Welcome to the training on Sickle Cell Disease and Trait provided by the Texas Health and Human Services Commission (HHSC) and the Texas Department of State Health Services (DSHS).
The goal of this module is to equip Texas Health Steps providers and others with the ability to coordinate care for children with sickle cell disease and trait, collaborate with other health-care professionals, and communicate effectively with patients and families.
Texas Health Steps providers and other interested health-care professionals.
Specific Learning Objectives
After completing the activities of this module, you will be able to:
1. Differentiate sickle cell disease from sickle cell trait.
2. Assess when a patient should be referred to a pediatric hematologist.
3. Manage ongoing care of patients with sickle cell disease and sickle cell trait in the primary care setting.
4. Specify culturally effective strategies for communicating with patients and families.
Please note this module expires on 11/14/2020.
This module was released on 11/14/2017.
Continuing Medical Education
The Texas Department of State Health Services, Continuing Education Service is accredited by the Texas Medical Association to provide continuing medical education for physicians.
The Texas Department of State Health Services, Continuing Education Service designates this enduring material for a maximum of 1.25 AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.
Continuing Nursing Education
The Texas Department of State Health Services, Continuing Education Service is accredited as a provider of continuing nursing education by the American Nurses Credentialing Center’s Commission on Accreditation. The Texas Department of State Health Services, Continuing Education Service has awarded 1.25 contact hour(s) of Continuing Nursing Education.
The Texas Department of State Health Services, Continuing Education Service under sponsor number CS3065 has been approved by the Texas State Board of Social Worker Examiners to offer continuing education contact hours to social workers. The approved status of The Texas Department of State Health Services, Continuing Education Service expires annually on December 31. The Texas Department of State Health Services, Continuing Education Service has awarded 1.25 contact hour(s) of Continuing Social Work Education.
Certificate of Attendance
The Texas Department of State Health Services, Continuing Education Service has designated 1.25 hour(s) for attendance.
Certified Community Health Worker
The Texas Department of State Health Services, Promotor(a)/Community Health Worker Training and Certification Program has certified this course for 1.25 contact hour(s) of continuing education for Certified Community Health Workers and Community Health Worker Instructors.
One of the requirements of continuing education is disclosure of the following information to the learner:
- Notice of requirements for successful completion of continuing education activity. To receive continuing education credit the learner must successfully complete the following activities:
- Create a Texas Health Steps account.
- Complete on-line registration process.
- Thoroughly read the content of the module.
- Complete the on-line examination.
- Complete the evaluation.
- Commercial Support.
The THSTEPS Web-based Continuing Education Series has received no commercial support.
- Disclosure of Relevant Financial Relationships.
The THSTEPS Continuing Education Planning Committee and the authors of these modules have no relevant financial relationships to disclose.
- Non-Endorsement Statement.
Accredited status does not imply endorsement of any commercial products or services by the Department of State Health Services, Continuing Education Service; Texas Medical Association; or American Nurse Credentialing Center.
- Off-Label Use.
Using a disclosure review process, the THSTEPS Continuing Education Planning Committee has examined documents and has concluded that the authors of these modules have not included content that discusses off-label use (use of products for a purpose other than that for which they were approved by the Food and Drug Administration).
The following are policies and definitions of terms related to continuing education disclosure:
The intent of disclosure is to allow Department of State Health Services (DSHS) Continuing Education Service the opportunity to resolve any potential conflicts of interest to assure balance, independence, objectivity and scientific rigor in all of its Continuing Education activities.
All faculty, planners, speakers and authors of Department of State Health Services (DSHS) Continuing Education Service sponsored activities are expected to disclose to the Department of State Health Services (DSHS) Continuing Education Service any relevant financial, relationships with any commercial or personal interest that produces health care goods or services concerned with the content of an educational presentation. Faculty, planners, speakers and authors must also disclose where there are any other potentially biasing relationships of a professional or personal nature.
Glossary of Terms
Conflict of Interest: Circumstances create a conflict of interest when an individual has an opportunity to affect Continuing Education content about products or services of a commercial interest with which she/he has a financial relationship or where there are any other potentially biasing relationships of a professional or personal nature.
