STEP BY STEP - Leukaemia CARE

STEP BY STEP Chronic Lymphocytic Leukaemia (CLL)

Introduction Being diagnosed with chronic lymphocytic leukaemia (CLL) can be a shock, particularly when you may never have heard of it. If you have questions about CLL – what causes it, who it affects, how it affects your body, what symptoms to expect and likely treatments – this booklet covers the basics for you. But for more information, talk to your haematologist, clinical nurse specialist (CNS) or hospital pharmacist. You’ll also find useful advice about how to get the best from your haematologist, plus practical advice on how to help important people in your life understand such a rare condition. Booklet compiled by Ken Campbell, MSc (Clinical Oncology) and peer reviewed by Deborah Stalkartt, Cancer Information Nurse at Cancer Research UK. We are also grateful to Gary Hunter, CLL patient reviewer, for his valuable contribution. Throughout this booklet you will see a number of quotations. These are the real experiences and words of CLL patients so will hopefully help you to understand your disease and situation a bit better.

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Next planned review: September 2017 Version: 1

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Introduction In this booklet 1. What is CLL?

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2. Symptoms and Diagnosis

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3. Treating CLL 4. Complications of CLL

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5. Living with CLL

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6. Talking about CLL

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7. Useful contacts and further support

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8. Symptom tracker

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9. Glossary

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10. Notes

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11. About Leukaemia CARE

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Useful personal information and contact details This is a place for you to note down important information relating to your diagnosis and key contacts Date of diagnosis My diagnosis Name

Contact details

My hospital Specialist nurse (CNS)

GP

Haematology clinic

Haematology ward Emergency contact number / Out of hours contacts

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Date

White cell count

Red cell count

Haemoglobin (Hb)

Platelets

Lymphocytes

Neutrophils

Record Sheet

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What is CLL? Chronic lymphocytic leukaemia (CLL) is a blood cancer that affects the lymphocytic (antibody-producing) white blood cells that are produced by the bone marrow. In CLL there is an excess number of lymphocytes in the circulating blood. These lymphocytes are abnormal and cannot help the body to defend against infections. They are sometimes called ‘smudge cells’ because the cells often break up easily and appear under the microscope as smudges. To understand CLL it is helpful to understand how blood cells are normally produced. Blood cells are formed in the bone marrow, which is spongy tissue found inside bones. Blood-forming stem cells divide to produce either more stem cells or immature cells that become mature blood cells over time. Only about one in 5,000 or fewer of the cells in the bone marrow is a stem cell. Each day, the bone marrow produces more than a trillion new blood cells, to replace those which are worn out. A blood stem cell, also called haematopoietic stem cells, may become a myeloid stem cell or a lymphoid stem cell. A myeloid stem cell becomes one of three types of mature blood cells:

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ed blood cells that carry oxygen and other substances to all R tissues of the body

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Platelets that form blood clots to stop bleeding

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hite blood cells (granulocytes) that fight infection and W disease. The shortest lived white cells are called neutrophils

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A lymphoid stem cell becomes a lymphoblast cell and then one of three types of lymphocytes (white blood cells):

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B lymphocytes that make antibodies to help fight infection

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lymphocytes that help B lymphocytes make the antibodies T that help fight infection

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Natural killer cells that attack cancer cells and viruses

It is the B lymphocytes that are affected in CLL. In CLL, the bone marrow is not able to make enough normal blood cells. CLL usually progresses slowly, meaning some patients may not need treatment as soon as they are diagnosed. In some cases the disease progresses more rapidly and may need early, and possibly more intensive, treatment. CLL is very similar to a type of lymphoma called small lymphocytic lymphoma (SLL). Although it is not always clear whether the diagnosis is CLL or SLL, fortunately this is not critical because

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both conditions respond to the same form of treatment. Most experts consider CLL and SLL to be two slightly different forms of the same disease.

How common is CLL? CLL is the most common form of leukaemia in adults in the Western world, with 3,200 people diagnosed with the disease in the UK each year. More men than women are diagnosed with CLL each year and it tends to affect older people, with the average age at diagnosis being around 70 years old. About one in ten cases of CLL is seen in people aged under 55 years old and CLL is rarely, if ever, seen in children. CLL is uncommon in Asian people, even when they live in the West or have a Western lifestyle – this suggests that the difference is genetic.

