Amyloidosis: Causes, Symptoms, Treatment and Cost

Amyloidosis is a set of disorders characterised by the accumulation of aberrant proteins known as amyloids in different organs or tissues. Amyloid is a word used to describe a protein that has been folded incorrectly. These aberrant proteins can accumulate in many organs, including the heart, liver, spleen, and kidneys, resulting in organ failure or other health issues.

The most common types of amyloidosis include:

• AL (primary) Amyloidosis: This type is caused by the abnormal production of an abnormal protein called light-chain amyloid in the bone marrow. It usually affects multiple organ systems including the heart, kidneys, liver, skin, nerves, and gastrointestinal tract.
• AA (secondary) Amyloidosis: This type is caused by chronic inflammation or infection such as rheumatoid arthritis or tuberculosis that leads to circulating immune complexes depositing in various tissues throughout the body. It typically affects the heart, kidneys, liver, spleen, nerves and gastrointestinal tract but can cause a wide range of symptoms depending on which organs are involved.
• Hereditary (familial) Amyloidosis: This type is caused by genetic mutations that cause abnormal proteins to accumulate in certain tissues throughout the body including the heart, nervous system, eyes and skin leading to tissue damage over time.

The aberrant buildup of an insoluble protein known as amyloid in the organs and tissues of the body is what leads to the disease known as amyloidosis.

The most common causes are aging, genetics, and chronic inflammation due to an underlying condition such as multiple myeloma or rheumatoid arthritis. There are several circumstances in which the specific reason may continue to be unclear.

The symptoms of amyloidosis vary depending on the kind of the disease as well as the location of the deposits inside the body. The following are examples of common symptoms:

• Legs, limbs, or the area surrounding the eyes may swell.
• Abdominal pain, swelling, or bloating.
• Fatigue
• Weight loss
• Shortness of breath.
• Numbness or tingling sensations in the hands and feet.
• Enlarged tongue
• An irregular heartbeat or palpitations.
• Difficulty swallowing.

The primary way to prevent Amyloidosis is to avoid the underlying conditions that can lead to it. This includes maintaining a healthy lifestyle and treating any existing medical conditions.Additionally, individuals should receive regular medical checkups in order to identify any potential health problems at an early stage and take steps to treat them immediately.

Finally, if you have already been diagnosed with an amyloidosis-causing condition such as multiple myeloma or rheumatoid arthritis, it is important that you follow the treatment plan prescribed by your doctor in order to prevent progression of the disease.

• Complete blood count (CBC): A complete blood count (CBC) for amyloidosis will include a white blood cell count, red blood cell count, hemoglobin and hematocrit levels, etc. It may also include a peripheral smear to look for abnormal cells.
• X-rays: X-rays can be useful in diagnosing amyloidosis. X-rays can show thickening of the heart wall and enlargement of the liver and spleen, which are both common findings in amyloidosis. Additionally, X-rays can help detect the presence of bone lesions which are commonly seen in certain types of amyloidosis.
• CT scan: A CT scan can detect abnormal accumulations of amyloid proteins in the body that may indicate amyloidosis. Additionally, a CT scan may be used to examine the effects of amyloidosis on other organs, such as the heart or kidneys.
• Echocardiogram (ultrasound of heart): When a person has amyloidosis, an echocardiography is done to evaluate the anatomy and function of the heart. It also helps to evaluate how well the heart is pumping, as well as detect any presence of tissue thickening or buildup from amyloid deposits.

Complications of amyloidosis can include organ damage and failure, heart problems, nerve damage, kidney failure, joint pain and inflammation, vision loss, cognitive decline or dementia due to brain amyloid deposits and increased risk of infection. It is potentially fatal if left untreated.

• Ashwagandha: Ashwagandha is an Ayurvedic herb that is known to help reduce inflammation, support healthy immune function, and promote detoxification.
• Amalaki: Amalaki is an Ayurvedic herb that helps balance the body's natural processes, including those associated with amyloidosis. It is known to be a powerful antioxidant and anti-inflammatory agent that can help reduce inflammation and protect the body from damage caused by free radicals.
• Guggul: Guggul is an Ayurvedic herb used to treat a variety of conditions, including amyloidosis. It helps remove toxins from the body and supports healthy digestion and metabolism.
• Vacha: Vacha is an Ayurvedic herb traditionally used for its anti-inflammatory properties which can help reduce inflammation associated with amyloidosis.

A diet rich in fruits, vegetables, whole grains, lean proteins and low-fat dairy products is best.