Commercial Interest: Any entity producing, marketing, re-selling, or distributing health care goods or services consumed by, or used on, patients.
Financial Relationships: Those relationships in which the individual benefits by receiving a salary, royalty, intellectual property rights, consulting fee, honoraria, ownership interest (e.g., stocks, stock options or other ownership interest, excluding diversified mutual funds), or other financial benefit. Financial benefits are usually associated with roles such as employment, management position, independent contractor (including contracted research), consulting, teaching, membership on advisory committees or review panels, board membership, and other activities for which remuneration is received or expected. Relevant financial relationships would include those within the past 12 months of the person involved in the activity and a spouse or partner. Relevant financial relationships of your spouse or partner are those of which you are aware at the time of this disclosure.
Off Label: Using products for a purpose other that that for which it was approved by the Food and Drug Administration (FDA).
Texas Health Steps courses are best viewed using a current browser. If you are using an out-of-date browser or a version of Internet Explorer less than 11, lesson progress and interactive features may not function properly.
- Electronic Preventive Services Selector, a web-based tool for mobile devices that allows for primary care clinicians to identify screening, counseling, and preventive medication services recommended by the U.S. Preventive Services Task Force.
- National Center for Biotechnology Information Genes and Disease, a collection of articles.
- National Institutes of Health, The Management of Sickle Cell Disease, fourth edition.
- Parent Companion First Five Years, a guide for Texas parents and caregivers of children with diagnosed or suspected disabilities from birth through 5 years of age.
- Sickle Cell Disease Association of America
- Sickle Cell Information Center
- Sickle Cell Trait Screening in Athletes: Pediatricians’ Attitudes and Concerns. Pediatrics,128(3):477-83.
- Texas Department of State Health Services, HbSS (sickle cell anemia) ACT Sheet and FACT Sheet.
- Texas Department of State Health Services, HbSΒ+-Thal (hemoglobin S/beta-thalassemia) ACT Sheet and FACT Sheet.
- Texas Department of State Health Services, HbSC (hemoglobin SC disease) ACT Sheet and FACT Sheet.
- Texas Department of State Health Services Sickle Cell Disease web page.
- Texas Department of State Health Services, Newborn Screening Web Application sign up. (Scroll down to Result Reports and Remote Data Systems). Complete and submit one Facility Security Agreement per facility and one Web User Access Agreement per user.
- Texas Department of State Health Services, list of genetic service providers in Texas.
- Texas Health Steps Periodicity Schedule.
- U.S. Department of Health and Human Services, Sickle Cell Disease: Increasing Access and Improving Care.
One of the key aspects of your relationship with patients and their families is to educate them about medical conditions and help them identify other sources for reliable information. A wealth of resources about sickle cell disease and sickle cell trait, including support groups, are available for families. Many resources are available for health-care providers as well. Below is a resource list to share with families and another list of resources for providers.
- American Sickle Cell Anemia Association
- Baby’s First Test, national newborn screening clearinghouse.
- Centers for Disease Control and Prevention, Sickle Cell Disease web page.
- Centers for Disease Control and Prevention. Do You Use the Emergency Department for Care of Sickle Cell Disease? What to Know Before You Go.
- Emory University, Sickle Cell Information Center
- March of Dimes: A Parent’s Guide to Newborn Screening (5:11-minute video)
- National Alliance for Caregiving
- National Athletic Trainers’ Association, Sickle Cell Trait
- National Collegiate Athletic Association (NCAA), Fact Sheet on Sickle Cell Trait for Student Athletes
- National Heart, Lung, and Blood Institute, What is Sickle Cell Disease?
- Navigate Life Texas, a multilingual website created by parents for parents of children with disabilities and special health-care needs. This unique site offers comprehensive, relevant, and reliable information for families, professionals, advocates, and anyone working with children who have disabilities and their families. Sponsored by the Texas Interagency Task Force on Children with Special Needs.
