Sickle cell trait or the carrier state for sickle cell disease occurs in high frequency among people of African-American and/or Hispanic descent, but it can also occur in people of all ethnicities. For nearly three decades, screening for sickle cell disease and related hemoglobinopathies has been part of state-mandated universal newborn screening programs in the US. When properly conducted, testing for hemoglobinopathies also detects the carrier state for sickle cell disease and other hemoglobinopathies. These results have lifelong validity and for most people testing does not need to be repeated.

The sickle cell carrier state is associated with normal hematological values and normal life span. Knowledge of carrier status is important for reproductive planning since carriers can have children with sickle cell disease. Although uncommon, persons who are carriers for sickle cell may develop specific rare symptoms that may be related to sickle cell trait. These symptoms include hematuria, mildly reduced ability to concentrate urine, and a tendency to develop glaucoma secondary to hyphema after eye injuries. Knowledge of medical problems possibly associated with sickle cell trait and their appropriate management is an expectation of good clinical practice.

Persons with sickle cell trait have been shown to be more vulnerable than those without trait to heat stroke and muscle breakdown (rhabdomyolysis) when subjected to strenuous forced exercise in military training under unfavorable environmental conditions. Follow up studies demonstrated that the incidence of this problem can be reduced in all recruits by avoiding dehydration and overheating during training. Although similar studies have not been conducted in athletes, and exercise-induced rhabdomyolysis occurs in persons without sickle cell trait, concern has been raised as to the vulnerability of athletes with sickle cell trait to rhabdomyolysis during strenuous conditioning regimens. Deaths of healthy young athletes during conditioning are extremely rare, and are associated with sickle cell trait in less than half of the cases. Moreover, in many cases, such deaths are attributed to sickle cell trait because of the presence of sickled cells in tissues at autopsy, even though the occurrence of post-mortem sickling is well documented in sickle cell trait and expected with death from any cause.

Screening athletes for sickle cell trait and subjecting carriers to alternative training regimens, as recommended by the National Collegiate Athletic Association (NCAA) and National Athletes Trainer’s Association (NATA), has not been demonstrated to reduce the incidence of training-related deaths. Nevertheless this approach carries great risk of stigmatization and discrimination against athletes with sickle cell trait. The NCAA mandate for sickle trait screening does not provide adequate assurance of the privacy of genetic information nor protection from the discriminatory use of such information.

The Sickle Cell Disease Association of America (SCDAA) supports the implementation of universal, safe training guidelines for all athletes, and to rigorously educate and improve the capacity of athletic coaches and trainers to recognize signs and symptoms of heat related illness and to provide medical care to athletes who become ill or injured under their supervision. Given the lack of scientific evidence that substantiates a significant correlation between sickle cell trait in athletes and training related sudden death, SCDAA does not support screening of athletes for sickle cell trait as a means to reduce heat related illness or death in athletes who are carriers. The common sense precautions recommended by NATA — building up exercise intensity gradually, responding to athletes reporting symptoms of physical distress, and avoiding overheating and dehydration – if applied universally, would make athletic training safer for all and would obviate the need to identify carriers of the sickle cell gene in the athletic setting. Therefore, the need to identify athletes who are carriers of the sickle cell allele in the athletic setting is unwarranted.