1 A practical guide to understanding cancer
2 About this booklet 1 About this booklet This booklet is about a type of cancer called mesothelioma. It is about the diagnosis and treatment of the two main types of mesothelioma: pleural and peritoneal mesothelioma. They are treated in different ways and the treatment of each type is covered separately. We hope it answers some of your questions about diagnosis and treatment, and addresses the feelings you may have. We can t advise you about the best treatment for yourself. This information can only come from your own doctor, who knows your full medical history. Pages have useful addresses and websites, and page 125 has space for you to write down any questions you want to ask your doctor or nurse. In this booklet we ve included quotes from people affected by cancer, which you might find helpful. Some are from healthtalk.org Others are from people who have chosen to share their experience on our online community website community.macmillan.org.uk Mesothelioma UK provides specialist information and support to people with mesothelioma. You can contact them on freephone (open Monday Friday, 8.30am 4.30pm).
3 2 Understanding mesothelioma If you would like to discuss this information, call the Macmillan Support Line free on , Monday Friday, 9am 8pm. If you re hard of hearing you can use textphone , or Text Relay. For non-english speakers, interpreters are available. If you have found this booklet helpful, you may like to pass it on to your family and friends so they can help and support you.
4 Contents 3 Contents Mesothelioma 5 Diagnosing mesothelioma 21 Treating mesothelioma 37 Af ter treatment 75 Financial help and work 81 Your feelings and relationships 97 Information for carers 107 Further information 113
5 As a family we knew this was what he wanted and we all agreed to rally around to help Joyce cope with his care Adrienne
6 Mesothelioma What is cancer? 6 The lymphatic system 8 Mesothelioma 9 Risk factors and causes 14 Symptoms 18
7 6 Understanding mesothelioma What is cancer? Cancer starts in cells in our body. Cells are tiny building blocks that make up the organs and tissues of our bodies. They divide to make new cells in a controlled way. This is how our bodies grow, heal and repair. Cells receive signals from the body telling them when to divide and grow and when to stop growing. When a cell is no longer needed or can t be repaired, it gets a signal to stop working and die. Cancer develops when the normal workings of a cell go wrong and the cell becomes abnormal. The abnormal cell keeps dividing making more and more abnormal cells. These eventually form a lump (tumour). Not all lumps are cancerous. Doctors can tell if a lump is cancerous by removing a small sample of tissue or cells from it. This is called a biopsy. The doctors examine the sample under a microscope to look for cancer cells. Normal cells Cells forming a tumour
8 Mesothelioma 7 A lump that is not cancerous (benign) may grow but cannot spread to anywhere else in the body. It usually only causes problems if it puts pressure on nearby organs. A lump that is cancer (malignant) can grow into nearby tissue. Sometimes, cancer cells spread from where the cancer first started (the primary site) to other parts of the body. They can travel through the blood or lymphatic system (see page 8). When the cells reach another part of the body, they may begin to grow and form another tumour. This is called a secondary cancer or a metastasis.
9 8 Understanding mesothelioma The lymphatic system The lymphatic system is part of the immune system the body s natural defence against infection and disease. It s made up of organs such as bone marrow, the thymus, the spleen, and lymph nodes. The lymph nodes throughout the body are connected by a network of tiny lymphatic tubes (ducts). The lymphatic system has two main roles: it helps to protect the body from infection and it drains fluid from the tissues. Mesothelioma can spread to the lymph nodes in the chest. The lymphatic system Neck (cervical) lymph nodes Thymus Armpit (axillary) lymph nodes Diaphragm Spleen Groin (inguinal) lymph nodes
10 Mesothelioma 9 Mesothelioma More than 2,500 people are diagnosed with mesothelioma in the UK each year. Mesothelioma is a tumour of the mesothelium. This is a thin lining (membrane) that covers the outer surface of most of our body s organs. The mesothelium has different names in different parts of the body. For example: in the chest it s called the pleura in the abdomen it s called the peritoneum. Cancer of the mesothelium is called malignant mesothelioma. However, it s usually referred to simply as mesothelioma. There are two main types of mesothelioma: pleural mesothelioma and peritoneal mesothelioma. Pleural mesothelioma is much more common than peritoneal mesothelioma.
