Myeloma Bone Disease and Bisphosphonates. Myeloma Infoguide Series.

Transcription

1 Myeloma Bone Disease and Bisphosphonates Myeloma Infoguide Series

2 Contents 4 Introduction 5 What is myeloma? 7 Treatment for myeloma 9 What is myeloma bone disease? 11 Normal activity of bone cells 13 Bone cell activity in myeloma 14 Tests and investigations to detect and monitor myeloma bone disease 18 What are the effects of myeloma bone disease? 21 Treatment and management of myeloma bone disease 27 Management of pain associated with myeloma bone disease 33 Managing problems with mobility 35 The future 36 Self-help checklist 37 Medical terms explained 40 Further information and useful organisations 52 With Myeloma UK you can Other information and support available from Myeloma UK 55 We need your help Disclaimer The information in this guide is not meant to replace the advice of your medical team. They are the best people to ask if you have questions about your individual situation.

3 Introduction This Infoguide is written for myeloma patients. It may also be helpful for their families and friends. It provides information on what myeloma bone disease is and how it is treated and managed. Some of the more technical or unusual words appear in bold the first time they are used and are described in the Medical terms explained on page 37. This Infoguide aims to: Help you understand more about myeloma bone disease Provide you with information about bisphosphonate treatment Answer some of the questions you may have about myeloma bone disease Help you make informed decisions about the treatment options available to you Myeloma UK also provides a range of specific Infoguides and Infosheets on all aspects of the treatment and management of myeloma. You will find a list of these at the back of this Infoguide. For more detailed information about myeloma and living with myeloma, see Myeloma Your Essential Guide and Living with Myeloma Your Essential Guide. To order your free copies call the Myeloma Infoline on or from Ireland. This information is also available to download at To talk to one of our Myeloma Information Specialists about any aspect of myeloma, call the Myeloma Infoline on or from Ireland. The Myeloma Infoline is open from Monday to Friday, 9am to 5pm and is free to phone from anywhere in the UK and Ireland. From outside the UK and Ireland, call +44 (0) (charged at normal rate). Information and support about myeloma is also available around the clock at 4

4 What is myeloma? Myeloma, also known as multiple myeloma, is a cancer arising from plasma cells that are normally found in the bone marrow. Plasma cells are a type of white blood cell which forms part of the immune system. Normal plasma cells produce different types of antibodies (also called immunoglobulins) to help fight infection. In myeloma, the plasma cells become malignant and release only one type of antibody, known as paraprotein, which has no useful function. It is often through the measurement of paraprotein that myeloma is diagnosed and detected. Bone marrow is the spongy material found in the centre of the larger bones in the body. As well as being home to plasma cells, the bone marrow is where blood cells (red blood cells, white blood cells and platelets) are made (see Figure 1). These all originate from blood stem cells. Plasma cells normally make up less than 5% of the total blood cells in the bone marrow. Figure 1. Bone marrow responsible for the production of blood cells Red blood cells White blood cells Natural killer cell Bone marrow Lymphocyte Plasma cell Blood stem cell Monocyte Neutrophil Eosinophil Basophil Platelets Myeloma Infoline: UK Ireland

5 Myeloma affects multiple places in the body (hence the term multiple myeloma ) where bone marrow is normally active i.e. within the bones of the spine, pelvis, rib cage and the areas around the shoulders and hips. The areas usually not affected are the extremities the hands and feet as the bones here do not contain much bone marrow. Most of the complications and symptoms of myeloma are caused by a build-up of myeloma cells in the bone marrow and the presence of paraprotein in the blood or in the urine. Common problems include: bone pain, bone fractures, tiredness due to anaemia, frequent or recurrent infections (such as bacterial pneumonia, urinary tract infections and shingles), kidney damage and hypercalcaemia. Myeloma most commonly occurs in people later in life over the age of 60. However, some myeloma patients are younger. It is also slightly more common in men than in women. The causes of myeloma are not known. Exposure to chemicals, radiation, viruses and a weakened immune system are thought to be potential causal or trigger factors. It is likely that myeloma develops when a susceptible individual has been exposed to one or more of these factors. There is a slight tendency for myeloma to occur in families. Although rare, this suggests that there is an inherited factor. Recent research indicates that inherited genetic variations may increase the risk of myeloma but other environmental factors are needed before myeloma develops. However, there are no screening tests available for myeloma at present. Basic facts There are approximately 4,000 new cases per year in the UK Between 14,000 20,000 people are living with myeloma in the UK Myeloma accounts for 15% of blood cancers and 1% of cancers generally Myeloma mostly affects older people, although it appears to be on the increase in younger people 6

