Myeloproliferative neoplasms (MPNs) have a high symptom burden that affects functional status, emotional well-being, and quality of life for patients. Symptom control continues to be a challenging therapeutic goal despite available pharmacologic interventions.
The following key factors will help your specialist to work out your best treatment options:
Watch and wait
If you have no symptoms when you are first diagnosed with MF, your specialist may suggest a “watch and wait” approach. This usually involves regular check-ups and blood counts, as well as your specialist advising you on ways to live a healthy lifestyle. If your symptoms develop or the disease progresses, you talk to your doctor about what options would be right for you.
Managing an enlarged spleen
An enlarged spleen is a common symptom of MF, often leading to pain, discomfort and a feeling of fullness or a loss of appetite. Treatment options include:
Managing your anemia
Anemia is common in people with MF. Symptoms include excessive tiredness, weakness and shortness of breath. If these symptoms are causing you difficulties, your specialist may suggest a blood transfusion.
Blood transfusions are usually done in an out-patient clinic and involves the transfer of red blood cells from a compatible donor into your body. This can increase red blood cell count and quickly reduce symptoms of anemia, often within 24 hours. Blood transfusions are a relatively safe procedure and don’t usually cause serious complications, however there is an increased risk of iron overload if you receive a series of transfusions. This usually happens over a number of years, or after a total of 10-20 transfusions have been given. Feeling hot, cold, shivery or in any way unwell during or after the procedure might be a sign that you are having an adverse reaction.
Agrylin® (anagrelide) is a drug option for lowering platelet counts in people who have thrombocythemia (too many platelets in the blood). In some cases it can cause serious side effects including trouble breathing or shortness of breath, chest pain, palpitations and swelling in the feet and ankles. Most common side effects are headache, diarrhea, weakness, nausea, abdominal pain and dizziness
The decision to begin any treatment should take place only after the risks and benefits are discussed with your treating physician.
Bone marrow or stem cell transplant
A bone marrow or stem cell transplant offers the only curative treatment for MF. Patients are individually assessed as transplants are not commonly recommended. This is due to a high risk of life-threatening side effects, as well as a risk that the new stem cells will react against your body’s healthy tissues. Transplants are often only considered an option for fit patients with advanced disease. The first step involves very high levels of chemotherapy or radiation therapy.
The decision to begin any treatment should take place only after the risks and benefits are discussed with your treating physician.
Management of PV is aimed at reducing the number of cells in your blood to control symptoms and reduce the risk of complications.
It is vital to monitor and control your hematocrit level as this is an important indicator of the severity of your condition. Keeping your hematocrit levels below 45% and normalizing your white blood cell and platelet counts can help to reduce the risk of major thrombosis and cardiovascular complications.
The effective management of your PV can also help alleviate some of the symptoms that these blood problems can cause. Avoiding situations that make symptoms worse can also help – for instance, hot baths can make itching worse.
Managing thrombocythemia
Agrylin® (anagrelide) is a drug option for lowering platelet counts in people who have thrombocythemia (too many platelets in the blood). In some cases it can cause serious side effects including trouble breathing or shortness of breath, chest pain, palpitations and swelling in the feet and ankles. Most common side effects are headache, diarrhea, weakness, nausea, abdominal pain and dizziness.
The decision to begin any treatment should take place only after the risks and benefits are discussed with your treating physician.
What you can expect
Your individual situation and health history, as well as the ways you respond to treatment, can all affect your prognosis. Your specialist will be able to provide you with a more accurate picture.
In general, patients with PV who do not progress to myelofibrosis (MF) or leukemia can expect to have a normal to slightly reduced life expectancy if properly monitored and treated as necessary.
In some patients the disease may remain stable or gradually progress over time. This means that you may never experience many of the symptoms and outcomes that you read about – or if you do, it may not be for many years. Many people with PV live normal lives for years without experiencing any complications.However, even if you are experiencing no symptoms, it is still important that your hematocrit level is controlled in order to reduce the risk of serious problems such as major thrombosis and cardiovascular complications.
In a minority of cases, PV can progress into myelofibrosis (MF) or acute myeloid leukemia (AML).Myelofibrosis is also an MPN and it occurs when the bone marrow is replaced by scar tissue, which affects the bone marrow’s ability to produce normal blood cells.AML is a malignant disease that develops when there are too many immature blood-forming cells in the blood and bone marrow, which in turn affects the development of your infection-fighting white blood cells.If you have any concerns, talk to your specialist.
Management of ET is aimed at managing symptoms and reducing complications, to help improve quality of life.