11 10 Understanding mesothelioma Pleural mesothelioma The lungs are covered by a membrane or lining, called the pleura. It has an inner and outer layer. The inner layer covers the lungs, and the outer layer lines the ribcage. The pleura produces a fluid that acts as a lubricant, which helps you to breathe easily and allows the lungs to move in and out smoothly. When mesothelioma develops in the pleura, the layers of the pleura thicken and press inwards on the lung. Fluid may also collect between the two layers. This is called a pleural effusion (see page 59).
12 Mesothelioma 11 Structure of the lungs and pleura Windpipe (trachea) Right bronchus Lymph nodes Outer pleura Left bronchus Pleural cavity Inner pleura
13 12 Understanding mesothelioma Peritoneal mesothelioma The organs in the tummy (abdomen) are covered by a membrane or lining called the peritoneum. It also has an inner and outer layer. The inner layer covers the abdominal organs and the outer layer lines the abdominal wall. The peritoneum helps protect the organs in the abdomen and keep them in place (shown as the thick line surrounding the abdominal organs in the illustration below). When mesothelioma develops in the peritoneum, it causes the layers of the peritoneum to thicken. Fluid may also collect between the two layers. This is known as ascites (see page 63). Side view of the abdomen Peritoneum Bowel Womb Bladder Rectum (back passage)
14 Mesothelioma 13 Cell types of mesothelioma Pleural and peritoneal mesothelioma can be grouped according to how the cells look under a microscope. There are three main types: epitheloid this is the most common sarcomatoid (fibrous) mixed (biphasic) this has both epitheloid and sarcomatoid cells. Knowing the type of cell involved may give your doctors an idea of how well the disease will respond to treatment. Rare types of mesothelioma Very rarely, mesothelioma may develop in the outer lining of the heart (pericardial mesothelioma) or in the outer lining of the testes (testicular mesothelioma). These are not discussed in this booklet. If you d like information about them, contact our cancer support specialists on
15 14 Understanding mesothelioma Risk factors and causes Asbestos Asbestos is the most common cause of mesothelioma. Up to 9 out of 10 cases of mesothelioma (90%) are caused by exposure to asbestos fibres. Asbestos is a natural mineral found in many countries. It acts as an insulator (to keep heat in and cold out) and it protects against fire and corrosion. There are three main types of asbestos: blue (crocidolite), brown (amosite) and white (chrysotile). These were used in UK industries until the ban on imports of blue and brown asbestos in the 1980s and on all types in Exposure to blue and brown asbestos is commonly linked with mesothelioma. However, exposure to all types of asbestos is harmful. Mesothelioma doesn t usually develop until many years after exposure to asbestos. It can take any time from years, although the average is about years after exposure.
16 Mesothelioma 15 Pleural mesothelioma When asbestos is disturbed or damaged, it releases tiny fibres. These can be breathed into the lungs. Asbestos fibres are very fine and can make their way into the smallest airways of the lungs. Once the fibres are in the lungs, the body s defence mechanisms try to break them down and remove them. This leads to inflammation in the lung tissue. The asbestos fibres can also travel through the lung tissue and settle in the outer lining of the lung (the pleura). Over many years they can cause pleural mesothelioma or other lung diseases to develop. Peritoneal mesothelioma Asbestos fibres can also be swallowed, and some of the fibres can stick in the digestive system. They can then move into the outer lining of the abdomen (the peritoneum). Here, they cause swelling and thickening of the lining and can lead to peritoneal mesothelioma.
17 16 Understanding mesothelioma Exposure to asbestos People most likely to have been exposed to asbestos at work include: joiners and construction workers plumbers electricians boilermakers shipbuilders. As these jobs were mostly done by men, mesothelioma is five times more common in men than in women. People who haven t worked directly with asbestos can also sometimes develop mesothelioma. These can include: family members of people who ve worked with asbestos and brought the dust home on their clothes people who lived near asbestos factories people who worked in buildings containing asbestos materials that were disturbed or damaged. If you develop an asbestos-related illness you may be entitled to certain benefits and compensation (see pages 82 93). We have further information on compensation claims in the Financial help section (pages 89 93).