6 Treatment for myeloma Treatments for myeloma can be very effective at halting its progress, reducing symptoms and complications and improving quality of life but as yet, they are not curative. In most cases, treatment is given in order to: Reduce the levels of myeloma as far as possible Control the myeloma for as long as possible when given as maintenance treatment Control the myeloma if it has come back again (relapse) Relieve the symptoms and reduce the complications the myeloma is causing Improve quality of life Prolong survival However, not everyone diagnosed with myeloma will need to start treatment immediately and it is usual to wait until the myeloma is actively causing symptoms and complications before starting treatment. Treatment for myeloma is often most effective when two or more drugs with different but complementary mechanisms of action are given together. In the past the number of treatment options for myeloma was somewhat limited but with the development of newer treatments in the last decade, there are now more options available. Before starting treatment, each option must be considered carefully so that the benefits of treatment are weighed against the possible risks of side-effects. In most cases, your age, overall health and fitness and any previous treatments will be taken into account. Myeloma Infoline: UK Ireland

7 The length of treatment varies depending on the type of treatment(s) being used and the stage at which the treatment is being given. Treatment is usually given over a number of weeks which may or may not be followed by a rest period. This pattern constitutes one cycle of treatment and a series of treatment cycles is referred to as a course of treatment. As well as treatment for the myeloma itself, you may also receive additional supportive treatments and procedures to help treat or prevent the symptoms and complications of myeloma. Specific treatments are available for the different symptoms and complications so it is important to let your doctor know if you are having any problems. The aim of supportive treatment is to improve your quality of life. 8

8 What is myeloma bone disease? Myeloma bone disease is the most common and often most debilitating feature of myeloma and bone pain is a very common symptom. Between 70 80% of patients have evidence of myeloma bone disease at the time of diagnosis and approximately 90% of patients have myeloma bone disease at some point during the course of their myeloma. Myeloma bone disease is due to the myeloma cells in the bone marrow affecting the surrounding bone, causing the bone to be broken down faster than it can be repaired. The extent of myeloma bone disease varies considerably from patient to patient. It most often occurs in the middle or lower back, the hips and the rib cage. The long bones of the upper arm and leg, and the shoulder may also be involved but the bones of the hands and feet are normally spared. Affected areas of bone often appear as holes on an X-ray. These holes are called lytic lesions. They represent thinned and weakened bone, increasing the risk of breaks without undue force or injury this is called a pathological fracture (see Figure 2). Figure 2. Lytic lesions show up as dark shadows on an X-ray. A fracture is visible in the X-ray (right) of a patient s arm Myeloma Infoline: UK Ireland

9 The thinning of the vertebrae (bones of the spine) can also result in fractures. When vertebrae fracture they tend to become compressed and collapse. This is known as a compression fracture. Compression fractures often result in loss of height and/or curvature of the spine known as kyphosis, as well as pain. In order to understand more about why myeloma bone disease occurs and the mechanisms that lie behind it, it is necessary to understand the normal activity within the bone as described in the next section. 10

10 Normal activity of bone cells Bones are made up of a dense outer shell of mineralised bone (bone cortex) with softer, spongier bone (cancellous bone), in the middle (see Figure 3). The bone marrow is contained within the spaces of the cancellous bone. Figure 3. Bone structure Cancellous bone Bone marrow Hollow inner bone Dense outer bone Although bone is made up of minerals and is hard, it is still a living tissue containing blood vessels, nerves and cells, including two very important cell types which play a key role in the normal activity of bones. These are: Osteoblasts (cells which form new bone) Osteoclasts (cells which break down old bone) Osteoblasts and osteoclasts work together to sustain a continuous cycle of bone formation and breakdown keeping the bone in a constant state of renewal throughout life. This ongoing process is known as bone remodelling (see Figure 4 on page 12) and maintains the thickness, strength and health of bones in the body. Normally, the rate of bone formation and the rate of bone breakdown are equal, so that the bone mass remains the same. As you will see in the next section, myeloma can interfere with this process resulting in a net loss of bone. Myeloma Infoline: UK Ireland

11 Figure 4. Normal bone remodelling Osteoclasts breaking down old bone Osteoblasts building new bone Bone New bone 12

12 Bone cell activity in myeloma Myeloma cells produce signals and substances known as cytokines and growth factors which activate bone breakdown but inhibit new bone formation. Specifically, these cytokines and growth factors (see Figure 5) increase the production and activity of the osteoclasts but at the same time reduce the activity of the osteoblasts. In addition, the osteoclasts produce their own signals which stimulate the myeloma cells to grow. This results in a vicious cycle of dependency between the myeloma cells and bone marrow cells, particularly osteoclasts and stromal cells, that occurs in what is known as the bone microenvironment. It is these processes within the bone microenvironment which result in a net loss of bone and lytic lesions in myeloma. Figure 5. Bone microenvironment in myeloma Myeloma cells Myeloma-derived osteoclast activation factors Osteoclastderived factors Myeloma-derived osteoblast inhibitory factors (+) (+) (-) Stromal cell Osteoclast Osteoblasts Bone Myeloma Infoline: UK Ireland

