Town hall sheds light on silent killer in Black community
By Fritz Esker
Contributing Writer Louisiana Weekly
Pharmaceutical giant Pfizer has become famous during the COVID-19 pandemic for their vaccines, but they have also been spreading awareness about a lesser-known-but-still-fatal condition called transthyretin amyloid cardiomyopathy (ATTR-CM). It’s a condition in which 90 percent of the victims are Black.
As part of the effort to spread awareness about ATTR-CM, Pfizer awarded $46,500 in sponsorship funding to the Minority Health Institute, Inc. (mhinst.org) to hold a town hall program called Voices for the Heart. According to data from the MHI, ATTR-CM affects four percent of the Black population in the United States, about 1.5 million people.
In the latest town hall in the series, Dr. Russell Ledet of Docs on Da Block discussed ATTR-CM on December 15. Ledet said ATTR-CM is often undiagnosed and misdiagnosed. The symptoms of the disease are often mistaken for other issues. For example, carpal tunnel syndrome can be a symptom of ATTR-CM.
ATTR-CM is also not a rapid onset disease. The symptoms slowly appear and build over time.
The website Your Heart’s Message (yourheartsmess-age.com) is a resource on ATTR-CM information. According to the website, some ATTR-CM patients report seeing up to five doctors before getting an accurate diagnosis. Dr. Selim Krim, a transplant cardiologist with the cardiomyopathy and heart transplant section of Ochsner’s Heart and Vascular Institute, said part of the reason for this is that the disease was a mystery even to doctors for a long time.
“This is a condition we did not know very much about 20-25 years ago,” Dr. Krim said.
What is ATTR-CM? The protein transthyretin travels through the bloodstream. In a patient with ATTR-CM due to aging or heredity, that protein misfolds. The misfiled proteins accumulate and drift into different parts of the bloodstream. It can cause the heart muscle to thicken and stiffen, leading to heart failure. The proteins can also travel all over the body, which is why some patients experience carpal tunnel syndrome. Aside from carpal tunnel syndrome, symptoms can also include the following: fatigue, tendon rupture, gastrointestinal issues, joint pain, swelling in the lower legs, eye disorders like glaucoma, shortness of breath, irregular heartbeat, pain or numbness in the lower back, and decreased sensation or a tingling sensation in the toes and feet.
Age-related ATTR-CM primarily affects white men, but the genetic version almost exclusively targets Black people. In extreme cases, a patient may require a heart transplant and a liver transplant since the protein is produced in the liver.
Ledet said the first step is to seek professional advice and assistance. The earlier it is diagnosed, the sooner it can be managed and treated. Treatments can both prolong life and improve the quality of life. When the disease progresses far enough, patients can neither lie flat nor exert themselves.
“Visiting your cardiologist is extremely important, especially if you’re between the ages of 50 and 60,” Ledet said.
For people unsure whether or not they should see a specialist about it, Ledet said they should mention it to their primary care doctor. A doctor will be able to refer a patient to a cardiologist if necessary.
“This is something you should be bringing up with your doctor when you visit the office,” Ledet said.
Ledet added that ATTR-CM is something Black people should be bringing up with their family members, especially if they receive an ATTR-CM diagnosis. Since there is a strong genetic component to ATTR-CM, doctors will do genetic testing to determine if someone has the gene mutation associated with hereditary ATTR-CM. So it’s important for those diagnosed with ATTR-CM to encourage their family members to get genetic testing.
Once diagnosed, patients will receive lifestyle modification plans similar to those used by patients with congestive heart failure. Dr. Krim said patients will go on a low sodium diet of less than 2000 mg of sodium a day (a challenge in a seafood-heavy city like New Orleans). They will also limit fluid intake to 50-60 ounces of fluid a day. This is also challenging because ATTR-CM patients will feel thirsty, but the reality is their bodies are over-hydrated so they need to resist that urge.
Dr. Krim said one important area where ATTR-CM treatments differ from those for other cardiomyopathies is in medication. The medicines used to treat the types of heart failure ATTR-CM can be mistaken for will lower the blood pressure. This is a problem since ATTR-CM causes low blood pressure. If the patient receives that medication, they will be prone to fainting at passing out. This is why proper diagnosis is so important. But there are specific medications that will effectively stabilize production of the faulty protein. For patients who are also experiencing nerve issues like carpal tunnel syndrome, there is a medication that will stop production of that protein altogether.
“We have great tools and medicines to help (people with ATTR-CM),” said Dr. Krim.
This article originally appeared in The Louisiana Weekly.