GATA2 Deficiency: Early Identification for Improved Clinical Outcomes.

BACKGROUND: Patients with GATA2 deficiency present with nontuberculous mycobacterial infections, severe viral infections (particularly refractory human papillomavirus disease), lymphedema, myelodysplastic syndrome (MDS), and acute myeloid leukemia. Patients with GATA2 deficiency who undergo allogeneic hematopoietic stem cell transplantation prior to the development of life-threatening infections or cytogenetic abnormalities may have optimal clinical outcomes. OBJECTIVES: The aim of this article is to determine ways in which oncology nurses can identify GATA2 deficiency in patients early and optimize treatment decisions. METHODS: A case study is presented of a 33-year-old man with recurrent infections and MDS and his two sons, all of whom were found to have the same GATA2 mutation. FINDINGS: Oncology nurses play an important role in early detection and identification by interviewing patients and obtaining a complete and thorough family history.

Patient perspectives on the usefulness of routine telephone follow-up as psychosocial support for hematologic malignancies: Australian findings.

PURPOSE/OBJECTIVES: To explore the use of routine telephone follow-up as a supportive care strategy for patients with hematologic malignancies from the patients' perspectives. RESEARCH APPROACH: A qualitative design based on a series of open-ended interviews and one focus group. SETTING: Leukaemia Foundation of Queensland, Australia. PARTICIPANTS: 50 participants recruited from the Leukaemia Foundation of Queensland database that represented a sample of major diagnostic groups, age, gender, and geographic location of patients with hematologic malignancies. METHODOLOGIC APPROACH: Interviews and focus groups were open-ended and were recorded, transcribed verbatim, coded, and thematically analyzed to form the basis of the findings. FINDINGS: The majority of participants in the study saw a perceived benefit in regular telephone follow-up as a supportive care service. Benefits included the positive aspects of individualized attention, potential support created for those not open to conventional types of support, and the positive effects of allowing support organizations to keep track of patient progress. However, some participants did not want to receive regular telephone follow-up support because of a desire to move on and access to other support through friends, family, or healthcare professionals. CONCLUSIONS: The results from the current study affirm previous research. Attitudes toward a telephone follow-up support service vary from patient to patient. Research demonstrates that patients with cancer responded with attitudes that range from favorable to unfavorable toward the benefits and usefulness of telephone support. INTERPRETATION: Oncology nurses provide supportive care for patients with hematologic malignancies, and they serve as a key professional group to provide follow-up telephone supportive care. Based on the findings from the current article, oncology nurses who provide supportive care should be aware of differing attitudes among patients with hematologic malignancies toward telephone follow-up support. Nurses participating in support initiatives should recognize the notion of patient receptivity and how it affects telephone support strategies.

Myelodysplastic syndromes: the challenge of developing clinical guidelines and supportive care strategies for a rare disease.

Myelodysplastic syndromes (MDS) represent a group of clonal myeloid malignancies with variability in clinical presentation and disease trajectory, as well as prognosis and treatment recommendations (Kurtin & Demakos, 2010). MDS is considered to be a rare disease that is most common in adults older than age 70. The disease is characterized by ineffective hematopoiesis, progressive bone marrow failure, and a variable risk of leukemic transformation thought to result from complex interactions between the malignant clone and the bone marrow microenvironment (Kurtin, 2011). This supplement is intended to provide the oncology clinician with an overview of MDS and provide tools for the clinical management and support of patients with MDS.

Update on the science of myelodysplastic syndromes.

Scientific research is only just beginning to shed light on the pathobiology underlying the various subtypes of myelodysplastic syndromes (MDS), a heterogeneous group of clonal stem cell disorders characterized by cytopenias that can progress to acute myeloid leukemia. Increased understanding of the disease and prognostic implications of specific clinical features has aided in the development of prescribing guidelines and new treatments for MDS. Because oncology nurses have frequent interactions with patients during diagnostic and therapeutic evaluations, an understanding of the science behind disease classification, prognostic scoring, and the goals of treatment for low- and high-risk disease is important to answer questions regarding diagnostic results, treatment outcomes, and adverse event monitoring.

