Disease Management Spotlight
Disease Management Spotlight | Sickle Cell Disease (SCD)
Valerie Mann-Jiles is an adult nurse practitioner board certified (DNP, ANP-BC) currently practicing at the Arthur G. James Cancer Hospital and Richard J. Solove Research Institute of Columbus, Ohio. She has been a nurse for 38 years.
"Sickle Cell Disease"
Management of the Adult Patient with Sickle Cell Disease
by Valerie Mann-Jiles, DNP, ANP-BC
Sickle cell disease (SCD) is a general term for a group of genetic disorders of hemoglobin S. Sickle cell anemia" is the name of a specific form of sickle cell disease in which there is homozygosity for the mutation that causes Hgb S.
The normal red blood cell contains three types of hemoglobin (Hb) A, Hb F or fetal, and Hb A2. The predominant hemoglobin present after six months of life is Hb A. The substitution of valine for glutamic acid is present in Hb S. Substituting lysine for glutamic acid results in hemoglobin C or Hb C.
Other forms of sickle cell disease include sickle-hemoglobin C disease, sickle beta-plus-thalassemia, and sickle beta-zero-thalassemia. Unlike sickle cell anemia, these other forms of sickle cell disease are compound heterozygous states in which the person has only one copy of the mutation that causes Hgb S and one copy of another abnormal hemoglobin gene.
It is important to know that "sickle cell anemia" is the proper name of a specific type of "sickle cell disease", and that "sickle cell disease" is a non-specific term. Because the different forms of sickle cell disease are quite different, one must be sure to specify the exact form of the disease in question.
Sickle cell anemia is one of the most common inherited blood anemias. The disease primarily affects Africans and African Americans. It is estimated that in the United States, some 70,000 African Americans are afflicted with the most severe form of sickle cell disease.
The sickled red blood cells tend to clog small blood vessels, depriving the tissues they serve of blood and oxygen. As a result the patient experiences a painful crisis or episode... Strokes or seizures can occur if the brain is affected. Lung infections resulting from the patient's reluctance to take deep breaths are a frequent complication. In addition, the sickled erythrocytes are fragile and subject to rupture and destruction, leading to hemolytic anemia reduction of oxygen-carrying hemoglobin caused by premature destruction of red blood cells and such symptoms as fatigue, jaundice, and headaches.
Patients with sickle cell anemia have steady-state or baseline anemia that varies in severity, with hemoglobin levels of 6-9 g/dl typical. Reticulocyte counts are elevated, reflecting new red blood cells replacing the rapidly destroyed older cells - red blood cell life span is markedly reduced in this disease (15 days vs. 120). Often, the white blood cell and platelet counts are elevated, and these cells may contribute to vaso-occlusion.
There is no universal cure for the disease, but advancements in treatment have improved median survival up to the fifth and sixth decades. Approximately 30 years ago the average life expectancy for persons with SCD was 14 years of age. Cerebral hemorrhage or shock is the usual cause of mortality in children. Recent studies have indicated that regular blood transfusions can prevent strokes in children. Anemia is treated with folic acid. Sickle cell crises may be treated with intravenous hydration, pain medication, antibiotics, oxygen, and transfusions. Hydroxyurea, formerly used as a cancer treatment, has been helpful to many adults with the disease, lessening the frequency and severity of crises.
The expanding adult sickle cell population places unique challenges on the health care delivery system. The application of evidence-based medicine to the management of adults with sickle cell disease is currently primarily driven by clinical expertise and patient preferences as there is a paucity of randomized controlled trials date to guide decision making.
The majority of patients with sickle cell disease do not receive care through comprehensive sickle cell centers. The management of acute complications, surgery and other interventions often involve physicians with limited experience in care for the adult patient with sickle cell disease. Thus there is a need to provide clinical guidelines that are useful for a wide spectrum of health care providers.