13 Tests and investigations to detect and monitor myeloma bone disease Myeloma bone disease is the most common presenting complication affecting 70 80% of patients at diagnosis. Therefore, bone tests will routinely be done alongside blood, urine and bone marrow tests to confirm the diagnosis and to decide on the need for treatment. Some of these tests are repeated during treatment and at follow-up appointments. Common tests and investigations to detect and monitor myeloma bone disease include: X-ray Standard X-rays are routinely used to detect evidence of myeloma bone disease. A series of X-rays, called a skeletal survey, is usually performed. This survey includes X-rays of the spine, skull, chest, pelvis and the long bones of the arms and legs. X-rays can show areas of thinning, lytic lesions and fractures, and remain the gold standard for detecting myeloma bone disease. The X-ray procedure itself takes seconds and is painless, but you may be asked to lie in certain positions that may be uncomfortable or painful. It may help to take pain-killers a few hours before your X-ray appointment and to use pillows and other aids to make you more comfortable when you have the X-rays. If the skeletal survey does not show up any areas of damage but myeloma bone disease is suspected, other imaging techniques may be used. Such techniques are more sensitive and can detect areas of bone damage not seen on an X-ray. These may include one or more of the following: Computerised tomography (CT) scans CT scans may be used if more detailed images of your bones are required or for detecting a localised collection of myeloma cells (plasmacytoma) that may exist outside of the bones, for example in soft tissue. They can also be used to pinpoint the exact area where radiotherapy treatment is to be given. 14

14 CT scans combine the X-ray procedure with a specialised computer to create detailed cross-sectional images of the body. The amount of radiation used in a CT scan is greater than for an X-ray but is more sensitive and can determine the presence or absence of myeloma bone disease more accurately. A CT scan may be done for the following reasons: If you have bone pain but your X-rays did not reveal any bone damage and you are unable to have an MRI scan Visualising a certain part of your body which was difficult to X-ray e.g. breast bone, certain rib areas, shoulder blade Confirming the presence of a plasmacytoma and whether it is affecting vital organs e.g. spinal cord compression Magnetic resonance imaging (MRI) MRI, like a CT scan, is more sensitive and can provide more detailed images of your bones than X-rays. It may therefore be used if X-rays are inconclusive or more detailed testing is needed. MRI involves a combination of radio-waves, a powerful magnetic field and a computer to generate images of the organs and tissues of the body by picking up signals sent out by water molecules. Unlike X-rays and CT scans, MRI does not involve the use of radiation. Whether you have an MRI or CT scan will depend on your clinical situation and on local hospital practice. There are subtle differences in what these scans are able to detect and your doctor will decide which is appropriate for you. You may not be able to have an MRI scan if you have a heart pacemaker or any metal implants in your body. It is important you lie still whilst you have your MRI scan. As this can take up to an hour to complete you may find it uncomfortable to remain still for the duration. You can arrange beforehand to be sedated when you have your scan. Myeloma Infoline: UK Ireland

15 MRI is especially useful for: Detecting small bone lesions Confirming suspected spinal cord compression Assessing the extent of myeloma in the bone marrow Identifying plasmacytomas outside of the bone Figure 6. Image from MRI scan of the spine showing bone marrow within the vertebrae and areas of myeloma bone disease Normal marrow is bright Abnormal areas are dark Positron emission tomography (PET) scanning PET scanning is a type of nuclear medicine imaging which shows how body tissues are working and what they look like. It is particularly useful for highlighting active cells. In preparation for a PET scan, radioactive sugar is injected into your vein. This is taken up and concentrated by cells which require a lot of energy, such as myeloma cells. Radioactive emissions from the sugar are then detected by a special camera which produces images of the body to show where the myeloma is (see Figure 7). 16

16 The most widely used radioactive sugar is 18Fluorine-fluoro-deoxyglucose (FDG), so the procedure is sometimes called FDG-PET. The dose of radioactivity injected is low and is equivalent to about as much as an X-ray. Of the imaging methods described, PET scanning is the most sensitive in terms of be able to identify myeloma bone disease early. It can detect the presence of myeloma when other scans have not. Unfortunately, PET scanners are expensive and are not available in all hospitals. Therefore, PET scans are not routinely used. In some cases, a PET/CT scan will be done. This is where a CT scan is combined with a PET scan. This method is potentially of great therapeutic benefit in myeloma but its clinical value has yet to be determined. Figure 7. Images from a PET scan showing areas of myeloma as dark spots before treatment (left) and after treatment (right) Myeloma Infoline: UK Ireland

17 What are the effects of myeloma bone disease? Myeloma bone disease can have the following effects: Osteopenia Osteopenia (bone thinning) means a general loss of mineralised bone. It is common in myeloma patients and can lead to small compression fractures of the spine and fractures of the ribs, causing pain and discomfort. It is because of these fractures that myeloma may often be first diagnosed. Lytic lesions Lytic lesions are areas in which the bone appears to have been eaten away, leaving a hole. They are most commonly found in the skull, spine, pelvis, ribs and the long bones of the arms and legs. Pathological fracture A pathological fracture is a broken bone that occurs in an area of weakened bone. It can occur spontaneously or with only slight injury, most often in the ribs, pelvis, sternum (breast bone) and the long bones of the arms and legs (see Figure 2 on page 9). Collapsed vertebrae/kyphosis/height loss When myeloma bone disease is extensive in the spine it can result in fractures of the vertebrae. In some cases, these fractures may become compressed, cause the damaged vertebrae to collapse and lead to severe back pain (see Figure 8). Sometimes the collapse changes the shape of the spine (kyphosis) and result in a loss of height. This can lead to varying degrees of immobility. Occasionally breathing difficulties and eating problems occur because of the pressure the curvature of the spine places on the chest. 18