18 Mesothelioma 17 Other causes Occasionally, mesothelioma develops in people who have never knowingly been exposed to asbestos. The other causes of the disease are not fully understood, but, in rare cases, mesothelioma has been linked to: exposure to radiation a mineral called erionite, which has been found in Turkey and North America. Mesothelioma isn t infectious and can t be passed on to other people. It isn t caused by inherited altered genes, so family members don t have an increased risk of developing it, unless they have also been exposed to asbestos.
19 18 Understanding mesothelioma Symptoms Symptoms of mesothelioma vary depending on whether it is in the pleura or peritoneum. Pleural mesothelioma The symptoms of pleural mesothelioma may include: shortness of breath chest pain that feels heavy, dull or aching fever weight loss loss of appetite a cough that doesn t go away fatigue. Peritoneal mesothelioma The symptoms of peritoneal mesothelioma may include: swelling in the tummy (abdomen) tummy pain loss of appetite weight loss feeling sick changes to your normal bowel pattern, such as constipation or diarrhoea.
20 Mesothelioma 19 All of these symptoms can be caused by conditions other than mesothelioma or cancer. If you have symptoms, you should always get them checked by your doctor, particularly if they don t go away after a couple of weeks.
21 As a family we knew this was what he wanted and we all agreed to rally around to help Joyce cope with his care Adrienne
22 Diagnosing mesothelioma How mesothelioma is diagnosed 22 Staging 33
23 22 Understanding mesothelioma How mesothelioma is diagnosed Usually, you begin by seeing your family doctor (GP). They will examine you and may arrange for you to have some blood tests and x-rays. Your GP should urgently refer you for a chest x-ray (see next page) if you have a history of asbestos exposure, and any of the following symptoms: cough fatigue shortness of breath chest pain weight loss loss of appetite. Depending on the results of any tests and x-rays, your GP may refer you to hospital for further tests and specialist advice and treatment. At the hospital, the specialist will ask you about your general health and any previous medical problems before they examine you.
24 Diagnosing mesothelioma 23 You may be asked questions about any possible exposure to asbestos in your past, such as during jobs you ve had or at places you ve worked. This is called your occupational history. You may also be asked if someone in your family, a parent for example, worked with asbestos. Don t worry if you can t remember all of the details at first. You can let your doctor know later about anything you remember. Tests for pleural mesothelioma Pleural mesothelioma often starts as tiny lumps (nodules) in the pleura. These may not show up on scans or x-rays until they re quite large. You may need to have a number of different tests before your doctors can make an accurate diagnosis. Tests you may have include: Chest x-ray A chest x-ray checks for any abnormalities in your lungs. CT (computerised tomography) scan A CT scan takes a series of x-rays, which build up a three dimensional picture of the inside of the body. The scan takes minutes and is painless. It uses a small amount of radiation, which is very unlikely to harm you and will not harm anyone you come into contact with. You will be asked not to eat or drink for at least four hours before the scan.
25 24 Understanding mesothelioma You may be given a drink or injection of a dye, which allows particular areas to be seen more clearly. This may make you feel hot all over for a few minutes. It s important to let your doctor know if you are allergic to iodine or have asthma, because you could have a more serious reaction to the injection. You ll probably be able to go home as soon as the scan is over. Someone having a CT scan
26 Diagnosing mesothelioma 25 Drainage of fluid from the pleura (pleural aspiration) Mesothelioma cells can irritate the pleura and cause a pocket of fluid to develop. This is called a pleural effusion (see page 59). Your doctor may take a sample of this fluid to send to the laboratory as mesothelioma cells are sometimes present. They will give you a local anaesthetic to numb the area. A needle is then inserted through the skin into the fluid and some fluid is removed. You will have an ultrasound scan while the procedure is being carried out. This helps the doctor guide the needle to the place where the fluid is. Pleural biopsy A biopsy is usually needed to help the doctors diagnose pleural mesothelioma. It involves taking a sample of tissue from the thickened pleura and, occasionally, from the lymph nodes in the middle of the chest. Your doctor may take a biopsy in different ways: CT or ultrasound guided biopsy A local anaesthetic is used to numb the area. Your doctor will then pass a special type of needle through the skin into the thickened area. An ultrasound or CT scanner will be used to help them guide the needle into the right place. The needle has a tip that can cut out a sample of tissue.
