CHRONIC lymphocytic LEUKAEMIA A guide for patients and families

1800 620 420 leukaemia.org.au

Contents

Acknowledgements 4

Introduction 5

The Leukaemia Foundation 6

Bone marrow, stem cells and blood 11

What is leukaemia? 16

What is CLL? 18

How common is CLL and who gets it? 19

What causes CLL? 19

What are the symptoms of CLL? 20

How is CLL diagnosed? 22

Staging and prognostic factors 27

Treating CLL 29

Supportive care 39

Making treatment decisions 43

Body image 44

Information and support 45

Useful internet addresses 46

Glossary of terms 47 Acknowledgements

The Leukaemia Foundation gratefully acknowledges the following groups who have assisted in the development and revision of the information in this booklet: people who have experienced CLL as a patient or carer, Leukaemia Foundation support staff, haematology nursing staff and clinical haematologists representing the various states and territories of Australia.

The Leukaemia Foundation values feedback from people 4 affected by CLL and the healthcare professionals working with them. If you would like to make suggestions, or tell us about your experience of using this booklet, please contact us at [email protected]. June 2017 Introduction

This booklet has been written We have used some medical words to help you and your family and terms which you may not be familiar with. Their meaning is explained understand more about chronic in the booklet and/or in the glossary lymphocytic leukaemia (also of terms at the back of the booklet. known as CLL). In some parts of the booklet we have provided additional information you It is important to acknowledge that for may wish to read on selected topics. many people CLL may never become Some of you may require more a problem and they can continue to information than is contained in this lead a normal life despite their diagnosis. booklet, so we have included some For others, especially where there is internet addresses that you might find 5 evidence of disease progression, CLL useful. In addition, many of you will may represent a more serious condition. receive written information from If you, or a loved one, have been the doctors and nurses at your diagnosed with CLL you may be feeling treating hospital. anxious or a little overwhelmed. This is It is not the intention of this booklet normal. Perhaps you have already to recommend any particular form of started treatment or you are discussing treatment to you. You need to discuss different treatment options with your your particular circumstances at all doctor and your family. times with your treating doctor and team. Whatever point you are at, we hope We hope you find this booklet useful in the information in this booklet is useful providing support and information. We in answering some of your questions. would appreciate any feedback from It may raise other questions, which you so we can continue to help you and you should discuss with your doctor your family in the future. or specialist nurse. You may not feel like reading this booklet from cover to cover. It might be more useful to look at the list of contents and read the parts that you think will be of most use at a particular point in time. The Leukaemia Foundation

The Leukaemia Foundation is Our transport service helps thousands get to and from medical appointments, the only national charity driving more than one million kilometres dedicated to helping those with each year to ensure people get the leukaemia, lymphoma, myeloma medicines they need to beat their and related blood disorders blood cancer. survive and then live a better The Leukaemia Foundation also quality of life. provides counselling, comprehensive information, education and support 6 It exists only because of the generous programs and financial assistance to and ongoing support of the Australian help the 60,000 Australians who are community. currently living with a blood cancer. Each year, the Leukaemia Foundation The Leukaemia Foundation also funds helps more than 750 families from researchers who are working tirelessly regional and rural Australia by providing to discover safer and more effective free accommodation in our capital cities treatments that will save lives and help so they can access life-saving treatment people lead a better quality of life. at major hospitals. Supporters ensure the Leukaemia Foundation can continue to give those impacted by blood cancer a strong voice, advocating for change and ensuring all Australians who need them have easy access to the very best blood cancer treatments. Leukaemia Foundation staff with health professional qualifications provide patients and their families with information and support across Australia.

Support Services Education & Support programs The Leukaemia Foundation offers The Leukaemia Foundation has you and your family both CLL-specific a team of highly trained and and general education and support caring support staff with programs throughout Australia. qualifications and experience in These programs are designed to empower you with information about nursing or allied health who various aspects of diagnosis and work across the country. treatment and how to support your general health and wellbeing. We can offer individual support and care to you and your family Emotional support 7 when it is needed. A diagnosis of CLL can have a dramatic impact on a person’s life. At times it can Support Services may include: be difficult to cope with the emotional Information stress involved. The Leukaemia The Leukaemia Foundation has a range Foundation’s support staff can provide of booklets, DVDs, fact sheets and you and your family with much needed other resources that are available free support during this time. of charge. These can be ordered via Blood Buddies the form at the back of this booklet or downloaded from leukaemia.org.au. This is a program for people newly diagnosed with CLL to be introduced to a trained ‘Buddy’ who has been living with CLL for at least two years, to share their experience, their learning, and to provide some support. Telephone discussion forums Accommodation This service enables anyone throughout Some people need to relocate for Australia who has or has been affected treatment and may need help with by CLL to share their experiences, accommodation. The Leukaemia provide tips, and receive education and Foundation’s staff can help you to find support in a relaxed forum. Each suitable accommodation close to your discussion is facilitated by a member hospital or treatment centre. In many of the Leukaemia Foundation areas, the Leukaemia Foundation’s fully support team who is a trained furnished self-contained units and health professional. houses can provide a ‘home away from home’ for you and your family. 8 Transport The Leukaemia Foundation also assists with transporting people to and from hospital for treatment. Courtesy cars and other services are available in many areas throughout the country.

We are not alone on the journey with CLL – support is available and real, and there are many ways to connect with others, either by support group meetings, phone support, seminars run by the Leukaemia Foundation, written materials or internet forums. I ask lots of questions on the Leukaemia Foundation’s CLL telephone forums, seeking information I can’t get anywhere else. It gives me some power over this disease which makes you feel quite impotent because you can’t do anything about it. I want to be able to fight it.

Practical assistance Contacting us The urgency and lengthy duration of The Leukaemia Foundation provides medical treatment can affect everyday free services and support across life for you and your family and there Australia. Every person’s experience of may be practical things the Leukaemia living with CLL is different. It’s not Foundation can do to help. In special always easy, but you don’t have to do circumstances, the Leukaemia it alone. Foundation provides financial support for patients who are experiencing Please call 1800 620 420 to speak to a financial difficulties or hardships as a support staff member or to find out result of their illness or its treatment. more about the services the Leukaemia This assistance is assessed on an Foundation offers. 9 individual basis. Alternatively, contact us via email by Advocacy sending a message to The Leukaemia Foundation is a source [email protected] or visit of support for you as you navigate the www.leukaemia.org.au. health system. While we do not provide treatment recommendations, we can support you while you weigh up your options. We may also provide information on other options such as special drug access programs, and available clinical trials. CLL

CLL is the most common type of leukaemia and is a form of blood cancer. Blood cancers occur in cells that originate in the bone marrow and are defined by the uncontrolled growth of faulty cells. 10 In CLL the majority of these cancerous cells are found in the blood in your circulatory system, but they can also be found in other areas of your body. To understand CLL and its effects on our body, we first need to understand the cells involved and where they are formed. For this, we need to understand the bone marrow and the cells that are formed there – the blood cells. Red Blood Cells Carry oxygen for the body to produce energy

Platelets Support blood White clotting to stop Blood Cells bleeding Form part of the immune system Getting to know your bone marrow, stem cells and blood

Bone marrow You might like to think of the bone 11 marrow as the blood cell factory. Bone marrow is the spongy The main workers at the factory are the tissue that fills the cavities stem cells. They are relatively small in inside your bones. Most of number but are able, when stimulated, to reproduce vital numbers of red cells, your blood cells are made white cells and platelets. All blood cells in your bone marrow. need to be replaced because they have limited life spans. The process by which blood cells are made is called haematopoiesis. There are two main families of stem There are three main types of blood cells, which develop into the various cells: red cells, white cells and platelets. types of blood cells.