Additionally, foods containing antioxidants such as blueberries, spinach and other leafy greens can be beneficial for maintaining overall health.

• In general, people with amyloidosis should avoid eating processed and high-fat foods, such as fried foods and fast food.
• Sugary foods, such as sweets and soda, must also be minimized.
• Red meat should be consumed no more than once a week.
• It is also important to reduce the amount of salt in your diet to help reduce fluid retention and swelling.

The main treatments for amyloidosis include:

• Medications: Medications such as corticosteroids and chemotherapy may be used to reduce inflammation and help slow down the progression of amyloid deposits in organs.
• Plasma Exchange: This procedure involves removing some of an affected person’s plasma (the liquid part of blood) and replacing it with donor plasma or a man-made solution known as albumin-replacement therapy (ART). The goal is to remove any abnormal proteins that are causing damage to organs and tissues.
• Surgery: If a localized area is affected by amyloid deposits, surgery may be recommended to remove them if they are causing symptoms or damage to nearby tissues or organs.
• Transplantation: In some cases, a transplant may be necessary if there is severe organ dysfunction due to amyloid deposits; this includes heart transplantation for those with cardiac involvement or kidney transplantation for those with advanced kidney failure due to amyloid deposition in their kidneys.
• Supportive Care: Supportive care measures such as diet modifications, physical therapy, occupational therapy, speech therapy and psychological counseling can also help improve quality of life for patients living with an advanced stage of the disease.

People with amyloidosis should consult a doctor who specializes in this condition, such as a hematologist or a cardiologist. The doctor will evaluate the patient's medical history, perform physical examinations, and order tests to diagnose amyloidosis and determine the underlying cause.

The best medicines for amyloidosis depend on the type and severity of the condition. Some common medications are:

• Corticosteroids: Corticosteroids are a type of medication that can be used to treat amyloidosis. They work by reducing inflammation and decreasing the production of proteins that cause amyloid deposits in the body. In some cases, corticosteroids may even help to slow the progression of amyloidosis and reduce its effects on the body.
• ACE inhibitors: ACE inhibitors may also be used to treat amyloidosis. They can help reduce the amount of protein buildup by blocking an enzyme that causes the proteins to form clumps. In addition, ACE inhibitors may help reduce inflammation caused by amyloidosis and slow down progression of the disease.
• Beta-blockers: Beta-blockers are commonly used to treat certain symptoms associated with amyloidosis, particularly when the disease is caused by an underlying condition such as hypertension or an irregular heartbeat. Beta-blockers act by inhibiting the effects of particular hormones that are responsible for these symptoms.

Recovery from Amyloidosis depends on a variety of factors, such as the type and severity of the condition, and the individual's overall health. In general, healing time might range from months to years.

Treatment for amyloidosis has different effects based on the kind and severity of the illness.

In some cases, treatment can be successful in reversing or slowing disease progression and even completely halting symptoms. In other cases, however, the disease may progress despite treatment, and symptoms may recur or worsen over time.

• In general, the post-surgical treatment guidelines for amyloidosis depend on the type of amyloidosis present.
• In some cases, a stem cell transplant may be performed to replace bone marrow cells lost by the illness. Following successful treatment, patients may need to undergo regular monitoring and follow-up with their doctor to ensure that any residual amyloid deposits do not progress further.
• For localized forms of amyloidosis such as familial amyloid polyneuropathy (FAP), doctors generally recommend lifestyle changes and drug treatments such as diuretics and ACE inhibitors to reduce fluid buildup in tissues. Surgery may be needed if tissue damage is severe or if a tumor is present.
• Patients should also take steps to prevent further organ damage by avoiding alcohol and reducing their salt intake.

Generally, the cost of medications and other treatments like chemotherapy, dialysis, and blood transfusions can range from anywhere between ₹25,000 to ₹5 lakhs.

Surgery may be necessary in some circumstances, which can cost up to 10 lakhs. Furthermore, many hospitals provide discounts to patients who are unable to pay the entire amount of their treatment.

Common side effects of amyloidosis treatments may include:

• Fatigue
• Nausea, vomiting, and diarrhea.
• Hair loss
• Low blood counts
• Neuropathy (nerve damage).
• Skin rash or itching
• Anemia (low red blood cell count).
• Kidney damage

If you are suffering from any complications relating to amyloidosis then you should consult a doctor nearby as they can cause complications like organ damage and failure, heart problems, nerve damage, etc. in which treatment course can range from a few months to years depending on the severity of the situation.