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Home , Blood cell, Myelodysplastic syndrome, Pancytopenia

Myelodysplastic Syndromes (MDS)

A Guide for Patients Introduction

Being diagnosed with a (MDS) can be a shock, particularly when you may never have heard of it. This booklet has been written to help you understand more about MDS. It describes what the various forms are, how they are diagnosed and treated and also the expected outcome (prognosis). It will also provide information on coping with the emotional impact of an MDS diagnosis. For more information, your Bowen, Honorary Professor of haematologist or clinical nurse Myeloid Leukaemia Studies and specialist will be able to provide Consultant Haematologist at St advice that is specific to your James’s Institute of . The diagnosis. booklet has also been reviewed by patients and we are grateful This booklet has been to Chris Dugmore and Claudia written by Dr Sally Killick, Richards for their valuable Consultant Haematologist; Dr contribution. Dominic Culligan, Consultant Haematologist; Philip Alexander, Throughout this booklet you will Counsellor and Cognitive see a number of quotations. These Behaviour Psychotherapist; are the real experiences of MDS Geke Ong and Janet Hayden, patients and will hopefully help Clinical Nurse Specialists; and you to understand your situation peer reviewed by Professor David a bit better.

If you would like any information on the sources used for this booklet, please email [email protected] for a list of references.

Version 2 Printed: 11/2018 2 www.leukaemiacare.org.uk Review date: 10/2021 In this booklet

Introduction 2

In this booklet 3

Acknowledgements 4

About Leukaemia Care 6

MDS at a glance 10

Who gets MDS and why? 12

Signs and symptoms 13

Diagnosis 14

What are the types of MDS? 17

What is the prognosis of MDS? 19

Treatment of MDS 22

The psychological impact of MDS 33

Living with MDS 44

MDS specialist centres and 53 other useful organisations

Glossary 58

Appendix 60

Helpline freephone 08088 010 444 3 Acknowledgements

This booklet has been compiled assistance and advice to by MDS UK Patient Support Group, patients and families affected Leukaemia Care and Bloodwise by myelodysplastic syndromes. in a joint collaboration. Although We offer a helpline, newsletter, you are reading the version website, chat forum and meeting supplied by Leukaemia Care, groups nationwide to facilitate all of the wording is the same contact with other MDS patients in each organisation’s booklet. and their families. Based at King’s This booklet does not endorse College Hospital, MDS UK is the any specific product or brand only national support group solely – any names mentioned are for dedicated to MDS. information only. About Leukaemia Care Acknowledgements and further Leukaemia Care is dedicated to thanks to The Irish Society providing information, support for their permission to use and advice to cancer information and images from patients, their carers and loved their MDS booklet. ones. This booklet has been endorsed Whether they need a listening ear by the Leukaemia & NI from our Patient Advocacy team, charity. a buddy to chat to who has been Leukaemia & Lymphoma NI funds in a similar position, a visit to research into the causes and one of our support groups or good cures of leukaemia, lymphoma quality, trusted information about and myeloma in Northern Ireland. a diagnosis, treatment or side effects, we are here for them all. Contributing charities About Bloodwise About MDS UK We’re the UK’s leading blood MDS UK Patient Support cancer research charity. We fund Group provides information, research to improve treatment

4 www.leukaemiacare.org.uk and care for people living with all types of blood cancer, and we provide anyone affected with information and support.

Helpline freephone 08088 010 444 5 About Leukaemia Care

Leukaemia Care is a national charity dedicated to ensuring that people affected by blood cancer have access to the right information, advice and support.

Our services found on our website at www. leukaemiacare.org.uk/support- Helpline and-information/help-and- Our helpline is available 9.00am - resources/information-booklets/ 10.00pm on weekdays and Support Groups 9.00am - 12.30pm on Saturdays. If you need someone to talk to, Our nationwide support groups call 08088 010 444 are a chance to meet and talk to other people who are going Nurse service through a similar experience. We have two trained nurses on For more information about a hand to answer your questions support group local to your area, and offer advice and support, go to www.leukaemiacare.org. whether it be through emailing uk/support-and-information/ [email protected], support-for-you/find-a-support- over the phone on 08088 010 444 group/ or via LiveChat. Buddy Support Patient Information Booklets We offer one-to-one phone We have a number of patient support with volunteers who have information booklets like had blood cancer themselves this available to anyone who or been affected by it in some has been affected by a blood way. You can speak to someone cancer. A full list of titles – both who knows what you are going specific and general through. For more information information titles – can be on how to get a buddy call

6 www.leukaemiacare.org.uk 08088 010 444 or email service, LiveChat (9am-5pm [email protected] weekdays). Online Forum Campaigning and Advocacy Our online forum, Leukaemia Care is involved in www.healthunlocked.com/ campaigning for patient well- leukaemia-care, is a place being, NHS funding and drug for people to ask questions and treatment availability. If you anonymously or to join in the would like an update on any of discussion with other people in a the work we are currently doing or similar situation. want to know how to get involved, email advocacy@leukaemiacare. Patient and carer conferences org.uk Our nationwide conferences provide an opportunity to Patient magazine ask questions and listen to Our quarterly magazine patient speakers and medical includes inspirational patient professionals who can provide and carer stories as well as valuable information and support. informative articles by medical professionals. To subscribe go Website to www.leukaemiacare.org.uk/ You can access up-to-date communication-preferences/ information on our website, www.leukaemiacare.org.uk, as well as speak to one of our care advisers on our online support

Helpline freephone 08088 010 444 7 Your details:

Date of diagnosis

My diagnosis/type of MDS

Cytogenic results

Date Details and Good, normal or poor affected

8 www.leukaemiacare.org.uk Name Contact details

Consultant haematologist

Specialist nurse

GP

Haematology day care unit

Haematology inpatient ward

Emergency contact number

Helpline freephone 08088 010 444 9 Myelodysplastic syndromes at a glance

What are This results in the number of blood cells in the bloodstream myelodysplastic being reduced. This is referred to syndromes? as a ‘cytopenia’. Some patients The myelodysplastic syndromes, have just one type of or MDS for short, are a group of that is low (such as red blood in which the production cells), however, sometimes MDS of blood cells by the can cause a reduction in all the is faulty. It is a type of cancer and types of blood cells. When this sometimes may be referred to as occurs, it is called ‘’. bone marrow failure. The bone marrow is located inside some of Can MDS to any your bones and it is the factory other conditions? where blood cells are made. It is In addition to low blood counts, here where the problem lies. the myelodysplastic syndromes The bone marrow makes three share a common tendency to main types of blood cells: develop into acute myeloid leukaemia (AML) over time. In 1. Red blood cells that carry MDS, the bone marrow has a oxygen around the body number of immature abnormal cells called blasts. In some 2. White blood cells that fight patients with MDS the number of blasts increases with time. 3. that prevent Leukaemia (AML) is defined as having more than 20% blast cells. What causes MDS? The risk of AML occurring depends In MDS, the bone marrow is on the type of MDS, but some usually more active than normal, patients may never progress to yet the blood cells it produces AML. are not healthy (we refer to that as ‘dysplastic’); do not work as You can find out more about well as they should and many AML in factsheets and booklets die either before they reach the available from Bloodwise and bloodstream or shortly afterwards. Leukaemia Care.

10 www.leukaemiacare.org.uk Is MDS a cancer? You do not need to MDS is a form of bone marrow learn everything about cancer, although its progression into leukaemia does not always MDS at once. You can occur. It is included in the World keep this booklet and Health Organisation Classification refer back to it, reading of Haematopoietic (blood and different sections as bone marrow) Tumours. and when you’re ready.

Helpline freephone 08088 010 444 11 Who gets MDS and why?

MDS is a rare disease. It may be previous or diagnosed at any age but it is very radiotherapy. rare in children and young adults. Inherited disorders – very This booklet deals with MDS •• rarely, MDS can be inherited or occurring in adults. may develop from another rare The typical age for patients to blood disorder. For this reason, develop MDS is around 75 years young patients may be tested old. About 9 out of 10 patients for any diseases that are linked are over 50 years at the time to MDS. However, for the vast of diagnosis. Men are slightly majority of patients, MDS will more likely than women to be not be passed down to children diagnosed with MDS. and is not an inherited genetic disease. The cause of MDS remains largely unknown, although there are ••Environmental factors – many research groups around the exposure to toxic chemicals world who are trying to improve such as may our understanding of why it marginally increase the risk of occurs and in whom. There are MDS, but such exposure is now certain factors that may increase uncommon. your chance of developing MDS and these include: MDS is not an ••Previous chemotherapy with infectious disease and or without radiotherapy – this it cannot be passed on treatment may have been given to other people. in the past (usually for other ). It is thought that the treatment damages the bone marrow and may cause MDS in some patients. This is called secondary or therapy-related MDS, as it is secondary to the

