as the nervous system, kidneys, eyes, joints and bones, gastrointestinal system, or genitourinary system.

Coordinating care among your care team

When working with multiple healthcare providers, it’s important that the different members of your team are in communication with one another. This is referred to as “care coordination.”

Here are a few strategies that can help:

Work with a specialized team

One of the best approaches is to work with a multidisciplinary team that specializes in the diagnosis and treatment of ATTR and/or ATTR-CM. A specialized team will be based at a single location, such as a hospital or medical center. This will make coordinating care between these healthcare providers easier and more straightforward.

However, the healthcare providers a person works with are often dictated by what healthcare providers are available where they live. Not everyone will have access to this type of specialized healthcare team.

If you do not have access to a center that specializes in ATTR-CM, you’ll need to work with a cardiologist who is able to consult with a specialized team and may need to coordinate care among different providers and teams.

Here are two additional strategies that can help.

Designate a point person

If you are working with multiple providers at multiple locations, it helps to designate one healthcare provider as your "point person." This provider should maintain an overview of the different aspects of your care and treatment for ATTR-CM—information about your diagnosis, treatment, and contact information for the other providers you’ve worked with. This may be your cardiologist or in some cases your primary care provider.

Keep a personal health record

This is a file that includes all paperwork and documents related to ATTR-CM as well as other aspects of your health and healthcare. This file should contain:

• Test results including results of tests like imaging tests, echocardiograms, electrocardiograms (ECGs), biopsies, and bloodwork
• Evaluations and workups from specialists
• Treatment records, including medications you've been prescribed and medical procedures
• Genetic testing results and family history information
• Contact information for all of your healthcare providers
• Insurance information
• Copies of bills, receipts, explanation of benefits documents, and other information related to the financial aspects of treatment

You can also include your own notes from appointments, including questions you've asked and information provided by healthcare providers. While this can take time and effort, it can also save you time and effort—for example, if you need to contact your insurance company or if you are referred to a specialist.