AL AmyloidosisYour guide to best cancer care

It can be overwhelming after being told that you have or may have AL amyloidosis. A lot can happen quickly, and you might have lots of questions, like 'What happens next after a diagnosis?'

This support resource can help to guide you, your family, and your friends through this experience.

Is Al amyloidosis a blood cancer?

Amyloidosis is the name given to a group of rare disorders in which abnormal proteins misfold forming amyloid fibrils. The body cannot break down amyloid proteins easily and they clump together and can deposit and/or accumulate in any tissue and organ of the body. As these deposits progressively build up, they interfere with the working of the body’s organs. Without treatment, this will eventually lead to organ failure.

Over 30 types of amyloidosis have been identified. This guide discusses one type only, AL amyloidosis. The prefix A stands for amyloid, and L stands for light chain. Normally the human body produces B cells and plasma cells which are found in the bone marrow. These cells are part of the body’s immune system. In AL amyloidosis, these plasma cells, or sometimes B cells, begin to grow abnormally, and begin to produce large amounts of a single type of free light chain.

Many people ask whether AL amyloidosis is a blood cancer. It is not. Although the amyloid forming protein is produced by an underlying bone marrow disorder, this is usually benign (non-cancerous) but in some cases the growth of plasma cells or B cells can be malignant (cancerous). AL amyloidosis is commonly linked with a plasma cell disorder called myeloma, and sometimes it is associated with a B-cell disorder called lymphoma, however, AL amyloidosis is not typically considered to be a cancer.

Information and support

If you need additional information or would like support, you can visit the optimal care pathway for AL amyloidosis, or call Cancer Council on 13 11 20 to talk to an experienced healthcare professional.

For more information about AL amyloidosis, visit the Leukaemia Foundation website. You can also view an educational video on amyloidosis available here.

The Australian Amyloidosis Network is a not-for-profit organisation of patients, carers, and health professionals working together to improve the lives of patients with all types of amyloidosis. For more information, visit the Australian Amyloidosis Network homepage.

To speak with an experienced health professional who can provide you with disease specific information, answer questions, talk through concerns and connect you to blood cancer support groups, call 1800 620 420 or visit the Leukaemia Foundation homepage. The team can also help with practical concerns such as accommodation close to treatment, transport to appointments and financial assistance.

If you need a translator, call TIS on 13 14 50 or visit the TIS National website.

Symptoms

Your general practitioner (GP) or other primary care physician will do a check-up to see if they can find out what is making you unwell. AL amyloidosis can present with many different symptoms, which will vary from patient to patient (e.g., fatigue and weight loss) and/or blood test abnormalities. These symptoms can mimic other diseases which may delay diagnosis.

You might have fatigue, weight loss, feeling faint, bruising of the skin especially around the eyes, a drop in blood pressure after sitting or lying down (postural hypotension), pain, numbness, tingling or weakness in different parts of your body (peripheral neuropathy), swelling in the legs and/or abdomen (oedema), diarrhoea or constipation, difficulty breathing or shortness of breath (dyspnoea), kidney and/or liver problems, and an enlarged or painful tongue.

Typically, your general practitioner will refer you to an appropriate specialist (e.g., a cardiologist for heart problems, a nephrologist for kidney problems, a neurologist for peripheral neuropathy or a health service with the relevant specialty services). Those specialists will then perform investigations to diagnose amyloidosis.

Initial tests you may have:

Referrals

If your GP or primary care physician thinks you may have the symptoms listed above, they will refer you to an appropriate specialist at a public hospital or in private practice for more tests. You may be referred to different specialists such as a haematologist (blood and bone marrow), cardiologist (heart), nephrologist (kidneys), neurologist (nervous system), or gastroenterologist (gastrointestinal tract) due to the symptoms you experience while the diagnosis is being investigated.

You can bring a family member or friend with you to your appointments.

Timeframes

The timing of your haematologist appointment will depend on your initial results of tests performed by your GP or referring specialist. For a small number of patients, an urgent appointment or hospital admission may be required, but for most people with no urgency, an appointment within four weeks is appropriate.

