Amyloidosis is a rare disease that develops when the body makes abnormal proteins. These proteins are not shaped correctly, which causes them to clump together. The protein clusters, also known as amyloid deposits or amyloid fibrils, build up in different places in the body. For example, amyloid can build up in the heart and in the wrist, causing heart disease and carpal tunnel syndrome. It can also build up in the blood vessels and the nervous system, leading to abnormalities in organ function and tissue damage. If amyloidosis goes untreated, organs can ultimately shut down.
Amyloidosis is diagnosed definitively by a biopsy, a tissue sample sent to a laboratory for analysis. As part of the workup for this condition, an echocardiogram (an ultrasound of the heart) will often be performed, as well as an MRI scan, to get better pictures of your organs. It is important to know that there are several types of amyloidosis, as this can affect treatment.
Amyloidosis is a condition that does not yet have a cure. However, some treatments can slow down the disease, preventing new amyloid proteins from forming. Additionally, changes in your diet may help relieve certain symptoms and expand treatment options by keeping you healthier.
There are several treatments that work in different ways to prevent or slow down amyloid buildup. The different treatment types also depend on the type of amyloidosis you have. Amyloidosis medications may:
In healthy individuals, the body is continually breaking down old proteins and making new ones. In people with amyloidosis, the body makes abnormal proteins faster than they can be removed. Treatments that stop the production of new amyloid proteins may give the body more time to break down and remove existing amyloid deposits.
Each type of amyloidosis is caused by a different abnormal protein. The treatment that a person receives depends on which type of amyloidosis they have. Certain medications that are used to treat multiple myeloma are also used in the treatment of amyloidosis.
Amyloid light-chain amyloidosis (AL amyloidosis or primary amyloidosis) develops when abnormal immunoglobulin light chain proteins build up. These proteins are made by abnormal plasma cells (a type of white blood cell) and can accumulate in the bone marrow, among other locations. Treatments that get rid of plasma cells help the body make less amyloid protein. These treatments may include:
AA amyloidosis or secondary amyloidosis is caused by an underlying infection or inflammatory disease. Examples of these underlying conditions include rheumatoid arthritis, systemic lupus erythematosus, chronic infections, and a variety of cancers. Treating the underlying disease will cause the body to make less of this type of amyloid protein, preventing additional buildup. If you have AA amyloidosis caused by another condition, speak with your doctor to find an effective treatment plan.
In hereditary or familial amyloidosis, the abnormal transthyretin (TTR) protein comes from the liver. A liver transplant can stop the production of new amyloid protein. Additionally, medications known as gene silencers may help. Onpattro (patisiran) and Tegsedi (inotersen) shut down the genes that cause hereditary amyloidosis. Researchers are also studying whether other medications can help remove amyloid for people with wild-type ATTR amyloidosis. For reference, wild-type ATTR amyloidosis was formerly known as senile systemic amyloidosis.
Amyloid proteins are often misfolded — the cell does not mold the proteins into the correct shape. This causes them to stick together. Some medications stabilize the amyloid proteins, preventing them from binding to each other.
Researchers are studying whether a medication called Kiacta (eprodisate) can stop amyloid deposits from building up in the kidneys or other organs. This medication may help treat people with AA amyloidosis.
A few different medications may block amyloid deposits caused by the TTR protein. Vyndamax (tafamidis) and Vyndaqel (tafamidis meglumine) are medications that attach to TTR and prevent it from forming amyloid fibrils. These medications are approved by the U.S. Food and Drug Administration to treat people with amyloidosis-related heart disease and heart failure. Dolobid (diflunisal) is a nonsteroidal anti-inflammatory medication approved to help stop amyloid from forming. This medication may help people with hereditary ATTR amyloidosis.
Researchers are interested in studying whether new medications may be able to remove amyloid proteins that have already formed deposits within organs. They are in the process of studying whether a medication called PRX004 can help treat hereditary or wild-type ATTR amyloidosis.
People who have been on dialysis for a long time due to kidney disease may develop dialysis-related amyloidosis. In this condition, the beta-2 microglobulin protein builds up in the joints and bones. A filter called a Lixelle column can help remove amyloid proteins from the blood.
Lifestyle changes will not prevent the body from making amyloid proteins or stop new amyloid deposits from building up. However, there are things you can do at home to improve your overall health and daily life.
Eating less sodium (salt) may help improve amyloidosis symptoms. Amyloidosis sometimes causes extra fluid to build up within the body, leading to fluid retention. This can make it harder for the heart and kidneys to work properly. A low-salt diet helps your body get rid of the excess fluid. It may also reduce swelling, lower blood pressure levels, and relieve symptoms like tiredness and shortness of breath. Eating less salt can also protect the kidneys from damage, keeping a person healthy while they undergo other treatments.
People who are experiencing kidney failure may also need to drink fewer liquids and avoid eating too much saturated fat and cholesterol. These dietary changes may help keep the kidneys healthy.
People with kidney disease may also need to eat less protein. High levels of protein can damage the kidneys. On the other hand, it is not necessary to reduce protein in the diet if the kidneys are functioning. Some people may think that they need to eat less protein because amyloidosis is caused by high levels of protein. However, amyloid proteins are made by cells in the body — they do not come from food. Talk with your doctor before making any changes to your diet.
One study suggested that green tea extract may slow down hereditary or wild-type amyloidosis. However, this research is in early stages. In the future, clinical trials may be able to show more clearly whether diet-based changes can help.
Ask your doctor whether you should change your diet. They may recommend eating or avoiding certain foods or drinks to improve your overall health.
MyAmyloidosisTeam is the social network for people with amyloidosis and their loved ones. On MyAmyloidosisTeam, members come together to ask questions, give advice, and share their stories with others who understand life with amyloidosis.
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