It is an inherited condition one that you are born with. The aim of the study was to assess self care management and selfefficacy among adult patients with sickle cell disease. It is a global health problem, affecting many races and ethnic groups. The patient is asked to identify factors that precipitated previous crisis and measures the patient uses to prevent and manage the crisis. Of these the most common and severe is sickle cell anaemia. The pathophysiology, medical and nursing management, and complications of scd are discussed. We give you the lowdown on treatments, collaborative care, and lifestyle adjustments to successfully manage this disease. Continuing nursing education cne credit a total of 1. Sickle cell disease scd is a group of inherited disorders caused by a mutation of the hemoglobin gene, resulting in the formation of abnormal, sickle shaped red blood cells. The primary treatment of acute pain consists of opioid and nonsteroidal antiinflammatory pain medications. Management of the acute painful crisis in sickle cell disease.
Anaemia often causes few symptoms and may not require specific treatment. Management of sickle cell disease in clinical simulation. Sickle cell disease scd is primarily characterized by pain. Nursing care plan for sickle cell anemia nrsng nursing. The article presents information about nursing management in sickle cell disease. Painful vasoocclusive events are the most common complication experienced by both children and adults with sickle cell.
Many people with sickle cell disease live long and productive lives. Sickle cell patients who are seen in the emergency room are dissatisfied with the quality of care received. Nice clinical guideline 143 sickle cell acute painful episode 5 receiving analgesia, insufficient or excessive doses, inappropriate analgesia, and stigmatising the patient as drug seeking. Adapted from understanding the complications of sickle cell disease. Screening and diagnosis of sickle cell disorders are discussed here. Nursing management for a patient with sickle cell anemia focus on the following. Sickle cell trait is a carrier condition for sickle cell disease. List evidence based treatment strategies for voc pain management.
The following guidelines represent a consensus document by health care providers who have both expertise and experience in working with sickle cell. Inheritance of the mutated gene from both parents results in sickle cell disease. The setting was the simulation lab at a local college of nursing in houston, texas. Introduction sickle cell disease also called sickle cell anemia is an inherited blood disorder that affects red blood cells. Comprehensive care includes early diagnosis, preentive measures, treatment of complications, and ongoing patient education. It is estimated that there are between 12,500 and 15,000 people with sickle cell disease in the uk1. Care of patients with sickle cell disease is largely supportive with hydroxyurea representing the only widely used drug which modifies disease pathogenesis. The aim of treatment for patients with sickle cell disease is symptom control and the prevention or management of complications. This publication, which was developed by physicians, nurses, psychologists, and social workers who specialize in the care of children and adults with sickle cell disease, describes the current approach to counseling and also to management of many of the medical complications of sickle cell disease.
Clinical guidelines for the management of sickle cell disease in adults are due to be published shortly under the aegis of the sickle cell society and will link in with the standards and guidelines for clinical care in children. To provide the professional nurse with an overview of scd and its symptoms, diagnosis, and treatment. Health maintenance for people with sickle cell disease. Most people affected are of african or africancaribbean origin, although the sickle gene is found in all ethnic groups. Health supervision for children with sickle cell disease. Sickle cell disease is not spread like a cold and cannot be caught from another person. Management of patients with sickle cell disease in the haematology day unit. Your healthcare team will help you learn more about sickle cell disease, and work with you to come up with an individual care plan that takes into account all your needs and health concerns. In addition, sickle cellhemoglobin c disease and sickle cell.
Sickle cell disease scd is the most common hemoglobinopathy in the united states and causes significant diseaserelated morbidity including multiorgan damage, chronic anemia, and debilitating pain crises. Sickle cell disease scd is a group of inherited red blood cell rbc disorders that cause the production of abnormal hemoglobin hb, the oxygencarrying component of the rbc. Apr 26, 2020 sickle cell disease scd and its variants are genetic disorders resulting from the presence of a mutated form of hemoglobin, hemoglobin s hbsref1 see the image below. In this article we plan to discuss the clinical features of the disorder and describe current concepts. The most severe form of scd is sickle cell anemia i. Obtain subjective reports of sickle cell disease scd pain for appropriate pain management and evaluation of treatment. Allogeneic stem cell transplantation is the only curative therapy available for sickle cell disease and has been used with some success.
This clinical handbook has been created to serve as a compendium of the evidencebased rationale and clinical consensus for sickle cell crisis. An improved interpretation of self care can benefit the team of health care by preparing the sickle cell patients with required resources and abilities essential to aid in managing their disease. Nonpharmacologic treatment of pain in sickle cell disease. Anaemia management in people with chronic kidney disease hyperphosphataemia in chronic kidney disease neutropenic sepsis sepsis sickle cell disease. Sickle cell disease can bring about chronic problems usually occur over a period of time can cause other types of pain should be included as part of assessment in other words not all pain is sickle cell pain. Scd results from any combination of the sickle cell gene with any other abnormal. Sep 21, 2012 sickle cell disease is an inherited abnormality of haemoglobin that results in lifelong anaemia and intermittent episodes of severe pain.