18 Figure 8. Left diagram: Normal spine showing three correctly aligned vertebrae, separated by vertebral discs. Right diagram: The middle vertebra has a compression fracture resulting in misalignment of the spine Spinal cord compression Spinal cord compression is the term used to describe pressure on the spinal cord. In myeloma this can be caused by collapsing vertebrae or by the growth of a plasmacytoma within the spinal canal. If compression is slight, symptoms can include discomfort in the back, weakness, tingling and changes in sensation, often affecting the legs and arms. If the compression is more severe, pain, numbness, significant weakness, and problems with passing urine and opening the bowels often occur. Cord compression is serious and is regarded as a medical emergency left untreated it can lead to paralysis. Therefore, it is important to contact your doctor immediately if you develop any of these symptoms. Myeloma Infoline: UK Ireland

19 Hypercalcaemia Bone is high in calcium and as it is broken down, a large amount of calcium is released into the blood. Once the body s ability to maintain normal levels is overwhelmed, calcium levels in the blood remain high. This is known as hypercalcaemia. Hypercalcaemia can cause a variety of symptoms including: loss of appetite, nausea, vomiting, constipation, increased thirst, confusion, general weakness and tiredness. As these symptoms are somewhat general, it is easy to put these effects down to the myeloma or its treatment. Hypercalcaemia most often presents at the time of diagnosis but is much less common once treatment has started. Pain Bone pain is the most frequent symptom of myeloma bone disease and is commonly felt in the mid and lower back, ribs and hips, or wherever there are areas of bone damage due to myeloma bone disease. The severity and intensity of the pain varies from patient to patient. It is usually aggravated by movement and relieved by lying down. Pain can get progressively worse over time or occur suddenly and severely, which can be a sign of a fractured bone. 20

20 Treatment and management of myeloma bone disease Treatment of the myeloma itself is one of the most effective ways of controlling further bone breakdown, correcting hypercalcaemia and relieving pain. In most cases, myeloma bone disease is likely to be an ongoing issue. However, treatments are available to slow down its activity, alleviate symptoms and sometimes correct the complications that occur. The treatment of myeloma bone disease has been revolutionised in recent years by a group of drugs called bisphosphonates. These and other treatments are discussed over the next few pages. Bisphosphonates Bisphosphonates are small molecules that bind to calcium and as a result are taken up into bone. They inhibit the activity of the osteoclasts and therefore interrupt the increased bone breakdown. Bisphosphonate treatment therefore has several potential beneficial effects including: Preventing/slowing down further bone breakdown Reducing bone pain and the need for pain-killers Preventing and correcting hypercalcaemia Reducing the need for radiotherapy Reducing the likelihood of pathological fractures due to myeloma bone disease Improving quality of life, particularly by decreasing pain and maintaining mobility Improving the chances of healing and recovery of strength of bone Myeloma Infoline: UK Ireland

21 Figure 9. Mechanisms of action of bisphosphonates Osteoblast Osteoclast 2. Released during bone breakdown and inhibits osteoclast activity BP BP 3. Reduced bone breakdown Bone BP BP BP 1. Concentrated in newly mineralising bone and under osteoclasts What are the different types of bisphosphonates? There are three bisphosphonates licensed for use in the UK to treat both myeloma bone disease and the hypercalcaemia resulting from it. These are shown in Table 1 together with the way they are given. Type Other names Method of administration Sodium clodronate Bonefos and Loron Oral tablets, taken once or twice per day Disodium pamidronate Aredia Intravenous infusion over minutes every month Zoledronic acid Zometa Intravenous infusion over minutes every month 22

22 What are the potential side-effects of bisphosphonates? Bisphosphonates are generally well tolerated; any side-effects are usually mild. The most common include: Fever and flu-like symptoms can occur shortly after intravenous (IV) infusions. They are typically mild and last for only two to three hours. The effects are usually successfully treated with paracetamol. Vein irritation may occur at the site of the infusion but is usually temporary, lasting one to two days. General bone aches and pains these are mostly linked to the onset of fever and/or flu-like symptoms. They can persist for a day or two after each infusion and can be managed with pain-killers such as paracetamol. Nausea may occur the first few times oral bisphosphonates are taken but is generally mild. Impaired kidney function probably the most important potential side-effect of bisphosphonates, especially those given intravenously. Since myeloma can already affect kidney function (e.g. due to paraprotein damage or hypercalcaemia) the possibility of kidney-related side-effects of treatment is carefully monitored. Your doctor will check your kidney function regularly by a simple blood test, especially if you already have kidney problems. To ensure the safe and effective use of bisphosphonate drugs and to help protect the kidneys, it is recommended you maintain a high fluid intake you should drink at least three litres of water per day. Pain and poor healing in the jaw (known as osteonecrosis of the jaw), particularly after tooth extraction, have been reported in a small number of cases. As a precaution, patients taking bisphosphonates should have regular dental check-ups and inform their doctor before any planned oral surgery/tooth extractions. Myeloma Infoline: UK Ireland