- Sickle Cell Association of Texas - Marc Thomas Foundation. Phone: 512-458-9767 (Austin); 210-315-7473 (San Antonio); 361-894-7635 (South Texas - Spanish speaking)
- Sickle Cell Disease Association of North Texas, Phone: 817-882-0990
- Texas Department of State Health Services, Case Management for Children and Pregnant Women
- Texas Department of State Health Services, Family Support Services. A program to help families care for children with special health-care needs at home. Services are provided by the Children with Special Health Care Needs (CSHCN) Services Program, a branch of the Texas Department of State Health Services.
- Texas Department of State Health Services, Sickle Cell Disease web page
- Texas Department of State Health Services, Things to Know About Sickle Cell Trait
- Texas Health and Human Services, Aging and Disability Resource Centers. Information about state and federal health benefits; local programs and services such as home care, meals, transportation, legal help, attendant care, respite support, and housing; and a list of resource center locations in Texas. ADRC help line at 855-937-2372 (855-YES-ADRC).
- Texas Health and Human Services, Take Time Texas, a web page with a state inventory of respite services.
- Texas Parent to Parent, offers peer support for parents of children with special health-care needs.
- Adelman, R. D., Tmanova, L. L., Delgado, D., Dion, S., & Lachs, M. S. (2014). Caregiver Burden: A Clinical Review. JAMA, The Journal of the American Medical Association, 311(10): 1052-1060.
- Amendah, D. D., Mvundura, M., Kavanagh, P. L., Sprinz, P. G., & Grosse, S. D. (2010). Sickle-cell disease related pediatric medical expenditures in the U.S. American Journal of Preventive Medicine, 38(4 Suppl): S550-6.
- American Academy of Pediatrics, Council On Sports Medicine and Fitness and Council on School Health. (2011). Policy Statement: Climatic Heat Stress and Exercising Children and Adolescents. Pediatrics, 128(3): e741-e747.
- American Academy of Pediatrics, Section on Hematology/Oncology and Committee on Genetics. (2002, reaffirmed 2006 & 2011). Health Supervision for Children with Sickle Cell Disease. Pediatrics, 109, 526-535. http://pediatrics.aappublications.org/content/109/3/526.full functional 7/7/17
- American College of Sports Medicine and the National Collegiate Athletic Association. (2012). Joint Statement (on) Sickle Cell Trait and Exercise.
- American Society of Hematology and the NCAA Sport Science Institute. Care for the Student-Athlete with Sickle Cell Trait. (n.d.).
- American Sickle Cell Anemia Association.
- Arkansas Department of Health. (2011). Newborn Screening.
- Brousseau, D. C., Panepinto, J. A., Nimmer, M., & Hoffmann, R. G. (2009). The number of people with sickle-cell disease in the United States: national and state estimates. American Journal of Hematology, 85 (1), 77-78.
- Boyd, J. H., Macklin, E. A., Strunk, R. C., & DeBaun, M. R. (2006). Asthma is associated with acute chest syndrome and pain in children with sickle cell anemia. Blood, 108(9), 2923-2927.
- Campinha-Bacote, J. (2002). The Process of Cultural Competence in the Delivery of Healthcare Services. Transcultural C.A.R.E. Associates, Cincinnati, OH.
- Carlson, K. J., Eisenstat, S. A., & Ziporyn, T. D. (2004). The New Harvard Guide to Women's Health. Cambridge, MA: Harvard University Press.
- Centers for Disease Control and Prevention. (2017). Sickle Cell Data Collection (SCDDC) Program.
- Centers for Disease Control and Prevention. (2016). Sickle Cell Disease: Data & Statistics.
- Centers for Disease Control and Prevention. (2014) Incidence of Sickle Cell Trait — United States, 2010. Morbidity and Mortality Weekly Report (MMWR), 63(49);1155-1158.
- Centers for Disease Control and Prevention. (2012). Sickle Cell Disease.
- Centers for Disease Control and Prevention. (n.d.). Do You Use the Emergency Department for Care of Sickle Cell Disease? What to Know Before You Go.
- Chapman, J., & Bhimji, S. (2017). Splenic Infarcts. StatPearls Publishing.
- DeBaun, M. R., & Telfair, J. (2012). Transition and Sickle Cell Disease. Pediatrics, 130(5): 926 -935.