If you can’t get an appointment within this timeframe, follow up with your GP.

Travel to access tests, treatment and care

You may have to travel to see a haematologist, undergo tests or access treatment, especially if you live in a rural or regional area. In some cases, you may have to leave home for an extended period of time. Accommodation and transportation support services are available, and it is encouraged to have family support with you whilst you undergo treatment. You can ask about what patient travel subsidy schemes are available to you.

See further information on Leukaemia Foundation’s accommodation and transport support by visiting their accommodation services page and transport services page.

Mental health and emotional well-being

A diagnosis of AL amyloidosis can affect your mental and emotional wellbeing. Talking with your GP or your hospital treatment team and being referred to patient support organisations (such as Leukaemia Foundation or the Australian Amyloidosis Network), or other health professionals such as a psychologist or social worker, can help you develop strategies to cope. They can help you access a mental health treatment plan if required.

Helpful tips

Let your doctor know what is most important to you in a specialist (e.g., cost, location, bedside manner, expertise).

Ask your GP to recommend trusted sources of information and support — you can share these with your family and friends too.

Talk to your GP/specialist about how to manage any other health conditions you may have during your treatment of AL amyloidosis and let them know if you have any concerns.

It’s a good idea to keep a written diary or digital record of treatment details and appointments with the GP and specialists. You can ask permission to record appointments. It’s also helpful to bring a list of questions and ask for a written treatment plan. This can be an important source of information for later in life.

Don’t ignore new signs and symptoms. Alert the GP or specialist. Trust yourself. It’s OKAY to be persistent.

If you haven’t seen a haematologist, you will be referred to one. Haematologists are trained in conditions that affect the blood and bone marrow. The haematologist will do more tests to confirm if you have AL amyloidosis and help determine the best treatment for you. This process of working out if you have a medical problem is called making a diagnosis.

You might have one test or a mix of tests:

The most frequently affected organs by AL amyloidosis are the heart, kidneys, gastrointestinal tract, nervous system, and liver.

These tests will give the specialist more information about the glioma, such as where exactly it is in your body, if the cancer is growing or has spread, and what stage it is at. This is called staging, which helps to work out the best treatment for you.

Timeframes

You should have most results back within four weeks.

Your haematologist will talk to you about your treatment options for AL amyloidosis. You may need more than one treatment type to get the best results, and you may require ongoing treatment to manage your AL amyloidosis for the rest of your life.

Treatment for AL amyloidosis focuses on stopping or slowing the production of amyloid and subsequently its deposition in the body’s organs and tissues, to preserve organ function, and to improve quality of life.

You might have one treatment or a mix of treatments:

Proteasome inhibitors (PIs). Drugs that are responsible for killing the affected plasma cells that are causing AL amyloidosis.

Chemotherapy. Drugs that are used to kill the affected plasma cells that are causing AL amyloidosis.

Monoclonal antibodies (mAbs) work in the same way as natural antibodies that your body produces; they fight infection and target the affected plasma cells that are causing AL amyloidosis.

Corticosteroids. Drugs that help kill the affected plasma cells that are causing AL amyloidosis.

Autologous stem cell transplant. Your haematologist will discuss whether this is an option for you.

Your treatment team will monitor you closely. If the treatment is not controlling the production of the amyloid quite quickly, treatment may be changed after discussion with you

For more information, visit Cancer Treatment or the Australian Amyloidosis Network page.

Supportive care (treatment or services that support you through a cancer experience) are also available, often delivered through a multidisciplinary team that may include nurses, pharmacists, psychologists, physiotherapists and dietitians.

You can also ask to be contacted by the Cancer Nurse Consultant/Cancer Care Navigator at your hospital or by a social worker if you have not yet seen one.

Timeframes

By evaluating your symptoms and test results, your specialist will determine when your treatment should start. For most people this will generally be within four weeks of the decision being made.

If your heart has been affected, treatment should start within two weeks. There may be instances where your treatment could be delayed beyond this timeframe. If this occurs your specialist will discuss this with you.

You can ask your GP for a referral to another specialist for a second opinion.