If you have no symptoms when you are first diagnosed, a “watch and wait” approach may be recommended if your platelet counts are at an acceptable level and if you are not in any other high-risk groups for developing blood clots. Your doctor may advise you on ways to live a healthy lifestyle, including giving you help to stop smoking.
Some ET patients may require various therapies, based on the symptoms and the results of routine monitoring by a physician. Treatment choice is generally based on a variety of risk factors including age, history of thrombotic events, and drug tolerance.
Reducing the risk of blood clots
Lowering blood platelet counts in people who have thrombocythemia (too many platelets in the blood) can reduce the risk of clotting and improve symptoms, including serious complications related to blockages in blood vessels and bleeding.
Managing thrombocythemia
Agrylin® (anagrelide) is a drug option for lowering platelet counts in people who have thrombocythemia (too many platelets in the blood). In some cases it can cause serious side effects including trouble breathing or shortness of breath, chest pain, palpitations and swelling in the feet and ankles. Most common side effects are headache, diarrhea, weakness, nausea, abdominal pain and dizziness.
Your treatment plan will depend on your own unique situation and take into account the symptoms you are experiencing as well as any lifestyle risk factors like high blood pressure and whether or not you smoke.
The decision to begin treatment should take place only after the risks and benefits are discussed with your treating physician.
What you can expect
Your individual situation and health history, as well as the ways you respond to treatment, can all affect your prognosis (the predicted outcome of the disease). Your hematologist will be able to provide you with a more accurate picture.
In general, patients with ET without severe clotting or bleeding complications have an excellent chance of living out a near-normal life span if properly monitored.
In some patients the disease may remain stable or gradually progress over time. This means that you may never experience many of the symptoms and outcomes that you read about – or if you do, it may not be for many years. Many people with ET live normal lives for years at a time without experiencing complications.
In a minority of cases, ET can progress into myelofibrosis (MF) or acute myeloid leukaemia (AML). MF is also an MPN and it occurs when the bone marrow is replaced by scar tissue, which affects the bone marrow’s ability to produce normal blood cells. AML is a malignant disease that develops when there are too many immature blood-forming cells in the blood and bone marrow, which in turn affects the development of your infection-fighting white blood cells. If you have any concerns, talk to your specialist.
Your doctor will discuss ways to manage your MPN, in order for you to feel as well as you can. The best part is that you may only have to make some simple lifestyle changes to have a real impact.
Your doctor will monitor your condition but you also play a vital role – alongside your doctor – to manage your condition and your symptoms. Following your doctor’s advice on how to manage your MPN, as well as adopting a healthier lifestyle can help lower your risk of complications. The goal is to help you to cope with your symptoms and reduce their impact so you can get on with enjoying the things you want to do – whether that’s meeting up with friends, keeping up with your hobbies, going to cultural events or playing with your grandchildren.
A balanced diet is important for everyone, but it’s particularly crucial if you have an MPN as eating well can help lower the risk of some complications:
SMALL CHANGES TO TRY
Sometimes, symptoms can get in the way of having a healthy diet. If you have an enlarged spleen, you may feel uncomfortably full, which can put you off eating more than a small amount, while fatigue can mean you don’t feel up to preparing nutritious food. Here are some easy ways to supercharge your diet:
Staying physically active is important for everyone, but exercise is particularly important for people with MPNs. Here’s why:
The amount of exercise you can do depends on how your condition affects you – some people with MPNs are able to exercise as they always have done, while others can do very little. Always speak to your doctor before you start any new exercise regime, to check it’s safe, and stop if you feel dizzy or breathless, or your heart’s racing, and seek medical advice. Your doctor may be able to help you draw up an activity plan that works for you.
SOME EXERCISE TIPS
When you don’t feel well…
Don’t stop exercising, even when you’re not feeling well. Whether you’re lying in bed or sitting in a chair, you can always do a little. Raise and lower your shoulders, wiggle your fingers and toes or open and close your hands repeatedly. On days when you’re feeling a little better, you can take a light walk, do some housework or gardening. On the other hand, if you feel extremely tired or in pain, listen to your body and rest.
Fatigue is one of the most disruptive symptoms of MPNs.Pacing yourself is important for getting the most from the energy you do have, as well as preventing yourself from getting more tired. Here are some tips to help you pace yourself:
Getting a good night’s sleep is especially vital to help you best manage fatigue and exhaustion. Sleeping well will help you cope better with fatigue when it happens and will likely also make you feel more calm.
Here are some tips for getting a good night’s sleep:
For everyone, smoking further raises the risk of heart disease and strokes, as well as being linked with a range of other serious issues such as lung cancer. So, if you smoke, quitting is one of the very best things you can do for your health.