27 26 Understanding mesothelioma Thoracoscopy This allows the doctors to look at the pleura and other structures around the lungs. The doctor can take biopsies, if required. You ll be given either a general anaesthetic or a local anaesthetic with a sedative to make you drowsy. A small cut is made in the chest wall and the doctor puts a thin tube with a light and camera at the end (thoracoscope) into your chest. An ultrasound scan may be used to guide the thoracoscope to the thickened area of pleura. Your doctor can then take a biopsy of the pleura. Sometimes doctors use a video camera with the thoracoscope. This is called video-assisted thoracoscopy (VATS) and it can help doctors get a better view of the area surrounding the lung. Pleurodesis If your doctor drains off fluid during any of these tests or procedures, they may also do a procedure called pleurodesis to try and prevent the fluid from returning. They will put sterile talcum powder through the tube in your chest and into the pleural space. This helps stop the fluid from building up again (see page 60). Mediastinoscopy Very occasionally, your doctor may arrange for you to have a mediastinoscopy. This test allows the doctor to look at the area at the centre of your chest (mediastinum) and the lymph nodes closest to the lungs. You may have this test if your doctors think the mesothelioma has spread to the lymph nodes in the middle of the chest (see page 8). It is done under a general anaesthetic and you will have a short stay in hospital.
28 Diagnosing mesothelioma 27 A small cut is made in the skin at the base of your neck, and a thin flexible tube is passed into your chest. The tube has a light and camera at the end (mediastinoscope), and can magnify the areas it looks at. The doctor can see any abnormal areas, and may also take samples of the cells and lymph nodes (biopsies) to examine under a microscope. Biopsy Having a biopsy can be uncomfortable but shouldn t be painful. If you have any pain during or after the procedure, let your doctor or nurse know. Once a biopsy has been taken, it s sent to the laboratory and examined by a pathologist (a doctor who advises on the type and spread of cancer). Sometimes, even after taking a biopsy, the doctors may still not be sure of the diagnosis. This is because pleural mesothelioma can be very difficult to tell apart from some other illnesses. In this situation, the biopsy samples may be sent to other laboratories to confirm the diagnosis. Some of your tests may need to be repeated or you may be referred to another hospital for a second opinion.
29 As a family we knew this was what he wanted and we all agreed to rally around to help Joyce cope with his care Adrienne
30 Diagnosing mesothelioma 29 Tests for peritoneal mesothelioma You may have a number of tests to help diagnose a peritoneal mesothelioma. These may include: Abdominal x-ray This checks for any abnormalities in your tummy. CT scan A CT scan takes a series of x-rays, which build up a three dimensional picture of the inside of the body. The scan takes minutes and is painless. It uses a small amount of radiation, which is very unlikely to harm you and will not harm anyone you come into contact with. You will be asked not to eat or drink for at least four hours before the scan. You may be given a drink or injection of a dye, which allows particular areas to be seen more clearly. This may make you feel hot all over for a few minutes. It s important to let your doctor know if you are allergic to iodine or have asthma, because you could have a more serious reaction to the injection. You ll probably be able to go home as soon as the scan is over.
31 30 Understanding mesothelioma MRI (magnetic resonance imaging) scan This test uses magnetism to build up a detailed picture of areas of your body. The scanner is a powerful magnet so you may be asked to complete and sign a checklist to make sure it s safe for you. The checklist asks about any metal implants you may have, such as a pacemaker, surgical clips or bone pins, etc. You should also tell your doctor if you ve ever worked with metal or in the metal industry as very tiny fragments of metal can sometimes lodge in the body. If you do have any metal in your body, it s likely that you won t be able to have an MRI scan. In this situation, another type of scan can be used. Before the scan, you ll be asked to remove any metal belongings including jewellery. Some people are given an injection of dye into a vein in the arm, which doesn t usually cause discomfort. This is called a contrast medium and can help the images from the scan to show up more clearly. During the test, you ll lie very still on a couch inside a long cylinder (tube) for about 30 minutes. It s painless but can be slightly uncomfortable, and some people feel a bit claustrophobic. It s also noisy, but you ll be given earplugs or headphones. You can hear, and speak to, the person operating the scanner.