Health Care Maintenance
The US Preventive Services Task Force (USPSTF) assessed at (www.ahrq.gov/clinical/uspstfix.htm) has recommendations applicable to patients with SCD. Interventions should routinely be implemented, including screening for high blood pressure, lipid disorders, colorectal cancer, breast cancer, depression, primary prevention of cardiovascular events and counseling for tobacco use. There are other health maintenance concerns specific to patients with SCD. Ophthalmologic examination, assessment of liver, pulmonary and renal function should be performed at least annually. Recommendations from the Advisory Committee on Immunization practices for adults with SCD include: immunizations with the 23-valent pneumococcal polysaccharide, Haemophilis influenzae type b, meningococcal and hepatitis B vaccines. Influenza vaccination on an annual basis and revaccination for pneumococcus one time after 5 years are also indicated.
Pain Episode (Crisis)
Pain is the most common presenting symptom and frequent painful episodes are associated with increased mortality. An evidence-based approach to the management of acute and chronic pain in SCD has been published by the American Pain Society (APS) and can be assessed at www.ampainsoc.org. General treatment measures include medications as well as IV hydration with hypotonic fluids (D5W or D5, ½ NS) to drive water into the RBCs. Oxygen should be administered only if there is an underlying pulmonary problem and hypoxia is documented by pulse oximetry or arterial blood gases. During initial treatment pain medication should be given on a fixed or continuous basis, maintaining a steady serum drug level to improve pain control, complications and patient anxiety. Keep in mind side effects of narcotic analgesics may include itching from histamine release, respiratory depression, nausea, vomiting, hypotension, constipation, increased bladder tone, urinary retention and decreased seizure threshold.
A Multicenter study of Hydroxyurea (HU) revealed statistically and clinically significant reductions in pain episodes for patients with sickle cell anemia 18 years or older. HU stimulates the production of protective fetal hemoglobin within RBCs. Fetal hemoglobin is the predominant hemoglobin in utero and has an affinity for oxygen. HU cuts in half the number of pain episodes, blood transfusions and hospitalizations in adults. Mortality is also reduced after nine years. Monitor frequently for bone marrow suppression with monthly CBC.
Minimum evaluation comprises a CBC with a WBC differential, pulse oximetry, and urinalysis. Consider CMP. Monitoring of labs should occur daily or every other day during prolonged hospitalization.
Transfusion remains a mainstay in the management of patients with SCD. Indications for transfusion: acute neurological event, (stroke), acute chest syndrome (ACS), multiorgan failure, or RBC sequestration in the spleen or liver, phenotypic packed RBC transfusions are indicated. Alloimmunization occurs in up to 30% of adult patients who receive frequent blood transfusions. The goal is to establish <30% HbS in the circulation, with a hemoglobin not to exceed 10. Increased hemoglobin levels of > 12 are associated with viscosity and complications. Transfusions during pregnancy are the same for the general SCD patient with the exception of refractory pre-eclampsia. Studies have revealed no correlation or significant difference between the degree of anemia in women with SCD and obstetrical complications, perinatal complications or birth weight. However, prophylactic transfusion was associated with a significant reduction in pain episodes.
Research to gain understanding of the psychosocial impact of SCD revealed significant burdens on patients which affect their physical, psychological, social and occupational wellbeing as well as level of independence. The day to day stress of the disease and fear of death may lead to depression in this patient population, which could increase the incidence and severity of painful episodes. Evaluation and treatment of depression, occupational and physical therapy, pharmacologic therapy, support system of family and health care providers may help. The growing adult SCD population provides many challenges for health care professionals, a better understanding of this adult SCD population and the seriousness of the disease and its complications may ultimately improve health care services for this population.
Anie, K., Steptoe, A., Ball, S., Dick, M., & Smalling, B. (2002). Coping and health service utilisation in a UK study of paediatric sickle pain. Archives of Disease in Childhood, 86, 325-329. Retrieved August 31, 2005, from http://adc.bmjjournals.com
Ashley-Koch, A., Yang, Q., & Olney, R. S. (2000). Sickle Hemoglobin (Hb S) Allele and sickle cell disease. American Journal of Genetics, 151(9), 839-845.