As an infant, haematopoiesis takes Myeloid (‘my-a-loid’) stem cells develop place at the centre of all bones. In later into red cells, white cells (neutrophils, life, it is limited mainly to the hips, ribs eosinophils, basophils and monocytes) and breast bone (sternum). Some of and platelets. you may have had a bone marrow biopsy taken from the bone at the Lymphoid (‘lim-foid’) stem cells develop back of your hip (the iliac crest). into other types of white cells (lymphocytes) including T-cells, B-cells and Natural Killer Cells. Blood cell formation:

Blood Stem Cells Lymphoid Myeloid Stem Cell Stem Cell Line Line (CLL pathway)

Red Cells White Cells Platelets B-cells T-cells

Neutrophils, Eosinophils, Natural Killer Cells Basophils, Monocytes

Growth factors Blood and cytokines Blood consists of blood cells and 12 All normal blood cells have a limited plasma. Plasma is a straw-coloured fluid lifespan in the circulation and need that blood cells use to travel around to be replaced on a continual basis. your body and also contains many This means that the bone marrow important proteins and chemicals. remains very active throughout your life. Natural chemicals circulating in your blood called growth factors (or cytokines) control this process of blood cell formation. Each of the different blood Plasma cells is produced from stem cells under the guidance of a different growth factor. Some of the growth factors can now Blood 55% be made in the laboratory (synthesised) Cells and are available for use in people with blood disorders. For example, granulocyte colony-stimulating factor 45% (G-CSF) stimulates the production of certain white cells, including neutrophils, while erythropoietin (EPO) stimulates the production of red cells. Blood cells

Red cells and haemoglobin Anaemia Red cells contain haemoglobin (Hb) Anaemia is a reduction in the number which gives the blood its red colour and of red cells or low haemoglobin. transports oxygen from your lungs to all Measuring either the haematocrit or the parts of your body. Your body uses this haemoglobin will provide information oxygen to create energy. regarding the degree of anaemia. Haematocrit If you are anaemic you may feel About 99% of all blood cells in rundown and weak. You may be pale circulation are red blood cells. and short of breath or you may tire The percentage of the blood that is easily because your body is not getting occupied by red blood cells is called enough oxygen. In this situation, a red cell transfusion may be given to restore the haematocrit. A low haematocrit 13 suggests that the number of red cells the red blood cell numbers and in the blood is lower than normal. therefore the haemoglobin to more normal levels. Normal ranges for adults:

Men Women

Haemoglobin (Hb) 130 – 170 g/L 120 – 160 g/L

Haematocrit (Hct) 40 – 52% 36 – 46%

White cell count (WBC) 3.7 – 11.0 x 109/L

Neutrophils (neut) 2.0 – 7.5 x 109/L

Platelets (Plt) 150 – 400 x 109/L White cells Neutropenia White cells, also known as leukocytes, Neutropenia is the term given to describe fight infection. The following is a list of a lower than normal neutrophil count. some of the different types of white cells: If you have a neutrophil count of less than 1 (1 x 109/L), you are at an increased Neutrophils: mainly kill bacteria risk of developing more frequent and and remove damaged tissue. sometimes severe infections. Neutrophils are often called the first line of defence when infections occur. Platelets They are often the first white blood cell at the site of infection and attempt to Platelets are cellular fragments that destroy the foreign pathogen before circulate in the blood and play an it becomes a problem to the body. important role in clot formation. They help to prevent bleeding. Eosinophils: mainly kill parasites. If a blood vessel is damaged (for 14 Basophils: mainly work with example by a cut) the platelets gather neutrophils to fight infection. at the site of the injury, stick together Monocytes: mainly work with neutrophils and form a plug to help stop the and lymphocytes to fight infection; bleeding. They also release chemicals they also act as scavengers to remove (called clotting factors) that are required dead tissue. These cells are known as for the formation of blood clots. monocytes when found in the blood, and called macrophages when they migrate into body tissue to help fight infection. B-cells: mainly make antibodies which target micro-organisms, particularly bacteria. T-cells: mainly kill viruses, parasites and cancer cells, and produce cytokines which can recruit other cells to make antibodies which target micro-organisms. These white cells work together to fight infection and also have unique individual roles in the fight against infection. Thrombocytopenia Thrombocytopenia is the term used to describe a reduction in the platelet count to below normal. If your platelet count drops too low, you are at an increased risk of bleeding and tend to bruise easily. Each treatment centre will have their own guidelines on the specific platelet count level when interventions may need to be taken. Platelet transfusions are sometimes given to return the platelet count to a safer level.

15

I have to be mindful with hygiene and I keep away from crowds. I’ve learnt to avoid touching my mouth, nose and eyes with my hands. I wash my hands frequently to decrease my chances of infection. What is leukaemia?

Leukaemia is the general name given to a group of cancers that develop in the bone marrow. Under normal conditions the bone marrow contains a small number of immature blood cells, sometimes called blast cells. These immature blood cells mature and develop into red cells, white cells and platelets, which are eventually released into the blood stream.

Leukaemia originates in developing blood cells, which have 16 undergone a malignant change. Instead of maturing properly these cells grow and multiply and interfere with normal blood cell production in the bone marrow. Most cases of leukaemia originate in developing white cells. In a small number of cases, leukaemia develops in other blood-forming cells, for example in developing red cells or developing platelets. What are the different Leukaemia can also be either myeloid or lymphoid. The terms myeloid and types of leukaemia? lymphoid refer to the types of cell There are several different lineage in which the leukaemia first types and subtypes of started (see diagram on page 12). leukaemia. Lymphoid leukaemias Leukaemia can be either acute or When leukaemia starts somewhere in chronic. The terms ‘acute’ and ‘chronic’ the myeloid cell line, it is called myeloid refer to how quickly the disease (myelocytic, myelogenous or develops and progresses. granulocytic) leukaemia. When leukaemia starts somewhere in Acute leukaemias the lymphoid cell line it is called Acute leukaemias develop and lymphoblastic, lymphocytic, or progress quickly and therefore need lymphatic leukaemia. 17 to be treated as soon as they are Therefore, there are four main types diagnosed. Acute leukaemias affect of leukaemia: very immature blood cells, preventing them from maturing properly. 1. acute myeloid leukaemia (AML)

Chronic leukaemias 2. acute lymphoblastic leukaemia (ALL) In chronic leukaemias there is an 3. chronic myeloid leukaemia (CML) accumulation of more mature but 4. chronic lymphocytic leukaemia (CLL). abnormal white cells. Chronic leukaemias can occur at all ages but Both adults and children can develop they are rarely seen in children. leukaemia but certain types are more common in different age groups. multiply in an uncontrolled way, live What is CLL? longer than they are supposed to and accumulate in the bone marrow, blood Chronic lymphocytic leukaemia stream, lymph nodes (glands), spleen and other parts of the body. (CLL) is a type of slow-growing leukaemia that affects CLL cells are abnormal and as such they developing B-cells. are unable to function properly. Left untreated over time, an excess number B-cells are specialised white blood cells of lymphocytes crowd the bone marrow which are an important component of and interfere with normal blood cell the immune system and they protect our production. The bone marrow begins to bodies against infection and disease in produce inadequate numbers of red many ways. Some B-cells differentiate cells, normal white cells and platelets, (mature) into plasma cells which produce making some people with CLL more 18 immunoglobulins (also called antibodies) susceptible to anaemia, recurrent that help protect our bodies against infections and to bruising and bleeding infection and disease. easily. Circulating red cells and platelets can also be damaged by abnormal In people with CLL, lymphocytes proteins made by the leukaemic cells. undergo a malignant (cancerous) change and become leukaemic cells. CLL usually develops slowly and For many people CLL remains stable for progresses slowly, over months and many months and years and has little, if years. Most people have no symptoms any, impact on their lifestyle or general of their disease when they are first health. Around 30-50% of people diagnosed. In these cases, people diagnosed with CLL never require any often require no treatment for a long treatment for their disease and can time (if at all), apart from regular survive for many years despite their checkups (often called ‘watch and diagnosis. For others, the leukaemic cells wait’ or ‘active monitoring’) with their doctor to carefully monitor their health. Others may need to be treated soon after they are diagnosed. How common is What causes CLL and who CLL? gets it? The cause of CLL is unknown and it does not develop as a consequence of Each year in Australia around 1400 someone’s activities or their diet. It is people are diagnosed with CLL. not contagious and you cannot ‘catch’ While CLL is a relatively uncommon CLL by being in contact with someone type of cancer, it is the most common who has it. type of leukaemia diagnosed in There is increasing appreciation that Australia and the western world. there may be an inherited tendency to The majority of people diagnosed with develop CLL in some patients. The risk CLL (almost 80%) are over the age of for developing CLL in a first degree 19 60. CLL is rare under the age of 40, relative (i.e. father, mother, brother, with less than 10 children diagnosed sister or child) of a patient with CLL is with the disease in Australia since approximately seven times higher than 1982 when records started. CLL is the population average. However, as more common in men than women. the background risk of developing CLL is very low, the vast majority of family members will never develop CLL. At present, screening for CLL simply because of a positive family history is not recommended. It would be recommended to seek further advice if you had several (usually more than three) close family members who had CLL, and/or related blood cancers.