12 www.leukaemiacare.org.uk

What are the signs and manifest itself as a rash on your skin. These are tiny symptoms of MDS? under the skin called petechiae Symptoms vary from person to and often appear where clothes person and depend on which are tight fitting like around the blood cells have become reduced ankles or waist. Nose or gum in your bloodstream. About 8 in bleeds can also be a sign of a low 10 patients have anaemia, whilst count. about 2 in 10 present to their doctor with infections or bleeding. Recurrent and persistent infections are another common Anaemia is due to a lack of red symptom of MDS due to low white blood cells (also referred to as low blood cell counts. haemoglobin), which may lead to and , Some MDS patients have no signs even on light exertion. or symptoms and are diagnosed by chance as a result of a routine "When I was diagnosed I was blood test. surprised; I had not heard of MDS before. But I also felt relieved; a reason why I was so fatigued." Anaemia is the most commonly experienced When your platelet count is low, you can suffer from easy bruising symptom in MDS. and bleeding. This can sometimes

Helpline freephone 08088 010 444 13 Diagnosis

How are the condition or monitor response to myelodysplastic treatment. syndromes diagnosed? What does a bone marrow test involve? Full blood count This is usually performed as an Low blood counts are picked outpatient, meaning you don’t up by a simple test called a full need to stay in hospital overnight blood count (FBC). The laboratory and can go home after the performing the test will then procedure. A small liquid bone examine the blood cells on a slide marrow sample is taken followed (called a ) under the by a biopsy, generally from the microscope. If you are found to pelvis. The doctor will numb the have changes on the blood film area with local anaesthetic and that suggest MDS, you will usually insert a needle into the bone be referred to a blood specialist marrow cavity in order to take the (haematologist). It is important sample. The procedure usually to rule out other causes of a low takes around 20-30 minutes, but blood count so the doctor will ask you should allow an hour of your general health questions and give time. It may be uncomfortable at you a physical examination. the time, or later that evening, but Bone marrow test simple paracetamol is usually enough to relieve the pain. You As MDS is a disease of the bone may be asked to lie on your back marrow, a bone marrow test is after the procedure for 10 minutes usually needed to diagnose the to reduce the risk of bleeding.

14 www.leukaemiacare.org.uk A small dressing or plaster is changes in the structure of applied to the site. the in the affected cells. In addition, there "I remember very little from when I is a general move towards was diagnosed as I was terrified and molecular testing (to look for in shock." DNA ), which we hope How long do I have to will lead to more precision and therefore make care more wait for the results? personalised. However, at the Preparing the sample and moment we are still learning what analysing all the results can take the molecular changes may mean two to three weeks. The bone for MDS patients. marrow sample is examined by If the bone marrow changes are a doctor under a microscope not clear and there is doubt about to look for changes seen in whether or not you have MDS, your MDS. Additional tests are often doctor may decide to monitor your requested on the bone marrow blood counts and repeat the bone sample, which may help to marrow test at a later stage. make the diagnosis and provide information about how the A bone marrow sample may not disease will behave (prognosis). be necessary in some cases, for example if the results are unlikely These tests include to affect treatment decisions for (sometimes called a ‘’), the person. which is the study of the

Helpline freephone 08088 010 444 15 Diagnosis (cont.)

You can find details of all the Waiting for your test different support organisations in Chapter 10: MDS specialist results may be an centres and other useful anxious time. Talk to organisations. your family and friends or contact patient support organisations which can assist in different ways. They can help put you in touch with other patients over the phone, in person or through online forums.

You can read about the experiences of other people who are going through or have been through something similar on the websites of patient support organisations. It is important at an early stage to get support and information from recommended and reputable sources, as the internet can present misleading and unvetted information.

"There is a lot of confusing and scary literature around, so talk with real people about it; it’s really helpful."

16 www.leukaemiacare.org.uk What are the types of MDS?

Your doctor will describe the type included in the current 2016 of MDS that you have, as each classification. These are: type can behave differently. The MDS with single lineage World Health Organization (WHO) 1. (MDS-SLD) has developed a regularly updated classification for separating the 2. MDS with multilineage different types of MDS. This was dysplasia (MDS-MLD) updated in 2016, and replaces the older classification of MDS in 3. MDS with ring sideroblasts 2008. Both classifications may (MDS-RS) still be referred to and therefore 4. MDS with excess blasts (MDS- the old classification can be seen EB); MDS-EB-1 and MDS-EB-2 as an Appendix at the end of the booklet. This system is based on 5. MDS, unclassifiable (MDS-U) the blood results, the appearance 6. MDS with isolated del(5q) or of the bone marrow, the number with 1 additional abnormality of abnormal immature blast cells and any chromosome changes Please note: Information on found. The immature cells are CMML (chronic myelomonocytic called blasts, which may be leukaemia) is available as a increased in some of the types of separate booklet, as it now MDS. falls under the WHO 2016 category of ‘Myelodysplastic/ There are six broad types of MDS Myeloproliferative neoplasms’.

Helpline freephone 08088 010 444 17 What are the types of MDS? (cont.)

is missing. There are many types What does high risk of MDS and this can be and low risk MDS difficult to understand. mean? Spend time talking to It is often easier to consider your doctor or nurse whether the type of MDS you have so you understand falls into what is called a LOW how your MDS will be risk group or a HIGH risk group. treated. It’s important The ‘risk’ refers to your chance of developing acute myeloid that you know and leukaemia (AML) and your life understand your exact expectancy (survival). In the low diagnosis. You could risk disease group a patient ask your treatment has about a 1 in 10 chance of team to write it in the progressing to AML. The low risk group includes MDS-SLD, MDS- front of this booklet, MLD, MDS-RS, MDS-U and MDS so that you have it to with del(5q) either in isolation or hand. with 1 additional abnormality. In contrast, the risk of developing leukaemia is greater in the high The terminology used can be risk disease group (MDS-EB). The difficult to understand, so ask separation into these groups is your doctor to explain which important as the treatment of type of MDS you have. Dysplasia patients with low risk and high means that the bone marrow cells risk disease can differ. Your doctor are abnormal in their appearance. may use the WHO classification Sideroblasts are young red blood to decide whether your disease cells that have a very distinctive is low or high risk; although ring of granules seen under it is usually better to use the the microscope. MDS with isolated prognosis score discussed next. del(5q) means the chromosome tests show part of

18 www.leukaemiacare.org.uk What is the prognosis of MDS?

Prognosis refers to the expected time your MDS is diagnosed outcome or survival from MDS The number of abnormal and is therefore different from •• immature cells (blasts) in your classification. Your prognosis bone marrow at diagnosis can depend on many factors, including those not related ••The chromosome test results to MDS, such as your general from your bone marrow at fitness and age. However, to diagnosis allow your doctor to make the right treatment decisions, MDS The calculator adds together the doctors and scientists around individual scores to give a final the world have designed a score, which puts you into one of number of scoring systems to the following IPSS-R risk groups: help predict how your MDS is ••Very low likely to behave. Over time, these scoring systems have evolved to ••Low give more accurate estimations. Intermediate The most commonly used •• scoring system is the revised ••High International Prognostic Scoring System (IPSS-R). This replaces the ••Very high previous IPSS. The risk groups describe the expected risk of developing acute Can you explain the myeloid leukaemia (AML) and scoring systems in expected survival. This helps your more detail? doctor to identify and discuss The British Society of with you the best treatment Haematology MDS Guidelines choices for you as an individual. recommend using the IPSS-R as a For more information about scoring system (see appendix). the risk and survival predicted in each group, see page 62 The IPSS-R score is calculated [appendix]. It is important to from: understand that the expected outcomes for each group give an ••Your blood count results at the

Helpline freephone 08088 010 444 19 What is the prognosis of MDS? (cont.)

an indication of what may happen UK MDS Forum and in that group of patients as a collective. This gives a useful British Society of framework for a discussion about Haematology (BSH) the future and the options for MDS Guidelines treatment, but it is not possible A group of expert haematologists, to give a precise outcome figure with a specialist interest in MDS, for an individual patient. As our has prepared guidelines for the understanding of the molecular diagnosis and therapy of adult changes seen in MDS increases, myelodysplastic syndromes. this may allow us to more These are updated periodically accurately work out an individual to reflect changes in medical patient’s outcome and guide practice. The content of the treatment further. treatment section of this booklet Doctors sometimes refer to ‘low is based on the BSH guidelines, risk’ and ‘high risk’ MDS. If doctors the full version of which can be are using the older IPSS, ‘low found online at www.b-s-h.org. risk’ refers to patients with Low uk/guidelines and Intermediate-1 categories, It is important to understand that whilst ‘high risk’ refers to although guidelines represent patients with IPSS Intermediate-2 the collected opinions of a group and High categories. The risk of experts based on best clinical designation is more difficult practice from available evidence, with the more recent IPSS-R, they are only guidelines. In most ‘low risk’ certainly including cases, a patient’s treatment will Very Low and Low categories and be based on these but a doctor ‘high risk’ including High and may decide that it is not in Very High categories. As yet, the the best interests of a specific risk designation for the IPSS-R patient to be treated exactly, or Intermediate group has not been even broadly, according to the confirmed. guidelines. If this is the case for you, then your doctor will discuss

20 www.leukaemiacare.org.uk the reasons for this.

The UK MDS Forum is an expert organisation open to those healthcare workers and scientists with an interest in MDS. The aim of the forum is to increase the awareness of MDS through education and increase access to clinical trials for patients with MDS across the UK.

