Hereditary Transthyretin Amyloidosis (hATTR)

Hereditary transthyretin (hATTR) amyloidosis is a rare genetic condition characterized by the abnormal accumulation of amyloid proteins throughout the body, particularly in the nervous and cardiac systems. hATTR amyloidosis may also affect the kidneys, eyes, and gastrointestinal tract. Common manifestations of this disease include progressive peripheral neuropathy, cardiomyopathy progressing to heart failure, and autonomic dysfunction, which may affect the digestive system.¹

hATTR-Specific Diets

According to some sources, no diet is specifically recommended for people with hATTR,² whereas others suggest that many factors, including chronic inflammation, geographical location, and eating habits, may influence the progression of hATTR.³  

Various studies have confirmed that increased salt consumption leads to enlargement of the interstitial space in cardiac muscle, the kidneys, and other tissues.³ Therefore, patients with the cardiac subtype of hATTR amyloidosis may benefit from a diet low in sodium.² It is recommended that they consume less than 6 grams of salt per day.³

Current recommendations for a low-sodium diet by the American Heart Association include choosing packaged and pre-prepared foods and condiments that are low in sodium, selecting canned foods that either are low in sodium or have no added salt, rinsing canned foods, avoiding highly salted or preserved meats, and using spices other than salt for seasoning.⁴ 

hATTR Impact on Nutritional Status

hATTR amyloidosis may affect nutritional status indirectly, through its effects on the autonomic nervous system, which cause digestive dysfunction, or directly, as amyloid is deposited within the gastrointestinal tract. Either process can result in gastrointestinal problems, especially gastroparesis, which causes early satiety, slow digestion, postprandial nausea and vomiting, anorexia, diarrhea, constipation, dehydration, vitamin deficiencies, and malnutrition/malabsorption.⁵

Chronic dehydration should be avoided because of the sensitivity of the interstitial space, where the hATTR amyloidosis disease process occurs, to fluid deficits. The daily recommendation is to consume between 25 to 30 mL of water per kilogram of body weight to ensure adequate hydration of the interstitial space.³

Water should be used to brew 1 L of green tea from 10 to 12 g of dried green tea leaves along with 200 mg of vitamin C for 3 minutes at 70℃ to 75℃. This amount of green tea should be consumed daily.³ In an observational study, the consumption of green tea extract for 1 year reduced amyloid fibril formation in the cardiac tissues, stabilizing left ventricular myocardial mass, in 25 men who had wild-type hATTR amyloidosis with cardiomyopathy.^2,6 

Increased consumption of fruits and vegetables, whole-grain carbohydrates, and natural, fresh meats and fish to maintain adequate nutritional status is suggested. Because potential digestive impairments caused by hATTR amyloidosis may lead to vitamin or mineral deficiencies, additional supplementation with vitamins, minerals, and trace elements should be considered.³

One source suggested that sugary foods, sausage products, and fried foods be avoided, and that consumption of milk fat, processed baked goods, and alcohol be reduced. Women with hATTR amyloidosis should not consume more than 15 g of alcohol per day, and men should not consume more than 30 g. Red wine is preferred.³

Nutrition and hATTR Quality of Life

A literature review analyzing methods to improve the quality of life of individuals with hATTR amyloidosis affecting the autonomic nervous system recommended pharmacologic interventions with vitamin supplementation, parenteral nutrition, and the daily intake of 12 g of cholestyramine to combat weight loss. Frequent small meals coupled with dopamine receptor antagonists and motilin receptor agonists were recommended for individuals affected by gastroparesis.⁵ 

Clinicians can use the modified body mass index to monitor the nutritional status of individuals with hATTR amyloidosis; poor nutritional status is indicated by a modified body mass index below 600.^7,8 Poor nutritional status predicts poor outcomes following organ transplant in patients with hATTR amyloidosis.⁸

References

1. Transthyretin amyloidosis. MedlinePlus. Accessed August 3, 2022.
2. Roberts JR. Transthyretin-related amyloidosis treatment and management: diet. Medscape. Updated July 19, 2022. Accessed August 3, 2022.
3. Altland K. A diet for familial transthyretin amyloidosis: strategies for delaying the onset and progression of the disease. Accessed August 3, 2022.
4. How to reduce sodium. American Heart Asssociation. Accessed August 3, 2022.
5. Gendre T, Planté-Bordeneuve V. Strategies to improve the quality of life in patients with hereditary transthyretin amyloidosis (hATTR) and autonomic neuropathy. Clin Auton Res. 2019;29(Suppl 1):25-31. doi:10.1007/s10286-019-00624-w
6. aus dem Siepen F, Bauer R, Aurich M, et al. Green tea extract as a treatment for patients with wild-type transthyretin amyloidosis: an observational study. Drug Des Devel Ther. 2015;9:6319-6325. doi:10.2147/DDDT.S96893
7. Luigetti M, Romano A, Di Paolantonio A, Bisogni G, Sabatelli M. Diagnosis and treatment of hereditary transthyretin amyloidosis (hATTR) polyneuropathy: current perspectives on improving patient care. Ther Clin Risk Manag. 2020;16:109-123. doi:10.2147/TCRM.S219979
8. Sekijima Y. Hereditary transthyretin amyloidosis. GeneReviews [Internet]. Revised June 17, 2021. Accessed August 3, 2022. 

Reviewed by Harshi Dhingra, MD, on 7/31/2022.

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Maria Arini Lopez, PT, DPT, CSCS, CMTPT, CIMT

Maria Arini Lopez, PT, DPT, CSCS, CMTPT, CIMT is a freelance medical writer and Doctor of Physical Therapy from Maryland. She has expertise in the therapeutic areas of orthopedics, neurology, chronic pain, gastrointestinal dysfunctions, and rare diseases especially Ehlers Danlos Syndrome.