What causes CLL? In most cases, there is no obvious cause for CLL. There are certain risk factors which are known to be linked to a higher chance of developing CLL. These are:

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ge – it is more common in older people A Gender – men are about twice as likely as women to develop CLL Ethnicity – CLL is more commonly seen in white people (Caucasians) than in any other ethnic group Family history – CLL is slightly more common in close relatives of people who have CLL (or similar conditions). The increased risk is small, so screening is only recommended if someone is being tested as a possible donor for a stem cell transplant, which is rarely done for CLL Monoclonal B-cell lymphocytosis (MBL) – some people have been found to have CLL-like cells in their blood but no symptoms. It is now thought that many, possibly most or all, people who develop CLL, previously had MBL

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Symptoms and diagnosis Before we discuss the symptoms of CLL, it’s important to understand how CLL affects the body, compared to someone who doesn’t have CLL. In someone without CLL, bone marrow (the soft, fatty tissue inside your bones) contains blood stem cells that in time develop into mature blood cells – red blood cells (to carry oxygen to the tissues of your body); white blood cells (to fight infection and disease); or platelets (to help prevent bleeding by causing blood clots to form). Production of new blood cells is very closely controlled to balance the loss of worn-out cells or cells lost by bleeding or damage. Bloodforming stem cells divide to produce more stem cells or to develop into working blood cells. The healthy number of different types of blood cells varies between people but is usually kept within fairly narrow limits. The white blood count may temporarily rise after exercise, but changes like this usually do not last very long. In someone with CLL, abnormal cells take over the bone marrow meaning the bone marrow is not able to make enough normal blood cells. This can result in an enlargement of the spleen as the spleen tries to compensate for the reduction in normal blood cells. In patients with CLL, the blood usually contains high numbers of large lymphocytes – these often break up when a blood film is prepared to examine under the microscope. When this happens they are referred to as ‘smudge cells’. Smudge cells can occasionally be seen in other circumstances but, if there are many of them, this strongly suggests CLL. Due to the inability of the bone marrow to make enough healthy blood cells, CLL patients often have lower than normal numbers of red blood cells (anaemia), white blood cells (neutropenia) and/

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or platelets (thrombocytopenia). When all types of blood cells are lower than normal this is called pancytopenia. These changes lead to some of the symptoms of CLL; you can read more about symptoms further on in the booklet. Sometimes CLL can present with signs, symptoms and blood results similar to other B-cell cancers. When this happens, there is a scoring system which looks at the results of special laboratory tests and can allow doctors to make a final diagnosis. One very important test looks for a protein known as CD5 on the abnormal cells. More than nine out ten people with CLL have this protein on their cells so if it is not found, the diagnosis may not be CLL. A condition called small lymphocytic lymphoma (SLL) is thought to be virtually identical to CLL, except for where in the body the abnormal cells are mainly found. SLL and CLL are treated in the same way, so the difference is not critical.

What are the most common symptoms of CLL? More than half of all patients with CLL have no symptoms at the time when they are diagnosed and many patients are diagnosed following a routine blood test for something else. Some symptoms you may have before you’re diagnosed, others you may experience after diagnosis. It’s also important to know that not everyone will experience the same symptoms. Symptoms which may be seen include:

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reathlessness B Fatigue Paleness Bruising and bleeding (this is uncommon at an early stage) Infections – these may be more frequent, persistent and/or more severe Enlarged lymph nodes

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The below are called ‘systemic symptoms’ and are uncommon at diagnosis. They are also known as ‘B symptoms’:

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evere or prolonged fever (high temperature) S Unexplained weight loss Drenching night sweats

Diagnosis of CLL If CLL is suspected, you’ll have a set of tests to confirm the diagnosis. If you’re diagnosed with CLL, you will also have further tests to ‘stage’ your cancer. Tests to confirm the diagnosis of CLL include: 1 . Full Blood Count (FBC) – this measures the number of red cells, white cells and platelets in the blood. 2. Immunophenotyping – An FBC alone will not confirm a diagnosis of CLL and more specialist tests will be required. Immunophenotyping is a technique which shows if you have abnormal CLL cells in your blood and how many. 3. Lymph node biopsy – You may need a lymph node biopsy if your lymph nodes are swollen. A lymph node biopsy is a minor surgical procedure where a small sample is taken from a lymph node then studied under a microscope.

T he thoughts that rushed through my mind when I was diagnosed were bizarre. I wondered how long I’d have left to live, how I’d tell my family and if I’d get through this. But I did get through it and remaining positive was the key. 11 11

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Staging of CLL There are two main systems used to stage CLL. The Rai system is most common in America, whilst in Europe, including the UK, the Binet system is most widely used. The purpose of staging is to assess how advanced the disease is and can be used to help decide when treatment should start and what treatment to recommend.

Binet staging system Stage A • No anaemia and a normal platelet count and • Fewer than three areas of lymphoid involvement Stage B • No anaemia and a normal platelet count and • Three or more areas of lymphoid involvement Stage C Anaemia and/or low platelet count Regardless of the number of areas of lymphoid involvement

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The lymphoid areas are the neck, the armpits, the groin, the spleen and the liver. The neck, armpits and groin each count as one whether one or both sides are affected.

