Question: We have a child on our football team with sickle cell disease, what should we watch outfor?
Answer:
Thank you for your question, and for taking the special effort to find out more about sickle cell disease. Sickle cell disease can be very different from one person to another. Mainly you need to check with the player's physician about any individual circumstances that make him able or not able to do specific things. I do not have experience with a sickle cell patient on a high school football team, but I do have suggestions based on experience from guiding summer camp activities for children with sickle cell disease, and from reading articles on exercise studies in people with sickle cell disease with normal people.
In general, people with sickle cell disease can be very functional, but they have less physiologic reserve. There have been small studies showing that people with sickle cell disease of different types may not be capable of increasing their exercise to the same level as their peers, may get exhausted more quickly with aerobic exercise, and have higher blood lactate levels after vigorous activity. Self-paced exercise will generally show this limitation, so that the self-paced walking distance or speed is likely to be lower than normal for a person without sickle cell disease. However, additional small studies indicate that training can improve endurance and submaximal exercise capacity in people with sickle cell disease, as indicated by Watts of energy output change in heart rate with exercise and the intensity of self-paced exercise. People with sickle cell disease type SS (sickle cell anemia) or S-beta-zero thalassemia have the lowest physiologic reserve, and those with sickle cell disease types SC, S-beta-plus-thalassemia, or SS-HPFH have better physiologic reserve. People with sickle trait have nearly normal physiologic reserve.
Specific precautions are necessary if the medical history includes:
1) Stroke – watch for neurologic impairments at baseline, possibly at risk for recurrent stroke or seizures.
2) Splenomegaly – watch for trauma to the left flank and abdomen, which could rupture the enlarged spleen
3) Implanted venous access device under the skin (“PortACath,” “InfusaPort”, “Bard CathLink” etc.) – watch for trauma to the device
4) Pulmonary hypertension – cardiopulmonary function may worsen with exercise, arrhythmias also possible. People with a diagnosis of pulmonary hypertension and sickle cell disease probably need clearance from a cardiologist to participate in vigorous sports.
5) Avascular necrosis of the femoral head – risk for further damage to the femur and hip joint with heavy load-bearing. Probably needs clearance from orthopedist to participate in vigorous sports.
6) Asthma – very common in children with sickle cell disease. Manage as with other asthma, but keep in mind that the combination of these 2 chronic illnesses can cause lung function to worsen very quickly (sickle cell acute chest syndrome or status asthmaticus)
General precautions for people with sickle cell disease are
1) be flexible with activity schedule & intensity – allow rest when tired (due to anemia, poor cardiovascular reserve). Pushing to exhaustion is probably not a good idea because lactic acidosis can trigger red blood cells to sickle and cause vaso-occlusive pain.
2) Maintain good hydration. Dehydration can occur more easily than in normal people, because the kidneys are damaged by sickle cells. People with sickle cell disease need to drink more often than normal people under the same conditions, drink in anticipation of heavy sweating & exertion
3) Avoid extremes of temperature. Being either too cold or too hot can trigger red blood cells to sickle & cause vaso-occlusive pain. The person should seek shade, wear hats, avoid excessive air-conditioning, avoid wet clothing. Watch out for chilling during swimming - take a break every 20 min to dry & warm up.
4) Encourage the player to sense his own body’s warning signs, try to head off pain or fatigue. Help the group to be supportive of the player, and enforce rest breaks & reasonable bedtime.
Sincerely,
-Lewis Hsu, MD, PhD
Pediatric Hematologist
For more frequently asked questions please see:
http://www.scinfo.org/faq.htm I came across this piece of info I would like to share with the forum.
Kathy