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.

1. Initial investigation and referral

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:

Physical exam

Your GP or specialist will check to see the cause of symptoms such as those noted above.

Blood tests

A sample of your blood is collected and sent for a full blood examination and may include a liver function test.

Electrocardiogram (ECG) and echocardiogram

Your doctor may want to check your heart by performing these tests.

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.

Questions you might want to ask

  • Are there any symptoms that I should watch out for while I am waiting for my appointment to see a specialist?
  • Can I choose the specialist I see?
  • What emotional and mental health support services are available and how do I access them?
  • Can I choose whether I go to a public or private hospital?
  • What are the differences of being treated in the public versus private system?

2. Diagnosis and staging

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:

Tissue biopsy

Tissue biopsy is done to check for amyloid deposits and further biopsies of other involved organs.

Bone marrow biopsy

A doctor will put a needle into your hip bone for a bone marrow biopsy. A sample of the bone marrow tissue will be sent to a laboratory for examination. This is usually done with a local anaesthetic, and inhalational pain relief or mild sedation.

Cardiac, renal, and liver tests

Cardiac tests include an echocardiogram to check heart function.

Renal tests include a urine test to check kidney function.

Liver tests check the size and functioning of the liver.

Clinical assessment

Clinical assessment for peripheral neuropathy to check for any damage to nerves.

Blood pressure test

Blood pressure test includes testing postural blood pressure (testing your blood pressure while sitting and standing).

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.

Questions you might want to ask

  • What is AL amyloidosis and where can I find more information about it?
  • What tests will I have?
  • Will my follow-up appointments be face-to-face or by tele/videohealth or a combination?
  • What’s my prognosis?
  • How much will appointments and tests cost and how much will I have to pay myself?
  • Can I choose where I have treatment?
  • Will I need to start treatment straightaway?
  • How long will treatment last?
  • Can I choose the type of treatment available for me?
  • Is there information that I can share with my family or friends?
  • What support services are available to me?

3. Treatment

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.

Clinical trials

You may be offered to take part in a clinical trial. Clinical trials are used to test whether new treatments are safe and work better than current treatments. Many people with cancer are now living longer, with a better quality of life, because of clinical trials.

Complementary therapies

Speak to your healthcare team about any complementary therapies (including dietary supplements like vitamins) you use or would like to use. Something as common as vitamins might not work well with your treatment.

Fertility and sexual health

Blood cancer related disorders such as AL amyloidosis and its treatment may cause fertility problems for both men and women. This will depend on age, the type of disorder, and the treatment received. If this is relevant, get advice from your treating team about contraception before, during, and after treatment. Discuss having children whilst undergoing treatment and the need for referral to a fertility specialist and options for fertility preservation before starting treatment. Diagnosis and treatment may affect your sex life or overall sexual health.

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.

Questions you might want to ask

  • What treatment do you recommend and why?
  • Are there alternatives?
  • What will happen if I don’t have treatment?
  • How long will I need to be on treatment?
  • What will treatment cost and how much of the cost will I have to pay myself?
  • What activities/exercise will help me manage my treatment and its side effects?
  • Is there any specific diet I should follow or foods or drink to avoid during treatment
  • How will the treatment affect my day-today life and can I still work?
  • Who is in my treatment team and who is the main contact person if I have any questions or feel unwell?
  • What side effects could I have from treatment and how should I manage them?
  • Will treatment affect my ability to have children and, if yes, what options should I consider?

4. After treatment

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.

Questions you might want to ask

  • Who should I contact if I am feeling unwell?
  • What can I do to be as healthy as possible?
  • What can I do to be as healthy as possible?
  • Who should I contact if I need advice?
  • Where can I get more help?

5. Living with cancer

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

Questions you might want to ask

  • What is the stage of my disease?
  • What is happening with my disease?
  • What are my treatment options?
  • What are the chances that the treatment will work this time?
  • Is there a clinical trial available?
  • What financial, practical, or emotional support is available?

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.

Questions you might want to ask

  • What can you do to reduce my symptoms, if any?
  • What extra support can I get if my family and friends care for me at home?
  • Can you help me to talk to my family about what is happening?
  • What support is available for my family or carer?
  • Can I be referred to a community support service?
  • What support services are available to me to help manage the ongoing nature of my AL amyloidosis?

6. Questions of cost

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.

7. Further support

AL Amyloidosis support

You can speak to specially trained staff at Cancer Council on 13 11 20. They can answer your questions about the effects of cancer, explain what will happen during treatment and link you to support groups and other community resources.

If you need an interpreter, call TIS (the Translating and Interpreting Service) on 13 14 50.

For support and advice for carers, call the Carers Association on 1800 242 636.

More support options

Australian Amyloidosis Network
A not-for-profit organisation of patients, carers, and health professionals working together to improve the lives of patients with all types of amyloidosis
• Website: www.aan.org.au

Leukaemia Foundation
A national charity dedicated to helping those with leukaemia, lymphoma, myeloma and related blood disorders to survive and live a better quality of life. The team can also help with practical concerns such as accommodation close to treatment, appointment transport, and financial assistance
• Telephone: 1800 620 420
• Website: www.leukaemia.org.au

Australian Cancer Survivorship Centre
Has general and tumour-specific information, primarily focused on the post-treatment survivorship phase
• Telephone: (03) 9656 5207
• Website: www.petermac.org/cancersurvivorship

Beyondblue
Information on depression, anxiety and related disorders, available treatment and support services
• Telephone: 1300 22 4636
• Website: www.beyondblue.org.au

Look Good, Feel Better
A non-medical, free community service program dedicated to teaching women how to manage the appearance-related side-effects caused by cancer treatment
• Telephone: 1800 650 960 (Monday to Thursday 9.00am to 5.00pm)
• Website: www.lgfb.org.au

Cancer Australia
Information on cancer prevention, screening, diagnosis, treatment and supportive care for Australians affected by cancer, and their families and carers
• Telephone: 1800 624 973
• Website: www.canceraustralia.gov.au

CanTeen
Australian organisation for young people living with cancer offers support, information, and resources
• Telephone: 1800 226 833
• Website: www.canteen.org.au

Care Search: Palliative Care Knowledge network
Information for patients and carers on living with illness, practical advice on how to care, and finding services
• Telephone: (08) 7221 8233
• Website: www.caresearch.com.au

National Health Services Directory
A directory providing information on local hospitable and community services
• Website: www.nhsd.com.au

Optimal care pathways have been developed for health professionals and health services. However, patients and carers may find useful information in this version to help understand the processes their treating health professionals are following.