It could be that I’ll never need treatment. I could be one of those lucky people and it (CLL) could be dormant forever. Like other types of leukaemia, CLL is thought to arise from an acquired What are the mutation (or change) in one or more of the special molecules of DNA symptoms (called ‘genes’) which control the growth and development of blood cells. of CLL? This change (or changes) will result in abnormal growth. This change occurs Many people have no symptoms in stem cells that produce your blood when they are first diagnosed. In these cells. This mutation is maintained when cases the disease may be diagnosed the affected stem cell divides and unexpectedly, for example during produces a series of ‘clones’: that is, a routine blood test or physical a group of identical cells all with the examination. Other people may go same defect. As such CLL is regarded to see their general practitioner (GP) as a clonal blood stem cell disorder. because they have some troubling 20 Why gene mutations occur in the symptoms of their disease. first place remains unknown but there These may include the following: are likely to be a number of as yet unidentified factors involved. These Symptoms caused by a lack of acquired mutations in genes are gained normal white cells and normal during a person’s lifetime, are not antibodies: present at birth and are usually not »» frequent or repeated infections. passed on to the next generation. Symptoms of anaemia due to a lack of normal red cells: »» persistent tiredness and fatigue »» weakness »» shortness of breath with minimal exercise »» looking pale »» feeling dizzy or lightheaded »» chest pain (angina) or palpitations. Symptoms caused by The spleen may also be enlarged as it a lack of normal platelets: attempts to rid the body of the excess »» bleeding or bruising more easily lymphocytes from the circulating blood. for no apparent reason Symptoms of an enlarged spleen (splenomegaly) include feelings of »» frequent or severe nose bleeds discomfort, pain or fullness in the upper or bleeding gums left-side of the abdomen. An enlarged »» the appearance of red or purple spleen may also cause pressure on the flat pinhead sized spots on the skin, stomach causing a feeling of fullness, especially on the legs initially. indigestion and a loss of appetite. These are due to small superficial In some cases the liver may also capillary bleeds known as petechiae be enlarged. (‘pe-tee-chi-a’). Some of the symptoms described above may also be seen in other ‘B’ symptoms (symptoms of simply illnesses, including viral infections. 21 having CLL) So, most people with these symptoms »» weight loss, especially unintentional don’t have leukaemia. However, it is loss of >10% of body weight important to see your doctor if you have any unusual symptoms, or symptoms »» fevers that persist much longer than expected »» night sweats so you can be examined and investigated properly. »» fatigue or poor energy. CLL can also cause a painless swelling of the lymph nodes (glands) in your neck, under your arms or in your groin. This is usually a result of lymphocytes accumulating in these tissues. How is CLL Which doctor? If your blood test results are abnormal diagnosed? and your GP suspects you might have leukaemia, you will be referred to another specialist doctor called CLL is diagnosed by examining a haematologist or an oncologist samples of your blood in the laboratory. for further tests and treatment. When you see your doctor about any A haematologist is a doctor who of the symptoms mentioned, the first specialises in the care of people with thing he or she will probably do is take diseases of the blood, bone marrow your full medical history, asking and immune system. questions about your general health and any illness or surgery you have had in the past, and give you a full 22 physical examination. The doctor will look and feel for any swelling of the glands in your neck, armpits and groin. Your abdomen and chest will also be examined for any signs of enlarged organs or fluid collection. The doctor will ask you about any other symptoms you might have and take some blood samples to check how well your bone marrow, liver and kidneys are functioning. Full blood count The first step in diagnosing CLL requires a simple blood test called a full blood count or full blood examination (FBC progressed and requires treatment. The or FBE). It involves taking a sample bone marrow examination may be done of blood from a vein in your arm, in the haematologist’s rooms or clinic and sending it to the laboratory for under local anaesthetic or, in selected examination under the microscope. cases, under a short general anaesthetic The number of red blood cells, white in a day procedure unit. blood cells and platelets, and their size and shape, is noted as these can all A mild sedative and a pain-killer are be abnormal in CLL. In CLL, the given beforehand and the skin is lymphocyte count is abnormally high, numbed using a local anaesthetic. and needs to be at least 5 x 109L for a This is given as an injection under the skin. The injection takes a minute or diagnosis of CLL. Anaemia and 23 thrombocytopenia (a lower than normal two, and you should feel only a mild platelet count) are common in more stinging sensation. After allowing time advanced disease. for the local anaesthetic to work, a long needle is inserted through the skin and Bone marrow examination outer layer of bone into the bone marrow cavity. A syringe is attached to the end of A bone marrow examination (biopsy) is the needle and a small sample of bone used in some cases to help confirm the marrow fluid is drawn out – this is called diagnosis of CLL. It can also provide a ‘bone marrow aspirate’. A small core of useful information about the likely bone marrow is then removed, which will course of the disease and to assess provide more detailed information about how well it is responding to treatment. the structure of the bone marrow and bone: this is known as a ‘bone marrow It involves taking a sample of bone trephine’. marrow, usually from the back of the hip bone (iliac crest) and sending it to the If you receive a sedative or painkiller laboratory for examination under the prior to your bone marrow tests you microscope. Bone marrow examinations might feel a bit drowsy afterwards, so are usually done if the disease has you should bring a family member or friend who can take you home. A small Cytogenetic dressing or plaster over the biopsy site can be removed the next day. There (‘cy-to-gen-etic’) tests may be some mild bruising or Cytogenetic tests such as chromosomal discomfort, which is usually managed analysis and fluorescent in situ effectively with paracetamol. More hybridisation (FISH) tests provide serious complications such as bleeding information about the genetic makeup or infection are very rare. of the leukaemic cells: in other words, the structure and number of chromosomes present. These tests may Immunophenotyping be used to provide more information about the likely course of your disease (‘im-u-no-feen-o-typing’) and the best way to treat it. Whether Immunophenotyping, or flow cytometry you receive cytogenetic testing tests, are commonly used to confirm or not depends on your individual 24 a suspected diagnosis of CLL. This circumstance. Usually these tests are technology uses special markers called performed when CLL is progressing and antigens found on the surface of cells. treatment is being considered. These antigens act like flags identifying Chromosomes are the structures that the abnormal characteristic of CLL. carry genes. Genes are collections Antigens are commonly referred to as of DNA, our body’s blueprint for life. ‘cluster of differentiation’ or CD antigens Certain cytogenetic changes, followed by a number. In CLL certain such as missing, extra or abnormal B-cell antigens like CD19, CD20, CD23 chromosomes help to confirm the type and CD5 and other surface markers of CLL you have, the likely course of are almost always expressed on the your disease and the best way to treat leukaemic cells. The presence of these it. These chromosomal changes are markers is key in diagnosing CLL, only found in the leukaemic cells. and distinguishes it from other diseases. These changes are usually acquired over time and are not passed down from parent to child.

