Link to article on MyHealthTeam
Myeloproliferative neoplasms (MPNs) are a category of blood cancers that include polycythemia vera (PV), primary myelofibrosis, and essential thrombocythemia (ET). Although MPNs can affect people of all ages, they are usually diagnosed later in life. This later diagnosis may be due in part because many people don’t experience symptoms for many years.
Some people with MPNs experience severe symptoms, especially later in life. They may be eligible for hematopoietic stem cell transplantation, which can cure some types of MPNs. Unfortunately, this procedure may be impractical for older adults. When a cure or reversal of disease is no longer possible (as with many other types of cancer), your health care team may refer you to palliative care, or hospice care.
What Is Palliative Care?
Palliative care encompasses care provided to people who are experiencing serious illness, focusing on reducing symptoms and improving quality of life. Palliative treatments do not focus on treating MPNs. Rather, they focus on relieving symptoms and discomfort. This kind of supportive care can include treatments that relieve pain and discomfort, increase mobility, or improve stamina.
These treatments can be provided in your home, at a specialized center, in a skilled nursing facility, or at an acute care facility like a hospital. Anything that relieves pain and reduces disease burden is considered palliative. This can also include emotional and spiritual support.
Some people may receive palliative care alongside treatments aimed at curing disease or slowing its progression. Therefore, some people can receive only palliative treatments, and others receive palliative care in conjunction with other treatments, depending on their medical situation.
What Is Hospice?
Hospice care falls under the umbrella of palliative care, although it refers to a specific type of care and situation. Hospice care is palliative care that is provided when someone decides they no longer wish to continue curative treatments or when a health care provider finds that the available treatments are no longer effective.
One criterion defining hospice care is that a health care provider must certify that the person in their care could pass away from their disease within six months. Some people referred to hospice may survive for many years thereafter and eventually resume treatment with their primary care provider. But for many, hospice means end-of-life care.
Home, Not Hospital
In the United States, hospice care generally focuses on keeping individuals comfortable and safe at home in the final stage of illness, rather than in the hospital. Studies show that a majority of people with cancer would prefer to live their last months at home and avoid aggressive treatments in their final days. This is one reason why many families report that care at the end of life is improved by hospice care.
When Should the Hospice Conversation Begin?
Families should begin a conversation about end-of-life wishes as soon as they are comfortable doing so. Regardless of age or disease status, early conversations about preferences are helpful as families make decisions later on. Because illness or injury can come at any time, families should consider open and direct conversations about the choices they would make if faced with a debilitating illness.
End-of-life conversations can be especially important when a family member has been diagnosed with cancer such as an MPN. Many people with MPNs live long lives, but it can shorten the lives of others. Talking with your family about your preferences for different types of care can be a good way to start the conversation.
Progression to Myelofibrosis
Although EV and PV are generally considered less aggressive forms of MPN, they can both progress to myelofibrosis. This progression can be referred to as “post-essential thrombocythemia MF,” “post-PV MF,” or secondary myelofibrosis. Some symptoms of myelofibrosis include night sweats, anemia and fatigue, weight loss, fever, and pain associated with an enlarged spleen. Some treatments associated with this disease process can include surgery to remove your spleen, blood transfusions, or hormone therapy to help stimulate your body to create red blood cells.
People with MPNs should talk with their families about their feelings regarding these treatment options. Would you be willing to receive regular transfusions? Would you be willing to undergo surgery? What type of care would you prefer if you become too fatigued to prepare meals every day? What if you became too weak to walk down a flight of stairs? These kinds of questions are important for families to consider when developing an individualized plan of care for their loved ones.
Progression to Acute Myeloid Leukemia
All MPNs have the possibility of progressing into acute myeloid leukemia (AML). This can be referred to as secondary AML (sAML), or blast phase (BP, or MPN-BP). This disease progression is difficult to treat and is often associated with a profound decline. End-of-life planning is crucial for those diagnosed with advanced cancer. People diagnosed with MPN-BP may receive a referral to hospice upon diagnosis, depending on their age and other medical factors.
If Treatment Becomes a Burden
Some people choose hospice when treatment and side effects become too burdensome. These issues can be due to the side effects of chemotherapy or radiation. For people with MPNs, treatment may become a burden when repeated transfusions are perceived to be too risky, when it becomes difficult to travel to treatment centers, or when someone decides they’ve simply been “poked enough” by needles.
In these cases, hospice care can be the right choice. Hospice services work to keep people safe and comfortable at home with their families. This can be done by providing support such as:
- Adaptive equipment, like shower benches to keep someone experiencing weakness safe while bathing
- Caregiver support and education
- Home health aides
- Respite care
- 24/7 nursing care services, for medical questions while at home
- Medication and pain management
- Emotional and spiritual support for the entire family
- Support from a social worker regarding legal or insurance matters
- Coordination with mortuary services
- Volunteer support
Infection, Isolation, and Social Support
Some MPNs are associated with immunosuppression. Doctors and other health care providers may recommend that people with MPNs isolate due to the risk of infectious disease. However, in the setting of hospice and end of life, this recommendation may be perceived as an unacceptable burden. All people, including those on hospice care, have the right to refuse or decline medical interventions, including advice from doctors. Therefore, if someone with an MPN chooses to be social rather than isolate at the end of life, every effort should be made to accommodate that preference.
Barriers to Hospice for People With MPNs
Researchers have noted possible barriers to hospice care for people with MPNs. For example, some individuals with MPNs become dependent on blood transfusions, especially as the disease progresses. This obstacle can create a problem, as most health insurance agencies, including Medicare, do not cover transfusions for people under hospice care.
As a result, some practitioners do not end up referring people with MPN symptoms to hospice care. In this case, some individuals with MPNs experience negative outcomes near the end of life. For example, these may include passing while in an inpatient setting like a hospital, receiving intensive care in the last month of life, and receiving chemotherapy in the last two weeks of life. Therefore, people with an MPN should communicate directly with their care team about their wishes regarding these scenarios.
A hospice care team can provide numerous benefits and support for those who would like to stay out of the hospital and in their own homes. Palliative care and hospice do not limit life nor do harm. Rather, they are a robust support system for people with severe illness, their caregivers, and their families.
Talk With Others Who Understand
On myMPNteam, the social network for people with myeloproliferative neoplasms, more than 2,000 members come together to ask questions, give advice, and share their stories with others who understand life with an MPN.
Have you or a family member been referred to hospice or palliative care? Share your experience in the comments below, or start a conversation by posting on your Activities page.
- Myeloproliferative Neoplasms — Leukemia & Lymphoma Society
- What Are Palliative Care and Hospice Care? — National Institute on Aging
- End-of-Life Care Quality Outcomes Among Medicare Beneficiaries With Hematologic Malignancies — American Society of Hematology
- Myeloproliferative Disorders Treatments — UCSF Health
- Acute Myeloid Leukemia Evolving from Myeloproliferative Neoplasms: Many Sides of a Challenging Disease — Journal of Clinical Medicine
- Immune Dysregulation and Infectious Complications in MPN Patients Treated With JAK Inhibitors — Frontiers in Immunology
- AMA Code of Medical Ethics’ Opinions Related to End-of-Life Care — AMA Journal of Ethics
- Hospice Care — MPN Voice