Helpline freephone 08088 010 444 21 Treatment of MDS

How is MDS treated? The MDT meetings The way that MDS behaves varies involve doctors, nurses from person to person, and and other healthcare depends on the type of MDS that professionals putting you have. The types of MDS have their heads together been described in detail earlier in this booklet. Treatment is based and deciding the best on British Guidelines agreed by treatment specifically MDS specialists, and your care for you. will be discussed in your local multidisciplinary team (MDT) meeting. Treatment planning Once the diagnosis of MDS has What is an MDT? been made, you and your doctors Your diagnosis and treatment will decide on the best treatment will be discussed and reviewed for you. The treatment that you are by a team of clinical specialists offered will depend on the type of including haematologists in your MDS you have, your own wishes, local area. They are called your your age, your general wellbeing multidisciplinary team (MDT). or fitness and the IPSS-R score, They hold regular meetings or in some cases the IPSS score which allow your case to be (see earlier section). Before your discussed by many doctors and treatment starts, your doctor or healthcare professionals in the nurse will explain the benefits Haematology clinical team. It and side effects of the chosen may also be necessary to ask for treatment so that you can give an opinion outside of this group your consent. It is likely that you to help either with the diagnosis will need to sign a consent form to or to discuss the best form of agree to the treatment. If you are treatment. unsure about anything, do ask, as MDS is a complicated disease to understand. The BSH MDS guidelines recommend that all patients who are newly diagnosed with MDS are discussed with a

22 www.leukaemiacare.org.uk regional or national expert in MDS so it can grow new healthy blood given that the disease is rare. You cells in the bone marrow are entitled to ask your doctor if Unfortunately, most patients’ they have done this. The NHS also MDS cannot be cured but MDS allows you to ask to see a regional can usually be controlled and or national expert in MDS if you often improved. think that this would help you. The first question that your Not all patients need active doctor will ask themselves is treatment, as some do not have whether there is a treatment any symptoms. If you are not option that has a chance of curing starting treatment, you will have the MDS. The only treatments regular check-ups which is often that can possibly cure MDS referred to as ‘watch and wait’ or are either a or bone ‘active monitoring’. marrow transplant from another "The challenge is predominantly person or, very rarely, intensive mental since it’s very hard to accept chemotherapy. that I have cancer but that it isn’t If a stem cell transplant is being treated." an option for you, you will be Broadly speaking, treatment of identified early so that a search MDS will include one or more of for donors can be started and a the following: transplant considered at an early stage. ••Supportive care – this aims to control the symptoms of MDS What is a ? ••Non-intensive treatment – this Research into MDS continues treatment tries to slow down worldwide to improve our the progression of MDS and knowledge of why MDS occurs, improve your blood counts how individual types of MDS behave and how best to treat the Intensive chemotherapy – this •• condition. Today we benefit from involves giving high doses of the thousands of patients who chemotherapy in hospital have been part of clinical trials ••Stem cell transplant – this and research studies in the past. gives your body new stem cells The words ‘research’, ‘trial’ or ‘new

Helpline freephone 08088 010 444 23 Treatment of MDS (cont.)

drug’ sometimes scare people, but your research team if you have rest assured, patients receiving any questions. new drugs in a trial are monitored very closely for side effects. Your Supportive care doctor may discuss with you a All patients will need supportive clinical trial available at your care at some stage, either alone hospital; however, you cannot be or to support other treatments entered into a trial without your being given. Supportive care is permission. The trial needs to be not directed at the underlying fully explained to you, and you disease but rather at controlling need to have time to think about the symptoms and complications the treatment before deciding. caused by the disease. The nature This is called ‘informed consent’. and extent of supportive care If you agree to be treated in a needed depends on which blood clinical trial, you can still change cells are affected and exactly your mind at any point and come how low the blood levels fall. out of the trial. If you decide not to Most patients will need blood go into a clinical trial, you will be transfusions at some stage. given the best-proven treatment available. Please ask if there are Treating anaemia any clinical trials suitable for you Most patients (but not all) available in your hospital or at diagnosed with MDS are anaemic. your nearest specialist centre. This can cause symptoms such as tiredness and shortness of When you have a bone marrow breath, affecting your quality test to diagnose MDS or monitor of life. Some patients continue how well treatment is working, having a normal or reasonable your doctor may ask your quality of life despite anaemia permission to store some of your and so will not necessarily need blood or bone marrow for future treatment for the anaemia at that research into the of MDS stage. Other patients will need (what causes MDS). There will be blood transfusions to improve an information sheet for you to the symptoms caused by the read and you will then be asked anaemia. The haemoglobin (Hb) to give your informed consent for level in your blood results will this research. Remember to ask show your level of anaemia.

24 www.leukaemiacare.org.uk Growth factors Blood transfusions Blood cell numbers can Blood transfusions are a very sometimes be increased by important part of your care. They the use of growth factors. will be considered if you have Growth factors are like natural symptoms from anaemia. There ‘hormones’ that stimulate our is no set haemoglobin level at blood production. We all make which a is these growth factors every day. given, but your doctor will assess For example, your symptoms and you will (sometimes known as ‘EPO’) is a decide together. The frequency that increases red of transfusions will vary between blood cell numbers. - patients; some need transfusions colony stimulating factor (or every few months whilst others ‘G-CSF’) increases white blood need one every couple of weeks. cell numbers. Not all patients Usually, once you have started are suitable for this treatment, having regular blood transfusions, and only some MDS patients will the length of time between respond. Your doctor can advise transfusions will gradually get you on your suitability for growth shorter. If you find that your factors. EPO is considered a safe symptoms of anaemia come back treatment in MDS. well before your next transfusion is due, contact your Haematology Growth factors are given as an team and discuss whether the injection under the skin. The interval between transfusions number of injections needed should be shorter, or the number will vary from patient to patient. of units of blood increased. This A district nurse can give the varies between patients. An injections, or you (or a family improvement in your symptoms member) can learn how to give after having a transfusion may the injections yourself. The skin not be immediate, sometimes around the injection site may it can take a few days to start become irritated, so it is best to feeling the benefit. regularly change the injection site. Do talk to your nurse(s) about this and also the common side effects that you may expect.

Helpline freephone 08088 010 444 25 Treatment of MDS (cont.)

you are offered iron It is important that therapy or not will depend on the you are transfused likely benefits versus the likely adequately to control disadvantages in your individual your symptoms as it case. This will be discussed with will help your quality you before you make a decision to start iron chelation. It is of life. Discuss this important that you do not reduce with your haematology the number of blood transfusions team.” you receive due to the concern about your iron levels, as this may not adequately control your symptoms. With every unit of blood you receive from a transfusion, you Desferal () is a will receive an excess amount of drug used to treat the build-up iron. Over time this can build up in of excess iron and is given as your body and may possibly cause a continuous subcutaneous damage to certain organs, like injection under your skin by a your heart or . Because blood pump. There are special teams transfusions are rich in iron, it is that can teach you how to important that you do not take administer the drug at home. additional iron tablets unless your Exjade is another iron chelator doctor prescribes them. There is and comes in tablet form. still uncertainty about whether However, in most cases this is too much iron in your body is only available for patients who always harmful. The level of iron in cannot tolerate subcutaneous the body will be regularly checked Desferal, who have serious side if you are on regular transfusions effects on Desferal or where it and treatment will be considered is thought not to be working if there is a build-up of excess adequately. Both treatments can iron. This treatment is called iron have certain side effects and chelation; however, there remains often need to be continued for a uncertainty about the benefits long period of time to be effective. of removing iron. Whether Your doctor can discuss this with

26 www.leukaemiacare.org.uk you. Don’t hesitate to discuss your , are on blood thinners iron levels with your doctor at any or have suffered from bleeding, time during your treatment. you might benefit from platelet transfusions to keep your platelet "Having a blood transfusion is an count at a higher level. Your doctor amazing feeling. People around me or nurse will inform you when this could see the colour returning to is necessary. my face. I had a shower and danced because at last it no longer hurt to stand and wash my hair. The relief It is important for you to was immediate." understand that patients with Platelet transfusions MDS have a higher risk of developing infections. Antibiotics About half of MDS patients will are not usually given to prevent have a reduced platelet count infections, as they cause side at diagnosis (this is called effects and may cause the ). The platelets bacteria to become resistant. But may also function poorly and this if you do get an infection, this means that bruising and bleeding should be treated quickly with can sometimes be a serious antibiotics, and you may need problem in MDS. If you have a to be admitted into hospital so low platelet count, it is usually that the antibiotics can be given advisable to avoid blood-thinning through a vein (intravenously). agents and non-steroidal anti- Most specialist units will have inflammatory drugs. However, this a direct phone number to call should be discussed with your for advice in the event of a doctor as there are exceptions occurring. where the benefit you will receive from these drugs outweighs the risks.