Immunoglobulin Other tests (antibody) levels Once a diagnosis of CLL is made, further tests may be done to find out the stage, Blood samples may also be taken to or extent of the disease in your body measure the levels of antibodies in your and the effect it is having on other blood. People with low levels of normal organs. They include a combination antibodies may be more susceptible of blood tests and imaging tests. These to repeated infections and some tests can also provide important may benefit from monthly intravenous information about your general health immunoglobulin (antibody) treatment and how well your kidneys, liver and to reduce the frequency of infections. other vital organs are functioning. The Blood tests may be repeated at regular results may be important in selecting intervals to monitor your disease and the best treatment for you. They can look for the presence of any additional also be used as a baseline and abnormalities that can be associated compared with later results to assess 25 with CLL. When therapy for CLL how well you are going. commences, patients are monitored Other blood tests closely for treatment-related complications and to determine how »» Kidney function tests their disease is responding to treatment. »» Liver function tests »» Coagulation tests (to see if your blood Lymph node biopsy is clotting properly) Rarely, a lymph node biopsy is »» Coombs test (direct antiglobulin test) necessary to help confirm the diagnosis to exclude autoimmune haemolytic of CLL. This usually involves a small anaemia, a rare complication of CLL surgical procedure where an enlarged where the body’s own immune lymph node is removed. You will need a system can attack and destroy its general anaesthetic for this and you own red blood cells. will have a few stitches afterwards. Imaging tests Once the lymph node is removed »» Chest x-ray (to detect a chest it is examined in the laboratory by a infection or any other abnormalities). pathologist. A pathologist is a doctor who is specially trained to examine »» A CT (computerised axial tomography) tissue specimens and cells to help scan may be ordered to see if your diagnose diseases such as cancer. spleen or liver is enlarged. This is discussed further on the next page. Computerised axial tomography Testing for SLL and CLL (CT scan or CAT scan) Small lymphocytic lymphoma (SLL) CT scans provide computer analysed, A small proportion of people with three dimensional (3D) images of cross CLL have the disease in their lymphatic sections of your body. This technology is system and/or bone marrow, and not able to detect tiny changes in tissue in the blood. These patients are density which might indicate the traditionally classified as having a form presence of an infection or a tumour. of non-Hodgkin lymphoma called small lymphocytic lymphoma (SLL). We now The CT scan does not hurt and it usually understand that this is actually the same takes less than an hour to complete. While disease as CLL, with the only difference the scan is being done you have to lie flat being that the cells in SLL do not and still on a cushioned table that moves circulate in the blood. SLL is treated slowly through the CT machine. The the same way as CLL, and behaves in 26 machine itself looks like a giant ring an almost identical manner. A lymph surrounding the table. node biopsy is usually required to confirm a SLL diagnosis. Sometimes a special dye is used to enhance the quality of the pictures taken. ‘Pre-CLL’ (Monoclonal The dye may be swallowed or injected B-lymphocytosis or MBL) into a vein in your hand or arm before the scan. The CT scanner picks up the dye as With the increasing use of advanced it moves through the body, highlighting testing that can pick up a very small areas to be examined more closely. number of abnormal cells in the blood, we now know that approximately 5-7% Waiting around for tests can be both of older people (over 60 years) will have stressful and boring. Remember to ask a small population of CLL cells in their beforehand how long the test will take blood. These people are diagnosed and what to expect afterwards. You incidentally and have no symptoms of might like to take a book, some music, leukaemia. Very few (approximately 1% or a friend for company and support. per year) will develop real CLL. This state is known as ‘monoclonal B-lymphocytosis’ (MBL) and it is not regarded as cancer, although there is a risk of developing CLL in the long-term. People with this condition require periodic medical follow-up only. Staging and Stage Findings prognostic < 3 lymphoid Binet stage A areas involved factors (equivalent to Rai stage 0) High lymphocyte Your doctor may want to document the count stage of your disease (the extent that the disease has affected your body) and 3 or more other factors that provide reliable Binet stage B lymphoid areas information about the likely course of (equivalent to involved your disease (your prognosis), and Rai stage I to II) High lymphocyte whether or not treatment should begin. count Traditionally the Rai and Binet staging Bone marrow 27 systems have been used to estimate Binet stage C failure: low red prognosis in CLL. Using these systems, (equivalent to cell count patients are assigned to one of three Rai stage (anaemia) +/- low major subgroups (good, intermediate III to IV) platelet count or poor prognosis) depending on the (thrombocytopenia) number of lymphoid areas affected by the disease (lymph nodes, spleen or liver), and the white cell (particularly lymphocytes), red cell and platelet counts in the circulating blood. Stage A is considered early stage disease and in nearly all cases people haven’t got any symptoms and don’t require treatment. People with stage B disease may occasionally require therapy, whereas stage C reflects more advanced disease which usually requires treatment. Learn about your disease and become a partner with your medical team, rather than a patient. A prognosis is an estimate of the likely These factors (cytogenetic course of a disease. It provides some abnormalities or immunoglobulin gene guide regarding the likelihood of your rearrangements) are increasingly being disease progressing, and the chances of used to help make therapeutic choices, controlling it for a given time. CLL particularly in younger people with CLL. is generally regarded as an incurable We know that patients with certain disease, but in many people the disease deletions or mutations of a particular remains stable for long periods of time chromosome may not respond well to and may never require treatment. In certain types of chemotherapy and some cases it progresses and when will have their treatment regimen necessary it can often be treated modified accordingly. effectively. The course of CLL can vary The rate at which the leukaemic cells considerably between individuals. are multiplying is also important. A fast As mentioned earlier, around 30-50% (high) lymphocyte doubling time (< six 28 of people diagnosed with CLL never months) where the amount of require any treatment for their disease lymphocytes double in number may be and can survive for many years despite associated with a poorer prognosis. their diagnosis. In 25-30% of cases, CLL This and other prognostic information tends to progress gradually over time, may be used to decide when to start eventually requiring treatment. In the treatment, especially in younger remainder of cases CLL presents as an patients and in patients with early aggressive disease, and needs to stage disease. be treated soon after it is diagnosed. Your doctor is the best person to give Over recent years, there has been you an accurate prognosis regarding significant progress in identifying your disease, as he or she has the factors (other than disease stage) most information to make this that provide reliable information about assessment. an individual patient’s prognosis and how quickly their disease is likely to progress. These include certain cytogenetic changes in affected lymphocytes and immunoglobulin (antibody) genes (IgVH genes) which may be associated with a less favourable prognosis than others. Clinical trials Treating CLL These trials (also called research The treatment chosen for your disease studies) test new treatments or ‘old’ will depend on several factors including treatments given in new ways to see the stage of your disease, whether or if they work better. Clinical trials are not you have symptoms of your disease, important because they provide vital how quickly your disease is progressing, information about how to improve your age and your general health. treatment by achieving better results Occasionally, treatment decisions with fewer side-effects. Remember, any may be influenced by other prognostic prescription medication you have ever factors, which may put some people taken is only available because people at a higher risk of disease progression were prepared to test it on a clinical trial than others, regardless of the stage at some time. Clinical trials can give of their disease. The principle aims of people access to new therapies not yet treatment are two-fold: to bring about funded by governments. 29 a long-lasting remission and to prevent If you are considering taking part and reduce any symptoms in a clinical trial make sure you of the disease. understand the reasons for the trial and Information gathered from hundreds what it involves for you. You also need of other people around the world who to understand the benefits and risks have had the same disease helps to of the trial before you can give your guide your doctor in recommending informed consent. Talk to your doctor the best treatment for you. who can guide you in making the best decision for you. Remember that no two people are the same. In helping you to make Informed consent the best treatment decision, your Giving an informed consent means that doctor will consider all the information you understand and accept the risks available including the details of your and benefits of a proposed procedure particular situation. or treatment. It means that you are Standard therapy happy that you have adequate information to make such a decision. Standard therapy refers to a type of treatment which is commonly used in Your informed consent is also required particular types and stages of disease. if you agree to take part in a clinical It has been tried and tested (in clinical trial, or if information is being collected trials) and has proven to be safe and about you or some aspect of your care effective in a given situation. (data collection). If you have any doubts or questions Advanced stage CLL regarding any proposed procedure or treatment please do not hesitate There is a general agreement that most to talk to the doctor or nurse again. people with advanced stage CLL (Rai stage III and IV, Binet stage B and C) Before giving your informed consent, need to be treated. you may ask for a second opinion if you still feel uncertain. Treatment for CLL may involve the use of: Early stage CLL »» chemotherapy »» monoclonal antibody therapy Many people with CLL, particularly in the early stages of disease (Rai stage 0 »» targeted therapies and I, Binet stage A), have no symptoms »» blood stem cell transplantation of their disease and don’t require any treatment. Instead the doctor may »» experimental treatments with drugs 30 recommend an ‘active monitoring’ not yet available for general use (sometimes called ‘watch and wait’) e.g. clinical trials or compassionate strategy involving regular check-ups access programs. and blood counts