Abstract
Juvenile Myelomonocytic Leukemia (JMML) has been perceived as a rare disease that predominates in young children under four years. The condition is most prevalent in male more compared to the female. There is miniature literature on the leading cause of the disease prevalence. However, research has indicated that the disease is commonly associated with cell and genetic mutations changing the DNA genetic codes in the blood cells. The purpose of the study is to present a critical analysis of the JMML, the risk factors, and the necessary nurse Aide needs for patients with the disease process. The review also addresses the importance of the nursing aide concepts concerning the patient’s outcomes and the nursing care.
Juvenile Myelomonocytic Leukemia
Introduction
Over the last few years, the world health organization has classified JMML as pediatric leukemia been recognized to share myelodysplastic and myeloproliferative neoplasm. The disease has been identified to share the same characteristics as these groups of disorders. Development disorders characterize the disease. The disease accounts for 1% of the pediatric leukemias, with prevalence ranging from about 1.2 per a million victims (NORD). Its prevalence has been commonly diagnosed to primarily occur in male children at the age of two years. The prevalence ratio between the male and the female ranges in about 1.2. In most cases, the disease has been detected and diagnosed before three years and six years. The disorder accounts for 20-40% of the pediatric MPN /MDS (Mayerhofer et al.).
The disorder develops a premature form of monocytes that collect in the bone marrow and outweigh diffusing other strong cells. The component generates unique structures that develop into white blood cells, red blood cells and platelets. These cells are absorbed into the blood vessels and circulate around the body, executing particular function. Once these abnormal cells are collected into the bone marrow, they affect the replication and the functions of the unaffected cells.
Children suffering from the disease may exhibit specific symptoms or a combination that develops in weeks or months. According to NORD, JMML may manifest itself through abnormal paleness of the skin, spleen, or liver abnormalities, weakness, dry cough, and fatigue (NORD). If the patient has an abnormal spleen or liver enlargement, their abdomen becomes distended, resulting in breathing difficulties and loss of appetite. Due to the crowding of the cancer cells within the bone marrow, blood circulation becomes limited, resulting in anemia and bone marrow abnormalities. The levels of platelets may also decrease, resulting in thrombocytopenia, which is associated with skin, nose, and mouth hemorrhage. Sometimes due to malfunctioning of the white blood cells, the child may develop tonsillitis and bronchitis (Locatelli, Franco, and Charlotte). Other manifestations may involve reduced appetite, irritability, poor weight gain, coffee-colored lesion on the skin and bone, and joint remission.
Risk Factors for the Disease
JMML has been poorly understood for years, and health clinicians have tried to understand the predisposing factors of the disease. However, numerous researches have indicated that most affected populations have a commonality of one or multiple cell mutations. According to the national organization for rare disorders, the patients likely to develop the disorder may have undergone abnormal structure and orientation of oncogenes of the tumor suppressor genes (NORD). These cells control the growth, development, and cell division and ensure that the cells are eliminated at the appropriate time. The specific cause of the cell change is unidentified. The current research has also indicated that children with DNA abnormalities responsible for carrying the body’s genetic factors JMML are known to occur sporadically for unknown reasons.
Locatelli, Franco, and Charlotte indicate that inherited diseases can also be predisposing factors for JMML. The diseases are known to occur in the early stages of the child’s life. In children with NF-1 symptoms, JMML increases more than 200 times, and it is commonly identified in children at one year (NORD). The recent research has also indicated that children suffering from Noonan syndrome, which is caused by PTPN11 genes, are also vulnerable to developing JMML disorder during their first year of life (Locatelli, Franco, and Charlotte) National organization for the rare disorder has also identified the presence of CBL abnormalities having higher risks of developing JMML conditions, characterized by severe blood vessel inflammations.
Nurse Aide Care
Communication Needs
Care for patients suffering from JMML deserves coordinated care from the physician, clinicians, and pediatrician who specialize in diagnosing and managing the disease. Sometimes the surgeons and the oncology nurses may also participate in planning the effective care treatment for the child (NORD). The nurses are also required to make effective communications between the patient’s family and themselves to build an excellent egalitarian relationship and enhance the patient’s safety and effective decisions (Berta, et al). They will also be required to communicate the patient’s progress, any healthcare needs, and any adverse manifestations of the diseases to the doctors and physicians for better patient treatment and medication and enhance patient safety. Since the condition is at a greater risk of developing psychological distress, communication between the nurse and the patient reduces the stress vulnerability. It increases the confidence of the patient in the treatment for better outcomes.
Pain Management
Treatment of patients with JMML may involve a combination of both the chemotherapeutic and therapeutic interventions and deserves particular attention from the nurse in charge. Chemotherapeutic interventions through cancer drugs have been perceived as a successful management model of the disorder as the patient waits for the transplant (Mayerhofer et al.). As a result, the patients require nurse assistance to ensure that chemotherapy doses are followed and monitor the disease progression. Patients subjected to chemotherapy treatment interventions are likely to suffer from health relapse and sometimes suffer more adverse complications associated with remissions. National organization for rare diseases presents that 50% of children with JMML happen to be treated through hematopoietic stem cell transplant have long-term remissions. About 30-40 % of the patient’s relapse in their first year of treatment (NORD). As a result, the patient will require nurse assistance to help them in pain management and ensure that the patient is on medication as per the goals and outcomes of the treatments.