Platelets can be transfused but because they only last about four days, they are not routinely given even when the platelet count is very low. But if you have an

Helpline freephone 08088 010 444 27 Treatment of MDS (cont.)

progression of the disease. It If you are feeling may be considered if your blood unwell, check your counts are quite low or falling, or temperature. If it if there are signs that the disease is developing into leukaemia. The is raised, or you idea is to treat the disease with experience shivers, as few side effects as possible, contact your specialist thereby maintaining a good nurses at the hospital quality of life. These treatments or call the helpline will not cure your MDS but may ‘modify’ the disease. These number you have been treatments are usually given as given. an outpatient. Hypomethylating agents (HMA) Treatment extending beyond supportive care can be classed Hypomethylating agents work as low-intensity, high-intensity on the behaviour of cancer or high-intensity with a stem cell cells at the DNA level and can transplant. turn on and off. Drugs such as work to improve bone marrow function Fact sheets for all and slow the progression to treatments are leukaemia. They are currently available. Please ask used in high risk MDS patients your nurse specialist (IPSS Intermediate-2 and High for the correct patient categories) who are not fit enough for a stem cell transplant. information sheet Azacitidine is usually given as to help you fully an injection under the skin. Side understand your effects can include: treatment. ••Mild nausea ••Diarrhoea or constipation Non-intensive treatment ••Skin irritation at the injection site Low-intensity or non-intensive treatment aims to slow the ••Becoming more prone to

28 www.leukaemiacare.org.uk infections (due to lowered blood In a small number of patients with counts) MDS, the number of bone marrow cells is unusually low (termed hypoplastic). This is similar to If you have a certain type of a blood disease called aplastic MDS which has deletion of part anaemia. Patients can sometimes of chromosome 5 (this may respond to drugs targeted at be referred to as the 5q minus suppressing the , syndrome or del 5q), you may be such as anti-thymocyte globulin offered lenalidomide if you are (ATG) or ciclosporin. anaemic. This is taken orally as a capsule and works in several Intensive ways to suppress the MDS cells, chemotherapy including altering the immune If you have high risk MDS, you system. Therefore, it is often may benefit from intensive referred to as a type of immune chemotherapy. It is the same modulation therapy. On starting treatment that is used to treat the treatment, your blood counts acute myeloid leukaemia fall before a response is seen. and aims to kill a significant During this early stage, you may proportion of the diseased cells need transfusions of blood and/ from your bone marrow to allow or platelets. Some patients also the bone marrow to work normally need G-CSF. Other side effects can again (remission). The treatment include: has a high number of side effects ••Rashes so you need to stay in hospital for four to six weeks for each course. ••Fatigue A small proportion of people ••Diarrhoea may be cured by intensive ••A small increased risk of blood chemotherapy alone, although clots usually when a donor is available, a stem cell transplant will follow. As lenalidomide can cause birth Achieving remission, even if not a defects, you must avoid getting cure for the disease, can improve pregnant whilst taking the drug. your quality of life (often almost to normal quality) as long as the Immunosuppressive therapy remission lasts.

Helpline freephone 08088 010 444 29 Treatment of MDS (cont.)

How is intensive chemotherapy ••Infections given? ••Bleeding Most chemotherapy is given as an infusion into a vein ••Anaemia (intravenously), but sometimes Other side effects can include: as a tablet. It is given as a course or cycle of treatment, whereby a ••Hair loss combination of chemotherapy Nausea is given over a number of days •• followed by a rest period. It is ••Vomiting often easier for you to have a Hickman line inserted, which ••Sore mouth allows all the drugs to be given ••Diarrhoea and blood tests to be taken. This is a line that is carefully inserted ••Loss of appetite and taste into a large vein and can stay ••Skin and nail changes in place for the duration of your treatment. ••Infertility What are the most common Allogeneic stem cell side effects from intensive transplant chemotherapy? A stem cell transplant, also The chemotherapy used in MDS referred to as a bone marrow is specially designed to kill the transplant, offers the chance of cancer cells in the bone marrow, curing the disease. so your blood counts will fall after the chemotherapy and In an allogeneic transplant, remain low for a number of weeks. healthy bone marrow or stem cells Healthy bone marrow cells are are taken from another person also ‘stunned’ in a type of ‘friendly whose tissue DNA is identical or fire’ but can recover better than almost identical to yours. This the MDS cells if remission is means the donor is compatible achieved. During this time there with you. The bone marrow or can be serious, sometimes life- stem cells are taken from a donor threatening side effects, the most – either a family member (usually common of which are: a sibling) or an unrelated donor.

30 www.leukaemiacare.org.uk The donor has a simple blood of this treatment to you as an test to see if they are matched to individual. Always try to take a you – they do not need to have a family member or friend to the bone marrow test. The results are appointments. usually available in two to three weeks. You can find out As medical knowledge and more about stem experience has progressed, more cell transplants in patients can now be considered several booklets: ‘The for a transplant. Reducing the intensity of the conditioning Seven Steps’ booklets, treatment done before the available from transplant also means the side Bloodwise and Anthony effects from the transplant itself Nolan, or those from are less severe. This approach Leukaemia Care. is called a reduced intensity conditioning (RIC) transplant. About one third of patients who Follow-up receive this treatment are free of Once MDS has been diagnosed, disease over many years but the your specialist will discuss disease may return (relapse). treatment options and follow-up. This treatment has many side For some patients, this will only effects and it is important mean infrequent outpatient visits that the decision to have an to check if the disease is showing allogeneic stem cell transplant signs of progressing. Sometimes is carefully thought through these check-ups can be shared by your healthcare team and with the GP. For those patients yourself. It is also important where the disease is thought to to know that the side effects be high risk or for those who have from a stem cell transplant can received active treatment, the continue for a number of years outpatient visits may be more after the transplant. If you are frequent. This will be individually suitable for a transplant, you will tailored to you. be referred to a specialist centre to discuss the benefits and risks

Helpline freephone 08088 010 444 31 The psychological impact of MDS

This chapter is about the these emotional ups and downs is emotional impact of having MDS, adjustment. which can be as significant as the "The diagnosis hit me like a ton physical impact of the illness. It of bricks. My emotions were on a is important to emphasise that rollercoaster." each person with MDS will cope in their own unique way, and your Adjustment is something every healthcare team are experienced person will experience as they go at considering your emotional through significant life events like needs, as well as your physical divorce, bereavement or illness. needs. It involves changes and losses of varying kinds and includes A Holistic Needs Assessment or both practical and mental Quality of Life questionnaire or adjustments. In the case of an assessment can often help to illness these include: identify certain problems, or help discuss them with staff. ••Getting used to being monitored Not everything in this chapter will ••Having medical appointments apply to you, but there are some and treatments common thoughts and feelings that you could be familiar with – ••Potential loss of – or reduction and, to some extent, your relatives in – some physical capabilities and carers too. which, in turn, could affect things like employment, or Adjustment personal roles and relationships People living with MDS ••Disruption to one’s usual life sometimes experience a range patterns and routines of complex thoughts and intense feelings as they try to cope with ••Questioning things normally the diagnosis, monitoring or taken for granted, like good treatment. This is often described health and future plans – as ‘being on an emotional perhaps making people more rollercoaster’. The formal term for worried about things than usual

32 www.leukaemiacare.org.uk Given the losses and changes issues. involved – which to some can "The emotions that went through my feel frightening – and the need to body cannot be explained – there adapt to and cope with something was anger, worry, fear and sadness. new, adjustment can be both But the overwhelming one was stressful and distressing. The determination that we would get good news is, while the emotional through this." ups and downs of adjustment aren’t always easy, with time most Coming to terms with people do adapt well to their new situation. your diagnosis Although everyone is different, What feelings might generally it is helpful to ‘process’ you experience and your thoughts and feelings, rather how can you help than ignore them. This means thinking about your diagnosis, yourself? including what it means to you This section describes many and how you might cope. It means of the common emotions (and being aware of your feelings and related thoughts) that people with being able to express them when MDS might experience. Following you want to. It can be helpful to each description are some talk about your situation with suggested coping strategies. other people, both professionals These are drawn from evidence- and those in your personal life. based psychological practices, as Writing thoughts and feelings well as feedback from patients down can help you to process about what has helped them. It them too. It is useful to strike a is important to remember that balance between thinking and not everyone will experience all talking about your situation, of these feelings, but it is equally and having periods in which you important to emphasise that if focus on other, meaningful and you have some – or all – of them, enjoyable things instead. you are not alone and it is not a sign of weakness or mental health "It’s so important to stay positive,

Helpline freephone 08088 010 444 33 share what’s going through your mind to change negative thoughts and and know that you’re not alone." thinking patterns. In short, the way we think affects the way we There is a link between feel. thoughts, feelings, physical sensations Managing thoughts and behaviour Writing negative thoughts and worries down can be helpful. Before we move on, it is useful You will notice that some of to explain that within every type them are ‘valid’ (this means of mood there are four elements: understandable and acceptable) thoughts, feelings, physical given your situation, but some sensations and behaviours of them are ‘catastrophic’ (the actions we take, or don’t (meaning they predict the worst take, to cope). Also, each of case scenario) or are very ‘black these elements interacts with and white’ (meaning things are and affects the other, as in this all good or all bad). Here are diagram (above). some examples: "because of It is hard to directly ‘access’ and this illness, my life’s ruined"; "I change an emotion, whereas know the treatment won’t work"; thoughts and behaviours and, "nothing ever goes right for me"; to some extent, physical states, "everything’s awful"; "there’s no are more easily changed. This hope"; "I must be a bad person". can help to improve emotional Take a step back and ask yourself feelings. It is particularly effective whether those thoughts are facts or opinions. Say to yourself "is

34 www.leukaemiacare.org.uk there another way of looking at even a little exercise, moderating this?", or "is that actually what my alcohol consumption, and medical team said to me?" Write connecting with other people will down alternative, more helpful help your mood. Another helpful thoughts next to the original behaviour is ‘pacing’. This means worries. This is not the same as doing a consistent amount of ‘positive thinking’, as you may activity on a regular basis, but have some valid concerns; it is not overextending yourself on a about maintaining perspective, good day. Overextending activities having a balanced view, and not tends to result in people being getting too caught up in your so exhausted that they cannot thoughts. function for a few days.