to carefully observe your health. This strategy may also be Chemotherapy appropriate in more advanced stages of Chemotherapy literally means therapy CLL, if your blood counts remain stable. with chemicals. Many chemotherapy Treatment is usually only given when drugs are also called cytotoxics you start to have troubling symptoms of (cell toxic) because they kill cells, CLL, or when there are signs that your especially ones that multiply quickly disease is starting to progress. Signs like cancer cells. Commonly, oral that your disease may be progressing chemotherapy (such as chlorambucil include a significant increase in the or cyclophosphamide) is prescribed. number of lymphocytes in your blood For patients who may benefit from more (short lymphocyte doubling time), intensive treatment, a combination of or rapidly-growing lymph nodes. two or more chemotherapy drugs may be used, for example fludarabine and cyclophosphamide. These may also be given with monoclonal antibodies. These combinations are often given How is through an injection into the vein. Your doctor will discuss which type chemotherapy given? of chemotherapy is best for you. There are many ways of giving chemotherapy. Some drugs are given Each drug, in a combination of drugs, in tablet form (orally). Others are given targets the cancer using different through a vein (intravenously or IV), mechanisms, and also has different usually in your arm or hand. side-effects. Therefore, a combination may be more effective than a single In most cases you don’t need to be drug in controlling your disease and the admitted to hospital for chemotherapy side-effects are kept to a minimum. as it is usually given in the hospital’s day treatment centre. Sometimes The names of the different regimens however, depending on the type of used are commonly derived from the chemotherapy being given and your first letters of each of the drugs given. general health, you may need to be Some examples of combinations of 31 admitted to the ward for a short while. drugs used to treat CLL are listed below. Side-effects of FC Fludarabine and chemotherapy cyclophosphamide Chemotherapy kills cells that multiply FCR Fludarabine, quickly, such as cancer cells. It also cyclophosphamide and causes damage to fast-growing normal rituximab (a monoclonal cells, including hair cells, and cells in antibody) your mouth, gut and bone marrow. The side-effects of chemotherapy Combinations of drugs are usually given occur as a result of this damage. in several cycles (or courses) with a rest Chemotherapy in tablet form is tolerated period in between each cycle. This is to well by most people and side-effects allow the body to recover from the side- tend to be few and mild. Intravenous effects of the drugs. chemotherapy can have more side- effects. The type and severity of these side-effects will vary from one person to another, depending on the type of chemotherapy used and how an individual responds to it. There is no doubt that side-effects neutropenic, which means that your can be very unpleasant at times but neutrophil count is low. Neutrophils are it’s good to remember that most of important white cells that help us to fight them are temporary and reversible. infection. A blood test may be arranged It is important that you report any side- for you during this time to check your effects you are experiencing to your blood count. nurse or doctor because many of them While your white blood cell count can be treated successfully, reducing is low you should take sensible any unnecessary discomfort for you. precautions to help prevent infection. Effects on the bone marrow These include avoiding crowds (for example shopping centres and Chemotherapy affects the bone movie theatres), avoiding close marrow’s ability to produce adequate contact with people with contagious numbers of blood cells. As a result, infections (for example colds, flu, your blood count (the number of chicken pox) and only eating food 32 white cells, platelets and red cells which has been properly prepared circulating in your blood) will generally and cooked (avoid reheated food, fall following treatment. The length of soft cheeses and salad bars). time it takes for your blood counts to fall and recover mainly depends on the Your treatment centre may have its type of chemotherapy given. own special dietary guidelines and may recommend a specific White cells ‘neutropenic diet’. The point at which your white cell Sometimes your doctor may decide count is at its lowest is called the nadir, to use a drug like G-CSF to help the which usually occurs seven to ten days recovery of your neutrophil count. after chemotherapy. During this time This drug works by stimulating the you will be at a higher risk of developing bone marrow to increase the production an infection. At this stage you may be of neutrophils. G-CSF is given as an need to be treated with antibiotics as injection under the skin (subcutaneous). soon as possible. This is quite a simple procedure and the If you think you have an infection nurses can usually teach you or a family while receiving chemotherapy, you member (or friend) to do this at home. must notify your doctor immediately. Your doctor and nurse will advise you If your doctor is not available (e.g. after on how to reduce your risk of infection hours or on a weekend), you must while your white cell count is low. go to the nearest Emergency Department immediately. Platelets Many medications provided for other Your platelet count may also be symptoms or diseases may affect the affected by your disease and by the blood’s ability to clot properly and may chemotherapy you are receiving and need to be ceased while you are you may become thrombocytopenic receiving chemotherapy. Medications (a reduction in the number of platelets in this category include non-steroidal 33 circulating in the blood). When your anti-inflammatory drugs (e.g. aspirin, platelet count is very low you can bruise ibuprofen) and anticoagulants (e.g. and bleed more easily. During this time warfarin, apixaban, rivaroxaban). it is helpful to avoid sharp objects in Your haematologist will make your mouth such as chop bones or recommendations on what medications potato chips as these can cut your need to be ceased during gums. Using a soft toothbrush also chemotherapy in consultation with your helps to protect your gums, and an GP or other healthcare practitioners. If electric razor should be used instead of you are not sure if the drug is safe, a razor blade. If your platelet count is please check with your doctor, nurse or very low, your doctor may prescribe pharmacist. A low platelet count may a platelet transfusion to reduce the also result from idiopathic risk of bleeding until the platelet thrombocytopenia purpura (ITP), a rare count recovers. complication of CLL where the immune system attacks and destroys the body’s If you do develop an infection you may own platelets. Specific treatment may experience a fever, which may or may be required for this; your doctor will let not be accompanied by an episode of you know if they are concerned. shivering (a rigor) where you shake uncontrollably. Infections while you are neutropenic may be very serious and Red cells Steroids (corticosteroids) If your red blood cell count and Corticosteroids are hormones that are haemoglobin level drop you will produced naturally by the body. They probably become anaemic, and you can also be made in the laboratory. may feel more tired and lethargic than usual. If your haemoglobin level is very Corticosteroids are used to treat low, your doctor may prescribe a red autoimmune haemolytic anaemia as well cell (blood) transfusion. as ITP, both complications of CLL where abnormal proteins (antibodies) destroy Fatigue healthy cells in the bloodstream and Most people experience some degree spleen. In autoimmune haemolytic of tiredness in the days and weeks anaemia there is a drop in the number following chemotherapy for CLL. Getting of red cells in the blood and symptoms plenty of rest and doing a little light of anaemia may develop such as breathlessness, paleness and tiredness, 34 exercise each day may help you feel better during this time. Getting out into and possibly a yellowing of the skin the fresh air and doing gentle exercise known as jaundice. In ITP the platelets is important for your general wellbeing are affected and if the platelet count falls and it may help to reduce your fatigue. It to a very low level you may notice is important to listen to your body and increased bruising or bleeding. If you rest when you are tired. Fatigue is also a develop any of these symptoms it is common side-effect of other CLL important for you contact your doctor treatments and a symptom of CLL itself. as soon as possible so you can be treated properly. Other side-effects Side-effects of Other possible side-effects of corticosteroids chemotherapy less commonly seen in the treatment of CLL include: The types of side-effects seen with corticosteroids depend largely on how »» nausea and vomiting long they are used for and the dose »» changes in taste and smell given. If you are using them for a short time you may notice that your appetite »» mucositis (sore mouth) increases or you may feel more restless »» diarrhoea or constipation than usual. Some people find it more difficult to get to sleep at night and »» hair loss is exceedingly rare sleeping tablets or other natural therapies with most forms of CLL treatment. are sometimes recommended. It may also be helpful if the corticosteroids are taken Long-term use, uncommon in CLL, early in the morning, so that the effects may cause some other effects such wear off by the time you go to bed. as fluid retention and an increased susceptibility to infections. Aching joints Corticosteroids can cause a rise in the such as the knees and hips have also blood sugar. Diabetics may find they been reported. Remember to tell your need more of their diabetes medication doctor and nurses about any side- while they are taking these drugs and effects you are having as they can some people who are not normally usually suggest ways to help you. diabetic may require treatment to keep their blood sugar at acceptable levels. Monoclonal antibody It is important to keep a check on the therapy blood sugar and keep a diary of the levels and the amount of diabetic Monoclonal antibodies are antibodies medication being taken. Diabetics will produced in a laboratory that act against specific infectious agents, toxic already know how to do this and can 35 seek additional assistance from their GP inflammatory substances or cancer cells. or, if appropriate, their endocrinologist. More recently improved results People whose blood sugar only goes up have been achieved by combining when they are on corticosteroids will be chemotherapy with a monoclonal given information on diet and taught how antibody like obinutuzumab, to measure their blood sugar and adjust ofatumumab or rituximab. their medication. Monoclonal antibodies work by binding Many of the side-effects of to the B-cell antigens (e.g. CD20) found corticosteroids are temporary and on the surface of the leukaemic cells. should pass once you finish taking them. These help the person’s own immune system to recognise these cells as Corticosteroids can impact your foreign (or faulty) and kill them. mental wellbeing. Feelings may be Monoclonal antibodies are given as heightened and some people who intravenous infusions, usually in the have had a history of mental illness outpatient’s department of the hospital. may have a recurrence of this.