23 For more information on managing side-effects, see The Kidney, Mouthcare and Osteonecrosis of the jaw Infosheets from Myeloma UK. To order your free copies call the Myeloma Infoline on or from Ireland. This information is also available to download at Who should and who shouldn t receive bisphosphonates? Current national guidelines on the diagnosis, treatment and management of myeloma recommend: The long-term use of bisphosphonates for all myeloma patients requiring treatment for their myeloma, whether or not they have myeloma bone disease Bisphosphonates should be used with caution in patients who have kidney problems Patients who have allergic reactions or who have a contraindication to bisphosphonate treatment should not take them Which bisphosphonate should I take? All the different types of bisphosphonates are effective in treating myeloma bone disease and hypercalcaemia, although some may be more potent than others. The national Myeloma IX study compared the effects of Bonefos versus Zometa in newly diagnosed myeloma patients. It is the only study done to date comparing oral and IV bisphosphonates in conjunction with anti-myeloma treatment. At the end of the study, the data showed that: Patients who received Zometa had fewer fractures, lytic lesions and other symptoms of myeloma bone disease than those receiving Bonefos The remission period was longer in patients receiving Zometa than those receiving Bonefos 24

24 Overall survival was greater in patients receiving Zometa than those receiving Bonefos Based on these results, national guidelines now recommend all newly diagnosed myeloma patients be given Zometa. However, when discussing the different bisphosphonate options, you and your doctor may consider that an alternative one is more appropriate depending on your situation and preferences. It is important to have this conversation so that the right choice is made. If you have any questions or queries in relation to the above, please contact your doctor in the first instance for clarification or call our Myeloma Infoline on or from Ireland to speak to one of our Myeloma Information Specialists. Do bisphosphonates have an anti-myeloma effect? It has been suspected for some time that certain bisphosphonates may have an anti-myeloma effect. Indeed, data from the Myeloma IX study described above indicates that this may be the case. In the study, Zometa was shown to have certain benefits over Bonefos. The study participants included patients with and without myeloma bone disease. Improvements in the length of remission and overall survival were seen in patients treated with Zometa who did not have myeloma bone disease, as well as in those with myeloma bone disease. This suggests that Zometa may have an anti-myeloma effect in newly diagnosed myeloma patients, as well as preventing myeloma bone disease. More research is required to further study the anti-myeloma effects of Zometa. For example, although it exerts a potential survival benefit in newly diagnosed myeloma patients, it is not known whether Zometa has the same effects in relapsed patients. Myeloma Infoline: UK Ireland

25 How long does bisphosphonate treatment last? There is no rule to the length of time you have bisphosphonate treatment. This is usually at the discretion of your doctor but will also depend on a number of factors, for example, if you are in remission; have kidney problems or if you require dental treatment. Generally speaking, it is recommended you have bisphosphonate treatment for a minimum of two years. 26

26 Management of pain associated with myeloma bone disease Bone pain is the most common symptom of myeloma bone disease. If left untreated or unmanaged, it can become debilitating and have a major impact on your quality of life. Managing the pain associated with myeloma bone disease is therefore a priority. It is important you tell your doctor or nurse of any pain you have, describe it as clearly as possible and explain how it affects you. This way the best approach to managing your pain can be devised. The following describes some of the treatments and procedures for managing pain associated with myeloma bone disease: Treating the underlying problem Pain caused by myeloma bone disease is often relieved by treatment of the myeloma itself. A response to anti-myeloma treatment is a major factor in reducing progression of myeloma bone disease, easing pain and improving quality of life. Radiotherapy Radiotherapy applied to a particular area may be helpful if you have localised severe pain. Radiotherapy kills off the myeloma cells in the bone, which in turn reduces bone pain. Pain relief from radiotherapy is sometimes more rapid than with drug treatment and may be given first. For more information see the Radiotherapy Infosheet from Myeloma UK. To order your free copy call the Myeloma Infoline on or from Ireland. This information is also available to download at Myeloma Infoline: UK Ireland

27 Pain-killers (analgesics) There are many different types of pain-killer used in myeloma. They broadly fall into the following categories: Over-the-counter pain-killers such as paracetamol for mild pain Weak opioids such as low-dose tramadol, co-codamol, codeine for moderate pain Strong opioids such as morphine, oxycodone, fentanyl for severe pain Nerve-type pain-killers such as gabapentin, pregabalin and amitriptyline When you are taking pain-killers, it is important that your doctor finds one that works best for you as no two patients are alike. You should tell your doctor if a particular pain-killer is not working or if you have side-effects so that another can be tried. It is usual to start with a low-dose or a milder pain-killer and increase to the maximum dose if need be, or to find the best combination of pain-killers that gives a balance between sufficient pain control and tolerable side-effects such as constipation and tiredness. Pain-killers can be given in a variety of forms by tablet, injection or patches where they are absorbed through the skin. Non-steroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen (Nurofen ) and diclofenac are common pain-killers, but should be avoided, particularly if you have kidney damage. Even for over-the-counter medicines, it is always best to check with your doctor regarding the best pain-killer to use. Nerve blocks such as gabapentin or pregabalin are sometimes used to help relieve pain by preventing pain signals from getting to the brain. 28