"I think at the beginning I did bottle "I have accepted that first thing in the my feelings up too much, trying to morning I am not going to leap out of stay strong for everyone else." bed; my whole body aches so I take it slowly. I feel human and am able to Changing behaviour cope with almost anything. I still play Some types of behaviour make golf a couple of times a week, and, emotional distress worse. For being a competitive person I have to example, when people avoid remind myself each time how lucky I activity, socialising and exercise am just to be out in the sunshine." (even doing basic things like having a shower and getting Specific emotions and dressed) they make depression coping strategies worse, rather than better. And, Shock, disbelief, helplessness when people avoid situations that and feeling out of control make them anxious, this also tends to make the problem worse, These feelings and sensations are rather than better. common when people experience something outside of the realm Behavioural change, which of their normal experience, like includes engaging in enjoyable a diagnosis of a serious illness. and meaningful activities, doing The situation can feel threatening

Helpline freephone 08088 010 444 35 The psychological impact of MDS (cont.)

and people wonder whether they ••‘Grounding’ techniques can be can cope. These thoughts and useful. These are simply things feelings can be so overwhelming people can do to bring their that people become shocked or awareness to the reality of the numb as a means of protecting present moment, in other words themselves. Some people describe to feel less detached or unreal. a sense of unreality. You can find more information on grounding techniques at "There was a total overwhelming www.healthyplace.com feeling of helplessness and being out of control of my everyday life. "At diagnosis I was shocked and But I had to carry on regardless for upset. But I was determined to fight it everyone else." and live a long life." Coping strategies Worry, anxiety and living with ••Time is needed for the uncertainty information to sink in and to be MDS often carries with it a ‘processed.’ degree of uncertainty which can lead to worry and anxiety. It is It can be helpful to talk things •• normal to experience fear about through with others and to something which is threatening. express feelings. This helps The emotional response to people to make sense of their fear is anxiety, or even panic. situation and to think about This tends to be driven by the how they will cope. physical response to fear, which ••Having access to the right is a release of adrenaline into information at this time is the bloodstream. This to important. It can be difficult many of the physical symptoms to take everything in, so it of anxiety like increased heart can be useful to write down rate and dry mouth. The mental questions that need answering aspect of fear is worry. This is or clarifying by the healthcare what people do as they try to team. predict and control things that might happen in the future.

36 www.leukaemiacare.org.uk While it is normal for people to ••A widely-used technique is worry about their illness to some called ‘progressive muscle degree, excessive worry will lead relaxation’. This gets people to chronic anxiety and exhaustion. to consciously tense and un- tense their muscles to induce "I was anxious a lot and focused on relaxation. my diagnosis and what might happen to me. I didn’t want to die." ••Notice your negative thoughts and worries and write them Coping strategies down to challenge them. ••Managing the physiological part Take gentle exercise. of anxiety is key as this will help •• to reduce the level of fear and ••Various forms of distraction, or stop the pulse - and thoughts - mental exercises like Sudoku, from racing. can help.

••To do this, it is necessary to "Living with uncertainty has been a reduce hyperventilation (over phrase which I have often used in the breathing) and excessive past. But living with MDS tests it to adrenaline production, which extremes. In reality, it’s very difficult are always present in anxiety. to live when you are uncertain what the next few months or years have ••Slow, controlled breathing is in store for you. Uncertainty breeds the most effective method. anxiety." Practices like mindfulness and meditation can be useful, Finally, a vital aspect of managing but there are also a number of worry is to accept that some different breathing exercises things you cannot know in that are helpful. For examples advance or control. Also, while of these online, visit www. it is possible to reduce some of getselfhelp.co.uk and www. the symptoms of anxiety, it is patient.co.uk not possible to eliminate them altogether. As human beings we ••Reducing tension in the all live with a degree of anxiety. muscles is another means of A useful means of managing alleviating anxiety. uncertainty is to focus on the

Helpline freephone 08088 010 444 37 The psychological impact of MDS (cont.)

‘here and now’ – on the things you common), dwelling on the can change, and on the things thoughts behind it tends to that you find meaningful and make the anger worse. Talk enjoyable in the present moment. things through with others. Anger ••Write down some of your thoughts and notice those It is common for people to that keep the anger going; try feel angry that they have been to change them or distance diagnosed with a serious illness. yourself from thoughts about For a number of reasons it can things that have no explanation feel confusing, unfair, or that it or cannot be changed. is outside of their control – for example if the illness is rare, ••Use relaxation techniques, if they believe that they have a or exercise, for managing healthy lifestyle, if there’s no the physiological symptoms history of similar illnesses in of anger (similar to those in their family, or if they believe that anxiety, and also driven by they have already had too many adrenaline). problems in life to cope with. Sometimes it can be difficult to ••‘Venting’ anger at others tends know what to do with feelings of to be self-defeating because it anger or to understand at what alienates people, rather than the anger is directed. As a result eliciting feelings of compassion anger can sometimes get directed from them. at loved ones, or even towards ••Self-soothe. Treat yourself to oneself. things you enjoy; treat yourself "At diagnosis I felt distraught, with compassion. devastated and angry. It was unfair. Stress Why me?" We experience stress when we Coping strategies feel that we are under too much ••Although a sense of disbelief pressure or have too many or injustice at being diagnosed demands being made of us and with MDS is valid (and that we don’t have the resources

38 www.leukaemiacare.org.uk to cope. Understandably, people ••Pace yourself, rather than doing can feel like this at times when too much or trying to cope with they are ill and they are trying everything at once. to cope with the demands Take breaks and ‘time out’. of treatment as well as with •• other concerns, for example ••Ask for help and support. financial issues, employment and relationships. The emotional ••Maintain a ‘here and now’ focus symptoms of stress can include on things that are enjoyable and low mood, anxiety and irritability. meaningful to you. Coping strategies Useful online resources include: ••Relaxation techniques ••www.helpguide.org (controlled breathing, www.getselfhelp.co.uk progressive muscle relaxation) •• and/or exercise to manage the ••www.nhs.uk/conditions/ physical, adrenaline-fuelled stress-anxiety-depression aspects of stress (which are the same as those in anxiety and Guilt and blame anger). Although becoming ill is never anyone’s fault, it is common for Notice the negative thoughts •• people to experience feelings which contribute to anxiety, of guilt and blame about their tension and irritability, illness. For example people might producing adrenaline as they question whether they became ill arise: "this is unbearable", "I because of something they did, or can’t cope". their ‘lifestyle’. Some people might ••Challenge negative thoughts by think that they are a burden on writing them down and coming other people because they need up with more helpful ones e.g. their help, or because they are "although this is difficult, I can not functioning as they once cope (especially with support)". did. Although these thoughts are common, they are not valid. They ••Plan, prioritise, break things can be part of people’s attempts down into manageable ‘chunks’ to find meaning in the situation, (writing this out is helpful).

Helpline freephone 08088 010 444 39 The psychological impact of MDS (cont.)

but what they do is to make days people can feel very down, people feel bad and distressed but usually these feelings are about themselves. temporary and people tend to say that they have ‘good days and bad "At diagnosis, I felt terror, grief, shock, days’. depression, helplessness." However, when people feel Coping strategies persistently sad and hopeless for ••Write guilt and blame thoughts two weeks or more – and when down and try to come up with these feelings are combined with more balanced and rational a lack of interest or pleasure in thoughts. life, disrupted sleep and appetite, feeling worthless, and thinking Don’t dwell on these negative •• they would be better off dead, this thoughts. is not sadness, but depression. Think about what you would •• To some extent, sadness, low say to a close friend or loved mood and clinical depression one if they were having these sit along a continuum. At one thoughts and feelings – and end, sadness tends to be an apply this to yourself. appropriate response to a specific ••Make a conscious effort to be situation, which fluctuates and more compassionate towards gradually diminishes over time. In yourself. the middle, people can have ‘down days’ or ‘feel blue’. At the other ••Do some things which are end of the spectrum, depression comforting and soothing. (often called clinical depression) Sadness, low mood, is more severe and persistent, hopelessness and despair and it affects the whole of a person’s life and stops them from It is natural and normal for people functioning. to experience feelings of intense sadness at times when they are ill. Dwelling on negative thoughts Sometimes it can feel that things is one of the things that makes aren’t going well or that they will low mood much worse and keeps never be the same again. Some it going. Often the thoughts are