- Dowling, M. M., Quinn, C. T., Rogers, Z. R., & Buchanan, G. R. (2010). Acute silent cerebral infarction in children with sickle cell anemia. Pediatric Blood & Cancer, 54(3), 461-464.
- Kark, J. (2000). Sickle Cell Trait. Howard University School of Medicine, Center for Sickle Cell Disease.
- Kaye, C. I. & American Academy of Pediatrics Committee on Genetics. (2006, reaffirmed 2011). Newborn Screening Fact Sheets. Pediatrics, 118(3), 1304-1312.
- Koopmans, J., Cox, L. A., Benjamin, H., Clayton, E. W., & Ross, L. F. (2011). Sickle Cell Trait Screening in Athletes: Pediatricians’ Attitudes and Concerns. Pediatrics, 128(3):477-83.
- Kristiansen, C. (2014). Research needed to treat sickle cell disease in Africa. Global Health Matters, 13(6), National Institutes of Health.
- Lanzkron, S., Carroll, C. P., & Haywood, C. Jr. (2010). The Burden of Emergency Department Use for Sickle Cell Disease: An Analysis of the National Emergency Department Sample Database. American Journal of Hematology, 85(10): 797-799.
- Lovett, P. B., Sule, H. P., & Lopez, B. C. (2014). Sickle cell disease in the emergency department. Emergency Medical Clinics of North America, 32(3): 629-647.
- March of Dimes. (2014). Sickle Cell Disease and Your Baby.
- Mayo Clinic. (2016). Sickle Cell Anemia.
- National Center for Medical Home Implementation. (2017). What is medical home?
- National Collegiate Athletic Association. (2010). A Fact Sheet for Student-Athletes, Sickle Cell Trait.
- National Diabetes Information Clearinghouse. (2014). Sickle Cell Trait and Other Hemoglobinopathies and Diabetes: Important Information for Physicians.
- National Heart, Lung, and Blood Institute. (2016) Who Is at Risk for Sickle Cell Disease?
- National Heart, Lung, and Blood Institute. (2013). Clinician-scientist sets sickle cell research agenda by engaging with the community, listening to families and patients.
- National Heart, Lung, and Blood Institute. Sickle Cell Disease Research & Care.
- National Heart, Lung, and Blood Institute. (2011). Our Story: Living with and Managing Sickle Cell Disease (Nicholas H.). (video)
- Nemours Foundation. (2015). TeensHealth. Sickle Cell Disease.
- Paulukonis, S. T., Feuchtbaum, L. B., Coates, T. D., Neumayr, L. D., Treadwell, M. J., Vichinsky, E. P., & Hulihan, M. M. (2017). Emergency department utilization by Californians with sickle cell disease, 2005-2014. Pediatric Blood & Cancer, 64(6): doi: 10.1002/pbc.26390.
- Quinn, C. T., Rogers, Z. R., McCavit, T. L., & Buchanan, G. R. (2010). Improved survival of children and adolescents with sickle cell disease. Blood, 115(17): 3447-3452.
- Shetty, A., & Matrana, M. R. (2014). Renal Medullary Carcinoma: A Case Report and Brief Review of the Literature. The Ochsner Journal, 14:270–275.
- Sickle Cell Disease Association of America. (2016). About SCT & SCD.
- Texas Department of State Health Services. (2016). ACT Sheets.
- Texas Department of State Health Services. (2014). Genetics Provider List.
- Texas Department of State Health Services. (2012). Pediatric Hemoglobinopathy Consultants.
- Texas Department of State Health Services. (2014). Newborn Screening Clinical Care Coordination.
- Texas Department of State Health Services. (2014). Newborn Screening Specimen Collection Video.
- Texas Department of State Health Services. (2017). Newborn Screening Laboratory.
- Texas Department of State Health Services. (2017). Texas Health Steps Medical Checkup Periodicity Schedule for Infants, Children and Adolescents.
- Texas Department of State Health Services. (2017). Newborn Screening Remote Data Services.
- U.S. Census Bureau. (2016). Hispanic origin.
