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Family Health Magazine - FAMILY MEDICINE

Multiple Myeloma
Treatment and hope

Multiple myeloma is a cancer of the bone marrow, the jellylike substance within large bones that makes new blood cells. Plasma cells are one type of cell in marrow. As part of the immune system, their job is to produce special proteins to prevent infection. In myeloma, these cells begin to grow and no longer follow their normal pattern. As with most other cancers, the problem is not rapid growth of cells. Rather, it is the high number of abnormal cells that appear when normal cells do not go through the full cycle of maturing and death.

Myeloma is related to other disorders of the bone marrow, including leukemia and lymphoma. Think of them as step­siblings – related but without the same parents.

With this group of disorders, an illness may progress over years from slightly more plasma cells to a single cluster of abnormal cells to full myeloma.

How common is it?

Myeloma is one of the more common blood cancers. Depending on ethnic background, it is generally the second most common bone marrow cancer after non-Hodgkin’s lymphoma. In 2009, 2200 Canadians were diagnosed with myeloma and 1400 died of the disease. The rate in men is about 1.5 times as high as in women. Those of African heritage are affected more often than people of other races. This fact may relate to both genetic and environmental factors. As with many cancers, it is most common in older patients, often appearing in and after the seventh decade of life.

What causes it?

No simple answer to this question exists for myeloma or any cancer. We believe that changes to normal control of cell growth leads to increasing numbers of cancerous cells. Those working in agriculture, certain industries or exposed to high levels of radiation as at a nuclear plant are thought to be at higher risk. While myeloma may run in a family, this is unusual as it is not an inherited disease.

What are the symptoms?

Myeloma has a wide range of symptoms. Sometimes it is found during routine blood tests. Fatigue, bone pain or sudden fractures, easy bruising and bleeding, and recurrent infections are common. Unfortunately, these symptoms are not limited to myeloma. It is not unusual to have tests done for weeks or even months before diagnosis.

This condition must be handled carefully. Unlike other bone marrow cancers, myeloma cells contain a protein that triggers bone thinning and breakdown. In someone with myeloma, even a small trauma may cause a fracture. As well, extra abnormal proteins from the myeloma cells can damage the kidneys. Since myeloma cells grow in marrow space, they may crowd out other cells. Having too few red blood cells can cause anemia, while too few white cells allows infection. Bruising may come from a lack of platelets, the cells which help blood clot. Managing myeloma involves all of these concerns.

Diagnosing myeloma

In most cases, blood and urine tests are done along with bone x-rays. A bone marrow test is usually done. Here, a small amount of bone marrow is removed with a needle from the back of the pelvic bone. Bone marrow testing may not be done in the very elderly or those for whom aggressive therapy is not appropriate. A number of other tests are also possible, including specialized imaging tests (CT scans, magnetic resonance scans, and other X-ray tests) and genetic analysis of the myeloma cells.

Both treatment and survival rates depend on the stage of the cancer. At one time, survival rates were less than two or three years. Happily, new treatments are more effective. Now, average survival ranges from four to five years. Forty per cent of patients survive 10 years. While not perfect, this improvement is due to intensive research and new treatments.

What happens next?

Both treatment and survival rates depend on the stage of the cancer. At one time, survival rates were less than two or three years. Happily, new treatments are more effective. Now, average survival ranges from four to five years. Forty percent of patients survive ten years. While not perfect, this improvement is due to intensive research and new treatments.

Since treatment depends on the type of disease, stage, age, and other factors, the description here is only a guide. Ask questions and discuss your treatment goals with your doctor. It may help to have a support person with you, as it can be difficult to absorb all of the complex information.

At first, treament must control immediate threats such as anemia, decreased kidney function, and high calcium in the blood from bone breakdown. Bone injuries or fractures may be causing pain. A radiation oncologist (a doctor who uses radiation to treat cancers) can often rapidly improve bone lesions. Protecting your kidneys is vital. Drinking lots of fluids helps kidney function. You must also avoid over-the-counter painkillers and non-steroidal anti-inflammatories, as they interfere with the kidneys. Check with your doctor before taking any pain relievers other than acetaminophen.

If you have diabetes, you are at particular risk of kidney problems. Tell your diabetes team about the myeloma immediately.

