A Review of Sleep Concerns in Paediatric Sickle Cell Disease
significant concern in this population. One study estimates that 59 % of families with a child with SCD have incomes that qualify the family for some type of government assistance.52
In addition to lower SES,
families of children with SCD are faced with many other stressors, such as living in urban environments, ethnic minority status and living in single-parent families.53
more daytime sleepiness,54,55 shorter night-time These are all important factors to
take into account when considering both health and sleep in children. Lower SES has been associated with parent reports of more bedtime problems,54 sleep durations55,56 breathing.45,57
been found to nap more frequently,58 tiredness,55
sleep for shorter periods at night, go to bed later56
exhibit higher rates of sleep-disordered breathing57 Caucasian children.
and a higher occurrence of sleep-disordered Additionally, children of ethnic minority status have report more daytime and
compared to
Differences in sleep habits across majority and minority ethnic groups emphasise the importance of developing more diverse groups when establishing normative sleep habits in children. Given the minority status of most children with SCD, it is important to examine sleep habits in comparison to African American children without SCD to understand the contribution of disease status to sleep. In our prior research we found that children with SCD demonstrate significantly more sleep problems than middle-class Caucasian children.44
The
differences between children with SCD and the demographically matched control group were less pronounced, which suggests that many problematic sleep patterns and behaviours are exacerbated by lower SES, living in an urban environment and minority status. Comparing children with SCD to SES-matched controls indicated that children with SCD experience more night waking, sleep-disordered breathing and enuresis, but similar elevated levels of bedtime behaviour problems and daytime sleepiness. This pattern of results suggests that sociodemographic factors are important determinants of sleep behaviours and quality in children. Within the SCD sample, SES was more highly related to sleep problems than most disease factors (i.e. disease complications, healthcare utilisation and genotype). It is possible that disease factors are better used as outcome measures than predictors of sleep problems, possibly refuting the assertion by previous research that there is a bidirectional relationship between sleep and disease processes in children with SCD.
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2. Rogers VE, Lewin DS, Winnie GB, Gieger-Brown J, Polysomnographic charateristics of a referred sample of children with sickle cell disease, J Clin Sleep Med, 2010;6(4):374–81.
3. Samuels MP, Stebbens VA, Davies SC, et al., Sleep related upper airway obstruction and hypoxaemia in sickle cell disease, Arch Dis Child, 1992;67(7):925–9.
4. Rogers VE, Marcus CL, Jawad AF, et al., Periodic limb movements and disrupted sleep in children with sickle cell disease, Sleep, 2011;34(7):899–908.
5. Barakat LP, Smith-Whitley K, Schulman S, et al., Nocturnal enuresis in pediatric sickle cell disease, J Dev Behav Pediatr, 2001;22(5):300–5.
6. Jordan SS, Hilker KA, Stoppelbein L, et al., Nocturnal enuresis and psychosocial problems in pediatric sickle cell disease and sibling controls, J Dev Behav Pediatr, 2005;26(6):404–11.
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Conclusions
Children with SCD are at an increased risk for sleep-disordered breathing,1–3
PLMS4 disease factors, including pain,34
and nocturnal enuresis,5,6 hypoxaemia39
as well as for several and sleepiness44
that
may affect sleep patterns and sleep quality. Additionally, contextual and demographic risk factors such as neighbourhood stress, poverty and minority status may also contribute to disrupted and insufficient sleep in children with SCD.44
Sleep problems may contribute to
disease complications, reduce the child’s ability to cope with disease complications and affect overall health status; thus, it is imperative that healthcare providers routinely screen children and adolescents for medical and behavioural sleep problems during regular healthcare appointments. Referrals should be made for children with SCD to see sleep specialists when warranted, especially in cases with sleep-disordered breathing, which may result in further disease complications. During hospital follow-up visits, healthcare providers should ask parents and older children with SCD about sleep quality before pain episodes to assess patterns that precede pain episodes and help parents intervene earlier with palliative methods. Lastly, it is important to work with caregivers to modify the sleeping environment (e.g. removing the television from the bedroom, using a white noise maker to drown out ambient noise) and use good sleep hygiene by keeping consistent bedtime routines and schedules so as to maximise sleep efficiency and sleep quality for children.