40 www.leukaemiacare.org.uk self-critical and hopeless in ••Connect with other people. nature; they are nearly always Consciously plan and schedule ‘black and white’ or catastrophic. •• activities, social time and It can be hard to ‘see’ or believe pleasurable things. anything positive about life, but all too easy to notice and believe ••Try to ‘capture’ negative negative things. As with the other thoughts and come up with emotions, writing down negative more helpful and balanced thoughts and trying to change thoughts. them can be helpful, as well as talking things through with ••Remember to ask yourself: others. This helps people to gain "Is this a fact or an opinion?"; some perspective and to feel less "What’s the evidence?"; "Is there hopeless. another way of seeing this?"; "What would I say to my best "Cancer is such a tough experience friend or partner if they thought and you can easily be filled with very this?" depressing or scary thoughts. Doing something positive is a very good Useful online resources: thing." ••www.getselfhelp.com The other thing that helps ••www.nhs.uk/Conditions/ depression is doing more and stress-anxiety-depression engaging with others. This helps to increase a sense of pleasure ••www.helpguide.org/articles/ and reward from life. depression/dealing-with- depression "The support of my family and local community kept me going and talking Please note: It is often helpful to with them helped me to offload." seek support if you are sad or low in mood, but it is essential to do Coping strategies so if you are depressed, especially ••Activate yourself as much as if you are having suicidal possible; do more, rather than thoughts. If you are feeling less. Take gentle exercise and depressed, talk to your GP and to don’t stay in bed or on the sofa. your healthcare team so that they

Helpline freephone 08088 010 444 41 can organise formal support for ••It can help to write down and you. Talking therapies can help, challenge thoughts like this. and if necessary. ••It is especially helpful if you can Grief talk about your feelings and about what, or who, has been Grief is what people experience lost. For a while, many people when they are mourning people, who are grieving need to be able things, or aspects of themselves to ‘tell their stories’ again and that they have lost. Grief includes again. most of the emotions described above, especially sadness, as Summary well as a kind of yearning for what has been lost (in other A diagnosis of MDS affects all words, you wish you could have of you, not just your body. It is it back). In the case of illness common to have a complex this might include previous roles mixture of thoughts and feelings. and responsibilities, previously Although this can be unpleasant, enjoyed activities, fertility, this is a normal response to certain freedoms and choices, a major life event. Emotional health and vitality. As grief is a distress of varying kinds can be normal response to loss, it cannot experienced at any point in your be rationalised away. Loss is ‘journey’ but it is important to something that people can learn remember there is support out to live with – and cope with – over there and ways in which you can time, especially as they adapt to help yourself. their changed circumstances and Talk to your specialist nurse and engage with other aspects of life. reach out to the MDS support Coping strategies groups that are widely available and can help. ••Although grief is a normal process, a preoccupation with certain types of negative thoughts and beliefs, e.g. "I cannot cope without X, my life isn’t worth living now", will make it worse and keep it going.

42 www.leukaemiacare.org.uk Living with MDS

Living with MDS – what a Centre of Excellence for MDS (further details are at the back of does this mean to me? this booklet). These are hospitals A diagnosis of MDS will have with expertise in this rare disease implications for your daily life. and the availability of new drugs Knowing in advance what to via clinical trials. You can ask your expect might alleviate some of the doctor if you would benefit from a stress or worries around this. referral to a Centre of Excellence. You can also ask for an additional Information about the disease opinion if you have questions and possible complications can about your treatment or disease help you recognise when to seek management. medical attention or ask for further support. It also can give The National Institute for Health you the feeling that you are more and Care Excellence (NICE) in control. guidance, published in 2003, promotes that all haemato- Hospital visits oncology patients should A diagnosis of MDS will inevitably have a designated clinical involve regular hospital visits. nurse specialist (CNS) who is The frequency of these can available to provide support and vary greatly depending on the information. If you have not been severity of the symptoms and introduced to a CNS, ask your the progression of the disease. doctor if there is one available in Patients with low risk disease the hospital where you are treated might only need monitoring every and request their contact details. few months or even yearly, while others might need weekly checks. Blood results Your doctor or nurse will give you Most patients will learn to keep guidance on how often visits and a record of their visits and blood blood tests will be needed. results. There are specially designed diaries or apps for your All patients diagnosed with MDS mobile phone that can help you have the right to be referred to

Helpline freephone 08088 010 444 43 Living with MDS (cont.)

record these results. This can be be neutropenic, and this can valuable information, especially make you more susceptible to if you are under the care of more infections. than one hospital or department. Knowing your own blood levels Infection and reducing can help you understand why the risk of infection you have certain symptoms and When you are neutropenic (have what to do in those situations. You low levels of ) and you can ask your nurse or doctor to feel unwell or feverish at home, it explain the results to you. is very important to immediately The most useful levels to know seek medical attention at your are: nearest hospital. If you are alone or too unwell to leave the house, ••Your Hb or haemoglobin call an ambulance so that you (contained in red blood cells) – can be brought to hospital. Hb carries oxygen around your Patients with the combination of body and is an indicator of an infection and can whether you are anaemic or not. deteriorate rapidly and should be ••Your platelet count (or started on intravenous antibiotics thrombocytes) – when these are as soon as possible. If you low you can be prone to bruising don’t have a thermometer, it is or bleeding. advisable to buy one so that you can measure your temperature ••Your count when you are at home. – these are cells that fight infections. Call your hospital team ••Your count – these if your temperature cells are a type of white blood goes above 38°C or cell. Neutrophils are the first you experience shivers line of defence when there is a bacterial infection. When these (rigors). cells are low, you are said to

44 www.leukaemiacare.org.uk Many patients with MDS will be are on treatment and especially more susceptible to infections, when your neutrophil level drops, because even if their white cells you may be asked to avoid are not low, they are ‘dysplastic’ certain foods. This varies from and might not work as well as one hospital to another. Ask your they should. Infections may doctor or nurse for instructions occur more frequently and also for your specific situation and get last longer than normal. Patients general advice from the patient receiving treatment for their support organisations in this MDS may be more vulnerable booklet. to infections especially if the When you are planning to travel, treatment causes a reduction in ask your doctor if you should white cell numbers. Sometimes take any antibiotics with you, they are started on drugs to especially if you have experienced prevent infections. picking up infections easily. This Good hand hygiene is the best may help to avoid any problems way to avoid catching bacterial while abroad. infections. There are lots of gels If your neutrophil count is low for sale but normal hand washing and you have regular infections, with water and soap is just as you may benefit from injections effective. In particular, make sure that can boost your neutrophil that you wash your hands after levels. G-CSF (granulocyte-colony using the toilet, when preparing stimulating factor) is a drug food, before you eat, and after that can be given by injection gardening or touching animals. under the skin. It is a hormone Try to avoid people who are unwell that is normally produced in the and ask your friends and family body and stimulates the growth not to visit when they have cold or of white blood cells in the bone flu symptoms. marrow. The injections can cause pain in the bones and muscles Normal food hygiene rules apply which can usually be relieved when you have MDS. When you by taking a mild painkiller, like

Helpline freephone 08088 010 444 45 Living with MDS (cont.)

paracetamol. You can be taught to your diet. give yourself these injections, or There is no specific guidance with a family member, GP or nurse can regards to exercise and MDS. As do it instead. long as your energy levels allow, Nutrition and exercise you can be as active as you like to be. If your platelets are low, Eating well and maintaining a however, you will need to avoid healthy weight will give you more contact sports and be careful strength and energy. A healthy with falls or bumps as these may diet includes: cause serious bleeding.

••Lots of fruit and vegetables In general the more active you ••A good proportion of remain before, during and after carbohydrates (bread, rice, treatment, the easier you will pasta, potatoes) recover after having treatment. Some hospitals will provide ••Some rich foods (meat, exercise programs for patients poultry, fish, nuts, eggs, pulses) during and after treatment. Ask ••Some dairy products your nurse or doctor if you want more information about this. ••Small proportion of foods that are high in salt, fat or sugar Fatigue ••Plenty of water Fatigue is one of the most widely reported side effects of You are unlikely to benefit from low haemoglobin levels. Fatigue an increase in iron rich foods as or lack of energy can seriously most patients with MDS will have impact on your quality of life. A normal or high levels of blood transfusion can improve (a protein that stores iron) levels. fatigue temporarily – for some the There are many theories about improvement lasts a few weeks, specific diets and cancer. Always and for others it may last longer. discuss with your nurse or doctor Treatment for MDS can initially before making any changes to make your fatigue worse but if the

46 www.leukaemiacare.org.uk treatment is effective and your ••Sit down when doing certain blood levels recover, fatigue levels jobs, like ironing or cooking. can improve over time. ••Reserve your energy for what When you feel fatigued you may you find important and build find it hard to concentrate or rest periods around those times. make decisions. The worries of Avoid stimulants before going to having MDS and dealing with •• bed such as alcohol, coffee, tea treatment can also add to the or chocolate, or using laptops, feeling of being tired all the time. tablets or mobile phones. Adjusting your lifestyle to your Keep your bedroom quiet and at energy levels can be a difficult •• a comfortable temperature. process. Some general tips on how to deal with fatigue include: ••Ask for help from family and friends. ••Have a regular sleep routine – try going to bed and waking up ••Talk about your worries with approximately the same time family, friends or your doctor every day and try to avoid lying or nurse, or patient support in. groups. ••Take part in regular, gentle ••Discuss your fatigue with your exercise to maintain your doctor or nurse. fitness levels as much as possible. Patient support organisations have booklets, factsheets and ••Use ready-made meals if videos on managing fatigue. You cooking is too tiring, so you can find contact details at the keep your energy just for eating. back of this booklet. Macmillan Cancer Support has also produced ••Wear clothes that are easy to a booklet about fatigue. You can put on and take off. download this at be.macmillan. ••Sit down on a plastic chair to org.uk shower, or wash your hair.

Helpline freephone 08088 010 444 47 Living with MDS (cont.)

