Myeloproliferative Neoplasms (MPNs) are rare progressive blood cancers that result when the bone marrow overproduces blood cells. Diagnosis often follows a routine blood test that reveals abnormal blood cell count.
Although an MPN can be diagnosed in a person of any age, they occur most commonly in adults over 60.
There are three main types of MPNs: essential thrombocythemia (ET), polycythemia vera (PV), and myelofibrosis (MF).
The three types
Patients who have ET produce too many platelets, which increases their risk of forming blood clots and may cause frequent headaches, visual disturbances, and fatigue.
Patients with PV produce too many red blood cells, and possibly too many platelets and white blood cells as well. Excessive red blood cells increase the risk for blood clots and may cause a feeling of fullness, headaches, fatigue, itching, and sweating.
Patients who have MF accumulate scar tissue in their bone marrow, which leads to a decrease in blood cell production and an enlarged spleen, causing myriad symptoms such as fatigue, shortness of breath, fullness, and bone pain.
The main risk factors for all patients who have MPNs are blood clots and cardiovascular events such as stroke and heart attack, although older adults are more likely to have multiple medical diagnoses that complicate their healthcare outcomes.
Because of the significant risk for blood clots in patients with MPNs, nurse practitioners should encourage patients to preserve their cardiovascular health by eating a heart-healthy diet, avoiding smoking, maintaining a normal blood pressure, monitoring diabetes closely, keeping blood sugar levels under control, and taking measures to decrease stress.
As the majority of people who live with MPNs are older adults, nurse practitioners can access the Gerontological Advanced Practice Nurse’s Association (GAPNA) as a resource for patient education, advocacy, and networking. Another resource for nurse practitioners is the evidence-based NCCN Clinical Practice Guidelines in Oncology for MPNs, which provides diagnostic guidelines, medication administration, and treatment options.
Nurse practitioners and hematologists should work together to educate patients and their families about symptom management, current treatment recommendations, and steps to maintain an optimal quality of life.
The most common symptom for patients with MPNs is fatigue, which can be caused by numerous factors such as increased or decreased blood cells, microvascular occurrences, inflammatory responses, and deconditioning from chronic fatigue and other comorbidities.
Nurse practitioners can assist with managing fatigue by encouraging patients to pace themselves during activity, engage in regular exercise, and develop proper sleep hygiene with a routine sleep schedule.
The symptom burden for patients who have MPNs is high, so caregivers should be educated to care for themselves as well as for the patient.
Advice for patients
If you are a patient who has been diagnosed with an MPN, learning about this rare condition may leave you feeling overwhelmed, but there are plenty of helpful online resources available.
For example, the MPN Research Foundation provides funding for researchers and is also a valuable resource for patient education and support.
In addition, the National Comprehensive Cancer Network produces a patient education booklet for people diagnosed with MPNs that explains diagnosis, symptoms, and treatment in an easy-to-read format.
By collaborating with your nurse practitioner and hematologist and following their advice, it is possible for you to meet your personal health goals.
