This article will highlight the Best Practices in Multiple Myeloma presented at the Academy of Oncology Nurse & Patient Navigators (AONN+) conference in May 2018. It will provide an overview of the diagnosis and treatment of multiple myeloma (MM). It will show the value of an oncology nurse navigator (NN) in helping patients with MM decrease barriers to care and stay on course. The article will also share the patient's perspective in undergoing therapy.
MM is a cancer of the plasma cell originating in the B-cell line, which is a type of white blood cell that is responsible for the production of antibodies that fight infection. It is a complex disease. In MM, the cells release too much protein (called immunoglobulin) into the bones and blood. As MM worsens, those plasma cells begin to spill out of the bone marrow and spread through the body. As the plasma cells build up in the body, they interfere with the immune system, causing organ damage and disruption of normal bone formation.
Approximately 30,000 new cases of MM are diagnosed each year. The median age for the development of MM is 69 years, and it affects African Americans more than it does white Americans and occurs more in males than females. The current 5-year survival rate is about 50%, which is significantly better than in the past. The cause of MM is unknown. Risk factors include drinking alcohol, obesity, radiation exposure, family history, and exposure to certain chemicals such as pesticides and herbicides. It is an incurable disease, but it is highly treatable because of the recent developments of many new therapies. Despite this, there are about 12,000 deaths a year attributed to MM.
Clinically, patients may present without any symptoms, and MM is often diagnosed as an incidental finding. Other times patients will present with back pain, fatigue, and anemia, but MM is not usually the diagnosis that comes to mind. MM is diagnosed based on blood or urine tests finding abnormal antibodies, bone marrow biopsy finding cancerous plasma cells, and radiology findings showing bone lesions. Another common finding is high blood calcium levels. Other symptoms may be recurrent infections, renal dysfunction, and plasmacytomas that are single soft mass lesions of the bone marrow.
Diagnostic testing for MM includes blood and urine tests looking for a high calcium level, low hemoglobin and hematocrit, elevated BUN, creatinine, serum protein electrophoresis (SPEP), urine protein electrophoresis (UPEP), and radiology studies. Bloodwork may reveal the M proteins produced by myeloma cells. Another abnormal protein produced by myeloma cells called β2-microglobulin may also be detected; it provides clues about the aggressiveness of the disease. A bone marrow biopsy will also be done to look for plasma cells. Specialized tests, such as fluorescence in situ hybridization, can analyze myeloma cells to understand their genetic abnormalities. Tests are also done to measure the rate at which the myeloma cells are dividing.
Radiology studies may be recommended to detect bone problems associated with MM. Tests may include x-rays of the skeletal system and MRI, CT, or PET scans.
There is a variety of options for the treatment for MM. Treatment depends on the stage of the disease, performance status, and goals of treatment. If the patient has monoclonal gammopathy of undetermined significance or smoldering myeloma, observation may be the treatment, whereas full-fledged MM requires treatment.
Treatment options may include the following:
Biological therapy: These drugs use the body's immune system to fight myeloma cells. They enhance the immune system cells that identify and attack cancer cells. These medications are commonly taken in pill form.
Chemotherapy: Chemotherapy drugs kill fast-growing cells, including myeloma cells. Chemotherapy drugs can be given orally or IV. High doses of chemotherapy drugs are used before a bone marrow transplant.
Corticosteroids: Corticosteroids regulate the immune system to control inflammation in the body. They are also active against myeloma cells. Corticosteroids can be taken orally or be administered IV.
Stem cell transplant: A stem cell transplant is a procedure to replace diseased bone marrow with healthy bone marrow. Before a stem cell transplant, blood-forming stem cells are collected from the blood. Patients receive high doses of chemotherapy to destroy the diseased bone marrow. Then stem cells are reinfused into the body, where they travel to the bone marrow and begin the rebuilding process.
Radiation therapy: This treatment uses beams of energy to damage myeloma cells and stop their growth. Radiation therapy may be used to quickly shrink myeloma cells in a specific area—for instance, when a collection of abnormal plasma cells forms a tumor (plasmacytoma) that's causing pain or destroying bone.
The MM treatment paradigm is exploding, with many new and novel therapies available to treat relapsed or refractory MM. (Table)
The following case study will illustrate the complexity of caring for patients with MM and the importance of a multidisciplinary approach to patient care.
Felix is a 62-year-old Spanish-speaking male who presents to his primary care physician (PCP) with back pain. His PCP orders an MRI study of the spine, and it shows lytic lesions. He also has bloodwork drawn. He is referred to the cancer center for further workup and is connected with the NN, who starts the initial assessment and triage process. Felix speaks limited English. Because of the language barrier, the NN utilizes interpreter services for her intake. The NN finds out that Felix lives 3 hours away from the cancer center. He is married and has 5 children, aged 20 to 38 years, and 4 grandchildren. He is a Jehovah's Witness. He previously worked at a factory, but he had to stop working because of health issues and is on Medicare. His medical history includes diabetes and hypertension. He takes Metformin 1000 mg daily and a thiazide diuretic. He tells the NN that he has had back pain for several months but did not go to the doctor. He complains of fatigue and decreased "stamina." He states he "lost his get-up-and-go" and can no longer play with the grandchildren, which used to bring him a lot of joy. The NN coordinates an appointment with a medical oncologist and provides Felix with all the details of his appointment. The NN will meet him and his family at their medical oncology visit.