Pdf sickle cell disease management in the emergency. The case manager s opportunity to improve patient outcomes jill brennancook, dnp, rn, cne, emily bonnabeau, ravenne aponte, christina augustin, and paula tanabe, phd, rn, faen, faan. Current trends and research findings related to improving the prognosis of children with scd are offered. Qualityofcare indicators for children with sickle cell. Lastly, a nursing care plan is offered which focuses on pertinent nursing diagnosis. Watch the video lecture pediatric sickle cell disease. Genetics of sickle cell disease sickle cell disease is a genetic condition occurring due to a mutation of the hemoglobin. Assessment data for a sickle cell anemia patient should include. Charache s, terrin ml, moore rd, dover gj, barton fb, eckert sv, et al. Nursing care plan for sickle cell anemia sickle cell anemia or sickle cell disease a genetic blood disorder, which passed down through families, characterized by red blood cells that assume an abnormal, rigid, sickle shape. The target audience is primary care providers and other clinicians, nurses, and staff who provide emergency or continuity care to individuals with scd. After reading this article and taking this test, youll be able to. Barriers to care for persons with sickle cell disease.
Sickle cell disease is the name given to a group of lifelong inherited conditions that affect haemoglobin. The management of sickle cell disease, 4th ed, national institutes of health, bethesda, md 2002. This statement provides pediatricians in primary care and subspecialty practice with an overview of the genetics, diagnosis, clinical manifestations, and treatment of. Children and adults with sickle cell disease are supported by a team of different healthcare professionals working together at a specialist sickle cell centre. Nursing care planning and goals for patients with sickle cell anemia include. Clinical handbook for sickle cell disease vasoocclusive crisis. It is a lifelong condition characterized by anemia, vasoocclusion, and decreased blood flow to vital tissues. Sickle cell patients are subject to delays and feel they wait longer to be see by the doctor haywood et al. However, its wider application is limited by the availability of suitable. Managing acute painful sickle cell episodes in hospital 21 september, 2012 an exclusive summary of new nice guidance for health professionals from a member of the guideline development group on the management of an acute painful sickle cell episode. Nursing care in sickle cell disease in the family health. Discussions of the clinical manifestations and management of sickle cell disorders are presented separately.
Manage pain of sc crisis, promote optimal perfusion and prevention of complications. The purpose of these guidelines is to help people living with sickle cell disease scd receive appropriate care by providing the best sciencebased recommendations to guide practice decisions. Rapidly initiate pain management with opioids for severe pain within. They include sickle cell anaemia, haemoglobin sickle cell disease and beta thalassaemia. Cultural competence case presentation sickle cell crisis.
Thus, pain management in sickle cell disease will likely progress to more aggressive outpatient regimens. This publications provides an overview of major sickle cell disease scdrelated health complications, as well as the nursing implications for each. This guideline addresses the management of an acute painful sickle cell episode in. Jul 22, 2003 sickle cell disorders are a group of inherited conditions that affect the red blood cells erythrocytes. Scd is a chronic disease that can cause many systemic complications, such as organ dysfunction and infection due to inadequate tissue perfusion and oxygenation. Sickle cell disease scd is characterized as the most prevalent genetic blood. Sickle cell disease scd is a pleiotropic genetic disorder of hemoglobin that has profound multiorgan effects. Chronic pain and the perception of addiction, implicit bias, frequent hospitalizations and emergency department visits, clinician and patient knowledge deficit and sickle cell disease stigma all impede the ability to provide evidencebased care for patients with. Develop care plans collaboratively with individuals with scd and their family members. Care coordination for patients with sickle cell disease.
Sickle cell disease can have a significant impact on morbidity and mortality. Here are six 6 nursing care plans ncp and nursing diagnosis ndx for patients with sickle cell anemia. Assess vital signs and grade the amplitude of peripheral pulses, presence of pain, skin color and temperature. Children with sickle cell disease should also have all the routine vaccinations, and possibly also additional vaccinations like the annual flu vaccine and the hepatitis b vaccine. Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Although the disease still is incurable, there is much the staff nurse and nurseclinician can do to minimize the suffering and disability it causes. Nursing management of children with sickle cell disease.
Sickle cell disease childrens hospital of wisconsin. Perinatal and neonatal implications of sickle cell disease. Sickle cell trait is a benign carrier condition characterized by heterozygosity for the sickle hemoglobin mutation. Acute nursing care and management of patients with sickle. Sickle cell disease is a term used for a group of conditions in which the pathology is due to the presence of hemoglobin s. This document has been prepared for informational purposes only. Investigators of the multicenter study of hydroxyurea in sickle cell anemia.
