 |  |  | | | THE NURSING MANAGEMENT OF MYELOMA | | | | |
| |  | |  | THE NURSING MANAGEMENT OF MYELOMA |
There are 3,300 new cases of myeloma each year in the UK. Patients are monitored long term with treatment determined according to physical condition and disease progression. Nursing care involves both supportive and definitive approaches of management
 | Author biography |
|  | | Monica Morris is Clinical Information Manager for CancerHelp UK, a patient information website by Cancer Research UK. Her role is to manage the production and review of the information on the site. Monica is a specialist oncology nurse, with a background in haematology, bone marrow transplant and psychosocial support of patients. Her interest in patient information spans her nursing career, and she has been responsible for producing written information in previous clinical posts. Her most recent role was at the International Myeloma Foundation UK, a national charity for those affected by myeloma. There she worked as a myeloma information and support specialist nurse, which included managing the patient information services within the charity. Monica co-founded and continues to help run the London myeloma patient support group. |
A diagnosis of myeloma is devastating. In a short space of time, newly diagnosed patients are faced with trying to understand a complicated disease, battle with often traumatic and debilitating symptoms and complications, and make decisions about treatment options that are increasingly complex. All this is made harder because most patients have never heard of myeloma before.
As well as providing treatment, care and support, nurses play a crucial role in helping patients to understand their disease and to make informed decisions about treatments. The following information provides a brief overview of myeloma for nurses, and highlights some key aspects of the nursing care of patients with myeloma
What is myeloma?
Myeloma is a relatively rare cancer of the plasma cells. In myeloma, a single defective plasma cell (a myeloma cell) multiplies rapidly, disrupts the immune system and crowds out normal haemopoietic cells in the bone marrow. This leads to bone marrow failure, with associated anaemia and immunosuppression. Myeloma cells have an affinity to bone, leading to bone damage and pain – the most common and distressing factor of the disease. The disease is characterised by periods of treatment and varying periods of remission. It is a heterogeneous disease, and can progress at different speeds in individual patients.
Aetiology and epidemiology
The causes of myeloma are uncertain, although it is thought that exposure to certain chemicals, viruses and a weakened immune system may be trigger factors. Exposure to radiation and benzene has traditionally been linked to myeloma, but the evidence is not conclusive (Joshua and Gibson 2002).
There are about 3700 new cases of myeloma diagnosed in the UK annually (Cancer Statistics Registrations 2003). The median age at diagnosis is 70 years with 2% of cases under 40 years (UKMF and NMSG 2005).
Symptoms
The most common presenting symptoms of myeloma are due to bone disease. They include:
 | pain |
| hypercalcaemia |
| osteoporosis |
| pathological fractures |
| loss of height |
| spinal cord compression |
Other symptoms relate to:
Impaired bone marrow function and immune paresis, causing
| recurrent or persistent infections |
| anaemia and associated fatigue |
| neutropenia and/or thrombocytopenia |
High levels of paraprotein in the blood and urine, causing
| renal dysfunction |
| raised plasma viscosity |
Prognostic indicators
High serum levels of β2-microglobulin and C-reactive protein, and low levels of albumin, are associated with poorer survival. Cytogenetic abnormalities are strong prognostic factors. Deletions of chromosome 13 and certain translocations of chromosome 14, t(4;14), t(14;16), are adverse prognostic indicators (UKMF and NMSG 2005).
Staging
The Durie/Salmon Staging System (Durie 1975) was devised in the 1970s to stage myeloma. In this system patients were put into one of three stages depending on their haemoglobin, calcium and paraprotein levels. The patient’s bone damage, renal impairment and myeloma cell mass were also taken into account.
A newer staging system uses B2 microglobulin and serum albumin to further differentiate patients. This is known as the International Prognostic Index (Greipp 2003). This is now recommended in preference to the Durie/Salmon staging system (UKMF and NMSG 2005).
Treatment
Myeloma is not yet considered curable, but is an increasingly treatable condition. Treatments are focused on:
| controlling the disease |
| prolonging survival |
| improving symptoms so that patients can live with myeloma and have the best possible quality of life. |
With current treatment the median survival is approximately 3-5 years (NICE 2003).
Patients with no symptoms (Stage IA or asymptomatic myeloma) may remain stable for a long period with no treatment. Treatment is only indicated when there is evidence of progression, or symptoms of bone disease. Regular monitoring of this group is required.
For patients who require treatment the standard treatment options include:
Standard chemotherapy
Alkylating agents such as melphalan and cyclophosphamide are effective in the treatment of myeloma. In older or less fit patients, who are not going on to have high dose therapy with stem cell transplantation the aim is to achieve the best response possible with the fewest side effects. A course of melphalan or cyclophosphamide, with or without steroids, is often used. Patients can have this orally or IV (intravenously).
