Weight Loss with Amyloidosis in South Africa

Types of Amyloidosis and Their Nutritional Impact

Amyloidosis is classified by the precursor protein that misfolds into amyloid fibrils. The three most clinically significant systemic types have distinct nutritional implications:

Wild-Type ATTR: The "Older Male" Amyloidosis

Wild-type ATTR (previously called "senile systemic amyloidosis") is an increasingly recognised cause of heart failure in older South African men (predominantly over age 70). It is substantially more common than once thought and is underdiagnosed because its echo findings overlap with hypertensive heart disease. Weight loss, early satiety, and GI symptoms in an older man with unexplained heart failure should trigger consideration of amyloid cardiomyopathy.

hATTR: ATTR in Black South Africans

Hereditary ATTR amyloidosis (hATTR) caused by the Val122Ile (V122I) TTR mutation is present in approximately 3–4% of Black individuals of West African ancestry — including a significant proportion of South Africa's Black population. This mutation causes amyloid cardiomyopathy with onset typically in the 60s–70s. This is an important South African consideration: unexplained cardiomyopathy in older Black South African patients warrants TTR gene testing and amyloid workup.

GI Amyloidosis: When the Gut is Infiltrated

Amyloid deposits in the gastrointestinal tract cause a range of symptoms depending on where deposits occur:

GI Motility Disorders

Amyloid infiltration of the autonomic nerves supplying the gut (particularly in ATTR and AL) causes autonomic neuropathy that disrupts normal GI motility:

• Gastroparesis: Delayed gastric emptying causes early satiety, nausea, vomiting, bloating, and unpredictable blood glucose in diabetic patients. Small, frequent meals replace standard three-meal patterns.
• Constipation: Slowed colonic transit causes constipation that can be severe — dietary fibre, hydration, and motility medications are required.
• Diarrhoea: Alternating with constipation; bacterial overgrowth in a dilated, poorly motile gut can cause chronic diarrhoea and malabsorption.
• Pseudo-obstruction: In severe cases, functional obstruction from absent colonic motility may require hospital management.

Malabsorption from Direct Mucosal Infiltration

When amyloid deposits infiltrate the intestinal villi directly, absorption of all macronutrients is compromised:

• Steatorrhoea (fatty stools) from fat malabsorption
• Protein-losing enteropathy — serum albumin falls as protein leaks into the gut lumen
• Micronutrient deficiencies: vitamins B12, D, zinc, iron, folate
• Progressive weight loss despite adequate dietary intake

Macroglossia: When the Tongue is Too Large

AL amyloidosis specifically causes macroglossia (tongue enlargement from amyloid deposits) in approximately 10% of cases. Macroglossia impairs chewing, swallowing, and speech. Dietary texture modification — soft, minced, puréed foods — and referral to a speech-language therapist is essential when macroglossia is present.

Dietary Strategies for GI Amyloidosis

For Gastroparesis

• Small, frequent meals: 5–6 small meals instead of 3 large ones. A full stomach in gastroparesis causes nausea and vomiting — small portions help.
• Low-fat meals: Fat slows gastric emptying further. In amyloid gastroparesis, reduce meal fat content (unlike CF or Huntington's where fat is encouraged). Cook with minimal oil; choose lean protein sources.
• Low-fibre meals: High-fibre foods form bezoars (undigested food masses) in gastroparetic stomachs. Avoid raw vegetables, fruit skins, seeds, and legumes if gastroparesis is significant. Puréed or well-cooked vegetables are safer.
• Liquid calories: Liquids empty faster than solids from the stomach. Smoothies, soups, and nutritional drinks (Ensure, Fresubin) may be better tolerated than solid meals.
• No lying down after meals: Remain upright for 2 hours after eating to use gravity to assist gastric emptying.

For Malabsorption and Protein-Losing Enteropathy

• High-protein intake: When albumin is low from protein loss, increase dietary protein to 1.5–2.0 g/kg/day. Focus on easily absorbed, high-quality protein: eggs, fish (pilchards, hake, tuna), chicken, amasi, low-fat dairy.
• Medium-chain triglycerides (MCT): MCT oil is absorbed directly into the portal blood without requiring bile acid emulsification — useful when fat malabsorption is present. Add MCT oil to shakes and soups (available at specialist dietitian-recommended suppliers).
• Oral nutritional supplements: High-protein, easily absorbed formulas (Ensure High Protein, Fresubin Protein Energy) help when food intake alone is insufficient.
• Micronutrient monitoring: Test and supplement vitamin B12, vitamin D, zinc, iron, and folate at least twice yearly.

Cardiac Amyloidosis: Nutrition Under Fluid and Sodium Restriction

Amyloid cardiomyopathy (most prominently in ATTR and AL types) causes a restrictive cardiomyopathy — the heart cannot fill adequately due to amyloid-stiffened walls. Heart failure management in amyloid cardiomyopathy differs importantly from standard heart failure:

Fluid Restriction

Fluid overload causes acute pulmonary oedema in amyloid cardiomyopathy. Fluid restriction is usually required:

• Typical target: 1,500–2,000 mL total fluid per day (all drinks, soups, ice cream, jelly)
• Measure and track all fluid intake — use a marked water bottle
• Hot weather in South Africa increases insensible fluid losses — discuss with cardiologist whether the target can be liberalised temporarily in extreme heat
• Thirst-quenching strategies: small ice chips, mouth rinses, sour sweets that stimulate saliva