Treatment of myelodysplastic syndromes: practical tools for effective management.

Myelodysplastic syndromes (MDS) are a heterogeneous group of myeloid malignancies with variability in clinical presentation, disease trajectory, treatment goals, and expected outcomes. The treatment of patients with MDS, therefore, often differs from patient to patient. Tools are needed to aid effective communication with patients, their caregivers, and their dedicated team of healthcare professionals. The use of methods often employed in clinical trials can help healthcare providers diagnose and classify risk status, track trends within patient responses, manage adverse events, set treatment expectations, and provide ongoing supportive care. This article discusses several tools and strategies available for the management of patients with MDS throughout the continuum of their disease.

The importance of quality of life for patients living with myelodysplastic syndromes.

Myelodysplastic syndromes (MDS) are a group of myeloid stem cell clonal disorders characterized by a wide variation in illness trajectory and potential treatment. The physical, functional, emotional, social, and spiritual well-being of individuals with MDS can be affected by both disease and treatment-related factors. As a result, the quality of life (QOL) in patients with MDS may vary throughout the course of the illness. To date, most research exploring QOL in patients with MDS has been conducted as part of clinical trials evaluating the effectiveness of a therapeutic intervention. Although data from those studies are useful, they do not fully address the issues critical to maintaining or maximizing QOL. Oncology nurses are in a key position to assist patients with MDS to maintain their QOL. Findings from comprehensive QOL assessments will guide nurses in providing relevant interventions and evaluating their outcomes. In this manner, oncology nurses can assist their patients to maximize QOL while living with this challenging illness.

Patient and family resources for living with myelodysplastic syndromes.

Primarily a disease affecting older adults, myelodysplastic syndromes (MDS) are a class of incurable myeloid malignancies with variable clinical presentation, treatment recommendations, and prognoses. Although effective communication between healthcare professionals and patients and their caregivers is a significant part of optimizing clinical outcomes, studies have shown that all three frequently have an incomplete understanding of MDS, its therapeutic options, and the fact that MDS is a malignancy. In addition, the advanced age of the patient population, high frequency of comorbidities, and variability of disease outcomes based on risk status require consistent communication across a wide number and type of healthcare providers as well as an individualized approach to patient and caregiver education. This article discusses these challenges and provides a number of resources designed to help educate healthcare professionals, patients, and caregivers.

Counselling and adverse event management for patients with myelodysplastic syndromes undergoing azacitidine therapy: a practice standard for Canadian nurses.

Azacitidine (5-azacytidine, VIDAZA) is a disease-modifying agent that improves survival, reduces transfusion dependence, and reduces progression to acute myeloid leukemia in patients with higher risk myelodysplastic syndromes. Azacitidine injection is associated with characteristic adverse events (AEs) that must be managed in order for patients to stay on therapy and achieve optimal therapeutic outcomes. These AEs include injection-site reactions, cytopenias, and gastrointestinal effects. Oncology nurses are uniquely positioned to provide patient support and counselling, thereby helping patients and their families set clear expectations for azacitidine therapy. This article presents a nursing standard designed to support Canadian oncology nurses in the key areas of counselling for patients initiating and continuing azacitidine, as well as nursing strategies for prevention and management of azacitidine-associated AEs. Many of the general principles discussed in this nursing standard can be applied broadly to many diseases and treatments.

Myelodysplastic syndromes: diagnosis, treatment planning, and clinical management.

Innovations in the diagnosis, risk stratification, and treatment of the myelodysplastic syndromes (MDS) have provided several new therapeutic options and renewed hope for patients with the disease. Optimal treatment requires careful evaluation of each patient using newly established criteria. Identifying the common symptoms in the MDS patient, integrating new therapies with novel mechanisms of anti-tumor activity and unique toxicity profiles, and developing tools to assist patients receiving these treatments have created unique challenges for the oncology nurse. Many of the emerging therapies have shown promise in tumor response and may be administered over extended periods of time. Most allow patients to be treated in an outpatient setting. This article will explore the diagnosis, treatment planning, and clinical management of patients with MDS.

