Men and sickle cell

According to a report by a task force with the National Athletic Trainers’ Association, acute rhabdomyolysis (tied to sickle cell) is one of the top four causes of death in college athletes.

During intense or extensive exertion, the sickle hemoglobin can change the shape of red cells from round to quarter-moon, or “sickle.” This change, called exertional sickling, can pose a grave risk for some athletes. In the past seven years, exertional sickling has killed nine athletes, ages 12 through 19.

Here’s what happens: sickle cells can “logjam” blood vessels and lead to collapse from ischemic rhabdomyolysis—the rapid breakdown of muscles starved of blood. Major metabolic problems from explosive rhabdomyolysis can threaten life. Sickling can begin in 2-3 minutes of any all-out exertion and can reach grave levels soon thereafter, if the athlete continues to struggle.

Heat, dehydration, altitude, and asthma can increase the risk for and worsen sickling, even when exercise is not all-out. Despite telltale features, collapse from exertional sickling in athletes is under-recognized and often misdiagnosed. Sickling collapse is a medical emergency.

Those with sickle cell disease are at high risk of dehydration, heat-related injury, exhaustion, painful episodes, and hip joint problems. Gradual acclimatization to heat, humidity and high altitude, slow conditioning over weeks and avoidance of dehydration are recommended for all adolescents with sickle cell disease to make their sport activity safe.

Those who inherit just one copy of the gene are said to have sickle-cell trait. Their blood cells look normal and they experience no side effects—most of the time. But there are a handful of situations that puts those with sickle-cell trait in danger—circumstances that deprive their body of oxygen, such as climbing a mountain, free diving (diving in the deep ocean without supplemental oxygen) or intensive exercise, reports the journal Scientific American.

In these low-oxygen conditions, the journal notes that something changes and individuals with sickle cell trait become more prone to sudden death. Researchers do not know exactly what causes mortality under these conditions, although they have some ideas. For some reason, the low-oxygen conditions seem to cause skeletal muscle to break down. This might result in the release of substances such as potassium, which can lead to heart failure, and the protein myoglobin, which can cause kidney failure.

Symptoms of exertional sickling include muscle cramping, pain, swelling, weakness, tenderness, inability to “catch the breath” and fatigue.

The Sickle Cell Disease Association of America (SCDAA) advises to educate individuals to create an environment that encourages athletes with sickle cell trait to immediately report any symptoms—such as fatigue, difficulty breathing, leg or low back pain, or leg or low back cramping. The association says to consider such incidents as a possible sickling incident.

Suggested medical steps according to SCDAA are:

1. Check vital signs.

2. Administer high-flow oxygen, 15 l pm (if available), with a non-rebreather face mask.

3. Cool the athlete, if necessary.

4. If the athlete is obtunded or as vital signs decline, call 911, attach an AED, start an IV, and get the individual to the hospital fast.