The NN identifies the following barriers to care in establishing a care plan for Felix:
Logistical: Felix will need help with transportation and lodging if he receives care at the cancer center. There are many options open to Felix and his family. They decide to stay at a Hosts for Hospitals home close to the cancer center. Hosts for Hospitals provides low-cost lodging and support for patients undergoing medical care. Volunteer hosts open their homes to patients in need of complex care that is unavailable close to home.
Financial: The patient has Medicare parts A and B but no secondary insurance. The NN knows that he will need to secure secondary insurance prior to undergoing a transplant. He does not have a prescription plan to cover the costs of his medication. The NN refers him to the financial advocates at the cancer center to help Felix obtain additional insurance. He tells his NN that he thought he had "good insurance" and would not need additional coverage.
Treatment: The NN assesses that education will be a challenge to help Felix and his family understand his plan of care. Arrangements are made to use interpreter services at all visits. The NN investigates patient educational materials available in Spanish to review with him.
The NN understands that the increase in oral oncolytic usage in MM can be confusing for patients and that the scheduling and timing of medication administration can be confusing. Hence, Felix will need a lot of education and reinforcement of the plan of care.
One of the challenges the NN faced was that she was located at one site and the bloodless medicine program was at another site within the health system, and that site did not have navigators.
Social: Felix has 5 children and several grandchildren. He has been married to his wife for 40 years, and he tells the NN that his family is very supportive. Both he and his wife help with the grandchildren, as all their children and spouses work.
Spiritual/Cultural: Felix and his family are Jehovah's Witnesses. Understanding the beliefs of the Jehovah's Witnesses religion will help the multidisciplinary team provide the best care for Felix by understanding his wishes and goals of care. They strictly follow a passage in the Book of Leviticus that states, "And you must not eat any blood in any place where you dwell...Any soul, who eats blood, that soul must be cut off from his people." Devout Jehovah's Witnesses take this command very seriously—even in the face of cancer treatment.
Jehovah's Witnesses will refuse the transfusion of most blood products, such as packed red blood cells, platelets, white cells, and plasma. They allow the transfusion of stem cells and some fractions of blood, such as albumin and cryoprecipitate.
Communication/Language barriers: The NN must be sure that Felix understands his treatment plan and that both written and oral information are provided in Spanish.
Emotional/Physical health: Felix tells his NN that he has a lot of support but is worried about the stress this will cause for his family. He also expresses that being away from his Jehovah's Witnesses community is a concern.
Felix and his family come in for the initial oncology appointment. He has a complete diagnostic workup, including bloodwork, bone marrow biopsy, and scans. The NN meets with Felix and his family and reviews the plan of care thus far with them. His team has a goals-of-care discussion with Felix and his family. His wishes are aggressive treatment so he can be "grandpa" again and enjoy his grandchildren. He wants to be able to participate in their activities. Because Felix is a practicing Jehovah's Witness, he is connected to the bloodless medicine and surgery team, which will coordinate his care with the NN and prepare Felix for a transplant, assuming his testing confirms a MM diagnosis. A follow-up appointment is scheduled in 1 week.
Felix's diagnostic workup confirms MM with the following results:
- His bone marrow biopsy shows 30% plasma cells with normal cytogenetics
- Elevated calcium
- M spike 3.1 g/dL IgG kappa light chains
- SPEP shows elevated protein levels
- 24-hour UPEP: 3.7 g/24 hr of monoclonal protein
- Elevated β2-microglobulin levels 2.9 mg/dL
A treatment plan is developed at this visit. The team discusses goals of care with Felix and his family and starts induction therapy with bortezomib/lenalidomide/dexamethasone for 3 to 4 cycles, with the intention of going for a stem cell transplant. Felix and his provider partner in a shared decision-making process. This includes 4 essential elements:
- Two participants: healthcare providers (MD/APP/RNs) and patient/caregiver
- Both parties share information
- Both parties take steps to build consensus about preferred treatment
- Mutual agreement is reached between patient and healthcare member on treatment approach
Shared decision-making has many benefits. Short-term benefits include increased confidence and satisfaction with treatment decisions, avoidance of decisional regrets, decreased patient and caregiver stress and anxiety related to cancer treatment decisions, and enhanced trust in the healthcare system. Long-term benefits of shared decision-making include treatment adherence, better quality of life, and improved treatment outcomes.