28 Surgical interventions These can be used to help pin or strengthen areas of bone that have fractured or are in danger of fracture. Surgery can also be used to help treat spinal cord compression and relieve pressure on the nerves surrounding the spine. Percutaneous Vertebroplasty and Balloon Kyphoplasty Percutaneous Vertebroplasty and Balloon Kyphoplasty are two relatively new surgical procedures developed to treat fractures of the spine. Both procedures are normally performed by a specialist spinal surgeon or interventional radiologist and can be done either under a local or general anaesthetic. Percutaneous Vertebroplasty is used to repair a compression fracture in one or several vertebrae and to relieve pain. It involves the injection of a small amount of bone cement through a hollow tube (cannula) into the vertebra to restore its strength. Up to two or three vertebrae can be treated at one time. Balloon Kyphoplasty is a similar procedure but in addition to stabilising, aims to reshape and restore the height of the damaged vertebra. This is achieved by inserting a balloon into the fractured vertebra and inflating it before the cement is inserted (see Figure 10 on page 30). This helps restore the vertebra to its original shape, before strengthening with cement. Although similar, Percutaneous Vertebroplasty and Balloon Kyphoplasty are not interchangeable and are indicated for different clinical situations. Therefore, doctors are very careful in deciding who might benefit from these procedures. In general, more conservative treatments for back pain will usually be tried first. Percutaneous Vertebroplasty or Balloon Kyphoplasty may then be suggested depending on the location of the pain, the type of vertebral compression fracture and the time elapsed since the fracture occurred. Myeloma Infoline: UK Ireland

29 Figure 10. Balloon Kyphoplasty A balloon is inserted into the centre of the compressed bone through a tiny tube The balloon is inflated, elevating the collapsed section The cavity is filled with bone cement The bone cement stabilises and preserves the reestablished height The following criteria apply to both procedures when selecting patients: Conventional treatment for relieving bone pain such as pain-killers and radiotherapy must be tried first Pain must have persisted for more than two months following conventional treatment Other causes of pain must be excluded Severely compressed vertebrae cannot be treated with these techniques These procedures must usually take place within 12 months of the collapse occurring 30

30 You may not be suitable for treatment because of other conditions, e.g. if the collapsed vertebra is causing nerve (neurological) problems, or if you have a bleeding disorder As Percutaneous Vertebroplasty and Balloon Kyphoplasty are relatively new procedures, they are not yet available in every NHS hospital. However, more and more surgeons and radiologists are being trained to carry out these procedures and availability is improving all the time. Recommendations are available from NICE (The National Institute for Health and Clinical Excellence) on the use of Percutaneous Vertebroplasty and Balloon Kyphoplasty. These are available on the NICE website For more information see the Percutaneous Vertebroplasty Infosheet and Balloon Kyphoplasty Infoguide from Myeloma UK. To order your free copies call the Myeloma Infoline on or from Ireland. This information is also available to download at Myeloma Infoline: UK Ireland

31 Non-medical treatments There are many non-medical treatments that can be used to help relieve your pain. The most common of these include: TENS machine and acupuncture: These techniques are used to stimulate nerves to the brain to make the body release its own pain-killers, called endorphins. They can be useful in treating specific areas of pain. Hot and cold compression packs: Hot water bottles and ice packs can be effective short-term pain relievers. It is best not to place them directly on the skin, and you may need to alternate between hot and cold. Relaxation techniques: Meditation, visualisation, relaxation or a combination of these can be helpful in relieving pain. Positioning: The way you sit or lie down can affect your pain. Move to get comfortable, use supportive pillows and ask for help from a family member if you need it. Bracing: An orthopaedic brace may sometimes be used to relieve pain associated with vertebral fractures or to stabilise areas where there is risk of fracture. Massage: This can help with both pain and relaxation. However, make sure it is gentle and not too vigorous. Diversion therapy: Watching TV, listening to music or chatting to a friend won t make your pain go away but it may distract your attention for a while. 32