If you have a history of mental illness, it is very important to share this with your doctor before taking corticosteroid treatment. Side-effects are common with the first It is important that you take your dose but can generally be easily medications as directed. Adhering to managed. They may include fever, chills your medication regimen is important and mild skin reactions. This therapy is in successfully treating your blood usually given in combination with cancer and taking a break from your chemotherapy, or very occasionally with medication can be harmful. Let your corticosteroids. There is an increased treating specialist know if you have risk of infection among patients being stopped taking your medication or you treated with monoclonal antibodies. are taking it differently to how it is prescribed. Some of the medications Other targeted therapies you take for other symptoms or Promising new and experimental oral diseases may interact with targeted medications are being developed and therapies and it is important that your are becoming available for use in treating haematologist is fully aware of Australia. Drugs used in these therapies all the prescribed and over-the-counter 36 target specific substances on the medications you are taking. Access to cancer cell and inhibit proliferation of these new therapies may be restricted B-cells (the blood cells affected in CLL). during this time. The Therapeutic Goods Administration If you are taking medications it is (TGA) has approved the following drugs important to check with your pharmacist for use in Australia: about interactions with other »» Ibrutinib medications and foods, for example ibrutinib interacts with Seville oranges » Idelalisib » and grapefruit. » Venetoclax. » Remember that no two people are the These drugs are taken orally every day same. In helping to make the best and can be used to treat adults with treatment decision about any side- relapsed or refractory CLL who have effects you may experience, your received at least one prior therapy, doctor will consider the specific details generally intravenous chemotherapy. of your situation. Do not be alarmed by Sometimes doses need to be altered this list of possible side-effects, as you due to side-effects, so always talk to may not experience any of them. your treating specialist if you are experiencing any kind of side-effect.