If a subsequent stem cell transplant is planned, a variety of chemotherapy combinations are used. For example VAD (vincristine, adriamycin and dexamethasone), or related regimes such as VAMP or C-VAMP. Because these regimes need a central line, alternative oral regimes such as Z-Dex may be considered. A recent study showed that Z-Dex may be a suitable oral alternative to VAD but it did not provide evidence for equivalence (Cook et al 2004).
High-dose therapy and autologous stem cell transplant
This treatment uses high doses of chemotherapy to consolidate standard chemotherapy. It avoids permanent damage to blood cell production because previously collected healthy stem cells are returned to the patient after high dose chemotherapy. This effectively ‘rescues’ the patient’s bone marrow and means normal healthy blood cell production can continue (IMF UK, 2004). High dose therapy is often the treatment of choice for patients under 60 years, but can be considered in people up to the age of 70. Recent studies have shown a survival benefit of about one year against standard chemotherapy alone (Child et al 2003).
Allogeneic transplants, usually with a sibling donor, are a potential option for a small group of younger patients (<50 years of age), and should be performed within the context of a clinical study. Reduced intensity conditioning (RIC) transplants have lower toxicity and transplant-related mortality, and may be considered in older patients (<70 years), again, within the context of a clinical trial.
Thalidomide
This treatment is arguably the most significant advance in the treatment of myeloma in the last 30 years (Cavenagh 2003). Thalidomide was first used as a treatment for relapsed disease, but is now increasingly used as a primary treatment. It is also used as a maintenance treatment following autologous stem cell transplant. Due to its history of teratogenicity, it is prescribed within strict risk management guidelines. A common side effect of thalidomide is peripheral neuropathy. This can mean some patients need to have their treatment reduced or stopped.
Bortezomib (Velcade)
Bortezomib is a targeted biological therapy. It acts on an enzyme complex that regulates cell function called the proteasome. Bortezomib inhibits the proteasome and can induce apoptosis in myeloma cells. Malignant cells are more sensitive to proteasome inhibition than non-malignant cells. Proteasome inhibition can induce apoptosis in myeloma cells that are resistant to conventional chemotherapy. This suggests that it works in a way that is not affected by the mechanisms associated with alkylating agent and steroid resistance (Morgan and Davies, 2005). Bortezomib is licensed for the treatment of progressive myeloma in patients who have received one prior treatment, and have already undergone or are unsuitable for stem cell transplantation.
Supportive Measures
There are various treatments that relieve the symptoms of myeloma.
| Bone damage and pain |
Bisphosphonate treatments have been shown to reduce bone pain, skeletal events, reduce hypercalcaemia and improve quality of life. They are recommended for all patients with myeloma requiring treatment with chemotherapy, whether or not bone lesions are evident (UKMF and NMSG 2005). Effective pain control is a priority of care and there are a variety of pain relieving methods that can be used with good effect.
Chemotherapy can relieve pain by treating the underlying disease. Radiotherapy is also an effective treatment, particularly at the site of painful lytic lesions. Opiate analgesia is often required and can be used alone or in combination with adjuvant methods. The use of NSAID’s (non-steroidal anti-inflammatory drugs) should be avoided due to their association with renal impairment.
Recently, percutaneous vertebroplasty has been used to treat back pain caused by vertebral bone disease, with some success. This procedure involves the injection of surgical cement into the vertebral space of collapsed vertebrae. The National Institute for Clinical Excellence (NICE) has produced guidelines on the use of vertebroplasty (NICE 2003). Spinal cord compression is an oncological emergency and often requires immediate treatment with steroids and radiotherapy (Haas 2003).
| Impaired bone marrow function |
Symptomatic anaemia (caused by both the disease and chemotherapy treatment) is managed by the use of blood transfusion or recombinant human erythropoietin (EPO). Antibiotic therapy, intravenous immunoglobulins, haematopoietic growth factors and vaccinations, are all used to treat or prevent complications associated with immunosuppression.
| Renal impairment |
Adequate hydration (3 litres daily) is essential to both prevent and treat renal impairment (MRC 1984). Other measures include treatment of the underlying myeloma. Sometimes plasmapheresis is needed to remove excess paraprotein from the blood. Up to 50% of patients have some degree of renal impairment (UKMF and NMSG 2005). Renal failure occurs in 3-12% of patients (Clarke et al 1999) and is managed by dialysis.
| Future treatments |
A variety of new therapies to treat myeloma are being studied. These include the thalidomide analogue lenalidamide (Revlimid), skeletal targeted radiotherapy, arsenic trioxide, and vaccines. How well these new treatments fit in with the current management profile is not yet known. The recent expansion in new treatments means the challenge that doctors now face is how to use them most effectively. Here are some of the questions that current clinical studies are hoping to provide answers to:
| Are new treatments better than existing treatments? |
| Which new treatment is most appropriate for an individual patient? |
| At what stage of disease should a patient have a new treatment? |
| Should a new treatment be given alone or in combination with other new or existing treatments? |
| What combination of treatments is most effective? |
Nursing Interventions
Nurses contribute to all aspects of the care of a patient with myeloma. Their role is central to helping patients manage their disease, and maintain the best possible quality of life. Myeloma patients are usually treated by a multidisciplinary team. Nurses often provide the continuity and coordination within the team that is essential for the efficient treatment of patients.
