Standard Operating Procedure

Monitoring and Care of Sickle Cell Disease/Trait

Policy: Monitoring athletes who suffer from either sickle cell disease or sickle cell trait is of vital
importance to be able to provide them with quality care in the field of sports. Those suffering from sickle cell disease or trait should be excluded from contact sports or sports of high exertional levels. These individuals also need to be monitored to help them maintain proper hydration and avoid overheating as these things may cause a flare-up of sickling. Certified athletic trainers should also be monitoring these athletes for a major condition resulting from having sickle cell disease or trait called ischemic rhabdomolysis, which can result in sickling-collapse syndrome. Sickling, or exertional sickling, occurs in those whose suffer from sickle cell disease or trait who are participating in high intensity or long duration exercises. It takes place during these bouts of exercise when the individuals red blood cells go from a round disc shape to a quarter moon, or sickle, shape. This change in shape can cause a blockage within a vessel which will result in a blockage of blood flow to the more peripheral vessels and organs/muscles of the body, which results in ischemic rhabdomolysis. Ischemic rhabdomolysis is the breakdown of muscles due to a lack of supply in blood, which presents with the signs and symptoms of cramping, pain, and/or weakness of the lower extremities. This is a medical emergency which can lead to sickling-collapse syndrome, and this is fatal if not treated right away. Before proper guidelines can be established as to the care of athletes that carry this disease, however, we must first determine what exactly this disease is. Sickle cell disease: A genetic disorder that alters the iron containing protein that is responsible for transporting oxygen in red blood cells, hemaglobin. It is quite rare as it only affects about 1% of African Americans in the United States. Complications that may arise due to this disease include anemia, anemic crises, pain crises, growth failure, infection, infarctions, and organ system complications. As these individuals get older early-onset congestive heart failure and chronic lung disease are two major conditions that can result from having this disease. Sickle cell trait: A condition where the carrier is benign having no hematologic complications. It affects about 8-10% of African Americans in the United States and only about .05% of non-African Americans in the United States. Complications that can arise from sickle cell trait are isosthenuria, splenic infarctions, urinary-tract infections, and hematuria. The four major factors that can lead to sickling in at risk individuals are: 1. Metabolic Acidosis 2. Red blood cell dehydration 3. Hyperthermia within the muscles 4. Severe hypoxemia

Procedures: The following guidelines and steps to take during an emergency are listed in order to aid
the acting medical professional to provide the best quality care to the at risk individual: Guidelines: o Before participation in any sport, a PPE should be conducted for possible at risk individuals o Athletes with these conditions should be conditioning year round at a predetermined pace to gradually acclimate them to the proper level of fitness needed to participate o These athletes should not participate in extremely high intensity drills such as full out sprints and timed goal runs as these will put them at a greater risk of sickling o If during any activity the athlete starts to present with symptoms they should be pulled out of participation to be evaluated o Proper education for these athletes is key to help the prevention of sickling and the causes of sickling such as dehydration and overheating o Take special considerations for environmental hazards such as participation out in a hot setting that could lead to heat related illnesses and dehydration EAP: In the event of sickling-collapse syndrome which is a medical emergency o Activate EMS o Check/monitor vital signs o Immediately start to cool of the patient if the cause of the incident may be heat related o Supply patient with high flow oxygen at 15 Lpm if available o If vital signs start to decline attach an AED to the patient and deliver shock if designated to do so o Continue to monitor vitals and treat symptomatically until EMS arrives

Wade Price, ATS

Resources: NATA Consensus Statement: Sickle Cell Trait and the Athlete Olsen II S. Sickling in Sports. Athletic Therapy Today [serial online]. March 2005;10(2):50-51. Available from: Academic Search Premier, Ipswich, MA. Accessed September 4, 2013.