Sodium Restriction

• Restrict sodium to 1,500–2,000 mg/day (<4 g salt/day) to reduce fluid retention
• Avoid adding salt at the table; use herbs and spices (dhania, turmeric, ginger, garlic, chillies) for flavour — South African cuisine is well-suited to flavourful low-sodium cooking
• Avoid processed foods: tinned soups, processed meats (polony, viennas), restaurant food, fast food, stock cubes
• Fresh rooibos tea is naturally sodium-free — an ideal fluid choice within daily limits

Caloric Balance in Cardiac Amyloidosis

The paradox of cardiac amyloidosis is that fluid restriction limits total food and drink volume, yet weight loss from systemic disease must be countered with adequate calories. Strategies:

• Choose calorie-dense foods that contain minimal water — nuts, nut butters, hard cheeses, dried fruit (small amounts), avocado
• Eat soups infrequently — they are mostly water and count toward fluid restriction
• Focus on solid meals with high caloric density to meet energy needs within fluid limits

Renal Amyloidosis (AA and AL): Dietary Modifications

AA amyloidosis primarily targets the kidneys, causing proteinuria, nephrotic syndrome, and progressive renal failure. Renal involvement also occurs in AL amyloidosis. Nutritional modifications for renal amyloidosis align with standard chronic kidney disease (CKD) dietary management:

• Protein: Historically restricted in CKD, but nephrotic syndrome protein losses may require maintained protein intake. Current guidance is individualised — discuss with nephrologist.
• Potassium: Restrict if eGFR falls below 30 mL/min/1.73m². High-potassium SA foods to limit: bananas, avocado, tomatoes, potatoes, butternut, dried fruit, paw paw, nuts.
• Phosphate: Restrict with advancing CKD. Avoid: cola drinks, processed meats, dairy in large quantities, nuts in large quantities. Use phosphate binders as prescribed.
• Fluid: Restrict if oedema and reduced urine output are present.
• Salt: Restrict to reduce blood pressure and proteinuria — standard 2 g sodium/day advice applies.

Exercise in Amyloidosis

Exercise capacity in amyloidosis depends entirely on cardiac involvement and overall disease status:

• Cardiac amyloidosis: Moderate exercise may be beneficial and safe in early-stage disease under cardiologist guidance. High-intensity exercise, heavy resistance training, and dehydrating activities are contraindicated. Gentle walking, tai chi, and light resistance work are generally appropriate. A 6-minute walk test by the cardiology team establishes baseline exercise capacity.
• Peripheral neuropathy (ATTR/AL): Balance is impaired — avoid activities with high fall risk. Seated exercise, pool walking, and physiotherapy-guided strengthening reduce fall risk while maintaining fitness.
• GI-dominant amyloidosis: Exercise tolerance is usually acceptable. Light to moderate aerobic exercise 3–5 times weekly supports gut motility and mental health.
• During active treatment (chemotherapy for AL): Rest periods between cycles are appropriate; gentle walking is beneficial even during treatment.

South African Resources for Amyloidosis

Amyloidosis is diagnosed and managed at tertiary academic hospitals in South Africa:

• Haematology (AL amyloidosis): Major academic hospitals — Charlotte Maxeke, Groote Schuur, Tygerberg, Inkosi Albert Luthuli — have haematology units experienced in plasma cell disorders. Stem cell transplant for eligible AL patients is performed at a small number of SA centres.
• Cardiology (ATTR amyloidosis): Cardiac amyloid diagnosis via nuclear pyrophosphate scan is available at selected SA centres. TTR genetic testing through clinical genetics services. New ATTR-specific treatments (tafamidis, patisiran, inotersen) are approved internationally and being accessed through SAHPRA pathways.
• Amyloidosis Foundation: amyloidosis.org — international patient resource with extensive nutrition and disease management guides
• UK National Amyloidosis Centre: ucl.ac.uk/amyloidosis — comprehensive disease-specific resources applicable to South African patients
• ADSA (Association for Dietetics in South Africa): adsa.org.za — locate a registered dietitian with experience in renal, cardiac, or oncological nutrition for amyloidosis support
• Medical aid PMB: Amyloidosis may qualify for PMB cover under relevant chronic conditions (CKD, heart failure) — check with your scheme's PMB coordinator

Key Takeaways

• Amyloidosis is not one disease — type (AL, ATTR, AA) and organ involvement determine the correct nutritional approach
• Weight loss is a consistent early feature of amyloidosis — countering it requires proactive dietitian involvement from diagnosis
• GI amyloidosis causes gastroparesis (small, low-fat, low-fibre meals), malabsorption (high protein, MCT oil, micronutrient supplementation), and protein-losing enteropathy
• Macroglossia (enlarged tongue in AL) requires texture-modified diet and speech-language therapy
• Cardiac amyloidosis requires fluid restriction (1,500–2,000 mL/day) and sodium restriction (under 2 g/day) — use calorie-dense solid foods within fluid limits
• Renal amyloidosis follows CKD dietary principles: restrict potassium, phosphate, sodium, and adjust protein per nephrologist guidance
• Exercise is organ-dependent: cardiac amyloidosis requires cardiologist-guided limits; neuropathy requires fall-prevention focus
• The Val122Ile TTR mutation causing cardiac amyloidosis is found in 3–4% of South Africans of West African descent — unexplained cardiomyopathy in older Black South African patients warrants amyloid workup
• New targeted treatments (tafamidis for ATTR, daratumumab-based regimens for AL) are transforming prognosis — specialist referral as early as possible improves outcomes

This article is for informational purposes only and does not constitute medical advice. Amyloidosis requires specialist haematological, cardiological, or nephrological management depending on type and organ involvement. Always consult your specialist team and registered dietitian before making dietary changes.