Rai staging system Stage 0 • Absolute lymphocytosis • No enlarged lymph nodes, spleen or liver • No anaemia, or low platelets

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Stage I • Absolute lymphocytosis • Enlarged lymph nodes • No enlarged spleen or liver, anaemia, or low platelets Stage II Absolute lymphocytosis Enlarged liver or enlarged spleen With or without enlarged lymph nodes Stage III • Absolute lymphocytosis and anaemia • With or without enlarged lymph nodes, spleen or liver Stage IV • Absolute lymphocytosis and low platelet count • With or without enlarged lymph nodes, spleen or liver or anaemia

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Absolute lymphocytosis means a lymphocyte count higher than 15,000 per cubic millimetre of blood.

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Treating CLL It is generally accepted that CLL is not curable with current treatments, but it is very treatable and it is usually possible to control the disease. Many patients will have a normal life-span with a good quality of life after diagnosis.

F eeling numb, I spent the next three days trawling the Internet and finding lots of out-of-date information that I assumed was current. It was scary and I went to my next appointment expecting a slim chance of survival. But in reality, what I was told by my consultant was much more positive and I walked out with hope. Not all patients with CLL start treatment after diagnosis, and some may never need treatment. The decision when, and if, to start treatment will be based on the results of your blood test results and possibly of bone marrow samples or scans, and on whether you have troublesome symptoms such as an enlarged spleen. It will also depend on your health, your individual condition and your wishes. You can refuse treatment at any time, but it is important that you clearly understand what might happen in this case. However, you can’t start treatment if your haematologist doesn’t think you need to. If you do not agree with them, you can ask for a second opinion at any time. As far as possible, all decisions about treatment will take your wishes into account.

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If you do not need to start treatment straightaway, you will be placed on something called ‘watch and wait’ or active monitoring. This means that you will have regular check-ups, blood tests

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and monitoring of your disease. It is important you attend these appointments as your consultant will be able to track your condition, talk about how you’re feeling and decide on if or when treatment may be needed. Some patients who have Binet stage A CLL will never need treatment. We have a booklet, Step-by-Step on watch and wait available in hard copy or download format. Visit www.leukaemiacare.org.uk/ resources or call 08088 010 444 for your copy.

Treatments for CLL If your haematologist is considering starting treatment, you will probably have further tests. This may include bone marrow tests, further scans and special tests on the abnormal CLL cells. Some of the investigations are for factors which influence how likely it is that your CLL will progress and how likely it is that certain treatments will (or will not) work for you. You may need to start treatment if:

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The stage of your CLL is advancing e.g. from Binet A to Binet B

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ou are Binet stage C, whether this is at diagnosis or at a later Y stage

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ou have autoimmune anaemia or low platelets which is getting Y worse

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he lymphocyte count has doubled in less than one year OR T your anaemia or platelet count has decreased

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You have increasing enlargement of lymph nodes or spleen

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You have developed B symptoms

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There are several tests which are known as prognostic tests or prognostic markers. These are not available at all hospitals but when they are available, they may be taken into account in deciding when to recommend starting treatment. These types of tests include:

IgHV mutation status

IgHV genes may be mutated or unmutated. When the genes are mutated, the disease usually progresses more slowly.

Zeta-associated protein (ZAP-70) and CD38 antigen status ZAP-70 is a protein found inside CLL cells and CD38 is found on the outside of CLL cells. In each case, high levels mean that the disease is likely to progress more quickly.

β2-Microglobulin (β2-M)

β2-Microglobulin levels give an indication of how many CLL cells there are – the higher the β2-M, the more likely it is that treatment will be recommended.

Cytogenetics

Cancer involves changes in the genes of the affected cells (this is different to inheriting genes from a family member). The study of these changes is called cytogenetics and investigates the genetic differences between CLL cells and normal cells. There are a number of different variations which may be seen but the most important is called the TP53 deletion, which means you’ll be less likely to respond to a drug called fludarabine, which is often used to treat CLL. It is important to understand that these prognostic markers indicate how CLL behaves on average. Your haematologists will take into account all the information about your case and will advise you on that basis. Not all hospitals carry out all of these tests and, if you do have results of any of these and you are worried, you should talk to your haematologist.

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Initial treatment The first treatment you have is called initial, or first-line, treatment. Options for initial treatment include:

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Taking part in a clinical trial if there is one available

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Fludarabine, cyclophosphamide and rituximab (FCR)

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Bendamustine and rituximab

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hlorambucil with anti-CD20 antibody (e.g. rituximab, C ofatumumab or obinotumumab)

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Alemtuzumab with or without high dose steroids

For less fit patients or for patients with poor kidney function, bendamustine or chlorambucil may be used alone. Some patients may be given the drug alemtuzumab, sometimes with highdoses of steroids. Alemtuzumab is a monoclonal antibody, like rituximab, ofatumumab and obinotumumab, but it works against a different protein, called CD52.