Important aspects of nursing a patient with myeloma include preventing and managing complications (of both the disease and its treatments), and being an accessible source of information and support. To achieve the latter, haematology nurses must keep up-to-date with the increasing number of treatment options for myeloma.
Optimum nursing management of a patient with myeloma will include:
| Provision of relevant, high-quality, up-to-date information, in a variety of formats, at all stages of their disease |
| Expert pain assessment and control |
| Education of patients about the need for adequate hydration |
| Prompt recognition of the signs of hypercalcaemia, spinal cord compression and sepsis |
| Recognition and management of fatigue |
| Prevention or management of constipation |
| Recognition and management of side effects, such as peripheral neuropathy, that occur with existing and new treatments |
| Provision of psychological and social support for patients and their families |
| References |
Cancer Statistics Registrations MB1 33, 2002
Cavenagh et al. Thalidomide in multiple myeloma: Current status and future prospects. Br J Haem. 2003 Jan 120(1) 10-17
Child J A, Morgan G J, Davies F E, Owen RG, Bell SE, Hawkins K, Brown J, Drayson MT, Selby PJ. High Dose Chemotherapy with Haematopoietic Stem Cell Rescue for Multiple Myeloma. N. England Journal of Medicine. 2003; 348: 1875-1883
Clarke, A.D., Shetty, A. & Soutar, R. (1999) Renal failure and multiple myeloma: pathogenesis and treatment of renal failure and management of underlying myeloma. Blood Reviews. 13. 79-90.
Durie BGM, Salmon SE. A clinical staging system for multiple myeloma. Cancer. 1975;36: 842-854
Cook G, Clark RE, Morris TC, Robertson M, Lucie NP, Anderson S, Paul J, Franklin IM. (2004) A randomized study (WOS MM1) comparing the oral regime Z-Dex (idarubicin and dexamethasone) with vincristine, adriamycin and dexamethasone as induction therapy for newly diagnosed patients with multiple myeloma. British Journal of Haematology, 126 (6) 792-8
Durie B.G.M et al (2003). Myeloma Management Guidelines. A Consensus Report from the Scientific Advisors of the International Myeloma Foundation.
Haas, F (2003). Management of malignant spinal cord compression. Nursing Times. 99 (15) p32
International Myeloma Foundation (UK) 2004 High dose therapy and Stem Cell transplantation in Myeloma – Your essential guide
Joshua D.E, Gibson J (2002). Epidemiology of plasma cell disorders. In: Mehta J, Singhal S (eds) Myeloma. London: Martin Dunitz, UK
Medical Research Council Working Party on leukaemia in adults. (1984) Analysis and management of renal failure in 4th MRC Myelomatosis trial. British Medical Journal. 288, 1411-16
Morgan G J and Davies F E (2005). Evolving treatment strategies for myeloma. British Journal of Cancer. 92, 217221.
NICE Improving Outcomes in Haematological Cancer Manual 2003
National Institute for Clinical Excellence. (2003) IPG 012 Percutaneous vertebroplasty, guidance.
UK Myeloma Forum (2001) The diagnosis and management of multiple myeloma. British Journal of Haematology; 115: 3, 522-540.
UK Myeloma Forum and Nordic Myeloma Study Group (2005) Guidelines on the diagnosis and management of multiple myeloma, 7 August 2005 - accessed on (BCSH) website British Committee for Standards in Haematology
| Further reading |
Mehta J, Singhal S (ed’s) 2002 Myeloma. Martin Dunitz, UK
Gahrton G, Durie B.G.M, Samson D, (ed’s). 2004. Multiple Myeloma and Related Disorders. Arnold
Malpas, James S.D.E, Bergsagel, R. A. Kyle, et al, (eds). Myeloma: Biology and Management. 2nd ed. Oxford: Oxford University Press, 1998.
| Websites |
International Myeloma Foundation UK
International Myeloma Foundation
UK Myeloma Forum
Multiple Myeloma Research Foundation
CancerHelp UK - the patient information website of Cancer Research UK
BCSH guidelines
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