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Sickle Cell Disease Awareness: Are You at Risk?.

What is Sickle Cell Disease?

According to the American Society of Hematology (ASH) (2018), Sickle Cell Disease (SCD) is the most common inherited blood disorder worldwide affecting more than 100,000 Americans, while one to three million Americans carry Sickle Cell Trait (SCT). Each individual possesses two hemoglobin genes, one from each parent. To have SCD means that a person has inherited two sickle cell genes, one from each parent. For an individual to have SCT, only one gene from one parent is passed on to a child. SCT is not a disease and individuals with SCT may be called carriers of the sickle cell gene.

Hemoglobin, found inside red blood cells, carries oxygen throughout the body. However, in SCD, red blood cells become abnormally shaped and cannot move easily through the blood vessels. A sickled hemoglobin cell, is "c" shaped, may become stuck inside blood vessels, blocking blood flow and causing damage to the body's normal cells and tissues. Because of this, patients with SCD develop serious health complications such as pain, infections, or worse, stroke or organ damage, just to name a few.

Why should nurses be aware of Sickle Cell Disease?

In young children, SCD is associated with increased multisystem morbidity (Quinn, Rogers, McCavit, & Buchanan, 2010). Quinn et al. (2010) noted a recent dramatic improvement in the survival rate of young children with SCD. This is thought to be related to early identification of disease on newborn screening and preventative therapies such as prophylactic penicillin and successful vaccination against Streptococcus pneumonia, Meningococcal and Haemophillus influenza type b. While it appears that mortality rates have decreased in younger children, the survival rate for young adults has shifted. Quinn et al. (2010) identified a higher risk of mortality in patients who are transitioning from pediatric medical care to adult medical care, thus, making them extremely vulnerable. There are many factors that may contribute to the increase of vulnerability of these patients. Crosby, Quinn and Kalinyak (2015) stated that there is an increased probability of chronic organ damage and a lack of comprehensive care programs in place for these adolescent/young adults. Care of the transitioning SCD patient is frequently left up to the primary care provider who may not possess the same expertise as a pediatric hematologist. As this population continues to age and transition, more awareness and education are needed for health care providers, caregivers, and patients.

The Impact of Sickle Cells Disease on Texans

Through state newborn screening programs, all newborns in the United States are screened for SCT and SCD (ASH, 2018). How many children are diagnosed with SCD or SCT in Texas? According to the Texas Department of State Health Services (DSHS) Newborn Screening Program, approximately 180 children are diagnosed with SCD and over 5,000 children are found to carry SCT annually in the State of Texas.

In 2016, DSHS appointed members to the Sickle Cell Advisory Committee, as required by 25 Texas Administrative Code [section]37.420. Their focus was to examine strategies for, and formulate recommendations to heighten public awareness of SCT and SCD. Through their work they recognized the need for educational materials (both in English and in Spanish) to provide resources not only for health care professionals, but also for patients and the public. In addition to the identified areas of need, the committee initiated and/or completed such tasks as creating a Sickle Cell Resources webpage, updating the DSHS website for Newborn Screening and identifying the potential for further online training via the Texas Health Steps Sickle Cell Disease and Trait online course (Department of State Health Services Sickle Cell Advisory Committee, 2017).

DSHS has a wealth of information for providers, patients, and their families on their website. Efforts put forth by this committee can be viewed on DSHS' website at: http://www.dshs.texas.gov/newborn/SCResources.aspx

In addition, Texas Health Steps also has a FREE online CE course available to healthcare professionals entitled, Sickle Cell Disease and Trait, accessible at: https://www.txhealthsteps.com/365-sickle-cell-disease-and-trait

References

American Society of Hematology. (2018). Sickle cell disease. Retrieved from: http://www.hematology.org/Patients/Anemia/Sickle-Cell.aspx

Crosby, L.E., Quinn, C.T., & Kalinyak, K.A. (2015). A biopsychosocial model for the management of patients with Sickle-Cell Disease transitioning to adult medical care. Advances in Therapy, 32(4), 293-305. doi: 10.1007/s12325-015-0197-1

Department of State Health Services Sickle Cell Advisory Committee. (2017). Sickle Cell Advisory Committee Report. Retrieved from: http://www.dshs.texas.gov/test/pdf/Sickle-Cell-Advisory-Committee-Report.doc

Quinn, C.T., Rogers, Z.R., McCavit, T.L., & Buchanan, G.R. (2010). Improved survival of children and adolescents with sickle cell disease. Blood Journal, 115(17), 3447-3452. doi: 10.1182/blood-2009-07-233700
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Publication:Texas Board of Nursing Bulletin
Geographic Code:1U7TX
Date:Oct 1, 2018
Words:782
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