Sexuality and fertility talk about it. Alternatively, you may want to talk to your GP or For some people, sex is an nurse who may be able to put you important part of life; for others in touch with a sexuality expert or it might be less important. A counsellor. diagnosis of MDS can impact your sex life in several ways. Certain treatments for MDS can For example, you may feel more have consequences for your fatigued and want to reserve your fertility, and during some of the energy for other activities; the treatments you will be asked treatment sometimes requires to take measures to prevent a you to be admitted to hospital pregnancy. Lenalidomide is a and as a consequence separates drug that is NICE approved for a you from your partner; you may specific group of MDS patients. It feel less attractive due to changes can cause birth defects and you to your body, such as hair loss, will be asked to use contraception skin changes or reduction in during and after this treatment. weight; or your worries may make The same applies for patients that you feel less interested in sex. are treated with chemotherapy Treatment with chemotherapy drugs. Most patients will be can also make sex more difficult permanently infertile after an as it can cause changes in the allogeneic transplant. mucous membranes resulting in Men have the option to freeze dryness and painful intercourse their sperm before starting their for women and erectile problems treatment. If treatment does for men. not need to be started urgently, If you are in a sexual relationship women also have the option of it may help to talk to your partner freezing their eggs. The medical about the changes you are team should discuss these experiencing and how you are options with you when treatment feeling. Your partner may struggle is required. Do not hesitate to with the subject as well and wait raise these issues yourself if you for a sign that you are ready to feel this is important to you.

48 www.leukaemiacare.org.uk Changes in sex life and possible with cancer. They can also give infertility may cause a range of you personal advice over the difficult feelings. Do speak with phone via their helpline and you someone close to you or your can discuss which benefits you doctor, nurse or counsellor if this are eligible for. Some Macmillan is negatively affecting you, and centres can arrange face to face ask for professional support if you meetings with a benefits advisor. feel you need this. Please visit the Macmillan website for more information. They can Work and finances also provide financial assistance MDS and its treatment can in the form of grants – ask your sometimes lead to difficulties nurse in the hospital how to apply relating to your work life. (see contact details for Macmillan Sometimes it leads to temporary at end of this booklet). sick leave or a reduction in As MDS is regarded as a cancer working hours, but it can also diagnosis you will also be mean that you have to stop work entitled to apply for a medical altogether. This will most likely exemption certificate which have financial consequences for means that you are entitled to you and your family. free NHS prescriptions. Your GP or Your consultant can guide you specialist nurse at the hospital in these decisions and either the can provide you with details hospital or your GP can arrange of how to apply for this. Most letters to confirm your situation hospitals will also have special to your employer. It is often worth arrangements for patients on taking time to explain MDS to benefits to claim their travel your employer, as it is likely expenses back. they will never have heard of the disease. Extra support at home You may find that the MDS or the Macmillan Cancer Support consequences of the treatment has published a booklet about impacts on your independence financial support when diagnosed

Helpline freephone 08088 010 444 49 Living with MDS (cont.)

at home. Most patients will have period of time, in hospital but family or friends who can step in also in the community. Some when certain tasks or activities medical treatments can be need to be taken over, but not fairly aggressive and treatments everyone will have this kind of provided by your palliative care support at home. team can help you tolerate the side effects of these treatments. Speak to your GP if you need additional help at home. They can Not all treatments, sadly, are arrange a social worker to assess successful and sometimes your situation and the support patients have to be told that that needs to be arranged. the disease is too progressive When you are in hospital, an for any treatment to control occupational therapist can assess it. Discussions around end of your abilities and discuss the life will then be initiated. That support and other resources that conversation will most likely you need at home. be started by your medical team. Most hospitals will have Palliative care palliative care teams that have Some treatments for MDS experience in dealing with end of can cause symptoms that life and related symptom control. require more than the normal Alternatively, you can be referred interventions. The palliative care to a community palliative care team can provide additional team connected with your local advice and support when hospice. Additional support can symptoms are not easily be organised either at home, at controlled. Symptoms such the hospital or at a hospice in as nausea and vomiting, accordance with the wishes of the breathlessness and pain are patient and carer(s). all examples of symptoms regularly dealt with by this Impact of MDS on specialist team. Their input can partners or carers be temporary or for a longer As with all cancer diagnoses,

50 www.leukaemiacare.org.uk MDS may also have an impact recognise the important role on other members of the family. a carer has in looking after As MDS is often diagnosed in the someone with MDS. Carers can elderly, there may be other health also attend support groups in issues in the family that require confidence and ask any questions attention. that may help them better understand the needs and care An initial diagnosis will inevitably of the person with MDS. Support cause feelings of concern and groups can also help with advice anxiety and may cause disruption when the need for information to the normal life of partners differs between carer and patient. and carers as well. All emotions mentioned earlier in this booklet If the responsibility of caring for and information about the someone gets too much, respite psychological impact of MDS may can be arranged via carers very well also apply to the rest of associations. Details of these the family or close friends. groups can be found at the back of this booklet. It sometimes happens that the information needs of the patient and the partner differ, where one The Caring for Carers wants to know more than the booklet is a great other. This can lead to feelings of frustration, misunderstanding resource to help carers or anger. Coping mechanisms deal with some of the may differ too and cause similar emotions they may feelings. face. Available through The support of the partner or Leukaemia Care. carers is very important. Carers who wish to find out more on the disease can contact the organisations involved in this booklet, or Macmillan, who all

Helpline freephone 08088 010 444 51 MDS specialist centres and other useful organisations

There are a number of UK centres ••Confirmation that your case was with a specialist interest in discussed at a regional MDT treating patients with MDS, (multidisciplinary) meeting some of which are recognised as A discussion covering a Centres of Excellence. •• monitoring/treatment plan You can request to be referred to A mention of the availability of these specialist centres for an •• clinical trials additional opinion. The referral request can be made via your ••Regular reviews with potential GP or your local haematologist. further biopsy if circumstances These referrals are particularly change helpful for the more complex MDS cases, or if you feel that the care ••Details of a CNS (Clinical Nurse or information you are receiving is Specialist) not sufficient for your needs. ••Information materials and Please note that additional details of support groups opinions are sought by many If you do seek an additional patients across the UK. These are opinion at a specialist centre, perfectly reasonable requests, you will still be treated at given that MDS is a rare and your local hospital. Your local complex disease. Although all haematologist will work together haematologists will be familiar with the specialist to provide you with the condition, not all of them with optimal care. This is called will have the specific expertise "shared care". found in specialist centres. A specialist centre will also In general, you should expect your provide you with a dedicated haematologist to provide you with Clinical Nurse Specialist, whom the following: you will be able to consult ••Your sub-type of MDS between appointments if necessary. ••Your IPSS or IPSS-R score The specialist centres include:

52 www.leukaemiacare.org.uk England: King’s College Hospital, London Professor Ghulam Mufti, Dr Austin Kulasekararaj (international expertise) St James’s Institute of Oncology, St. James’s Professor David Bowen (international University Hospital, Leeds expertise) Royal Bournemouth Hospital, Bournemouth Dr Sally Killick Queen Elizabeth Hospital, Birmingham Professor Charles Craddock, Dr Manoj Raghavan Brighton and Sussex University Hospital, Dr Tim Chevassut Brighton MRC Laboratory, Addenbrookes NHS Trust, Professor Alan Warren Cambridge Radcliffe Hospitals and University of Oxford Professor Paresh Vyas The Christie, Manchester Dr Dan Wiseman Northern Centre for Cancer Care, Newcastle Dr Gail Jones Nottingham University Hospital Dr Rohini Radia Great Western Hospital, Swindon Dr Alex Sternberg Royal Cornwall Hospital, Truro Dr Bryson Pottinger Worcestershire Acute Hospitals NHS Trust Dr Juliet Mills and Birmingham NHS Foundation Trust St Bartholomew’s Hospital, London Professor Jamie Cavenagh Northampton General Hospital, Northampton Dr Jane Parker University Hospital Southampton, Dr Chris Dalley Southampton Castle Hill Hospital, Cottingham, Hull Dr Simone Green Scotland: Aberdeen Royal Infirmary, Aberdeen Dr Dominic Culligan Beatson West of Scotland Cancer Centre, Dr Mark Drummond Glasgow Wales: University Hospital of Wales, Cardiff Dr Jonathan Kell, Dr Wendy Ingram Ireland: Tallaght Hospital, Dublin Dr Helen Enright

Helpline freephone 08088 010 444 53 MDS specialist centres and other useful organisations (cont.)

UK patient MPD Voice organisations and Provides support specifically for support groups those with myeloproliferative disorders MDS UK (see author information at the beginning of 07934 689 354 (Monday - Friday this booklet) between 8am & 5pm) 020 7733 7558 (Helpline Mon-Fri www.mpdvoice.org.uk 9am - 6pm) [email protected] www.mdspatientsupport.org.uk (website and forum) Macmillan Cancer Support Provides a helpline, information [email protected] on all types of cancer, financial Leukaemia Care (see author advice and travel insurance information at the beginning of recommendations. this booklet) 0808 808 00 00 08088 010 444 (Freephone www.macmillan.org.uk helpline open weekdays 9:00am - 10:00pm, Saturdays 9:00am - Anthony Nolan 12:30pm) Provides information specifically www.leukaemiacare.org.uk to patients, families and stem cell donors regarding stem cell [email protected] transplantation and donation.