Future research should address sleep in children with SCD using objective (e.g. actigraphy, PSG) and subjective (e.g. perceptions of sleep quality, daytime sleepiness) reports in concert to describe sleep habits and behaviours in these children. Other predictors of sleep problems should also be examined within this population to better understand the causes of sleep disruptions in children with SCD. Further examination of the pain and sleep connection using prospective measures, diaries and objective measurement is important to understand relationships between these factors. Research that addresses the long-term effects of sleep on disease outcomes and general health are essential next steps. Finally, intervention studies are important for children with SCD, with a specific focus on modifying sleep behaviours and sleep problems in relation to their effects on disease outcome. n
11. National Heart Lung and Blood Institute, National Sleep Disorders Research Plan, Bethesda: National Institutes of Health Publication 2003, No. 03-5209.
12. Owens JA, Neurocognitive and behavioral impact of sleep disordered breathing in children, Pediatr Pulmonol, 2009;44(5):417–22.
13. Marcus CL, Sleep-disordered breathing in children, Am J Respir Crit Care Med, 2001;164(1):16–30.
14. Salles C, Ramos R, Daltro C, et al., Prevalence of obstructive sleep apnea in children and adolescents with sickle cell anemia, J Brasileiro Pneumol, 2009;35(11):1075–83.
15. Souza LA, Viegas C, Quality of sleep and pulmonary function in clinically stable adolescents with sickle cell anemia, J Brasileiro Pneumol, 2007;33(3):275–81.
16. Needleman JP, Franco ME, Varlotta L, et al., Mechanisms of nocturnal oxyhemoglobin desaturation in children and adolescents with sickle cell disease, Pediatr Pulmonol, 1999;28(6):418–22.
17. Wittig RM, Roth T, Keenum AJ, Sarnaik S, Snoring, daytime sleepiness, and sickle cell anemia, Am J Dis Child, 1988;142(6):589.
18. Maddern BR, Ohene-Frempong K, Reed HT, Beckerman RC, Obstructive sleep apnea syndrome in sickle cell disease, Ann Otol Rhinol Laryngol, 1989;98(3):174–8.
19. Khatwa U, Kothare SV, Restless legs syndrome and periodic limb movements disorder in the pediatric population, Curr Opin Pulm Med, 2010;16(6):559–67.
20. Allen RP, Picchietti D, Hening WA, et al., Restless legs syndrome: diagnostic criteria, special considerations, and epidemiology: a report from the restless legs syndrome
diagnosis and epidemiology workshop at the National Institutes of Health, Sleep Med, 2003;4(2):101–19.
21. Kirk V, Bohn S, Periodic limb movements in children: prevalence in a referred population, Sleep 2004;27(2):313–5.
22. Hedo CC, Aken'Ova YA, Okpala IE, et al., Acute phase reactants and severity of homozygous sickle cell disease, J Intern Med, 1993;233(6):467–70.
23. Mizuno S, Mihara T, Miyaoka T, et al., CSF iron, ferritin and transferrin levels in restless legs syndrome, J Sleep Res, 2005;14(1):43–7.
24. Walter PB, Harmatz P, Vichinsky E, Iron metabolism and iron chelation in sickle cell disease, Acta Haematol, 2009;122(2–3):174–83.
25. Nevéus T, The role of sleep and arousal in nocturnal enuresis, Acta Pædiatrica 2003;92(10):1118–23.
26. Noll J, Newman A, Gross S, Enuresis and nocturia in sickle cell disease, J Pediatr, 1967;70(6):965–7.
27. Saxena UH, Scott RB, Ferguson AD, Studies in sickle cell anemia. XXV. Observations on fluid intake and output, J Pediatr, 1966;69(2):220–4.
28. Figueroa TE, Benaim E, Griggs ST, Hvizdala EV, Enuresis in sickle cell disease, J Urol, 1995;153(6):1987–9.
29. Onen SH, Onen F, Courpron P, et al., How pain and analgesics disturb sleep, Clin J Pain, 2005;21(5):422–31.
30. Long AC, Krishnamurthy V, Palermo TM, Sleep disturbances in school-age children with chronic pain, J Pediatr Psychol, 2008;33(3):258–68.
31. Dampier C, Ely E, Eggleston B, et al., Physical and cognitive– behavioral activities used in the home management of sickle pain: a daily diary study in children and adolescents,
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