Managing side-effects of treatments

Potential side-effects Potential remedies Diarrhoea Avoid sorbitol, mannitol, maltitol (common ingredients in sugar free foods) Psyllium seed - which increases fibre Anti-diarrhoeal medications (e.g. loperamide) after discussion with your doctor Drink plenty of fluids Fatigue Check for anaemia 37 Moderate regular exercise, yoga, meditation Take a daily nap if you need it Reduce stress Nausea and vomiting Anti-nausea drugs will usually be prescribed by your doctor to prevent nausea Record what you ate each time you felt sick, as some foods may cause an upset stomach more than others. Constipation Increase fruits and vegetables in diet Drink plenty of fluids, not alcohol Stool softeners/laxatives may be required Increase fibre intake 20-30 min gentle exercise per day Bleeding/bruising Notify doctor/dentist if you need any operations or procedures Report irregular bleeding/bruising to your doctor attack them, causing Graft-versus-host disease which can be life-threatening Allogeneic stem cell or disabling. This is the reason why transplantation (stem cell allogeneic stem cell transplantation is only considered in young patients with transplant or bone marrow very aggressive disease. After transplant) allogenic stem cell transplant, there is For a very small number of people with an increased risk of severe infection for CLL a stem cell transplant may be done. many months. Your age, fitness level and CLL A transplant is usually only offered if prognostic factor would all be taken into your doctor feels it will be of most consideration in determining if a benefit to you. transplant is the best option for you. This procedure replaces the non- Treatment for relapsed 38 functioning bone marrow of the person and resistant CLL with CLL with that of one from a healthy Finding out your CLL has come back person, usually a brother or sister, (relapsed) or is resistant to standard although sometimes an unrelated donor treatment (refractory) can be devastating. may be used. In order to determine if a It is important to remember there may suitable donor is available, blood still be several options for treating the samples are taken from the patient and disease and getting it back under potential donors for compatibility testing control. These include corticosteroids, in a procedure known as tissue typing. more chemotherapy, monoclonal Unfortunately, suitable donors cannot antibody therapy, and in selected cases be found for all patients. A highly a stem cell transplant. matched donor decreases the risk of complications, but transplants with Promising, new and experimental lower matches can still be performed. treatments are being developed for CLL all the time. Some of these The introduction of a new bone marrow treatments are currently being used will rebuild your body’s blood and in clinical trials in Australia and other immune system. This can often induce countries. Your doctor will discuss with a potential immune attack against the you all of the treatment options CLL cells, and may be curative. suitable for you. However, the risk is that the new immune system will recognise normal Clinical trials are an essential part of organs in the body as foreign and modern day medicine. Mostly these drugs have been extensively evaluated for safety and trials set out to find Supportive exactly how effective the new agents are and which patients would most care benefit from their use. Trials are strictly governed and patient care is Supportive care plays an important role paramount. You may like to consider in the treatment of many people with participating in a trial if your CLL. This involves making every effort haematologist suggests one to you. to improve your quality of life by Every drug you have ever taken has relieving any symptoms you might have only been available to you because and by preventing and treating any others have agreed to participate in complications that arise from your clinical trials. disease or treatment. Blood transfusions, antibiotics and, in Palliative care some cases, the use of growth factors 39 If a decision is made not to continue which promote the production of blood with anti-cancer treatments there are cells in your bone marrow, are all still many things that can be done to important elements of supportive care. help you to stay as healthy and comfortable as possible. Palliative Blood and platelet care aims to relieve any symptoms or transfusions pain you might be experiencing as a result of your disease or its treatment, If symptoms of anaemia are interfering rather than trying to cure or control it. with your normal daily activities, your You may still receive chemotherapy to doctor may recommend that you relieve your symptoms even though have a blood transfusion. Platelet you are in the care of the palliative transfusions are sometimes given to care team. prevent or treat bleeding if your platelet count is very low. You do not need to be admitted to hospital for a red blood cell or platelet transfusion and they are usually given in the outpatient department. Transfusions these days are considered safe and they don’t usually cause any serious hospital if you develop any of the complications. Nevertheless, you will be following signs of infection so that you carefully monitored throughout the can be treated appropriately, with transfusion. You will need to call the antibiotics and other drugs if necessary: nursing staff if you are feeling hot, cold »» a temperature of 38oC and/or an and shivery or in any way unwell, as this episode of shivering (where you might indicate that you are having a shake uncontrollably) reaction to the transfusion. Steps can be taken to minimise these effects and »» coughing or shortness of breath ensure that they don’t happen again. »» a sore throat and/or a head cold Infections »» passing urine frequently or a stinging pain when passing urine Infections are a common complication of CLL and its treatment. Infections »» if you are feeling generally unwell. 40 may be more common for several reasons, including lower levels of You also need to be seen by a doctor normally protective antibodies if you: (hypogammaglobulinaemia) and »» cut or otherwise injure yourself inadequate numbers of normally severely functioning white cells circulating in the bloodstream. Infections can occur »» develop excessive bleeding (for anywhere in the body. Common sites example blood in your urine, stools, include the upper and lower respiratory and/or sputum, bleeding gums or a tract (snius or chest infections), urinary persistent nose bleed) or start tract (bladder infections) and skin. You bruising easily may be prescribed preventive »» have any surgery planned by another (prophylactic) antibiotics especially medical practitioner, as advice may during and after particular types be required from your haematologist of treatment. as to the best supportive treatment Some people are given monthly with red cells, platelets and antibiotics, intravenous infusions of immunoglobulin to ensure your surgery is completed to help fight infection. successfully without problems due to your disease. Don’t hesitate to contact your doctor or Growth factors clothes and a hat if you are heading outdoors. This is especially important As mentioned earlier, growth factors are if you are having chemotherapy and natural chemicals in your blood that radiotherapy. Your doctor may refer you stimulate the bone marrow to produce to a skin specialist (dermatologist) for different types of blood cells. Some of regular check-ups. them can be made in the laboratory and used to help manage your CLL. Nutrition Granulocyte-colony stimulating factor A healthy, varied and nutritious diet is (G-CSF) is an example of a growth important to help your body cope with factor which may be given to stimulate your disease and treatment*. Talk to the bone marrow to produce more your doctor or nurse if you have any white cells, particularly neutrophils. questions about your diet or if you are These white cells help fight bacterial considering making any radical changes and fungal infections. to the way you eat. You may wish to see 41 Growth factors are given as an injection a nutritionist or dietitian who can advise under the skin (subcutaneous). They you on planning a balanced and don’t usually cause any major side- nutritious diet. effects but some people experience If you are thinking about using herbs or fevers, chills, headaches and some vitamins it is very important to talk this bone pain. Your doctor may recommend over with your doctor first. Some of you take paracetamol to relieve any these substances can interfere with the discomfort you may be feeling. effectiveness of chemotherapy or other Skin care and treatment you are having. sun protection * There is a separate Leukaemia Foundation booklet called ‘Eating Well’. People with CLL are at increased risk of developing skin cancer. It is important that Exercise you take care of your skin from the first Blood Cancer Related Fatigue (BCRF) day of CLL diagnosis with basic measures is tiredness or exhaustion that is directly such as applying sun cream to exposed related to blood cancer or blood cancer skin areas, and wearing long-sleeve treatment. Fatigue is the most common Complementary therapies side-effect of all cancer, affecting 70 – 90% of people. It is not just a physical Complementary therapies are therapies sensation, it can affect concentration which are not considered standard levels and your ability to finish or even medical therapies. Many people find start complex tasks, which leads to that they are helpful in coping with their frustration and distress. treatment and recovery from disease. There are many different types of ‘Moderate’ activity and exercise has complementary therapies. These proven to be effective in easing BCRF include yoga, exercise, meditation, and has many positive benefits for prayer, acupuncture and relaxation. emotional and psychological wellbeing. Complementary therapies Doing too much too soon can should ‘complement’ or assist with negatively impact energy levels, so it is recommended medical treatment important to seek advice from a health 42 for CLL. They should not be used professional before undertaking instead of or as an alternative to exercise activities after your diagnosis. medical treatment. It is important to realise that no complementary or Vaccination alternative treatment alone has proven Vaccinations are important to protect to be effective against CLL, and against infection. Vaccination against complementary medicines do not have influenza and pneumococcal infection, to pass the same rigorous testing as as per Department of Health guidelines, prescribed medications to prove their should be considered in all patients effectiveness in treating disease or with CLL. symptoms. Some complementary therapies may interfere with the way Live vaccines (e.g. Zostavax to protect your body handles chemotherapy against shingles) are NOT drugs, potentially making them less recommended in patients with CLL. effective or more toxic. Please tell your doctor if you are on complementary Please discuss your vaccination therapies, including herbs, special diets schedule with your GP. and supplements. Before going to see your doctor make a Making list of the questions you want to ask. It is handy to keep a notebook or some treatment paper and a pen handy as many questions are thought of in the early decisions hours of the morning. Your treating doctor will spend time Many people feel overwhelmed when discussing with you and your family they are diagnosed with CLL. In addition what he or she feels is the best option to this, waiting for test results and then for you. Feel free to ask as many having to make decisions about questions as you need to at any stage. proceeding with the recommended You are involved in making important treatment can be very stressful. Some decisions regarding your wellbeing. people do not feel they have enough You should feel that you have enough information to make such decisions while information to do this and that the 43 others feel overwhelmed by the amount decisions made are in your best of information they are given, or that they interests. Remember, you can always are being rushed into making a decision. request a second opinion if you feel this It is important you feel you have enough is necessary. information about your illness and all of the treatment options available, so you can make your own decisions about which treatment to have. Sometimes it is hard to remember everything the doctor has said. It helps to bring a family member or a friend along who can write down the answers to your questions, prompt you to ask others, be an extra set of ears or simply be there to support you. Body image

For some people, particularly for those with more advanced disease, it is likely that the diagnosis and treatment of CLL will have some impact on how they feel about themselves as a man or a woman and as a ‘sexual being’. Hair loss, skin changes, and fatigue can all interfere with feeling attractive. Look Good...Feel Better is a free community service that runs programs on how to manage the appearance-related side- effects of cancer treatments. 44 You might like to visit their website at lgfb.org.au or call them on 1800 650 960. There may be a CLL support group Information near you. You may wish to contact the Leukaemia Foundation for and support more information. If you have a psychological or People cope with a diagnosis of CLL psychiatric condition please inform in different ways and there is no right your doctor and don’t hesitate to or wrong or standard reaction. For request additional support from some people the diagnosis can trigger a mental health professional. any number of emotional responses ranging from denial to devastation. It is Many people are concerned about not uncommon to feel angry, helpless the social and financial impact of the and confused. Naturally people fear for diagnosis and treatment on their their own lives or that of a loved one. families. Normal family routines are On the other hand, people who don’t often disrupted and other members 45 currently require treatment may wonder of the family may suddenly have to if they are sick at all. fulfil roles they are not familiar with, for example cooking, cleaning, doing It is worth remembering that information the banking and taking care of children. can often help to take away the fear of the unknown. It is best for patients and There is a variety of programs families to speak directly to their doctor designed to help ease the emotional regarding any questions they might and financial strain created by cancer. have about their disease or treatment. The Leukaemia Foundation is here It can also be helpful to talk to other to provide you and your family with health professionals including social information and support to help you workers or nurses who have been cope during this time. Contact details specially educated to take care of for the Leukaemia Foundation are people with haematological diseases. provided on the back of this booklet. Some people find it useful to talk with other patients and family members who understand the complexity of feelings and the kinds of issues that come up for people living with an illness of this nature. Useful internet addresses

Leukaemia Foundation Health Unlocked www.leukaemia.org.au www.healthunlocked.com

American Cancer Society Leukemia & Lymphoma www.cancer.org Society of America www.lls.org Bloodwise (UK) www.bloodwise.org.uk Look Good...Feel Better program 46 www.lgfb.org.au Cancer Council of Australia www.cancer.org.au National Cancer Institute (USA) www.cancer.gov/cancerinfo Cancer Clinical Trials in Australia Patient Power www.australiancancertrials.gov.au www.patientpower.info/chronic- CLL Canada lymphocytic-leukemia www.cllcanada.ca

CLL Support Association (UK) www.cllsupport.org.uk CLL Global Research Foundation www.cllglobal.org

CLL Forum (discussion group for those with CLL) www.cllforum.com Glossary of B-cell A type of white (lymphocyte) cell terms normally involved in the production of antibodies to combat infection.

