Myelodysplastic Syndromes (MDS) are a group of malignant hematopoietic stem cell diseases characterized by bone marrow failure, with resultant cytopenias namely anemia, which can progress to acute myeloid leukemia.
I recently took a graduate level public health course on the survey of human diseases. Much to my dismay, MDS was not discussed as hematological malignancy. This is not surprising, as most patients and many health care providers do not recognize MDS as a malignancy. This encouraged me to divulge even deeper into the understanding of MDS, and I wrote my term paper with a focus on the epidemiology and prevalence of the disease. These are important components that are relevant to a nurse for a higher understanding of the disease. This understanding will allow for a greater role in patient advocacy and a more individualized plan of care for patients.
Etiology and epidemiology of MDS reveal that it dramatically increases as an individual ages, that is slightly more common in men rather than women, that it is not contagious, and that environmental agents and genetic abnormalities collectively play a role in disease etiology. The median age of diagnosis is 65 years of age. MDS is common hematological malignancy with estimates of 40,000 new cases per year in the USA.
Those patients who have received chemotherapy or radiation for other malignancies, or those that have experienced occupational exposures to solvents or agricultural chemicals are at an increased risk of developing MDS. Inherited genetic disorders with increased risk for developing MDS include Diamond-Blackfan Syndrome, dyskeratosis congenita, Shwachman-Diamond Syndrome, and Fanconi’s anemia. The pathogenesis of the disease is complex including interplay between a neoplastic clone and an inflammatory environment that early in the disease leads to accelerated apoptosis and later to impaired differentiation and proliferation.
After assuring a patient’s accurate diagnosis, the first step in managing MDS is staging. The international prognostic scoring system (IPSS) or its revised version (R-IPSS)are the most commonly used tools. The patients are classified as lower risk with a step wise approach and treatment aimed to improve cytopenias. Available options include erythroid stimulating agents, lenalidomide for patients with deletion 5 q MDS, hypomethylating agents and immunosuppressive therapy in selected cases. Among patients classified as higher risk, the goal of treatment is to improve survival. Allogeneic hematopoietic stem cell transplant and hypomethyalting agents are the treatment options to be considered.
In my literature search I found the Life Beyond Limits, MDS treatment matters global initiative. The mission statement includes initiatives to “Explore and address the reasons why older patients with (MDS) are treated less aggressively than younger patients, educate and motivate patients to become self-advocates for their health, and mobilize the MDS community to improve the treatment for older cancer patients.’’ Although progress has been made in the treatment of MDS, the elderly still experience discrepancies in their treatment because of poorly observed responses, unwillingness to treat and impact of therapy to overall quality of life.
As a nurse, I have recognized the importance of a patient specific plan of treatment and that it cannot be solely based on chronological age. As advocates, we nurses play a huge role in patients’ management. Nurses are closely involved with MDS patients on a daily basis through the assurance of an accurate diagnosis, staging of the disease, selection of appropriate therapy, monitoring and recognizing adverse events. Our role in educating patients and setting their expectations should always be emphasized.
Christa Roe, RN, BS, OCN
H.Lee Moffitt Cancer Center and Research Institute
Department of Malignant Hematology