Treatment options for CLL The standard of care for most patients who require treatment for CLL is called chemo-immunotherapy. This is a combination of chemotherapy and immunotherapy, which are described below.

Chemotherapy

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. It has a very high success rate in the treatment of CLL. It does not cure the disease but it gives good control for most patients. Chemotherapy will also damage some normal cells, which means that there are side effects. The main chemotherapy drug used in treatment of CLL is called fludarabine. Fludarabine is usually given together with another drug called an alkylating agent. The alkylating agents most commonly used are cyclophosphamide, bendamustine or chlorambucil. Fludarabine is a very effective drug but it is usually reserved for patients with

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good kidney function. If the CLL cells have a particular abnormality called TP53 deletion, most forms of chemotherapy will not work very well, or at all. Treatment of CLL with TP53 deletion is described further in the booklet.

Fludarabine

Fludarabine is a type of drug called a purine analogue. Purine analogues affect your body’s immune system and may reduce your blood counts by affecting the bone marrow’s production of normal blood cells. It is recommended that any patient who has been treated with purine analogues and who later needs a blood transfusion should be given irradiated blood. This is to reduce the risk of a condition called ‘transfusion-related graft versus host disease’. This can happen when a patient’s immune system is not working properly and healthy immune cells in the donor’s blood damage the patient’s tissues. Irradiated blood is not radioactive and is just as safe and effective as any other blood transfusion. While you are being treated with fludarabine, you will be carefully watched for any sign of infection. You may be given drugs to prevent some virus infections if your lymphocyte count is very low. If this applies to you then you will be given detailed information. Your haematologist or clinical nurse specialist will explain any special precautions you may need to take and will answer all your questions. Fludarabine may cause nausea and/or vomiting but this can usually be controlled by taking drugs called anti-emetics at the same time.

Alkylating agents

Alkylating agents include bendamustine, cyclophosphamide or chlorambucil. They are a group of anti-cancer drugs which damage DNA and kill CLL cells and are usually used with fludarabine. For some patients, who are less fit or who have poor kidney function, alkylating agents may be given alone.

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Immunotherapy

Immunotherapy uses monoclonal antibodies to attack and destroy CLL cells. Monoclonal antibodies are drugs that recognise, target and stick to particular proteins on the surface of cancer

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cells. They can stimulate the body’s immune system to destroy these cells. The most common target for immunotherapy is a protein called CD20, which is found on nearly all CLL cells. A drug called rituximab is the most commonly used anti-CD20 treatment. Other anti-CD20 drugs include ofatumumab and obinutuzumab. There is another drug called alemtuzumab, which works in the same way on a different target called CD52. You can find more information about the drugs used to treat you CLL (and any other medicines you are taking) at the eMC Medicine Guides web site www.medicines.org.uk/guides.

Stem cell transplant

A stem cell transplant involves the use of high-dose treatment to kill as many as possible of the leukaemia cells. This also destroys the bone marrow’s ability to make new blood cells, so the patient is given healthy stem cells. The stem cells may be the patient’s own healthy stem cells, collected after treatment (autologous transplant) or healthy stem cells from a matched donor (a donor or allogeneic transplant). This option is only suitable for a small number of patients because the risks associated with a stem cell transplant aren’t justified for patients with a slowly progressing disease like CLL. If this is an option for you, then your haematologist will discuss it with you and give you a chance to ask questions. This is generally considered the only potential cure for CLL, but for most patients the risk of a transplant is greater than the benefit.

Radiotherapy

Most patients with CLL don’t have radiotherapy. However, if your spleen is swollen, radiation may help to reduce its size.

Splenectomy

On very rare occasions, some patients have an operation to remove the spleen (splenectomy).

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Treatment of relapse and of TP53 deletion CLL Although CLL is highly treatable, it is not generally considered curable and some patients may experience a relapse. A relapse is a return of the disease after a period of time without symptoms or signs of CLL in blood counts. There are two oral (taken by mouth) drugs now being used for treatment of relapsed CLL and also for treatment of patients who have TP53 deficient CLL. The two drugs are:

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I brutinib which blocks a protein called BTK Idelalisib which blocks a different protein called PI3K

CLL cells are more dependent on these proteins than normal cells so they are vulnerable to ibrutinib and to idelalisib. Because of the way they work, these drugs are just as effective when TP53 is deleted. This is an important option for patients with TP53 deficient CLL because normal chemotherapy is not successful in this form of the disease and immunotherapy by itself is not very effective. An anti-CD52 drug called alemtuzumab may also be effective in TP53 deficient CLL and may be used for relapsed CLL. The main risk of alemtuzumab is severe infection.