Anaemia Bone marrow A reduction in the haemoglobin level The tissue found at the centre of many in the blood. Haemoglobin normally flat or big bones of the body. Active or carries oxygen to all the body’s tissues. red bone marrow contains stem cells Anaemia causes tiredness, paleness from which all blood cells are made and and sometimes shortness of breath. in adults this is found mainly in the bones making up the axial skeleton – Antiemetic hips, ribs, spine, skull and breastbone A drug which prevents or (sternum). The other bones contain reduces feelings of sickness. inactive or (yellow) fatty marrow, which, 47 as its name suggests, consists mostly Antigen of fat cells. A substance, usually on the surface of a foreign body such as a virus or bacteria Cancer that stimulates the cells of the body’s A malignant disease characterised immune system to react against it by by uncontrolled growth, division, producing antibodies. accumulation and invasion into other tissues of abnormal cells from the Antibody original site where the cancer started. Cancer cells can grow and multiply to Naturally-produced substance in the the extent that they eventually form a blood, made by white blood cells lump or swelling. This is a mass of called B-lymphocytes or B-cells. cancer cells known as a tumour. Not Antibodies target antigens on other all tumours are due to cancer, in which substances such as bacteria, viruses case they are referred to as non- and some cancer cells and cause malignant or benign tumours. their destruction. Cannula Blood count A routine blood test that measures the A plastic tube which can be inserted number and type of cells circulating into a vein to allow fluid to enter in the blood. Also called a full blood the bloodstream. count (FBC). Central venous access device Echocardiogram A line or tube passed through the large A special ultrasound scan of the heart. veins of the arm, neck, chest or groin and into the central blood circulation. It can Electrocardiogram (ECG) be used for taking samples of blood, Recording of the electrical activity of giving intravenous fluids, blood, the heart. chemotherapy and other drugs without the need for repeated needles. Growth factors and cytokines A complex family of proteins produced Chemotherapy by the body to control the growth, division and maturation of blood cells Single drugs or combinations of drugs by the bone marrow. Some are now which may be used to kill and prevent available as drugs as a result of genetic the growth and division of cancer cells. engineering and may be used to Although aimed at cancer cells, stimulate normal blood cell production chemotherapy can also affect rapidly following chemotherapy or bone dividing normal cells and this is marrow or peripheral blood stem responsible for some common side- cell transplantation. effects including hair loss and a sore 48 mouth (mucositis). Nausea and vomiting are also common side-effects, but Haematologist nowadays largely preventable with A doctor who specialises in the modern anti-nausea medication. Most diagnosis and treatment of diseases side-effects from chemotherapy are of the blood, bone marrow and temporary and reversible. immune system.

Computerised axial tomography (CT Haemopoiesis scan or CAT scan) The process involved in blood A specialised x-ray or imaging cell formation. technique that produces a series of detailed three dimensional (3D) images Hairy cell leukaemia of cross sections of the body. A rare type of CLL in which abnormal B-lymphocytes accumulate in the bone Cytopenia marrow, liver and spleen. Under the A deficiency of some cellular microscope, these cells are seen to have element of the blood. tiny hair-like projections on their surface. High dose therapy Leukaemic blasts The use of higher than normal doses of Abnormal blast cells which multiple chemotherapy to kill off resistant and/or in an uncontrolled manner, crowding residual (leftover) cancer cells that have out the bone marrow and preventing survived standard-dose therapy. it from producing normal blood cells. These abnormal cells also spill out Hypogammaglobulinaemia into the bloodstream and can accumulate in other organs. The term used to describe low immunoglobulins (IgA, IgG, IgM). Leukopheresis Immune system A procedure that uses a special machine called a ‘cell separator’ to remove the The body’s defence system excess white cells in the blood while against infection and disease. returning the rest of the blood to the patient. Immunocompromised When someone has decreased Lymphocytes immune function. Specialised white blood cells involved in 49 defending the body against disease and Intravenous immunoglobulin (IVIG) infection. There are different types of The pooled immunoglobulin (antibody) lymphocytes including: B-cells, T-cells G (IgG), extracted from the plasma of and Natural Killer Cells. many blood donors and may be provided to assist immunocompromised Lymphocyte doubling time patients. IVIG’s effects last between The time it takes for lymphocyte counts two weeks and three months. to double.

Leukocytes Lymphocytosis Another name for white blood cells. An abnormal increase in the number of lymphocytes in the circulating blood. Leukaemia A cancer of the blood and bone Lymphomas marrow characterised by the General name given to cancers widespread, uncontrolled production of of the lymphatic system. large numbers of abnormal blood cells. These cells take over the bone marrow often causing a fall in blood counts. If Malignancy they spill out into the bloodstream A term applied to tumours however they can cause very high characterised by uncontrolled growth abnormal white cell counts. and division of cells (see cancer). Minimal Residual Disease (MRD) Pathologist The name given to small numbers A doctor who specialises in the of leukaemic cells that remain in the laboratory diagnosis of disease, patient during treatment, or after and how disease is affecting the treatment when the patient is in organs of the body. remission. It is the major cause of relapse in cancer and leukaemia. Peripherally inserted central venous catheter (PICC) Mucositis A type of central venous catheter which Inflammation of the lining of the mouth is inserted into a large vein in the arm. and throat, which also can extend to the lining of the whole of the gastro- Plasma cells intestinal tract (stomach and intestines). Activated B-cells that produce antibodies to help the body fight infection. 50 Neutropenia A reduction in the number of circulating Prolymphocytic leukaemia (PLL) neutrophils, an important type of white A rare type of chronic lymphocytic blood cell. Neutropenia is associated leukaemia in which abnormal with an increased risk of infection. lymphocytes known as prolymphocytes are found in Neutropenic diet the bone marrow and bloodstream. A diet which seeks to reduce the amount of food introduced into the Prognosis body that has high levels of bacteria. An estimate of the likely course of a disease. Neutrophils Neutrophils are the most common type Resistant or refractory disease of white blood cell. They are needed This means that the disease to mount an effective fight against is not responding to treatment. infection, especially bacteria and fungi.

Remission (or complete remission) Oncologist When there is no evidence of disease General term used for a specialist detectable in the body.Note: this is not doctor who treats cancer by different always equivalent to a cure as relapse means, e.g. medical, radiation, may still occur. surgical oncologist. Spleen of a wide range of blood cancers An organ that accumulates including leukaemias, lymphomas, lymphocytes, acts as a reservoir for myeloma, certain solid tumours, red cells for emergencies, and destroys and other serious diseases. blood cells at the end of their lifespan. The spleen is found high in the T-cell abdomen on the left-hand side. A type of white cell (lymphocyte) It cannot normally be felt on involved in controlling immune reactions. examination unless it is enlarged. It is often enlarged in diseases of the Thrombocytopenia blood – this is known as splenomegaly. A reduced platelet (thrombocyte) count. Splenomegaly Tumour Another term used to describe an enlarged spleen. An abnormal mass of cells which 51 may be non-malignant (benign) or malignant (cancerous). Standard therapy The most effective and safest Ultrasound therapy currently being used. Pictures of the body’s internal organs built up from the interpretation Stem cells of reflected sound waves. Stem cells are primitive blood cells that can give rise to more than one cell type. White blood cells (white cells) There are many different types of stem cell in the body. Bone marrow stem cells Specialised cells of the immune system have the ability to grow and produce all that protect the body against infection. the different blood cells including red There are five main types of white blood cells, white cells and platelets. cells: neutrophils, eosinophils, basophils, monocytes and lymphocytes. Stem cell transplant (peripheral blood stem cell X-ray or bone marrow transplant) A form of radiation used in diagnosis These treatments are used to support and treatment. the use of high-dose chemotherapy and/or radiotherapy in the treatment Notes

52 Making a donation

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55 This information booklet is produced by the Leukaemia Foundation and is one in a series on leukaemia, lymphoma, myeloma, MDS, MPN and related blood disorders. Copies of this booklet can be obtained from the Leukaemia Foundation by contacting us. The Leukaemia Foundation is a not-for-profit organisation that depends on donations and support from the community. Please support the Leukaemia Foundation today. June 2017

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