- U.S. Census Bureau. (2011). Overview of Race and Hispanic Origin: 2010.
- U.S. Department of Health and Human Services, Health Resources and Services Administration. (2017). Baby’s First Test.
- Williams, T. M., Mwangi, T. W., Roberts, D. J., Alexander, N. D., Wambua, S., Kortok, M. . . . Marsh, K. (2005). An immune basis for malaria protection by the sickle cell trait. PLOS Medicine, 2(5): e128.
- World Health Organization. (2011). Sickle-cell disease and other haemoglobin disorders. Fact Sheet, #308.
- Yawn, B. P., Buchanan, G. R., Afenyi-Annan, A. N., Ballas, S. K., Hassell, K. L., James, A. H., . . . John-Sowah, J. (2014). Management of Sickle Cell Disease: Summary of the 2014 Evidence-Based Report by Expert Panel Members. JAMA,312(10):1033-1048.
- Yusuf, H. R., Atrash, H. K., Grosse, S. D., Parker, C. S., & Grant, A. M. (2010). Emergency department visits made by patients with sickle cell disease: A descriptive study, 1999-2007. American Journal of Preventive Medicine, 38(4 Suppl): S536-41.
- Aidoo, M., Terlouw, D. J., Kolczak, M. S., McElroy, P. D., ter Kuile, F., Kariuki S., … Udhayakumar, V. (2002). Protective effects of the sickle cell gene against malaria morbidity and mortality. The Lancet, 359:9314, 1311-1312.
- Ashley-Koch, A., Yang, Q., & Olney, R. S. (2000). Sickle hemoglobin (Hb S) and sickle cell disease: A HuGE review. American Journal of Epidemiology, 151:9, 839-845.
- Forget, B. G., Higgs, D. R., Steinberg, M., & Nagel, R. L. (2009). Disorders of Hemoglobin: Genetics, Pathophysiology and Clinical Management. Second Edition. Cambridge University Press; Cambridge, UK.
- Hamideh, D. & Alvarez, O. (2013). Sickle cell disease related mortality in the United States (1999–2009). Pediatric Blood Cancer, 60(9):1482–1486.
- Harmon, K. G., Asif, I. M., Klossner, D., & Drezner, J. A. (2011). Incidence of sudden cardiac death in national collegiate athletic association athletes. Circulation, 123 (15): 1594–600.
- Information Center for Sickle Cell and Thalassemic Disorders.
- Jones, A. M, Seibert, J. J., Nichols, F. T., Kinder, D. L., Cox, K., Luden, J., . . . Adams, R. J. (2001). Comparison of transcranial color Doppler imaging (TCDI) and transcranial Doppler (TCD) in children with sickle-cell anemia. Pediatric Radiology, 31(7):461-9.
- Lab Tests Online. (2013). Hemoglobinopathy Evaluation.
- Lin, K. & Barton, M. (2007). Screening for Hemoglobinopathies in Newborns: Reaffirmation Update for the U.S. Preventive Services Task Force. Evidence Synthesis No. 52. Rockville, MD: Agency for Healthcare Research and Quality. AHRQ Publication No. 07-05104-EF-1.
- Pegelow, C. H., Macklin, E. A., Moser, F. G., Wang, W. C., Bello, J. A., Miller, S. T., . . . Kinney, T. R. (2002). Longitudinal changes in brain magnetic resonance imaging findings in children with sickle cell disease. Blood, 99(8), 3014-18.
- Smith, W. R., Penberthy, C. T., Bovbjerg, V. E., McClish, D. K., Roberts, J. D., Dahman, B., . . . Roseff, S. D. (2008). Daily assessment of pain in adults with sickle cell disease. Annals of Internal Medicine, 148, 94-101.
- Tarini, B. A., Brooks, M. A., & Bundy, D. G. (2012). A policy impact analysis of the mandatory NCAA sickle cell trait screening program. Health Services Research, 47:446-461.
The medical definitions in this module were adapted from the Centers for Disease Control and Prevention (CDC), MedicineNet.com, Merriam-Webster Dictionary, and the U.S. National Library of Medicine.