Ballas, S. (2001). Sickle cell disease: Current clinical management. Seminars in Hematology, 38(4), 307-314.
Bloom, M. (1995). Understanding sickle cell disease. Jackson, MS: University Press of Mississippi.
Brown, R. T., Buchanan, I., Doepke, K., Eckman, J. R., Baldwin, K., Goonan, B., et al. (1993). Cognitive and academic functioning in children with sickle-cell disease. Journal of Clinical Child Psychology, 22(2), 207-218.
Lottenberg, R., & Hassell, K., (2005). An evidence-based approach to the treatment of adults with sickle cell disease. [ Electronic version]. American Society of Hematology 58-65.
National Institutes of Health National Heart, Lung and Blood Institute. (2002). The management of sickle cell disease (4th ed.). Bethesda, MD: Author.
Thomas, V. & Taylor, L. (2002). The psychosocial experience of people with sickle cell disease and its impact on QOL: Qualitative findings from focus groups [Electronic version]. British Journal of Health Psychology 7(3), 345-363.
©2013 Valerie Mann-Jiles, DNP, ANP-BC All Rights Reserved.
Dr. Valerie Mann-Jiles
Adult Nurse Practitioner
Arthur G. James Cancer Hospital and Richard J. Solove Research Institute
Valerie is a certified nurse practitioner at the Arthur G, James Cancer Hospital with the Adult Comprehensive Sickle Cell Program.
Prior to working at the Arthur G, James Cancer Hospital, she devoted 17 years of her nursing career in the minority community at the Columbus Health Department In various roles and positions. These positions include home health nurse, outpatient clinic nurse, nurse manager, occupational health assistant supervisor and case manager for the Breast and Cervical Cancer Program.
Her nursing career began in 1979 as a license practical nurse. Since moving to Columbus Valerie has received an Associate Degree in nursing, from Hocking Technical College, a Bachelor of Science degree in nursing from Ohio University, Master's Degree in Nursing from Capital University, a Post Master's-Nurse Practitioner Certificate from The Ohio State University and Valerie completed the Doctor of Nursing Practice Degree at Case Western Reserve University in 2007.
Valerie is a member of several organizations, The Ohio Association of Advanced Practice Nurses, American Academy of Nurse Practitioners, and Sigma Theta Tau honor society. Valerie is a member of the Integrative care, Diversity and Support Group Facilitator's committees at the James. Previous memberships have included the Columbus Black Nurses Association and The National Black Leadership Initiative on Cancer.
Research interests are spiritual care, health care disparities, cultural competency and coping with chronic illness related to the sickle cell population. She has written an unpublished thesis on Clinic Nurses' Attitudes and Beliefs toward Spiritual Care in an Urban Setting and in 2009 published an article in the American Academy of Nurse Practitioner's titled Quality of Life of Adult Patients with Sickle Cell Disease.
As a member of the Central Seventh Day Adventist church she has served as an ordained deaconess, religious liberty leader, and a member of the health, and temperance department. In addition to church related activities, she has served as a volunteer for the Columbus Health and Human Services Mentorship Program, and visitation chaplain at the Riverside Methodist Hospital. Valerie is currently a member of the Worthington Seventh Day Adventist Church, she serves on the welcome committee as a greeter, and teaches the adult Sabbath school class.
Recognition awards were received from the Central Seventh Day Adventist Church, Columbus Health Department, and Columbus Health and Human Services Mentorship Program and The Ohio State University/James Care Center for excellence in Patient Education and excellence in Patient Satisfaction.
Most important, Dr. Mann-Jiles loves the Lord, and has been a Christian for 50 years. She is a theologian and Christian educator, and has studied courses in theological studies at Capital University, of Columbus, Ohio. In addition, she enjoys ministering, serving, and teaching the "Word of God" to others. She is a Faith Community Nurse (FCN), and committed to educating the local community, congregates, and churches in relevant areas of Health Ministry (mind, body, spirit).
Valerie is a native of Trenton, NJ, the wife of Melvin Jiles and mother of one son Michael.