Advances in the management of low- to intermediate-risk myelodysplastic syndrome: Integrating the National Comprehensive Cancer Network guidelines.

Myelodysplastic syndromes (MDSs) are a group of hematologic diseases that present unique challenges for oncology nurses, especially because patients with the disorders are being seen more often in oncology practices. An increasing array of therapeutic options are available, and the National Comprehensive Cancer Network published its first clinical practice guidelines for MDSs in 2004. This article provides oncology nurses with the most recent data on supportive care as well as emerging therapies for patients with low- to intermediate-risk MDS.

Advances in myelodysplastic syndrome: nursing implications of azacitidine.

Myelodysplastic syndrome (MDS) is a group of clonal hematopoietic stem cell disorders characterized by ineffective hematopoiesis, leading to bone marrow failure and peripheral blood cytopenias. MDS is difficult to diagnose because of the absence of symptoms in the early stage of the disease; it often is discovered accidentally during routine physical examinations or blood tests. The U.S. Food and Drug Administration approved azacitidine (Vidaza, Pharmion Corporation, Boulder, CO) for the treatment of MDS. Prior to the approval of azacitidine, no approved therapies were available for the treatment of MDS. Azacitidine is believed to exert its anticancer effects by induction of hypomethylation and cytotoxicity. In clinical studies, the most common adverse events during treatment with azacitidine included nausea, anemia, thrombocytopenia, vomiting, pyrexia, leukopenia, diarrhea, fatigue, injection-site erythema, constipation, neutropenia, and ecchymosis. To ensure proper treatment with azacitidine, nurses should have an understanding of dosage and administration guidelines, commonly observed adverse events, monitoring and care of adverse events, and monitoring of laboratory tests. Having a comprehensive understanding of MDS, its underlying disease characteristics, and current treatments will enable oncology nurses to provide optimal patient care.

Myelodysplastic syndromes: a review for nurses.

The Myelodysplastic Syndromes (MDS) are a group of hematologic disorders that resemble hematologic malignancies but are often treated much differently. These syndromes result from a clonal disorder of certain stem cells in the bone marrow. Treatment can range from simple supportive care to new and innovative approaches such as immunotherapy. In general, treatment is dictated by the severity of the presenting disease. Oncology nurses, because of their familiarity with the manifestations of cancer, are particularly qualified to intervene for the patient and family experiencing MDS. Nursing interventions, based on a firm understanding of the underlying disease, include patients and family education. In addition, emotional support and symptom management are important nursing roles.

Myelodysplastic syndromes.

A cure for MDS has yet to be found. The aim of therapy is to attempt to restore normal hematopoiesis and prevent evolution to acute leukemia. The major trend is supportive care. Blood counts and bone marrow aspirations are taken to evaluate the disease, and transfusions of blood products and antibiotics are given when necessary. A new encouraging modality of therapy is the use of hematopoietic growth factors to reverse cytopenias. As there is no curative treatment for MDS, the patient is likely to be offered investigational drugs either singly or in combination. Future trends in the treatment of MDS will be combinations of agents including biological agents with retinoic acid or vitamin D, low-dose Ara-C, the interferons, and colony-stimulating factors.

Nursing care of the patient receiving subcutaneous low-dose Ara-C therapy.

Although a number of reports have suggested clinical efficacy of low-dose Ara-C (LoDAC) in the treatment of acute non-lymphocytic leukemia (ANLL) and myelodysplastic syndromes (MDS), this therapy remains controversial. Clinical trials presently are being conducted to determine the efficacy of LoDAC in these hematologic disorders. Patient education related to self-administration of the agent and monitoring of potential toxicities are primary components of nursing management. It is likely that nurses in acute care, ambulatory, and community settings will be involved in the management of patients receiving this therapy.