Myeloproliferative NeoplasmsYour guide to best cancer care

It can be overwhelming after being told that you have or may have myeloproliferative neoplasms (MPN). 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. Other international resources are available online but may discuss approaches to testing and treatment that are not standard of care in Australia.

There are three main types of MPNs which include essential thrombocythaemia (ET), polycythaemia vera (PV), and myelofibrosis (MF).

Information and support

For more information about MPN, visit the Leukaemia Foundation website, or call Cancer Council on 13 11 20 to talk to an experienced healthcare professional.

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

MPN Alliance Australia is a small team of volunteer Australian MPN patients who provide evidence-based information and support for MPN patients via their website. The website includes an information and support section for newly diagnosed patients, the latest MPN articles, treatment information, and several support options, including from other MPN patients.

More information is available in the ‘Resource’ section of the optimal care pathway for myeloproliferative neoplasms.

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

Symptoms

Many patients with an MPN may be unaware of having symptoms, and your condition may be first noticed by your doctor as unusual results in a routine blood test. If you have presented to your general practitioner (GP) or other primary care physician with symptoms, they will do a check-up to see if they can find out what is making you unwell.

They will ask you about any symptoms you might be having such as fatigue, concentration problems, fevers or night sweats, abdominal discomfort, weight loss, bone pain, itchy skin (particularly after exposure to water), gout, or burning pain in the hands and feet in response to heat. Please discuss any symptoms with your doctor.

Initial tests you may have:

Referrals

If your GP/physician thinks you may have an MPN, they’ll refer you to a haematologist or medical oncologist (specialist) at a public hospital or in private practice for more tests. Haematologists are highly trained in conditions that affect blood and bone marrow.

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

Timeframes

The timing of your specialist appointment will depend on your initial results of tests performed by your GP or doctor. 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 these timeframes, follow up with your GP.

Travel to access tests, treatment and care

You may have to travel to see a specialist, 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. Accommodation and transportation support services are available, and it is encouraged to have family support with you whilst you undergo treatment. Most MPN patients require ongoing treatment and management via regular blood tests and visits to their haematologist.

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

Even though most MPNs are a chronic blood cancer, a blood cancer diagnosis can affect your mental and emotional well-being. Many people being treated for a blood cancer experience a range of feelings and it is not uncommon to feel low, depressed, or anxious. Patient support organisations such as the Leukaemia Foundation or Cancer Council, your GP, or another health professional 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., that they specialise in ALL, 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 blood cancer treatment and let them know if you have any concerns.

It is very helpful to request and keep copies of your blood test results, as well as reports of any scans or biopsies, etc. You are then able to take them with you when you see any other health provider for other health conditions, so that your care may be best suited to your particular needs.

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.

The haematologist/specialist will do more tests to determine if you have an MPN to 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:

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 eight weeks.

Your specialist will talk to you about your treatment options for your MPN. You may need more than one treatment type to get the best results, and you will require monitoring and are likely to require ongoing treatment to manage your MPN for the rest of your life.

Some patients will initially be managed with a ‘Watch and Wait’ approach which involves regular blood testing and appointments with your haematologist to check for symptoms or changes in your blood test results.

You might have one treatment or a mix of treatments:

Anti-platelet & anti-coagulant therapy. Small daily doses of antiplatelet medication like aspirin or an anticoagulant medication like warfarin for most people with MPNs prevent platelets from clumping together and reduce the risk of blood clots.

Venesection/phlebotomy. Used for the treatment of PV and is a procedure where a controlled amount of blood is removed from the bloodstream to reduce the high red blood cell count.

Transfusions. If you have low red blood cell counts, you may need a blood transfusion which is a slow injection of red blood cells into a vein (intravenously or IV). Similarly, if you have a low platelet count, you may need a platelet transfusion where you will be given a slow injection of platelets into a vein.

Cytoreductive therapy. Considered for high-risk patients with MPN. These medications include chemotherapy drugs, immunotherapy drugs such as Interferon, platelet reducing agents such as Agrylin, and JAK inhibitors such as Ruxolitinib, which help to control the number of red and white blood cells and platelets in the bloodstream as well as the size of the spleen.

Allogeneic stem cell transplant. This is reserved for high-risk patients only; your haematologist will discuss if this is an option for you.

For more information, visit Cancer Treatment. 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.

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 eight weeks of the decision being made.

For people who are at a high-risk for blood clots, treatment should begin immediately, usually starting with taking anti-platelet or anti-coagulant medications and venesections if haematocrit is very high in PV patients. 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.

A priority for treating MPNs is optimising symptom management and quality of life for patients through using combined medical approaches and supportive therapies to support your physical, psychological, nutritional, and general wellbeing.

Comprehensive support and side effect management should be part of your routine ongoing care. It is important to ask your GP and follow health advice on vaccinations including for COVID-19 and other infections such as influenza or shingles.

Ongoing care management plan

You will need regular ongoing follow-up appointments and care, so your specialist and healthcare team will work with you to make a chronic disease management plan for you and your GP. This 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 get help quickly if you have an urgent problem, e.g., blood clots and unusual bleeding
• 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 symptoms of the disease and how to manage them
• 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.

Patients with MPN have an increased risk of other malignancies and therefore should have preventative strategies and appropriate screening (such as bowel, cervical, breast, and prostate cancer screening).

Monitoring of blood counts, renal, and liver function and tolerance to treatment should be undertaken by practitioners who have experience in managing patients with MPNs. These can be tailored to the patient’s treatment regimen and risk factor profile.

Other treatment options may include:

• standard anti-leukaemia strategies (considered on a case-by-case basis)
• clinical trials
• referral to a specialist bone marrow (stem cell) transplant centre
• supportive care and symptom control

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.

What can I do to help myself now that I have an MPN?

Many people are living longer with cancer, and many people with MPNs can have an almost normal life expectancy. It is important that people receive ongoing care and support in finding a ‘new normal’, managing general health, the effects of the cancer and/or treatment, and coping with the emotional impact of cancer and fear of progression.

It is important to take good care of yourself and there are many things you can do to improve your general health and wellbeing including, eating a balanced diet, maintaining a normal weight, exercising, quitting smoking, and considering wellness activities such as yoga and meditation.

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.