New and future treatments There are several new types of drugs being studied for treatment of CLL. Most of these fall into three groups:

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Anti-apoptotic drugs Immunomodulatory drugs B-cell receptor inhibitors

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Apoptosis is a natural process in which cells which are worn-out, or no longer needed by the body trigger a ‘suicide’ pathway. Many anti-cancer drugs work by triggering apoptosis but cancer cells, including CLL cells, find ways to block apoptosis. These are called

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anti-apoptosis pathways and, despite the name, anti-apoptotic drugs are designed to switch apoptosis back on. This means that anti-cancer drugs are more able to kill cancer cells at lower doses, which means fewer side effects.

Immunomodulatory drugs

Immunomodulatory drugs, or IMiDs, are drugs which modify, or modulate, the way in which the immune system behaves. They have been widely used for treatment of other forms of blood cancer, and are now being studied for use in treatment of CLL. One of the advantages of IMiDs is that they do not kill all dividing cells, this means that, although they do have side effects, these are not the same as other anti-cancer drugs. This is called nonoverlapping toxicity and, for patients, it means better cancerkilling without more severe side effects.

B-cell receptor inhibitors

The abnormal cells in CLL are B-lymphocytes and, like normal B-lymphocytes, they have proteins on the outside called B-cell receptors or BCRs. When a protein binds to a BCR it sends the cell a signal to divide. Unfortunately, CLL cells are particularly sensitive to BCR signals, which means they divide and produce more CLL cells. One way to stop this is to design a BCR inhibitor, which is a drug which blocks, or inhibits, the BCR signal. Anti-apoptotic drugs, IMiDs and BCR inhibitors are all drugs which have a good chance of having fewer side effects than existing drugs and also a better chance of being successful when CLL has stopped responding to present drugs.

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Complications of CLL CLL may cause complications such as those outlined below.

Risk of infection Neutrophils are a type of white blood cell and are particularly important in fighting infections. When there is a low number of neutrophils (a type of white blood cell) in the blood you are ‘neutropenic’. Patients with CLL are more susceptible to becoming neutropenic as they are unable to fight infections as well as normal. This is known as immunosuppression. If you have immunosuppression, ordinary infections may occur more often and be more severe or longer lasting. You may also get ill from germs which normally live in the body without causing problems but which grow more rapidly when your immune system is not working – these are called ‘opportunistic infections’. You will be given detailed advice by your healthcare team on precautions to take to reduce the risk of infection. Common symptoms of infection include: • Fever – a temperature of 38⁰C or greater • Aching muscles • Diarrhoea • Headaches • Excessive tiredness Keep a thermometer handy so that you can easily check your temperature. Call your hospital team if your temperature goes above 38⁰C. If you develop a fever or any other symptoms that might indicate infection, it is very important that you contact your doctor or specialist nurse immediately as early treatment is necessary.

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The signs and symptoms of infection may be less obvious when you have CLL, so if you are in any doubt it is best to contact your doctor and ask for advice.

How to avoid infection Good hand hygiene is the best way to prevent catching bacterial infections. Make sure that you wash your hands after using the toilet, when preparing food, before you eat, after gardening, being in crowded public places, using public transport and touching animals. Try to avoid people who are unwell and ask your friends and family not to visit when they have cold or flu an upset tummy. Normal food hygiene rules apply for patients with CLL. When you are on treatment and especially when your neutrophil level drops, you may be asked to avoid certain foods as having neutropenia means you are more at risk of developing food-borne infections, but following basic food safety guidelines can help to lower this risk. You may come across something called a ‘neutropenic diet’. The aim of this type of diet is to cut out the foods that are more likely to contain infection-causing microbes. There is further information on the neutropenic diet at www.leukaemiacare.org.uk/ looking-after-you. Your hospital is likely to give you advice on food and drinks to avoid, especially if you are severely neutropenic. Always ask your medical team for advice as they will know and understand your individual situation. Avoiding infections is not always possible. It is useful to have a thermometer in the house in case you develop a fever so that you can check and inform your doctor or nurse. They can advise you better if they have an accurate temperature level. Most hospitals will have emergency contact details in case you become unwell at home. Ask your doctor or nurse about the arrangements in your local hospital.

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D on’t get me wrong, it’s hard living with a chronic condition. I get tired a lot and have to be careful of picking up infections, as my immune system is lower than others. But all in all, I will not let it take over my life. I don’t intend on fighting it; it will have to fight me. Disease transformation In about one case in ten, CLL may change (transform) into a condition called prolymphocytic leukaemia (PLL). More rarely, about one case in twenty, or fewer, CLL may transform into a condition called lymphoma. If your CLL transforms in this way, your doctor will explain what this means in terms of any changes in treatment or in outlook.