32 Managing problems with mobility Exercise Exercise can help maintain fitness, strength and boost feelings of wellbeing. However, having myeloma may make exercise more difficult because of the effects of myeloma bone disease and also the side-effects of treatment, muscle weakness and fatigue. Although there is a lack of research on the benefits of exercise in myeloma patients, studies have shown that it can help strengthen bones in osteoporosis patients. Exercise can benefit you in many ways. It can: boost your energy levels; reduce fatigue; help you maintain a range of movement in your joints and muscles; decrease anxiety and improve your appetite. The type of exercise you do will depend on the extent of your bone disease and the amount of pain you have. Generally, low-impact gentle exercise, such as walking, swimming or cycling, is recommended. High-impact exercise, such as jogging, golf or contact sports, is not usually recommended. It is important to talk to your doctor or nurse before taking part in any new exercise or sport to make sure you are not putting yourself in danger. For more specific advice, your doctor may be able to refer you to a physiotherapist. Help with mobility There are a variety of aids such as walking sticks and zimmer frames to help if you have mobility problems or if you are worried about falling. Discuss this with your GP, doctor or nurse they can refer you to a physiotherapist or occupational therapist. If your walking difficulties are permanent or long-term, you can get a wheelchair from the NHS. Details of local wheelchair services are available from your GP, local health centre and the physiotherapy or occupational therapy departments of your local hospital. Wheelchairs can also be hired from the British Red Cross ( , Myeloma Infoline: UK Ireland

33 There are many disability associations that have information about more general travel issues for those with mobility problems. A good starting point is the Royal Association for Disability and Rehabilitation (RADAR) ( , Benefits This allowance is paid to people under 65 years of age, who have difficulty with personal care or have mobility problems. This allowance is tax-free and not meanstested. It is made up of two components care and mobility, both of which have different rates depending on your individual circumstances. Some people are entitled to receive just one component, others will receive both. A medical assessment might be necessary. If you are awarded DLA at the higher rate for mobility, the vehicle you use may be exempt from road tax. The Blue Badge Scheme The Blue Badge Scheme provides a range of parking concessions for people with severe mobility problems who have difficulty using public transport. The Blue Badge enables badge holders to park close to where they need to go. The Scheme operates throughout the UK; however, parking concessions may be different depending on where you live in the UK. Contact your council to apply. 34

34 The future Much research is ongoing to further understand the complex relationship between myeloma cells, bone cells and the bone microenvironment. By understanding the key players involved in myeloma bone disease, it may be possible to find treatments that can disrupt these mechanisms. This may lead to better ways of reducing or preventing myeloma bone disease. An example of a new treatment approach is with a drug called denosumab, currently the subject of a number of clinical studies around the world. Denosumab is an antibody which binds specifically to a protein called RANKL that has been identified as a critical player in breaking down bone. By binding to RANKL, denosumab prevents it from activating osteoblasts and the process of bone breakdown is reduced. Therefore, the net loss of bone in myeloma is halted. Research is also ongoing to determine whether denosumab has an anti-myeloma effect. More generally, much emphasis is being placed on understanding the genetics of myeloma. It is hoped that this information may identify features that underlie myeloma bone disease that can be used to help predict a patient s response to treatment. In future, this may lead to more personalised treatment options for myeloma bone disease as well as to the development of newer and more effective treatments for it. Myeloma Infoline: UK Ireland

35 Self-help checklist Report any new symptoms to your doctor Report pain to your doctor or nurse so that it can be treated Take pain-killing drugs as prescribed It is useful to keep a record of the pain-killers you have been taking to show your doctor or nurse If your usual combination of pain-killers becomes less effective, contact your doctor or nurse If you are on oral bisphosphonates, such as Bonefos or Loron, take them as prescribed, avoiding food an hour before and after you take them Try to take regular gentle exercise talk to your doctor if you are worried about the risks of exercise or are considering trying something new Check to see if you are entitled to any benefits or financial assistance because of mobility problems If you are having problems walking around and carrying out your usual daily activities talk to your GP. You may be able to improve your mobility with help and support from specialist healthcare professionals If you are seeing an orthopaedic surgeon or radiotherapist make sure they are liaising with your myeloma specialist and keeping each other informed of changes in your condition or treatment 36

36 Medical terms explained Anaemia: A decrease in the number of red blood cells or haemoglobin level in the blood. Antibodies: Also known as immunoglobulins, antibodies are proteins produced by certain white blood cells (plasma cells) to fight infection and disease. Bisphosphonate: A type of drug which binds to bone and protects the bone from the being broken down by osteoclasts. Commonly used bisphosphonates include sodium clodronate (Bonefos), disodium pamidronate (Aredia) and zoledronic acid (Zometa). In myeloma, these drugs may be used to treat hypercalcaemia. Bone marrow: The soft spongy tissue in the centre of bones where red blood cells, white blood cells and platelets are produced. Bone remodelling: The lifelong process whereby mature bone tissue is removed from the skeleton and new bone tissue is formed. Hypercalcaemia: A higher than normal level of calcium in blood. In myeloma patients, this is usually due to bone destruction and the release of calcium into the bloodstream. Immunoglobulins: Also known as antibodies, immunoglobulins are proteins found in the blood which are produced by cells of the immune system, called plasma cells. Their function is to bind to substances in the body that are recognised as foreign antigens found on the surface of bacteria and viruses. By tagging the antigens, immunoglobulins enable other cells of the immune system to destroy and remove them, thereby helping to fight infection. Immune system: The complex group of cells and organs that protect the body against infection and disease. Myeloma Infoline: UK Ireland