32 Diagnosing mesothelioma 31 Drainage of fluid from the abdomen (peritoneal aspiration) Mesothelioma cells can cause fluid to collect in the abdomen. This is called ascites (see page 63 64). Your doctor may take a sample of this fluid to send to the laboratory as mesothelioma cells are sometimes present. Your doctor will give you a local anaesthetic to numb the area. A needle will then be inserted through the skin into the fluid and some fluid is removed. Sometimes the sample of fluid is taken while you re having an ultrasound scan. The scan helps the doctor guide the needle to the fluid. Peritoneal biopsy A biopsy of the peritoneum is usually needed to help the doctors diagnose peritoneal mesothelioma. It can be carried out by: Laparoscopy This procedure allows the doctor to look at other structures in your abdomen and take further biopsies if required. You ll either be given a general anaesthetic or a local anaesthetic with a sedative to make you drowsy. Your doctor will make a small cut in the tummy wall. They will then put a thin tube with a light and camera at the end (laparoscope) into your tummy. Your doctor can then take a small biopsy of the peritoneum. CT or ultrasound guided biopsy You will have a local anaesthetic to numb the area. Your doctor will then pass a special type of needle through the tummy into the area they want to biopsy. They will use an ultrasound or a CT scanner to help them guide the needle into the right place. The needle has a tip that can cut out a sample of tissue.
33 32 Understanding mesothelioma Biopsy Having a biopsy can be uncomfortable but shouldn t be painful. If you have any pain during or after the procedure, let your doctor or nurse know. Once a biopsy has been taken, it s sent to the laboratory and examined by a pathologist (a doctor who advises on the type and spread of cancer). Sometimes, even after taking a biopsy, the doctors may still not be sure of the diagnosis. This is because peritoneal mesothelioma can be very difficult to tell apart from some other illnesses. In this situation, the biopsy samples may be sent to other laboratories to confirm the diagnosis. Some of your tests may need to be repeated or you may be referred to another hospital for a second opinion. Waiting for test results Waiting for test results can be a difficult time. It may take from a few days to a couple of weeks for the results of your tests to be ready. You may find it helpful to talk with your partner, family or a close friend. Your specialist nurse or one of the organisations listed on pages , can also provide support. You can also talk things over with one of our cancer support specialists on
34 Diagnosing mesothelioma 33 Staging The stage of a cancer refers to its size and whether it has spread beyond the area of the body where it first started. Knowing the extent of the cancer helps the doctors decide on the most appropriate treatment for you. There are a number of different staging systems. Many are based on the TNM system where: T describes the size of the cancer. N describes whether the cancer has spread to the lymph nodes and which nodes are involved. For example, N0 means that no lymph nodes are affected, while N1 means there are cancer cells in the lymph nodes. M describes whether the cancer has spread (metastasised) to another part of the body. For example, M0 means the cancer has not spread to other parts of the body, while M1 means the cancer has spread to other parts of the body. Pleural mesothelioma The staging system that s most commonly used for pleural mesothelioma is called the International Mesothelioma Interest Group (IMIG) system. It s based on the TNM system. A simplified version of the IMIG system is described on pages You may find it helpful to refer to the diagram showing the structure of the lungs and pleura on page 11.
35 34 Understanding mesothelioma Stage 1 Cancer cells are found in the pleura around the lung on one side of the chest. Stage 1a Cancer cells are only found in the outer layer of the pleura (parietal pleura). Stage 1b Cancer cells are also found in the inner layer of the pleura (visceral pleura). Stage 2 The cancer has spread to both layers of the pleura and has either spread into the muscle of the diaphragm or into the lung tissue on one side of the chest. Stage 3 The cancer has spread deeper into the tissues around the pleura. It may have spread to the: chest wall fatty tissue in the centre of the chest tissue that separates the ribs from the pleura outer lining of the heart (pericardium). Or, it may have spread to local lymph nodes.
36 Diagnosing mesothelioma 35 Stage 4 The cancer has spread deeply into the tissues close to the pleura. It may have spread to the: pleura on the other side of the chest diaphragm and the lining surrounding the abdomen (peritoneum) spine internal surface of the heart pericardium and into the muscle of the heart. Or, it may have spread to the lymph nodes on the other side of the chest or the lymph nodes above the collarbone on the same or other side of the chest. Or, it may have spread to distant organs or tissues. Peritoneal mesothelioma There isn t a specific staging system for peritoneal mesothelioma but doctors sometimes use the TNM described on page 33.
