Monday, July 28, 2014

Those with sickle cell anemia are more likely to have sleep apnea

A study shows that children who have sickle cell anemia are more likely to have sleep apnea.

Children with sickle cell anemia are more likely to suffer from sleep apnea than children who are otherwise healthy, according to the findings of a study published online in the journal Pediatrics.
The study, which was published last week, was funded by the National Institutes of Health. 
The findings underscore the importance for physicians to screen sickle cell patients on a routine basis for sleep apnea, said one of the study's co-investigators, Dr. Carol Rosen, a pediatric sleep medicine specialist and medical director of the Pediatric Sleep Center at University Hospitals Rainbow Babies & Children's Hospital and professor of pediatrics at Case Western Reserve University.
Obstructive sleep apnea is a sleep-related breathing problem that can reduce blood oxygen levels during the night. Patients with the disorder stop breathing for periods lasting from a few seconds to a few minutes, dozens of times during the night.
An estimated 1 to 5 percent of all children in the United States suffer from it, according to the American Sleep Apnea Association. Risk factors include chronic swollen tonsils and adenoids, smaller upper airways and obesity.
Sickle cell disease is one of the most common genetic diseases in the country and is typically diagnosed in newborns.  In the United States, it's estimated that sickle cell anemia affects 70,000–100,000 people, mainly African Americans. The disease occurs in about 1 out of every 500 African American births.
It can cause frequent bouts of joint or organ pain, vulnerability to infection, stroke, slow growth or internal organ damage. When red blood cells sickle, or become malformed (shaped like a sickle or a crescent), it makes blood flow and oxygen flow to nearby tissue more difficult, causing pain and organ injury.
Children with sickle cell anemia – the most common form of sickle cell disease – have lower oxygen levels than healthy children, and may be even more vulnerable to the effects of low oxygen that come with asthma or sleep apnea.
Dr. Michael DeBaun (now at Vanderbilt University School of Medicine) and Dr. Robert Strunk (Washington University School of Medicine) conceived of the study while both were colleagues at St. Louis Children's Hospital.  They noticed a relationship between breathing problems like asthma and increased hospitalizations for pain and chest problems in children with sickle cell anemia.
Collaborating with researchers at UH Rainbow and University College of London Institute of Child Health and Great Ormond Street Hospital in England, they launched a study.  They wanted to see if their hypothesis - that children with sickle cell anemia would have a higher-than-expected prevalence of sleep apnea – was correct.
Of the 243 sickle cell anemia patients enrolled in the study, 59 were patients at UH Rainbow. Nearly all of the patients were of African heritage.
Data was collected through the use of questionnaires for medical history (including asthma, allergies and sleep); physical exams that included breath tests and measuring oxygen levels; and sleep studies.
What they found was a higher prevalence of sleep apnea in children with sickle cell anemia. Those with sleep apnea had more aggravated symptoms of their sickle cell anemia than patients who did not have sleep apnea.
"Having low oxygen is a trigger for [the cells] sickling," Rosen said. "If you have sleep apnea with episodes of low oxygen, cells may be more likely to sickle and cause additional health problems."
Study participants have now been followed for several years.  The next step will be to look at whether having sleep apnea is a risk factor for future problems like increased hospitalizations for pain or a serious lung condition called acute chest syndrome.
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