In all types of amyloidosis, it is important that treatment should support the function of organs containing amyloid. In ATTR amyloidosis this may include:
Treatment for heart disease
ATTR amyloid deposits in the heart cause the heart to stiffen which can lead to symptoms of heart failure. Patients can benefit from supportive treatment measures for heart failure. However, many standard medications used for heart failure are not helpful for patients with cardiac amyloidosis. Careful attention to fluid balance is important, as explained in the question below.
In patients with low blood pressure, drugs such as fludrocortisone or midodrine may help to maintain blood pressure and allow higher diuretic doses.
Some patients may experience light-headedness, fatigue on minimal exertion or fainting due to drops in blood pressure. They may benefit from instruction in how to change position carefully from lying to sitting, sitting to standing and standing to walking.
Heart transplantation
For hereditary, 'variant' ATTR amyloidosis, combined heart and liver transplant has been performed in a few dozen cases around the world. This operation is only an option for a minority of patients, and it carries significant risks.
Most patients with wild-type ATTR amyloidosis are too elderly to undergo a heart transplant. The risk of complications from this major operation is high with advanced age. But heart transplantation may be an option for younger, otherwise healthy patients with this condition.
Treatment of peripheral neuropathy symptoms
Medications that may help to alleviate neuropathic pain include gabapentin, pregabalin and duloxetine. Medical staff can give advice regarding appropriate foot care and footwear. This is important to prevent painless ulcers at pressure points and to protect areas of the foot that lack sensation.
Treatment of autonomic neuropathy symptoms
If there is orthostatic hypotension (drops in blood pressure and faintness on standing up from sitting or lying positions), elastic stockings may be recommended. Drug treatment with midodrine may also be helpful. Care should be taken to avoid dehydration if there is vomiting and diarrhea. Intravenous fluids and anti‑nausea drugs may be necessary, but it is important to avoid fluid overload if there is heart disease. There are drugs that can help to control diarrhea and constipation, and others that can help to combat erectile dysfunction.
The place of new drugs in the treatment of ATTR amyloidosis is discussed separately in other questions in this section.
How can I avoid fluid balance problems?
Many patients with ATTR cardiac amyloidosis should limit their fluid intake. This advice is extremely important but is often overlooked.
The most important principle of treatment for cardiac amyloidosis is strict fluid balance control. Specialist heart failure nurse involvement may help patients to achieve this.
When there is cardiac amyloidosis, the heart may be too stiff to pump the blood efficiently around the body. This can lead to fluid build- up, causing leg swelling (edema) and breathlessness due to fluid in the lungs. This problem is exacerbated if the patient drinks too much fluid.
Fluid excess can be avoided by careful attention to the 3 Ds:
- Diet
- Diuretics
- Daily weights
Diet:
Fluid intake should be steady and should usually not exceed 1.5 liters per day.
Salt intake should be limited. This includes attention not just to salt deliberately added to the food during cooking or at the table but also to ready prepared foods with high salt content such as processed foods, canned meats, canned soups, and smoked fish. Apart from that, a balanced, healthy diet is always advisable. It can be extremely helpful to meet with a dietician for precise and personalized dietary advice.
Diuretics:
Doctors will often prescribe diuretics (water tablets) which increase the amount of urine produced and help the body to lose excess salt and water in the urine. This can help to reduce ankle swelling and breathlessness. Diuretics prescribed may include furosemide and spironolactone. Taking these drugs is not a substitute for avoidance of excessive dietary salt and water.
Patients should follow their doctor’s advice carefully regarding the dose of diuretic and the time of day when the tablet should be taken.
Daily weights:
Some patients benefit from recording their weight regularly, usually daily or weekly. It is important that weight should be measured consistently - using the same scales, at the same time of day. This is usually best done first thing in the morning after passing urine, just wearing underclothes. Several liters of fluid can accumulate in the body without it being very noticeable. An increase in weight can be an early sign of fluid overload. The doctor or nurse can then recommend appropriate measures such as increased diuretic dose before the patient even feels unwell because of the fluid overload.