Felix and his team agree on a plan, and he moves forward with treatment. He had been referred to the financial advocates, who met with him to work out insurance issues and to assist in copay assistance. He is started on bisphosphonates because his bone scan showed osteopenia and the original scan showed lytic lesions and fracture in the lumbar area. He is placed on aspirin for venous thromboembolism (VTE) prophylaxis due to the higher risk of VTE while on lenalidomide. Extensive education is provided by the team in Spanish to ensure that Felix understands his medication schedule and plan of care. Teaching is done regarding the expected toxicities of his regimen, as well as what to report to the team. He is given a sheet with all of this information, as well as contact phone numbers if he needs to reach his providers. A consult is sent for social work, physical therapy, and nutrition to work with Felix and his family as he moves toward the transplant. The NN follows Felix through his treatment to help him stay compliant and to provide support. Felix has an issue with understanding his medication schedule, and his NN is able to intervene and get him on track.
Because Felix is a Jehovah's Witness, he is enrolled in the bloodless medicine and surgery program. For many patients, blood transfusion is not an option, whether for religious reasons or safety concerns. Bloodless medicine and surgery teams understand and respect this view. A wide variety of patient blood conservation methods and techniques are available to manage patients without transfusions.
The following techniques may be used to manage patients without transfusion support:
- Cell Saver: continually cleans and recirculates the patient's own blood during surgery
- Aquamantys: effectively stops bleeding by sealing the soft tissue and bone during or after surgery
- Hemodilution: dilution of the patient's blood with nonblood volume expanders within a closed circuit attached to patient's circulatory system
- Ultrasonic scalpel, lasers, and electrocautery: devices used to reduce blood loss during surgery
- Oximetry: tracks oxygen levels during and after surgery
- Erythropoietin: stimulates bone marrow to produce red blood cells
- Volume expanders: intravenous fluids that enhance the circulation of your own blood
- Minimal blood draws and use of pediatric tubes when blood specimens are needed
While completing his induction therapy, Felix receives a very good partial response. He obtains secondary insurance and a prescription plan thanks to the financial advocates. He undergoes dental clearance with help from his NN. Dental clearance pretransplant is a challenge when patients do not have dental insurance and are on a fixed income.Some resources for patients are local dental clinics that may provide care on a sliding scale, dental schools, or local providers who have partnered with the cancer center to provide dental hygiene and care to patients in need of treatment. Felix and his family meet with the social work team and are assisted in obtaining local lodging at a nominal cost while undergoing transplant. Felix and his family are connected to the local Jehovah's Witnesses community for additional support.
Felix is cleared for transplant and has his cells harvested. Again, there is extensive education provided on this process and what to expect while hospitalized. He is admitted to the hospital for 2 weeks. Prior to admission, the NN collaborates with the inpatient team to ensure that things go according to plan. There are many novice nurses who are new to bloodless medicine and surgery patients being transplanted. In conjunction with the inpatient clinical educator, the NN provides an overview of Felix's case, as well as education and serving as a resource for the staff. The NN also helps with Felix's transition of care back to the outpatient setting and maintenance therapy.
Felix does well after his stem cell infusion. He is discharged in stable condition with adequate blood counts. He stays close to the cancer center and is monitored frequently posttransplant. His NN makes sure he is reconnected with his PCP and a local oncologist for follow-up since he is 3 hours away. Felix is put on maintenance therapy of lenalidomide 10 mg po days 1 through 21. He is provided information on various community resources such as:
Felix is now 2 years posttransplant and continues to do well.
Yelak Biru gave an inspiring talk about living with MM. His goal is to live with myeloma for as long as possible with the greatest quality of life. He serves on many committees and boards and as an advocate for MM patients. He spoke about his journey and the changes that have occurred in the MM landscape that have allowed him to live for 22 years. He spoke about being empowered. Living with MM requires a lot of things. Three key factors Yelak spoke about were knowledge, relationships, and response to treatment. Knowledge requires patients to educate themselves about the disease, advocate for others, and ensure treatment choices to maximize efficacy and quality of life. He spoke about shared decision-
making and the importance of being a partner in the care team. Relationships refer to taking ownership of one's care and building a survival team. That team includes many MM specialists. When he spoke about response to treatment, he said that MM will knock you down, and you need to choose to get up and fight the battle. One of his goals was to maximize outcomes and minimize adverse effects.
Yelak has been on multiple lines of therapy, including participating in clinical trials that have led to new MM treatments. He spoke about the myths and misconceptions patients have about participating in clinical trials. He addressed the importance of advocating and educating patients on all treatment options, and that clinical trials should be part of every discussion. If it were not for clinical trials, he would not be alive today to tell his amazing story. He provided many inspiring tips to share with patients. These included the following: know you are unique and form partnerships with those around you; seek clinical trials as treatment options; have long- and short-term goals; know your options; and communicate your needs, especially regarding quality of life, with your team.
Yelak's team did not have an NN. He served as his own navigator, and his story provided great insight into how NNs can improve patient care. Yelak's journey was long and had many ups and downs, but he learned a great many lessons throughout his journey that he was able to share. The lessons and tips that Yelak shared should be part of every navigator's toolbox.
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