37 Interventional radiologist: Interventional radiologists specialise in invasive procedures that can biopsy internal organs, open blocked arteries and veins, drain abscesses and cysts and treat many other conditions and/or disorders. Interventional radiologists are often involved in the treatment of myeloma bone disease. In many interventional radiology procedures, patients are treated on an outpatient basis and are back to their normal routines quickly. Intravenous (IV) injection: An injection into the vein. Kyphosis: An abnormal curvature of the spine. Lytic lesions: The damaged area of bone that shows up as a dark spot on an X-ray and is evidence that the bone is being weakened. Maintenance treatment: Treatment (often lower dose) given over an extended period of time after the main standard dose of treatment has finished. Malignant: Cancerous cells which have the ability to invade and destroy tissue Osteonecrosis of the jaw (ONJ): A condition in which the bones of the jaw do not heal properly causing ongoing, sometimes painful, complications. Paraprotein: An abnormal immunoglobulin (antibody) produced by myeloma cells and measured in the blood. Measurements of paraprotein are used to monitor disease. Pathological fracture: A broken bone caused by the weakening of bone as a result of disease. Often the break occurs spontaneously or with little injury. Plasma cell: A specialised type of white blood cell that produces immunoglobulins to fight infection. Myeloma cells are cancerous plasma cells that do not function properly. Plasmacytoma: A collection of myeloma cells found in a single location in the bone marrow, bone or soft tissue. 38

38 Platelets: Small blood cells involved in blood clotting. Quality of life: A term that refers to a person s level of comfort, enjoyment and ability to pursue daily activities. It is a measure of an overall sense of wellbeing. Red blood cells: Blood cells that transport oxygen around the body. Relapse: The point where myeloma returns or becomes more active after a period of remission or stable disease. Side-effects: Problems that occur when treatment affects healthy cells. Common side-effects of standard cancer treatments are fatigue, nausea, vomiting, decreased blood cell counts, hair loss and mouth sores. Stem cells: The cells from which all blood cells develop. Normal stem cells give rise to red blood cells, white blood cells and platelets. Stem cells are normally located in the bone marrow and can be harvested for transplant. Stromal cells: Cells in the bone marrow that are not directly involved in the blood cell making process but instead generate bone, cartilage, fat and connective tissue. Supportive treatment: Treatment intended to relieve symptoms and complications rather than treating the myeloma. Vertebra: A bone which forms part of the spinal column. White blood cells: Blood cells involved in the body s immune system. Myeloma Infoline: UK Ireland

39 Further information and useful organisations United Kingdom Benefit Enquiry Line (Monday Friday, 8.30am 6.30pm; Saturday 9am 1pm) The Benefit Enquiry Line is a confidential advice and information service for people with disabilities, and their carers and representatives. The enquiry line provides information about social security benefits and how to claim them, and can provide assistance, over the phone, with filling out benefit application forms. Blue Badge Scheme (Blue Badge Helpline; Monday Friday, 9am 5pm) The Blue Badge Scheme provides a national arrangement of on-street parking concessions enabling people with severe walking difficulties who travel, either as drivers or passengers, to park close to their destinations. To apply for a badge, contact the Social Services Department (or in Scotland the Social Work Department) of your local authority or council. British Association for Counselling and Psychotherapy (BACP) (General enquiries; Monday Friday, 8.45am 5pm) (Client Information Helpline; Monday Friday, 8.45am 5pm) BACP provides advice on a range of services to help meet the needs of anyone seeking information about counselling and psychotherapy. To find a local counsellor call the Client Information Helpline, or use the search facility on their website. British Red Cross (General enquiries; Monday Friday, 9am 5pm) Volunteers assist with a range of local services including care in the home, transport and medical loans to help those with health issues lead a full and independent life. The Medical Equipment Service has a wide range of products and equipment available for short-term loan. The Home from Hospital Service provides short-term practical assistance and support to help people settle back into their own homes. A Transport and Escort Service offers help to people who cannot get about easily or use ordinary transport. 40

40 Cancer Black Care (Monday Friday, 9am 5pm) Cancer Black Care provides a unique service of information, advice and support for the black and minority ethnic community. The Cancer Counselling Trust (Monday Friday, 9am 5pm) The Cancer Counselling Trust offers free telephone counselling across the UK. It supports cancer patients, their families, friends and care givers who seek counselling to help them through the difficult issues precipitated by a cancer diagnosis. It provides free, specialist counselling for anyone impacted by cancer, across the UK. Cancer Research UK (Nurse information line; Monday Friday, 9am 5pm) CancerHelp UK is the patient information website of Cancer Research UK. It provides a free information service about cancer and cancer care for patients and their families. Carer s Allowance Unit (Switchboard, ask for the Carer s Allowance Unit; Monday Thursday, 9am 5pm; Friday 9am 4.30pm) General information about the carer s allowance, and assistance with filling in the application form. Carers UK (Wednesday and Thursday, 10am 12noon, 2pm 4pm) Carers UK provides advice, information and support for carers. It produces a directory of national and local carer organisations and can show you where to get help in your area. Myeloma Infoline: UK Ireland