Patient and Family Support
In reference to the family and the patient’s support, the individuals deserve to receive adequate psychological support. According to NORD, the disease is known to cause psychological distress, anxiety, and stress once the patient is diagnosed with the disorder. As a result, before and after initiating the diagnosis process, the patient and the victim need psychological counseling and support to understand their health and the possible treatment measures to be taken. This can be done either professionally or through enrollment in the support groups.
Nutritional Requirements
Since children with specific cell mutations act as pathways for the disease, taking certain food substances may inhibit or activate the disease pathways and signaling. All food substances have been tested for nutritional supplements with active ingredients supporting specific molecular actions on distinct biochemical pathways. Hence, the uptake of certain dietary supplements may aid in treating the disease, while others may lead to adverse manifestations and progression of the disease (Locatelli, Franco, and Charlotte). Nurses should provide nutritional support and education to the patient’s parents to avoid the adverse progression of the disease and promote healing. Foods containing folic acid, Retinol, and vitamin c should be avoided as they activate the Azacytidine, causing the MYC signaling. Nutritional supplements with turnips should also be avoided. Conversely, taking foods containing active ingredients such as Benzyl Isothiocyanate, Brasinin and crocetins inhibit biochemical pathways promoting a positive effect. Additionally, foods with beta-sitosterol and citric acid and Azacytidine treatments decelerate disease progression and enhance a positive impact.
ADL support
Additionally, the patient requires assistance with their activities of daily living, such as ambulating personal hygiene (Berta, et al.). Since the condition is a complication in the child’s bone marrow, this may affect the ability of the patient relocate from one position to another thus enhancing their mobility. This will also reduce the incidences of falls and promote patient safety.
Importance of the Nursing Aide concepts to Patients with JMML
The Nursing Aide in the treatment process of the patients suffering from JMML is essential. It will provide the necessary physical care and emotional and psychological support to the patients and their families. As mentioned earlier, patients and families of the victim need psychological support before and after JMML to diagnose since it increases one’s vulnerability to mental disorders such as anxiety and distress. The concepts help in ensuring the physical, psychological, and emotional well-being of the patients (Berta, et al.). The program serves as the vital concept of the overall treatment of the patients and will help the patient carry on with their daily activities with the nurse’s help. With the aid of ADL, clinicians can assess the patient’s status and promote effective planning for the next phase of the patient’s treatments (Berta, et al.). It also helps determine the patient’s level of accomplishing functional capabilities to ensure that the patient can independently live and perform personal care. Finally, the concepts allow the clinician to assess the patient’s progression by determining the patient’s vital signs and conclude the efficiency of the treatment interventions. The primary aim of the concept is to ensure the patient’s outcomes are in line with the treatment and the care goals for a positive health outcome.
Socioeconomic, Cultural, and Religious Factors Impacting Aide Care
The nursing care provided to patients suffering from JMML may encounter socioeconomic, religious, or cultural impediments inhibiting adequate care delivery. These variables will influence the decisions made by the nurse towards care delivery. From a religious perspective, the patients’ religious and spiritual beliefs may impact the chemotherapeutic and nutrition interventions to aid in managing the condition. Additionary, religious and spiritual beliefs may also inhibit the implementation of allogeneic hematopoietic stem cell transplant due to the strong perceptions of organ transplant. Secondly, Patients’ income and occupational status may influence how patients perceive the quality of care in health facilities when receiving outpatient care services. The patient’s family may have limited resources to cover the healthcare expense and the knowledge of the type of care needed for the patients. The nurse should be able to deliver the best care by demonstrating cultural awareness and employing cultural competency.
Conclusion
Juvenile Myelomonocytic Leukemia is known to affect children under the age of four. It’s characterized by premature growth of white blood cells in the bone marrow cells, which overcrowds the existing healthy cells. This condition leads to an impact on the cell’s functions and ability to multiply. Its prevalence is more in males than female children. Its primary cause is unknown, but researchers believe it is caused by the mutation of cells found in the bone marrow. Gender, age, and abnormal cell development or mutation have been identified as the primary risks factors to JMML. Patients diagnosed with the disorder require nursing assistance in numerous ways, such as psychological, emotional, and physical support that promotes effective management of the condition. A combination of chemotherapeutic and cell transplant interventions has been perceived to be more effective compared to therapies. However, implementation of nursing assistance might be ineffective due to religious, cultural, and socioeconomic factors, but nurses should demonstrate high cultural awareness and implement appropriate cultural competency when providing care.
Work Cited
Berta, Whitney, et al. “The evolving role of health care aides in the long-term care and home and community care sectors in Canada.” Human resources for health 11.1 (2013): 1-6. https://dx.doi.org/10.1186%2F1478-4491-11-25
Locatelli, Franco, and Charlotte M. Niemeyer. “How I treat juvenile myelomonocytic leukemia.” Blood, The Journal of the American Society of Hematology 125.7 (2015): 1083-1090. https://doi.org/10.1182/blood-2014-08-550483
Mayerhofer, Christina, Charlotte M. Niemeyer, and Christian Flotho. “Current Treatment of Juvenile Myelomonocytic Leukemia.” Journal of Clinical Medicine 10.14 (2021): 3084. https://doi.org/10.3390/jcm10143084
National organization for the rare disorder (NORD). “Juvenile Myelomonocytic Leukemia.” NORD, 27 June 2017, rarediseases.org/rare-diseases/juvenile-myelomonocytic-leukemia/.
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