Some tips for quitting:
For most people, having an MPN doesn’t have to mean no more holidays. Whether you’re planning a weekend away or an exotic trip, follow these tips to get organized:
place for long periods of time. Your doctor will tell you whether it’s safe for you to travel. If it is, you can further help to reduce the risk by:
Your doctor isn’t the only one who can help you cope with your MPN – seeking support from other people in your life can make things that much easier.
It’s natural to feel anxious about certain aspects of your life when you have a long-term condition. Here are some troubleshooting tips to tackle some possible concerns.
I feel scared about having a disease I’ve never heard of:
This is a very natural reaction to a condition like an MPN. Share your concerns with others who have MPNs through online or in-person support groups. Listening to what others have to say can help you feel a lot less isolated and hearing how they’ve dealt with their worries may ease some of your concerns.
I hate to think of being a burden:
A lot of people with chronic illnesses get anxious about the thought of becoming a burden to loved ones. If you feel concerned you may be putting too much strain on your friends and family, you may try to struggle on by yourself, which could affect the way you feel, so it’s important to tackle this worry. We all have different times in life where we can give help to others and need help from others. Ask for help, but also tell others you don’t want to be a burden so they are free to say no; they will likely reassure you.
I’ll be treated differently at work if people know I’m ill:
It’s best to be honest with your HR department and employer about your condition, so they have the full picture and understand why you might need time off. Lots of people have different health issues and it’s likely you’re not the only one in your work place. When you talk to your manager, keep it simple: tell them about the condition you have and explain it’s being managed. You may even be able to come to a flexible arrangement, such as working from home some of the week to save your energy rather than having to do a tiring commute. You might also want to tell some of your colleagues about your situation, so everyone understands – they may be able to help take on some of your work on days you don’t feel well. However, this depends on the relationship you have with people you work with, and you don’t need to tell colleagues if you don’t feel comfortable doing so.
I’m concerned about what will happen to my finances if I can’t work in future:
This is a common worry for people with chronic illnesses. There are lots of “What if…” situations in life and this is one of them for many reasons. If it bothers you, talk to a financial advisor and make a plan. Share your concerns with trusted family and friends who may also have positive ideas to help.
My relationship with my partner will change because of my condition:
It’s true that having a long-term condition like an MPN can have an impact on your intimate relationship. At first, you may both be focused on coming to terms with the diagnosis, and symptoms such as discomfort and fatigue can affect your sex life and the amount of time you spend doing pleasurable things, such as going out for meals or seeing friends. It’s important for both of you to be honest about this impact and your concerns to work out solutions.
Relax with mindfulness meditation:
Research has found a type of meditation called mindfulness meditation can be very effective in reducing anxiety.[ Kabat-Zinn J, Effectiveness of a meditation-based stress reduction program in the treatment of anxiety disorders, Am J Psychiatry 1992;149:936-943.] So try this mini-meditation whenever you need to unwind:
If you or someone you care about has an MPN, you may be dealing with it for a long time. One of your vital partners in this journey is your doctor, the MPN specialist who will follow your progress and who will be responsible for your treatment. It’s important that you have a good and open relationship with your doctor and that you do everything you can to plan for your appointments. Below you will find information to help guide that process.
When you have an illness like an MPN, that is rare and that you will have the rest of your life, a good relationship with your doctor is particularly vital.
Here are some tips for having constructive interactions with your doctors.
If you are having emotional problems, your doctor may have dealt with these issues many times before. Your mood can impact how you feel and how you manage your condition, so open up to your doctor.
If you have any doubts about your diagnosis or how your doctor has suggested managing your condition, you need to discuss this with them.
For example, perhaps your doctor has suggested a “watch and wait” approach for now, and you feel worried about this method. It’s important to let your doctor know how you feel. The chances are your doctor will welcome the opportunity to explain their decision, and will be able to reassure you.
Conversation starters:
If you feel concerned about your doctor’s approach to your MPN, you could ask for a second opinion. While this can feel awkward, it’s common for people with rare conditions like yours to ask for second opinions.
When doctors may not have seen the condition many times, seeking the input of another can mean you end up with a pool of knowledge, which can reassure you you’re getting the best advice and care. You might end up seeing a doctor who has more experience with MPNs and knows more about recent developments than your first doctor. On the down side, you may end up having further tests and you may be given different suggestions and even a different diagnosis, which could be confusing.
If you do decide to seek a second opinion, let your doctor know so both doctors have a full picture of your case.
Conversation starters:
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Myeloproliferative neoplasms (MPNs) are the name given to a group of conditions sometimes referred to as blood cancers.
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