Bloodwise (see author 0303 303 0303 information at the beginning of this booklet) www.anthonynolan.org 0808 2080 888 [email protected] www.bloodwise.org.uk Maggie’s Centres [email protected] Maggie’s offers free practical,

54 www.leukaemiacare.org.uk emotional and social support anaemia, myelodysplastic to people with cancer and their syndromes (MDS), paroxysmal families. nocturnal haemoglobinuria (PNH), and related bone marrow failure www.maggiescentres.org diseases.

International patient www.aamds.org organisations and [email protected] support groups MDS Support Group Ireland (& Other MDS resources Northern Ireland) MDS-Europe 086 200 1402 The website for the MDS-RIGHT programme in Europe now [email protected] hosting MDS blogs, international MDS Foundation patient resources (including UK) and interactive MDS guidelines International information and for diagnosis and management support group based in the USA. Provides further information on www.mds-europe.org MDS – and contact with American MDS patients via a chat forum. Financial assistance www.mds-foundation.org and benefits advice Macmillan Cancer Support patientliaison@mds-foundation. org 0808 808 00 00 Aplastic Anaemia and MDS Your local Citizens Advice International Foundation Bureau International organisation, based www.adviceguide.org.uk in the USA, supporting patients Turn2us and families living with aplastic 0808 802 2000

Helpline freephone 08088 010 444 55 MDS specialist centres and other useful organisations (cont.)

www.turn2us.org.uk www.carers.org Travel insurance [email protected] Whilst it can often be difficult to get travel insurance if you have a pre-existing medical condition such as MDS, you can still go on holiday.

Fact sheets and information about travel insurance and recommended insurance providers are available from MDS UK and Leukaemia Care. For carers Carers UK 0808 808 7777

www.carersuk.org

[email protected] Carers Trust 0844 800 4361

56 www.leukaemiacare.org.uk Glossary

Acute Myeloid Leukaemia (AML) blood or one of its components is given to a person through AML is a cancer of the blood and an intravenous (IV) line into bone marrow. It is characterised the bloodstream. A red blood by an increase in the number of cell transfusion or a platelet myeloid cells in the marrow that transfusion can help some do not mature and interfere with patients with low blood counts. the production of healthy blood cells. Bone marrow Allogeneic stem cell transplant The soft blood-forming tissue that fills the cavities of bones A procedure where bone marrow and contains fat, immature and stem cells are taken from a mature blood cells, including genetically matched donor and white blood cells, red blood cells, given to the patient through an and platelets. intravenous (IV) line. The donor may be related or unrelated. Chemotherapy Anaemia Therapy for cancer using chemicals that stop the growth of A medical condition in which cells. the count or haemoglobin is less than normal. Clinical trial On a normal blood result, RBC will A medical research study show as approx: Red Blood Count involving patients with the aim of (RBC) Males: 4.5-6.5; Females 3.8- improving treatments and their 5.8 - 1012/L side effects. You will always be Blast cell informed if your treatment is part of a trial. An abnormal (dysplastic), immature blood cell found in the Cytogenetics bone marrow or peripheral blood. The study of chromosomes As they are not mature, these cells (DNA), the part of the cell that are unable to fulfil their intended contains genetic information. function. AML develops from these Some cytogenetic abnormalities blast cells. are linked to different forms Blood transfusion of myelodysplastic syndromes (MDS). A procedure in which whole

Helpline freephone 08088 010 444 57 Glossary (cont.)

Fatigue helps fight infection. Extreme tiredness, which is Platelet not alleviated by sleep or rest. A disc-shaped element in the Fatigue can be acute and come on blood that assists in blood suddenly or it can be chronic and clotting. During normal blood persistent. clotting, the platelets clump Full blood count or FBC together (aggregate). Although platelets are often classed as A blood test that counts the blood cells, they are actually number of different blood cells. fragments of large bone marrow Genomics cells (). The study of genes and their Platelet count functions, which is increasingly A normal platelet count in a important to refine the prognosis healthy individual is between of the various sub-types of MDS. 150,000 and 450,000 per In future this information may microlitre of blood. In general, low also help to personalise MDS platelet counts increase bleeding treatments. risks. Normal platelet Count (PLT) Haemoglobin 150-450 - 109/L A protein in the red blood cells. Stem cells Haemoglobin picks up oxygen in Cells that have the potential to the and brings it to cells develop into many different or in all parts of the body. Normal specialised cell types. counts are approx: Haemoglobin (HB) Males 130-180; Females 115- White blood cell 165 g/L One of the cells the body makes Neutropenia to help fight infections. There are several types of white blood cells. A condition in which the number The two most common types are of neutrophils (a type of white the and neutrophils. blood cell) in the bloodstream is Normal White Cell Count (WBC) decreased. 4-11 - 109/L Neutrophil A type of white blood cell that

58 www.leukaemiacare.org.uk Appendix

International has five categories which allow a more accurate idea of expected Prognostic Scoring outcome. System for MDS Both scoring systems should The International Prognostic only be used at diagnosis and not Scoring System (IPSS) was during the course of the disease. developed by analysing information on almost 1,000 MDS IPSS-R Cytogenetic patients, who mostly received only Prognostic Subgroups supportive care, and determining which factors best predicted The cytogenetic result from the disease progression and outcome. patient’s bone marrow sample is This was then used to create an important part of the IPSS-R a scoring system based on score. The table below shows percentage of blasts in the bone cytogenetic risk categories: marrow, cytogenetics and the number of cell types affected in Cytogenetic Cytogenetic the circulating blood. The IPSS is category abnormalities now only used to decide whether Very good -Y, del(11q) patients are suitable for certain treatments that were developed Good Normal, in the IPSS era (1995-2012). Since del(5q), 2012, all discussions with patients del(12p), about prognosis should be based del(20q), on the IPSS-R: double including The Revised IPSS del(5q) (IPSS-R) Intermediate del(7q), +8, +19, i(17q), any This scoring system has used other single the data gathered from patients or double in the IPSS to further categorise independent patients into more defined risk clones groups from over 7,000 untreated patients with MDS. The score now

Helpline freephone 08088 010 444 59 Appendix (cont.)

Poor -7, inv(3)/ IPSS-R Prognostic t(3q), double Score Values (below) including To calculate a person’s prognostic -7/del(7q), score, we use the variables in the complex: 3 left hand column. Each variable abnormalities has a number or ‘score’ between Very Poor Complex: >3 0 and 4. The numbers are then abnormalities added together to give your final IPSS-R score.

Prognostic 0 0.5 1 1.5 2 3 4 variable

Cytogenetics Very Good Good Intermediate Poor Very Poor

Bone marrow ≤2 >2-<5 5-10 >10 blast %

Haemoglobin ≥100 80-<100 <80 concentration (g/l)

Platelet count ≥100 50-<100 <50 (x 109/l)

Neutrophil ≥0.8 <0.8 count (x 109/l)

60 www.leukaemiacare.org.uk IPSS-R Prognostic Risk Categories/Scores and Clinical Outcomes The table below shows what the predicted clinical outcomes for people with MDS in different risk groups are.

Risk Risk Survival (median 25% AML catergory score – years**) evolution* (median – years**)

Very Low ≤1.5 8.8 NR Low >1.5-3 5.3 10.8 Intermediate >3-4.5 3.0 3.2 High >4.5-6 1.6 1.4 Very High >6 0.8 0.73

* This column (25% AML evolution) is the average length of time that it would take for one quarter of patients to develop AML in that IPSS-R group.

** median = The midpoint of data after being ranked from lowest to highest. It shows that for the 25% of patients who do progress to AML, half of them would progress before this time period and half of them after it.

Helpline freephone 08088 010 444 61 Appendix (cont.)

Definitions used in the The individual scores for bone marrow blast percentage, IPSS karyotype and cytopenias are Karyotype (cytogenetic added together to give the IPSS abnormalities) score. The scores for the risk ••Good – normal, deletion of Y groups are as follows: chromosome, del(5q), del(20q) Low risk 0 Poor – complex (more than 3 •• INT-1 risk 0.5-1.0 abnormalities), abnormalities INT-2 risk 1.5-2.0 ••Intermediate – all other High risk >2.5 abnormalities Cytopenias (low blood counts) ••Haemoglobin – less than 100g/l (or 10g/dl ) ••Neutrophils – less than 1.8 x 109/l ••Platelets – less than 100 x 109/l IPSS score table

Score 0 0.5 1 1.5 2

% of blasts <5 5-10 11-20 21-30 in the bone marrow

Karyotype Good Intermediate Poor

Number of 0/1 2/3 cytopenias

62 www.leukaemiacare.org.uk World Health ••MDS associated with del(5q), Organization (WHO) including the 5q- syndrome 2008 Classification (for updated 2016 version, see section 5) ••Refractory cytopenia with unilineage dysplasia (RCUD) ••Refractory anaemia with ring sideroblasts (RARS) ••Refractory cytopenia with multilineage dysplasia (RCMD) ••Refractory anaemia with excess blasts (RAEB) ••Myelodysplastic syndrome unclassified (MDS-U)

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Helpline freephone 08088 010 444 63 Leukaemia Care is a national charity dedicated to providing information, advice and support to anyone affected by a blood cancer.

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