37 As a family we knew this was what he wanted and we all agreed to rally around to help Joyce cope with his care Adrienne
38 Treating mesothelioma Treatment overview 38 Treatment for pleural mesothelioma 44 Treatment for peritoneal mesothelioma 56 Other treatments (supportive therapies) 59 Treatments to control other symptoms of mesothelioma 66 Research clinical trials 71
39 38 Understanding mesothelioma Treatment overview Chemotherapy, radiotherapy and surgery may all be used to treat mesothelioma. Pleural and peritoneal mesothelioma are treated in different ways. We have split the information about treatment into two sections so you can read the information that s relevant to your type of mesothelioma. You can read about treatment for pleural mesothelioma on page 44. You can read about treatment for peritoneal mesothelioma on page 56. Some information about treatment is relevant to both types of mesothelioma. This includes information about: treatments to control symptoms and help you feel more comfortable. This is known as supportive or palliative care (see page 59). taking part in a clinical trial (see pages 71 72). The next section explains how treatment is planned. There s also information about the benefits and disadvantages of treatment and giving your consent.
40 Treating mesothelioma 39 Multidisciplinary team If your tests show that you have mesothelioma, a team of specialists, called a multidisciplinary team (MDT) will plan your treatment and care. In a number of areas of the UK there are regional specialist mesothelioma MDTs. This team will normally include: chest physicians doctors experienced in lung disease (if you have pleural mesothelioma) oncologists doctors experienced in using chemotherapy and radiotherapy to treat mesothelioma surgeons experienced in chest surgery (if you have pleural mesothelioma) or abdominal surgery (if you have peritoneal mesothelioma) specialist nurses who give information and support symptom-control specialists radiologists who help analyse x-rays pathologists who advise on the type and extent of cancer. It may also include other healthcare professionals, such as a physiotherapist, counsellor, psychologist, social worker or dietitian. The MDT will take a number of factors into account when planning your treatment and care. These include the type and stage of your mesothelioma and your general health. Occasionally, you may be offered a choice of treatments. If this happens, make sure you have enough information about the different treatments, what s involved and the possible side effects, to help you make the right decision.
41 40 Understanding mesothelioma If you have any questions about your treatment, don t be afraid to ask your doctor or nurse. It often helps to make a list of questions and to take a relative or close friend with you. You can use page 125 of this booklet to write down your questions and the answers you receive. The benefits and disadvantages of treatment Many people are frightened at the idea of having cancer treatments, particularly because of the side effects that can occur. However, these can usually be controlled with medicines. Treatment can be given for different reasons and the potential benefits will vary depending upon your individual situation. For many people with mesothelioma, the cancer has already spread when it s diagnosed and treatment is given with the aim of slowing the growth of the cancer. This can lead to an improvement in symptoms and a better quality of life. However, for some people, the treatment will have no effect on the cancer and they will get the side effects of the treatment with little benefit. Making decisions about treatment in these circumstances is always difficult, and you may need to discuss in detail with your doctor or specialist nurse whether you wish to have treatment. If you choose not to have it, you will be offered supportive (palliative) care, with medicines to control any symptoms. It s important that you ask your doctors and nurses any questions you have about your treatment. The more you understand about your treatment, the easier it will be for you and them. You may find it useful to read our booklet, Making treatment decisions. We can send you a copy.
42 Treating mesothelioma 41 Giving your consent Before you have any treatment, your doctor will explain its aims. They will usually ask you to sign a form saying that you give permission (consent) for the hospital staff to give you the treatment. No medical treatment can be given without your consent, and before you are asked to sign the form you should be given full information about: the type and extent of the treatment its advantages and disadvantages any significant risks or side effects any other treatments that may be available. If you don t understand what you ve been told, let the staff know straight away, so they can explain again. Some cancer treatments are complex, so it s not unusual to need repeated explanations. It s a good idea to have a relative or friend with you when the treatment is explained, to help you remember the discussion. You may also find it useful to write a list of questions before your appointment. People sometimes feel that hospital staff are too busy to answer their questions, but it s important for you to know how the treatment is likely to affect you. The staff should be willing to make time for your questions. You can always ask for more time if you feel that you can t make a decision when your treatment is first explained to you.