Treatment of myeloma has changed dramatically in the last 15 years. Stem cell transplants and three new drugs - thalidomide, bortezomib, and lenalidomide – have made a big difference. Unless you are not a candidate for aggressive therapy, seek help from a centre or doctor specializing in myeloma and related disorders. Hematologists (doctors who specialize in blood disorders), oncologists (doctors who specialize in cancer care), and many internal medicine specialists are experts in this area. As well, they can often quickly refer you to other specialists such as radiation oncologists.

Autologous stem cell transplant, which uses your own stem cells, is an option for many. It particularly helps those under the age of 65 to 70. There is nothing magical about this procedure. It uses high dose chemotherapy to try to lower the number of myeloma cells in the body, while adding stem cells to lessen side effects from low blood counts. Using stem cells is like removing healthy seeds from a garden overgrown with weeds before cleaning it out with a can of gasoline and a match. The saved seeds re-establish the garden once the bad cells have been killed. The procedure cannot cure myeloma. Instead, it attempts to reduce the number of tumour cells and give a partial or complete remission. Most often, responses last about two to three years.

The introduction of thalidomide, bortezomib, and lenalidomide has added to our treatment options. Thalidomide has a bad reputation because of the birth defects it can cause. However, it is very useful in treating several kinds of cancers. Provided that great caution is taken to avoid pregnancy, it can be very beneficial. Side effects include loss of sensation and strength of peripheral nerves in the hands and feet, and some degree of sedation. Thalidomide is usually combined with other drugs, such as the steroid drugs prednisone and dexamethasone. Although it is not licensed here, your doctor can obtain it by making a special application to Health Canada.

Bortezomib (Velcade®) is given both at diagnosis and after stem cell transplant. It seems particularly useful for those with kidney problems or whose myeloma has specific genetic factors. However, many patients receive bortezomib at some point. It should be carefully monitored to avoid potential nerve damage. If this happens, it can be more painful than with thalidomide. In many cases, bortezomib is combined with other drugs, usually steroids or melphalan. Bortezomib is licensed in Canada for the treatment of myeloma.

The newest drug available, lenalidomide (Revlimid®), is a much more potent derivative of thalidomide. It is usually used along with steroid drugs (prednisone or dexamethasone). Generally it is given after initial therapy, although its role at the beginning of treatment is being studied. Lenalidomide does not cause nerve damage. However, blood counts may drop and it must be used very carefully in patients with kidney impairment. As it comes from thalidomide it may cause birth defects, so avoiding pregnancy is crucial. Anyone treated with lenalidomide must be registered in a safety program provided by the manufacturer. Finally, like thalidomide, lenalidomide has a risk of blood clots. Those taking it must be aware of this possible complication. Lenalidomide is licensed in Canada for the treatment of myeloma.

Various research drugs are also being assessed. Talk to your doctor about alternatives. Participating in a clinical trial offers the chance to receive a new treatment, while adding to knowledge of the disease.

Additional treatment

Like all patients with bone marrow cancers, myeloma patients are at risk of infection, bleeding, and anemia. Be aware of these complications and discuss them with your doctor. It is critical that you advise other members of your health care team, including your dentist. Mention the possible effects of myeloma on kidney function and bones.

Bisphosphonate drugs can be used to strengthen bones. The most commonly used version is called pamidronate. Be sure to tell your dentist, as certain steps must be taken to avoid complications following dental work.

Some patients with anemia, especially combined with kidney problems, may benefit from drugs that stimulate red blood cell production. These must be used carefully to ensure that blood count does not rise too quickly or too far. A specialist physician should always supervise their use.

Controlling pain is important, and narcotic painkillers may be necessary. Talk to your doctor about drugs you can safely use. It is very important to avoid non-steroidal painkillers – your doctor will know which ones they are. As with any serious disease, it is always wise to check with a doctor or pharmacist before taking any medication.

Hope for the future

Although myeloma is generally not considered curable, the outlook has improved in the last 20 years. Stem cell transplant, supportive medications, and the new drugs described and even newer treatments being researched may allow patients a longer and better quality of life. For now, the best approach is to understand the disease, discuss treatment and potential problems with your doctor, ask questions when necessary, and actively participate in your own care. Informed patients make better decisions.

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