Comorbid obstructive sleep apnea and increased risk for sickle cell disease morbidity

Pediatrics • Original Article
  • 26 Downloads

Abstract

Purpose

Sickle cell disease (SCD) imparts an increased risk for obstructive sleep apnea (OSA) in childhood. Studies of pediatric SCD have identified an increased risk for pain and neurologic complications with comorbid OSA. We determined the rate of a broad range of SCD-related medical complications to better characterize the spectrum of SCD complications related to OSA.

Methods

Retrospective chart review at a single hematology clinic identified 641 youth with SCD who received consistent screenings for OSA as part of routine hematological health maintenance visits over an 11-year period. Medical complication rates in the 136 children with OSA determined by polysomnography exams were compared to 136 matched controls at lower risk for OSA due to negative OSA screenings or exams.

Results

Children with SCD and OSA had higher overall rates of SCD complications than low OSA-risk controls; lung morbidity showed the largest effect size. Infection, cardiovascular, and neurologic complications occurred at higher rates in children with OSA. Children with comorbid OSA had higher rates of SCD complications both before and after OSA diagnosis.

Conclusions

OSA in children with SCD is associated with higher rates of a broad range of SCD complications, including pneumonia and acute chest syndrome. Routine screenings, diagnosis, and increased therapeutic intervention for children with comorbid OSA could decrease SCD morbidity.

Keywords

Cell diseases, sickle Apnea, obstructive sleep Sleep-disordered breathing Complications Hospitalization 

Notes

Acknowledgments

This project was completed as part of doctoral degree requirements of the first author. No funding was received for this research.

Compliance with ethical standards

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. For this type of study, formal consent is not required.

Conflict of interest

The authors declare that they have no conflict of interest.