Sickle cell anemia is most commonly seen in children and young adults of both sexes. This guideline addresses the management of an acute painful sickle cell episode in patients presenting to hospital until discharge. Sickle cell acute painful episode nice clinical guideline 143 full. This chronic pain with acute exacerbations is the most common reason for hospital visits, admissions, and readmissions, particularly in young adults aged 1839 years. Managing acute painful sickle cell episodes in hospital. Major complications of sickle cell disease and nursing. Sickle cell disease pain management and the medical home. Many adolescents with sickle cell disease experience delayed. Management of acute sickle cell pain recommendations rapid assessment. The painful episodes occur unpredictably and vary in both severity and frequency. Sickle cell disease pain management in adolescents. Opioids have common and significant side effects, including hyperalgesia, tolerance, and dependence. Anemia sickle cell, nursing care, family health strategy, health centers.
To develop a set of qualityof care indicators for the management of children with sickle cell disease scd who are cared for in a variety of settings by addressing the broad spectrum of complications relevant to their illness. Hemoglobin functions to move red blood cells through small blood vessels and. Individuals with sickle cell disease can pursue a variety of vocations and professions. Management of sickle cell disease, expert panel report 2014,1 is intended to support, enhance, and expand the knowledge of basic aspects of care for patients with scd. Sickle cell disease is one of the most common inherited anemias of the hematopoietic system. A biopsychosocialspiritual model of chronic pain in adults with sickle cell disease. Patients with sickle cell disease require multidisciplinary approach to their management and primary care is pivotal in overseeing delivery of care sickle cell disease scd is a group of inherited disorders of the blood that causes a chronic multisystem disease, leading to organ damage and premature death.
The methodology included a pre and postconference of student performance in the management of sickle cell patients in the simulation lab and clinical setting. Describe complications and management of sickle cell disease during pregnancy. Nursing care plan for sickle cell anemia nrsng nursing courses. The pathophysiology, medical and nursing management, and. Cultural competence case presentation sickle cell crisis race, gender heather hollowell md, marcus l. One of every 600 black people in the united states has sickle cell anemia. Sickle cell disease is a chronic illness that impacts physical and social development, often requiring multiple hospitalizations. Standards for the management of sickle cell disease in. Major complications of sickle cell disease and nursing implications.
Usually associated with lower respiratory symptoms, hypoxaemia and a new infiltrate on cxr. To develop appropriate pain care plans across environments of care, especially in the ed, nurses need to understand the contextual basis of the pathophysiology. Doctor of philosophy in nursing program, rush university, chicago il. Sickle cell disorders are a group of inherited conditions that affect the red blood cells erythrocytes. Ensure patients understand how to take pain medicines to manage acute pain. Primary care physicians play a key role in the medical home model of care for adults with scd. Erythromycin prophylaxis may be used as an alternative for suspected penicillin allergy. Sickle cell disease, complication, fetomaternal risk, active nursing management. Lennette benjamin 1 1comprehensive sickle cell center, montefiore medical center, new york, ny people with sickle cell disease scd are living longer, but their lives are impacted even more by the unpredictable intermittent or constant pain that is often. Panel members were selected by the national heart, lung, and blood institutes nhlbis leadership. Pediatric nurse care for sickle cell infants and children. Sickle cell anemia, or homozygous sickle cell disease, results from the inheritance of a sickle cell gene from both parents.
Sickle cell disease can produce an acute illness related to infarction of the lung tissue. With sickle cell disease, early intervention and longterm management through primary care is key to fighting andor preventing complications caused by infections in immunocompromised patients. Last, hydroxyurea has been found beneficial even in children as young as 2 years of age. The intervention included a simulation covering the care of a sickle cell patient in crisis. People at risk for inheriting the gene for sickle cell, are descended from people who are or were. Sickle cell trait originated many years ago in areas of the world where malaria was present. Sickle cell disease is a chronic, complex syndrome that requires a multidisciplinary approach in order for patients to receive the best care possible. Assess for causes of pain unrelated to sickle cell. The sickle cell membranes display abnormal charge topography, most probably caused by increased lipid peroxidation sickle reticulocytes show abnormaly high expression of antigen cd36 and integrin. Describe nursing care of a pregnant woman with sickle cell disease.
Maryland state school health services guideline management of sickle cell disease in schools2019 2 sickle cell disease sickle cell disease is a group of inherited red blood cell disorders affecting the protein known as hemoglobin. Sickle cell is a complex disease that can affect all parts of the body, which is why successful management plans focus on the patients overall health. A person who inherits the mutated gene from only one parent is a carrier of the trait. Help patients to understand potential triggers and avoidance strategies. Sickle cell pain crisis guidlines virginia premier. Martin md, leighann jones case scenariohistory a 22 year old africanamerican male comes to the ed with a case of sickle cell crisis and requests pain medicine. The most common types include sickle cell anemia hb ss, the sickle betathalassemias hb s. An update deborah gribbons, mn, rn lina kurdahi zahr, dnsc, rn susan r.
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