For more information on clinical trials, visit the Australian Cancer Trials page or search the Australian New Zealand Clinical Trials Register. Participation in a clinical trial is voluntary, and you can choose not to participate in a clinical trial or withdraw from a clinical trial at any point if you wish.

For relationship support and, if necessary, referral to counselling services, visit the relationships and sex page on Leukaemia Austalia. Canteen Australia also provides some helpful information on fertility options after a cancer diagnosis.

You can ask for more time to think about your treatment options, and you can ask your GP for a referral to another specialist for a second opinion.

Patients with AL amyloidosis will need to be followed up by their medical team for the rest of their lives. Although there is a good chance that many patients with AL amyloidosis will, with treatment, achieve remission (the amyloid is no longer being produced and deposited in any organs) they may still require maintenance treatment or further treatment if the amyloid production starts again. Comprehensive supportive care including emotional and social support should be part of routine ongoing care.

Survivorship care plan

Current therapies mean that most people with AL amyloidosis will go on to live long lives. You may need regular ongoing follow-up appointments and care, so your specialist and healthcare team will work with you to make a survivorship care plan for you and your GP.

Your survivorship care plan may need to be updated over time if your needs change. This plan will explain:

• who your main contact person is after treatment
• how often you should have check-ups and what tests this will include
• understanding and dealing with potential side effects of treatment
• how to get help quickly if you have an urgent problem
• how to look after your overall health and wellbeing
• what healthcare and community support services are available to you and how to access them.

Your specialist and healthcare team will talk to you about your needs and can refer you to other health professionals and community support services.

Other information you get might be about:

• the side effects of treatment and the specialists you may need to see
• how to make healthy lifestyle choices to give you the best chance of staying well

For more information, visit After Cancer Treatment.

Over time, most patients with AL amyloidosis will relapse after responding to initial treatment. Relapse is not certain and may not occur soon after treatment. As people respond differently, many patients can live in long-term remission. Access to the best available therapies, including clinical trials and treatment with a multidisciplinary team, are crucial to achieving the best outcomes for relapsed disease.

Treatment will depend on how far your amyloidosis has progressed and the different organs that may be affected.

AL amyloidosis patients will be closely monitored throughout treatment. If treatment is stopped because it is not controlling the amyloid production or the amyloid starts depositing again and the patient comes out of remission, a full assessment by the treatment team might be called for to determine the present status of the disease.

Options may include:

• using a different drug regimen that contains drugs of a different class or a different drug of the same class
• ‘Watch and Wait’
• retreatment with a previous regimen
• clinical trial, if appropriate
• stem cell transplantation in some patients
• supportive and/or palliative care

Advance care planning

Your GP or healthcare team may talk with you, your family, and carer about your future treatment and medical needs.

Advance care directive

Sometimes known as a living will, an advance care directive is a legally binding document that you prepare to let your family and healthcare team know about the treatment and care you might want or not want in case you become too unwell to make those decisions yourself.

For more information, visit Advance Care Planning Australia.

Palliative care

Your specialist may refer you to palliative care services, but this doesn’t always mean end-of-life care. Today, people can be referred to these services much earlier if they’re living with cancer or if their cancer returns. Palliative care can help you to live as well as you can, including managing pain and symptoms. This care may be at home, in a hospital or at another location you choose.

Speak to your GP or specialist or visit Palliative Care Australia.

Making treatment decisions

You may decide not to have treatment at all, or to only have some treatment to reduce pain and discomfort. You may want to discuss your decision with your healthcare team, GP, family and carer.

For more information, visit Advanced Cancer Treatment.

You may have to pay for some appointments, tests, medications, accommodation, travel or parking. Speak with your GP, specialist or private health insurer (if you have one) to understand what is covered and what your out-of-pocket costs may be.

If you have concerns about costs, talk to your healthcare team or a social worker about:

• being bulk-billed or being treated in the public system
• help with accommodation during treatment
• the possible financial impact of your treatment

You can call Cancer Council on 13 11 20 to speak to a healthcare professional about financial support. For more information about costs, visit Practical and financial assistance and What will I have to pay for treatment.