Vaccines Vaccinations may not have the same effect or work as well when you have CLL, but it is still recommended that you have your annual flu jab. This will still reduce the risk of getting ill and will not cause you any harm. Some forms of vaccines, called ‘live’ vaccines may be dangerous when you have CLL, so if a vaccine is recommended by someone other than your CLL specialist you should check that it is safe.

Shingles If you have had chickenpox previously, you may develop a painful nerve condition called shingles. You’re more likely to develop shingles if your immune system isn’t working well, like with CLL. The symptoms of shingles include a rash, itching or burning sensation and blisters which form crusty sores. If it is treated early, the symptoms won’t be as bad. You may be able to receive a vaccine against shingles but you should talk to your doctor about this. If you think you have shingles, please talk to your medical team as soon as possible.

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Autoimmune haemolytic anaemia This is a condition in which your immune system does not recognise your red blood cells and destroys them, causing you to become anaemic. ‘Autoimmune’ refers to the fact that the immune system is damaging your own cells and ‘haemolytic’ means that the anaemia is occurring because red blood cells are being destroyed. Occasionally, a similar problem may affect platelets, this is called ‘autoimmune thrombocytopenic purpura’. ‘Thrombocytopenic’ means too few platelets (thrombocyte is another name for a platelet) and ‘purpura’ refers to small purple bruises which may be seen in the skin.

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Living with CLL After a diagnosis of CLL, you may find that it affects you both physically and emotionally. This chapter will talk about both of these aspects.

Emotional impact of CLL Being told you have cancer can be very upsetting. CLL is a rare condition and, because of this, you may need emotional, as well as practical, support. Being diagnosed with a rare disease can affect the whole of you, not just your body, and can impact you emotionally at any point of your ‘journey’. It is likely that you will experience a range of complex thoughts and emotions, some of which may feel strange or unfamiliar to you. It is important to know that these feelings are all valid and a normal response to your illness. Our booklet, Step-by-Step on the emotional impact of a blood cancer provides lots of useful information that could help you cope. To request your copy, call 08088 010 444 or download through the website at www.leukaemiacare.org.uk/resources

T he way I dealt with my diagnosis was to be armed with information about the condition – the more I knew the better. Although CLL does not always need treatment, it is a blood cancer and, when treatment is needed, it may be extended over a long period of time. Some patients who are placed on ‘watch and wait’ describe it as ‘watch and worry’.

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It can be stressful if you know you have a blood cancer but you are not having any treatment, and probably not what you were expecting to hear after a cancer diagnosis. You are likely to need a lot of emotional, and practical, support.

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We have a booklet, Step-by-Step on watch and wait which explains it in more detail, helps you to understand what you can expect and information on coping and helping yourself.

Staying active One of the most commonly reported symptoms of CLL is fatigue. This isn’t normal tiredness and doesn’t improve with sleep. The idea of getting out and being active may be the last thing you want to do when you’re fatigued, but it is important to try and stay as active as possible as it could make your symptoms less severe. Some general tips how to deal with fatigue include:

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ave a regular lifestyle – try going to bed and waking up H approximately the same time every day and try to avoid lying in

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ake part in regular, gentle exercise to maintain your fitness T levels as much as possible

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eserve your energy for what you find important and build rest R periods around those times

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efore going to bed avoid stimulants such as alcohol, coffee, B tea or chocolate, or using laptops, tablets or mobile phones Keep your bedroom quiet and at a comfortable temperature

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alk about your worries with family, friends or your doctor or T nurse, or patient support groups

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Discuss your fatigue with your doctor or nurse

Practical support Work and finances

Being diagnosed with CLL can sometimes lead to difficulties relating to your work life. You may need to ask for special adjustments at work e.g. to help you avoid infections, especially if your job brings you into close contact with people more likely to carry infections. Your diagnosis may lead to temporary sick leave

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or a reduction in working hours but it can also mean that you have to stop work altogether. You may need to make an arrangement with your employer for times when you may need to go into hospital or for those times when you may not be well enough to go into work. Your consultant or your GP can arrange letters to confirm your diagnosis and the effects it may have on your work life to your employer. It is often worth taking time to explain CLL to your employer, as it is likely they will never have heard of the disease. It is important for you to know that people with any form of cancer are covered by law by the Equality Act. This means that legally your employer cannot discriminate against you and must make reasonable arrangements relating to your disease. Macmillan has published a booklet about financial support following a diagnosis of cancer. They can also give you personal advice over the phone via their helpline and you can discuss which benefits you are eligible for. Some Macmillan centres can arrange face-to-face meetings with a benefits advisor. They can also provide financial assistance in the form of grants – ask your nurse in the hospital how to apply. As CLL is regarded as a cancer, you will also be entitled to apply for a medical exemption certificate which means that you are entitled to free NHS prescriptions. Your GP or specialist nurse at the hospital can provide you with the details how to apply for this.

