Table of Contents
Patients, Families and Friends
Health Care Providers
|Specific Problems: Sickle Cell Trait|
|Health Care Providers - Problem Oriented Clinical Guidelines|
by James Eckman, M.D. and Allan Platt, PA-C
Individuals with sickle cell trait are generally asymptomatic and have no abnormal physical findings. Their laboratory evaluation is normal with no anemia, no evidence of hemolysis, and no laboratory abnormalities other than hemoglobin AS on hemoglobin electrophoresis. Many individuals will have decreased ability to concentrate their urine. There may be an increased incidence of urinary tract infection during pregnancy. Painless hematurea does occur in 1 to 4 % of individuals with sickle cell trait . This complication is usually not a significant problem, however, a minority of individuals may have significant problems with recurrent hematurea requiring medical intervention, transfusion, and iron therapy. Complications such as splenic infarction, pain episodes, and sudden death may be induced by severe hypoxia, severe dehydration, and exertion at the limits of human endurance.
Present Illness. No symptoms
Past Medical History No medical problems
Family History. Document history of sickle hemoglobin, thalassemia, and other hemoglobin variants.
Review of Symptoms. Asymptomatic
Individuals with sickle trait are at an increased risk of serious complications when exercising at the extremes of human endurance. Adequate hydration and avoidance of excessive fluid loss with minimize problems. With extreme hypoxia and dehydration, rarely complications like splenic infarction and pain episodes may occur. Hydration and grade exercise are important preventive measures. White individuals with sickle cell trait may be at higher risk for these complications. All individuals participating in sports, whether or not they have sickle cell trait, should have adequate hydration before during and after exertion in hot conditions.
Individuals with hematuria should actively hydrate themselves before during and after physical exertion. In individuals who have frequent, severe or persistent hematuria may need to avoid activities that regularly cause episodes. Hydration and bed rest may abort episodes if done imediately.
Parent and Patient Education
Patients and their families should be counseled about their risks of having a child with sickle cell disease based on their and their partners genotype. Both structural hemoglobin variants and beta thalassemia need to be excluded before providing such education.
Individuals with hematuria need to be educated about the benign nature of hematuria in sickle cell trait. Preventive measures and avoidance of precipitating activities need to be stressed in the rare individual with persistent or recurrent episodes. See our FAQ page on sickle cell trait by Clicking here
New Sports Guidelines for Sickle Cell Trait 8/17/2004 12:57:00 PM - New Youth Football Recommendations Emphasize Practice, Training Safety in the Heat; Expert Panel: Youth Football Coaches Key to Safety
Sickle Cell Trait article by Dr. John Kark at http://sickle.bwh.harvard.edu/sickle_trait.html - An excellent and comprehensive review of potential problems facing those with sickle cell trait
Steinberg MH Sickle cell trait in Disorders of Hemoglobin: Genetics, Pathophysiology and Clinical Management. Steinberg MH, Forget BG, Higgs DR, Nagel RL. Cambridge University Press Cambridge UK 2001. Pp. 811-830.
Sears DA. Sickle Cell Trait in Sickle Cell Disease: Basic Principles and Clinical Practice Embury SH, Hebbel RP, Mohandas N, Steinberg MH eds. Raven Press NewYork pp. 381-394.
Sears DA. The morbidity of sickle cell trait: A review of the literature. Am J Med 64:1021-1036, 1978.
Kark JA, Posey DM, Schumacher HR et al. Sickle-cell trait as a risk factor for sudden death in physical training. New Engl J Med 317:781-787, 1987.
Kark JA, Ward FT. Exercise and hemoglobin S. Semin. Hematol 181-225, 1994.
McInnes BK III. The management of hamaturia associated with sickle hemoglobinopathies. J Urol 124:171-174, 1980.
Baldree LA, Ault BH, Chesney C, Stapleton FB. Intravenous desmopressin acetate in children with sickle cell trait and persistant macroscopic hematurea. Pediatrics 86:238-243, 1990.