The defective protein forms aggregates within the red blood cells, making them stiff and giving them a sickle shape. These abnormal red blood cells stick to the inner lining of small blood vessels, preventing blood flow, lowering blood oxygen levels, and causing episodes of pain called vaso-occlusive crises.
Sickle cell anemia patients also experience sleep-related complications such as sleep-disordered breathing.
Sleep-disordered breathing refers to a group of conditions where complete or partial cessation of breathing occurs several times during sleep. Obstructive sleep apnea syndrome is the most common form of sleep-disordered breathing, where the patient experiences decreased or no breathing for several seconds during sleep.
This can occur several times during sleep, decreasing the level of oxygen in the blood. Obstructive sleep apnea syndrome causes sleepiness and fatigue during the day.
Children with sickle cell anemia and sleep
Children with sickle cell anemia have a higher prevalence of sleep-disordered breathing compared to children without the disease. In these children, sleep-disordered breathing contributes to behavioral problems, learning difficulties, higher blood pressure, nighttime bed-wetting, and reduced growth.
Adults with sickle cell anemia and sleep
Pain and sleep disturbance are two key symptoms of adult sickle cell patients. Pain disturbs sleep, which increases the severity of pain and the risk of developing chronic pain.
The prevalence of sleep disturbances in adults with sickle cell anemia is high, with 44 percent of adult patients diagnosed with sleep-disordered breathing. Sickle cell patients sleep less at night (decreased sleep duration), take more time to fall asleep (late sleep onset), or spend more time awake during the night (increased sleep fragmentation). These sleep disturbances are thought to ultimately cause more severe pain the following day.
Clinical trials on sickle cell anemia and sleep
A clinical trial (NCT01565954) called the SOMMEDREP study is recruiting 62 children with sickle cell anemia, ages 2-6, to evaluate breathing difficulties during sleep and its influence on disease complications. The study is being conducted at Assistance Publique – Hôpitaux de Paris and is expected to be completed this year.
Another clinical trial (NCT02539771), the DREPASOM study, aims to evaluate sleep in 150 adults with sickle cell anemia who also experience painful complications such as vaso-occlusive crises and persistent and painful erection during the night.
The trial aims to investigate the relationship between nocturnal painful complications and obstructive sleep apnea syndrome with low blood oxygen levels. The study is recruiting participants in France and is expected to be completed in September 2018.
A randomized trial (NCT03150433) is being conducted at Johns Hopkins University in Maryland to test the effects of disturbed sleep on pain and brain function in 120 adult patients with sickle cell anemia.
The study also aims to determine whether the treatment of sleep disturbances can reduce pain and pain-related complications in these patients. The study is still recruiting participants and is expected to be completed in May 2021.
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