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Talking about CLL Talking to your haematologist CLL, although the most common form of leukaemia in adults, is still a rare condition. It is important for you to develop a good working relationship with your haematologist so you are given the best treatment possible for you. The following gives advice on working well with your haematologist:

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If it’s an initial consultation, take along a list of your current medications and doses, and a list of any allergies you may have

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If you have a complicated medical history, take a list of diagnoses, previous procedures and/or complications

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Make a list of questions to take to your appointment. This will help the discussion with your haematologist

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It can be useful to repeat back what you have heard so that you can be sure that you fully understood

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Note information down to help you remember what was said

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Be open when you discuss your symptoms and how you are coping. Good patient-doctor communication tends to improve outcomes for patients Other tips:

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Bring someone along to your appointment. They can provide support, ask questions and take notes

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Don’t be afraid to ask for a second opinion – most haematologists are happy for you to ask

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You need to tell your haematologist if... You’re having any medical treatment or taking any products such as prescribed medicines, over the counter treatments or vitamins. It is important to understand that treatments, including complementary therapies which are perfectly safe for most people, may not be safe if you are being treated for CLL. Remember, if you choose to start any form of complementary therapy outside of your medical treatment, consult your haematology consultant or CNS (clinical nurse specialist), prior to beginning it. It is important to understand the difference between complementary therapies, used alongside standard treatment, and alternative therapies, used instead of standard treatment. There is no evidence that any form of alternative therapy can treat CLL. For help with talking to your haematologist, you can download free copies of our ‘Questions to ask your medical team’ at www. leukaemiacare.org.uk/resources which features a list of questions which you may want to ask.

Talking to other people Telling people you have a rare condition like CLL can be hard to explain. You might find it useful to let your close family and friends, as well as your employer know about your health condition. It might be easier to provide people with basic information and give them information leaflets about CLL if they want to know more in-depth details.

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It is probably best to focus conversations on the symptoms that you are experiencing, how the condition affects you and how you feel about it. Often people misunderstand and, unfortunately, it will mostly fall to you to you to educate them as best as you can. Where possible, it’s advisable to let people know what you find helpful and unhelpful, in terms of what others say and do. Often people make assumptions and do what they think helps. For example, saying you look well, recounting stories of others they know with a similar diagnosis, encouraging you to look ahead and stay positive, which isn’t always what people really want to hear. In many ways, the more you communicate with them the better.

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These points may help you: • Explain that you have a condition that means your bone marrow does not function properly, and this affects the number of blood cells it produces

• Explain your symptoms (maybe you are tired, or have a lot of pain) • Explain what you need (maybe more help day-to-day, or someone to talk to)

You could also consider the following when telling people about your diagnosis:

Find out more

Try to find out as much as you can about your condition, from reliable internet sources, charitable organisations or your consultant haematologist. The more you know, the more you can share.

Have a print-out to hand

It may help to have a factsheet to hand to share with family and friends. This will take the pressure off you having to remember everything they may want to know. We have one on our website for you to download.

Explain your needs

Try and be clear about what your needs may be. Perhaps you need help with the weekly food shop, help with cooking dinner, or someone to drive you to and from appointments. You may find that friends and family are pleased that they can do something to help you.

Be open about how you feel

Don’t be afraid of opening up about how you feel, as people who care will want to help you as best they can. Talk as and when you feel comfortable, so those around you will know when you need them most.

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Useful contacts There are a number of helpful sources to support you during your diagnosis, treatment and beyond, including:

• • • • •

Your haematologist and healthcare team Your family and friends Your psychologist (ask your haematologist or CNS for a referral) Reliable online sources, such as Leukaemia CARE Charitable organisations

There are a number of organisations, including ourselves, who provide expert advice and information.

Leukaemia CARE We are a charity dedicated to supporting anyone affected by the diagnosis of any blood cancer. We provide emotional support through a range of support services including a 24 hour CARE Line, patient and carer conferences, support group, informative website, one-to-one buddy service and high quality patient information. Care Line: 08088 010 444 (Freephone 24 hours a day) www.leukaemiacare.org.uk [email protected]

CLL Support Association (CLLSA) The CLLSA is a patient-led UK charity, with a mission to support and empower CLL patients, their families and their carers through education and access to reliable, relevant and current information. You can connect with other patients through their online community. 0800 977 4396 www.cllsupport.org.uk www.healthunlocked.com/cllsupport (online community)

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Bloodwise Bloodwise is the leading charity into the research of blood cancers. They also offer support to patients, their family and friends through patient services. 020 7504 2200 www.bloodwise.org.uk