References

  1. 1.
    Maddern BR, Reed HT, Ohene-Frempong K, Beckerman RC (1989) Obstructive sleep apnea syndrome in sickle cell disease. Ann Otol Rhinol Laryngol 98(3):174–178.  https://doi.org/10.1177/000348948909800302 CrossRefPubMedGoogle Scholar
  2. 2.
    Robertson PL, Aldrich MS, Hanash SM, Goldstein GW (1988) Stroke associated with obstructive sleep apnea in a child with sickle cell anemia. Ann Neurol 23(6):614–616.  https://doi.org/10.1002/ana.410230615 CrossRefPubMedGoogle Scholar
  3. 3.
    Brooks LJ, Koziol SM, Chiarucci KM, Berman BW (1996) Does sleep-disordered breathing contribute to the clinical severity of sickle cell anemia? J Pediatr Hematol Oncol 18(2):135–139.  https://doi.org/10.1097/00043426-199605000-00007 CrossRefPubMedGoogle Scholar
  4. 4.
    Rosen CL, Debaun MR, Strunk RC, Redline S, Seicean S, Craven DI, Gavlak JCD, Wilkey O, Inusa B, Roberts I, Goodpaster RL, Malow B, Rodeghier M, Kirkham FJ (2014) Obstructive sleep apnea and sickle cell anemia. Pediatrics 134(2):273–281.  https://doi.org/10.1542/peds.2013-4223 CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Rees DC, Williams TN, Gladwin MT (2010) Sickle-cell disease. Lancet 376(9757):2018–2031.  https://doi.org/10.1016/S0140-6736(10)61029-X CrossRefPubMedGoogle Scholar
  6. 6.
    Hassell KL (2010) Population estimates of sickle cell disease in the U.S. Am J Prev Med 38((4):S512–S521CrossRefGoogle Scholar
  7. 7.
    Kato GJ, Gladwin MT, Steinberg MH (2007) Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes. Blood Rev 21(1):37–47.  https://doi.org/10.1016/j.blre.2006.07.001 CrossRefPubMedGoogle Scholar
  8. 8.
    Gozal D, Kheirandish-Gozal L (2007) Neurocognitive and behavioral morbidity in children with sleep disorders. Curr Opin Pulm Med 13(6):505–509.  https://doi.org/10.1097/MCP.0b013e3282ef6880 CrossRefPubMedGoogle Scholar
  9. 9.
    Marcus CL, Brooks LJ, Ward SD et al (2012) Diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics 130(3):e714-e755CrossRefGoogle Scholar
  10. 10.
    Abou-Elhamd KE (2012) Otorhinolaryngological manifestations of sickle cell disease. Int J Pediatr Otorhinolaryngol 76(1):1–4.  https://doi.org/10.1016/j.ijporl.2011.10.004 CrossRefPubMedGoogle Scholar
  11. 11.
    Davies S, Stebbens VA, Samuels MP, Southall DP (1989) Upper airways obstruction and cerebrovascular accident in children with sickle cell anaemia. Lancet 334(8657):283–284.  https://doi.org/10.1016/S0140-6736(89)90477-7 CrossRefGoogle Scholar
  12. 12.
    Kemp JS (1996) Obstructive sleep apnea and sickle cell disease. J Pediatr Hematol Oncol 18(2):104–105.  https://doi.org/10.1097/00043426-199605000-00002 CrossRefPubMedGoogle Scholar
  13. 13.
    Kirkham FJ, Hewes DK, Prengler M, Wade A, Lane R, Evans JP (2001) Nocturnal hypoxaemia and central-nervous-system events in sickle-cell disease. Lancet 357(9269):1656–1659.  https://doi.org/10.1016/S0140-6736(00)04821-2 CrossRefPubMedGoogle Scholar
  14. 14.
    Hargrave DR, Wade A, Evans JP, Hewes DK, Kirkham FJ (2003) Nocturnal oxygen saturation and painful sickle cell crises in children. Blood 101(3):846–848.  https://doi.org/10.1182/blood-2002-05-1392 CrossRefPubMedGoogle Scholar
  15. 15.
    Adams RJ (2005) TCD in sickle cell disease: an important and useful test. Pediatr Radiol 35(3):229–234.  https://doi.org/10.1007/s00247-005-1409-7 CrossRefPubMedGoogle Scholar
  16. 16.
    American Thoracic Society (1996) Standards and indications for cardiopulmonary sleep studies in children. Am J Respir Crit Care Med 153(2):866–878CrossRefGoogle Scholar
  17. 17.
    Whitesell PL, Owoyemi O, Oneal P, Nouraie M, Klings ES, Rock A, Mellman TA, Berihun T, Lavella J, Taylor RE, Perrine SP (2016) Sleep-disordered breathing and nocturnal hypoxemia in young adults with sickle cell disease. Sleep Med 22:47–49.  https://doi.org/10.1016/j.sleep.2016.05.006 CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Su VY, Liu CJ, Wang HK, Wu LA, Chang SC, Perng DW, Su WJ, Chen YM, Lin EY, Chen TJ, al CKT (2014) Sleep apnea and risk of pneumonia: a nationwide population-based study. CMAJ 186(6):415–421.  https://doi.org/10.1503/cmaj.131547 CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Oga T, Chin K, Tabuchi A, Kawato M, Morimoto T, Takahashi K, Handa T, Takahashi K, Taniguchi R, Kondo H, Mishima M, Kita T, Horiuchi H (2008) Effects of obstructive sleep apnea with intermittent hypoxia on platelet aggregability. J Atheroscler Thromb 16(6):862–869CrossRefGoogle Scholar
  20. 20.
    Yanni E Grosse SD, Yang QH, Olney RS (2009) Trends in pediatric sickle cell disease-related mortality in the United States, 1983-2002. J Peds. 154(4):541–545.  https://doi.org/10.1016/j.jpeds.2008.09.052 CrossRefGoogle Scholar
  21. 21.
    Tauman R, Gozal D (2011) Obstructive sleep apnea syndrome in children. Expert Rev Respir Med 5(3):425–440.  https://doi.org/10.1586/ers.11.7 CrossRefPubMedGoogle Scholar
  22. 22.
    Nur E, Biemond BJ, Otten HM, Brandjes DP, Schnog JJ, CURAMA Study Group (2011) Oxidative stress in sickle cell disease; pathophysiology and potential implications for disease management. Am J Hematol 86(6):484–489.  https://doi.org/10.1002/ajh.22012 CrossRefPubMedGoogle Scholar
  23. 23.
    Muzumdar H, Raanan A (2013) Physiological effects of obstructive sleep apnea syndrome in childhood. Respir Physiol Neurobiol 188(3):370–382.  https://doi.org/10.1016/j.resp.2013.05.006 CrossRefPubMedPubMedCentralGoogle Scholar
  24. 24.
    Kheirandish-Gozal L, Gozal D, Pépin JL (2015) Inflammation in sleep debt and sleep disorders. Mediat Inflamm 2015:1–2.  https://doi.org/10.1155/2015/343265 CrossRefGoogle Scholar
  25. 25.
    Grady AJ, Hankins JS, Haberman B, Schoumacher R, Stocks RM (2017) Hydroxyurea treatment effect on children with sickle cell disease and obstructive sleep apnea. Sleep Breath 21(3):697–701.  https://doi.org/10.1007/s11325-017-1458-9 CrossRefPubMedGoogle Scholar
  26. 26.
    Narang I, Kadmon G, Lai D, Dhanju S, Kirby-Allen M, Odame I, Amin R, Lu Z, Al-Saleh S (2015) Higher nocturnal and awake oxygen saturations in children with sickle cell disease receiving hydroxyurea therapy. Annals of the American Thoracic Society 12(7):1044–1049.  https://doi.org/10.1513/AnnalsATS.201410-473OC CrossRefPubMedGoogle Scholar
  27. 27.
    Brietzke SE, Katz ES, Roberson DW (2004) Can history and physical examination reliably diagnose pediatric obstructive sleep apnea/hypopnea syndrome? A systematic review of the literature. Otolaryngol Head Neck Surg 131(6):827–832.  https://doi.org/10.1016/j.otohns.2004.07.002 CrossRefPubMedGoogle Scholar
  28. 28.
    Hankins JS, Ware RE, Rogers ZR, Wynn LW, Lane PA, Scott JP, Wang WC (2005 Oct 1) Long-term hydroxyurea therapy for infants with sickle cell anemia: the HUSOFT extension study. Blood 106(7):2269–2275.  https://doi.org/10.1182/blood-2004-12-4973 CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Scott JP, Hillery CA, Brown ER, Misiewicz V, Labotka RJ (1996) Hydroxyurea therapy in children severely affected with sickle cell disease. J Peds 128(6):820–828.  https://doi.org/10.1016/S0022-3476(96)70335-9 CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Psychology, University of South CarolinaColumbiaUSA
  2. 2.Department of PediatricsUniversity of South CarolinaColumbiaUSA

Personalised recommendations