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Understanding Your Myelofibrosis Diagnosis
- September 06, 2019
- Amber Specialty Pharmacy
The American Society of Hematology recognizes three main types of blood cancer: leukemia, lymphoma and myeloma. Within those three categories, there are many blood cancers distinguished by specific characteristics. Primary myelofibrosis (MF), a rare bone marrow cancer, is a type of leukemia. As the lead pharmacist on Amber Specialty Pharmacy’s Oncology Center of Excellence, I counsel patients every day to help them manage treatment for myelofibrosis.
What is Myelofibrosis?
Myelofibrosis (MF) is a chronic blood cancer. Patients with MF have scarring (fibrosis) that occurs in the bone marrow (myelo) which interferes with our body’s ability to produce blood cells. Myelofibrosis falls under a broader category of blood disorders called myeloproliferative neoplasms (MPNs) where the bone marrow produces too many abnormal blood cells. Stem cells are cells in our body that have the ability to grow and change into more specialized cells such as red blood cells, white blood cells and platelets. In MF, stem cells develop genetic mutations that prevent them from developing into these healthy specialized cells. Instead, you see an out-of-control production of these mutated cells which eventually take over and do not allow our bone marrow to make normal healthy blood cells.
Patients can have low blood cell counts leading to the signs and symptoms of MF. A low red blood cell count, called anemia, can cause tiredness, weakness and shortness of breath. A decrease in platelets can lead to easy bruising and bleeding. Night sweats and fever are also common. Patients may also have pain, tenderness and a feeling of fullness on their left side under the ribs. This is due to swelling in the spleen as a result of our body trying to produce blood cells in other areas of the body due to the scarring that occurs in the bone marrow.
What causes Myelofibrosis?
The cause of MF is not known, but there are some factors that are known to increase your risk. Your age is one such factor. Myelofibrosis can affect anyone but it is more common in those greater than 50 years of age. Those who have other MPNs (essential thrombocythemia or polycythemia vera) can develop MF. Exposure to certain chemicals and radiation have also been found to increase the risk of developing MF.
Treatment of Myelofibrosis
There are three goals with the treatment of MF: 1) decrease symptoms, 2) improve blood counts and 3) decrease the chance of MF progressing to acute myelogenous leukemia (AML). Treatment may include observation, medication, stem cell transplant or a clinical trial. Based on your current symptoms and risk category you and your doctor will discuss the best course of treatment for your case. Your pharmacist at Amber Specialty Pharmacy is here to answer your questions about your medications, how to manage side effects from those medications and symptoms you may have from your MF.
Recently, Amber Specialty Pharmacy was selected to be a specialty pharmacy network partner for Celgene’s new product Inrebic® (fedratinib). The U.S. Food and Drug Administration approved Inrebic® on Aug.16, 2019, for the treatment of adult patients with certain types of MF.
“We believe that Inrebic® will play an important role in the treatment of patients with MF and we are honored to be selected as a specialty pharmacy provider for this medication,” said Aaron Wiese, President and CEO of Amber Specialty Pharmacy. “Our high-touch patient support services and oncology clinical expertise offer best-in-class program practices that address all aspects of the health and well-being of our MF patients, and their healthcare team.”
Amber Specialty Pharmacy is a specialty and infusion pharmacy that offers exceptional patient support services and condition-specific Centers of Excellence. People diagnosed with MF can benefit from the specialized clinical expertise offered by team members on the Oncology Center of Excellence. Amber Specialty Pharmacy offers a whole-health approach to managing MF and other cancers. If you have questions about managing medication side effects, eating a nutritious diet or finding a local MF support group, we can help.