Lymphoma Association The Lymphoma Association provides emotional support and information to anyone affected by a lymphatic cancer. 0808 808 5555 www.lymphomas.org.uk

Macmillan Macmillan provides free practical, medical and financial support for people facing cancer. 0808 808 0000 www.macmillan.org.uk

Maggie’s Centres Maggie’s offers free practical, emotional and social support to people with cancer and their families and friends. 0300 123 1801 www.maggiescentres.org

Citizens Advice Bureau (CAB) Offers advice on benefits and financial assistance. 08444 111 444 www.adviceguide.org.uk

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Symptoms Tracker How are you feeling? Keeping track of how you are feeling, and how your symptoms affect your lifestyle, can be difficult. We have included symptom trackers, which may help you track any changes, so you can discuss them with your haematologist. Identify the symptoms that are most troublesome to you. Think back over the past seven days, and consider how much each symptom gets in the way of your ability to sleep, interact with others, work, go about other normal daily activities, or generally enjoy your day. Use the trackers in this booklet to mark the level that most closely describes how much that symptom has affected your life that week. Try and complete the tracker on the same day each week so you can compare how the symptom is affecting you. Remember to share this information with your haematologist or clinical nurse specialist.

Symptom Tracker Symptom: Days:

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Glossary Anaemia A medical condition in which the red blood cell count or haemoglobin is less than normal.

B lymphocyte or B cell A type of lymphocyte (white blood cells) which produces antibodies to fight infection.

Bone marrow The soft blood-forming tissue that fills the cavities of bones and contains fat, immature and mature blood cells, including white blood cells, red blood cells, and platelets.

Chemotherapy Therapy for cancer using chemicals that stop the growth of cells.

Clinical trial A medical research study involving patients with the aim of improving treatments and their side effects. You will always be informed if your treatment is part of a trial.

Fatigue Extreme tiredness, which is not alleviated by sleep or rest. Fatigue can be acute and come on suddenly or chronic and persist.

Full blood count or FBC A blood test that counts the number of different blood cells.

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A cancer of the blood with many different subtypes. Some forms are acute (develop quickly) and others are chronic (develop slowly). Leukaemia is an excess number of abnormal cells in the

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blood, usually white blood cells, which stop the bone marrow working properly.

Lymph node or lymph gland An oval-shaped organ of the lymphatic system that catches viruses and bacteria. It contains white blood cells that fight infections.

Lymphocyte A type of white blood cell which forms the body’s immune system.

Neutropenia A condition in which the number of neutrophils (a type of white blood cell) in the bloodstream is decreased.

Neutrophil A type of white blood cell that helps fight infection.

Platelet A disc-shaped element in the blood that assists in blood clotting. During normal blood clotting, the platelets clump together (aggregate). Although platelets are often classed as blood cells, they are actually fragments of large bone marrow cells (megakaryocytes).

Platelet count A normal platelet count in a healthy individual is between 150,000 and 450,000 per microlitre of blood. In general, low platelet counts increase bleeding risks.

Radiotherapy The use of radiation in treatment. It is sometimes used in the treatment of CLL if the spleen is swollen.

Red blood cell The blood cell that carries oxygen. Red cells contain haemoglobin, which permits them to transport oxygen (and carbon dioxide).

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Spleen An organ that filters the blood. It removes old blood cells and helps to fight infection. It sits under the ribs on the left of the body.

Stem cells Cells that have the potential to develop into many different or specialised cell types.

White blood cell One of the cells the body makes to help fight infections. There are several types of white blood cells. The two most common types are the lymphocytes and neutrophils.

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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 CARE Line Our 24-hour CARE Line is available any time of the day or night.

Support groups Our nationwide support groups are a chance to meet and talk to other people who are going through a similar experience.

Patient and carer conferences Our nationwide conferences provide an opportunity to ask questions and listen to patient speakers and medical professionals who can provide valuable information and support.

Website You can access up-to-date information on our website, www.leukaemiacare.org.uk, as well as speak to one of our CARE advisers on our online support service, LiveChat (9am-5pm weekdays).

Campaigning and Advocacy Leukaemia CARE is involved in campaigning for patient well-being, NHS funding and drug and treatment availability.

Journey magazine Our quarterly magazine includes inspirational patient and carer stories as well as informative articles by medical professionals.

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Leukaemia CARE is a national charity dedicated to providing information, advice and support to anyone affected by a blood cancer.

Around 34,000 new cases of blood cancer are diagnosed in the UK each year. We are here to support you, whether you’re a patient, carer or family member.

Want to talk?

CARE Line: 08088 010 444

(free from landlines and all major mobile networks)

Office Line: 01905 755977 www.leukaemiacare.org.uk [email protected]

Leukaemia CARE, One Birch Court, Blackpole East, Worcester, WR3 8SG Registered charity 259483 and SC039207