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Sleep Disorders in Different Diseases

Sleep Disorders in Different Diseases. Indra Narang Director, Sleep Medicine Hospital For Sick Children, Toronto Assistant Professor, University of Toronto. Overview. Obesity Down syndrome Sickle cell disease Prader-Willi Syndrome Cardiac disease

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Sleep Disorders in Different Diseases

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  1. Sleep Disorders in Different Diseases IndraNarang Director, Sleep Medicine Hospital For Sick Children, Toronto Assistant Professor, University of Toronto

  2. Overview • Obesity • Down syndrome • Sickle cell disease • Prader-Willi Syndrome • Cardiac disease • Craniofacial disorders • Central apnea/hypoventilation disorders

  3. Sleep and Down syndrome • Sleep disordered breathing (SDB) in DS constitutes 15% of all referrals at SickKids • OSA is between 30-60% 1 • Central apnea/hypoventilation disorders- prevalence unclear • Non- respiratory related sleep problems in > 50% 2 • Bedtime resistance • Night wakenings • Parasomnias • Daytime sleepiness • 1. Marcus CL. Paediatrics 1991, 2. Carter M. Arch Dis Childhood 2009

  4. Sleep and Down syndrome • Parents unable to predict OSAS in DS: • Prospective cohort study 2 • 56 subjects had a PSG and questionnaires • Mean age: 42 months (range=4-63 ) • 57% met criteria for OSA • 69% parents who DID NOT report sleep problems: • 54% had an abnormal PSG • Parents who did report sleep problems: • 36% had an abnormal PSG 1. Marcus CL, Paediatrics 1991 2.. Shott S, 2006, Arch Oto Head & Neck Surg • Marcus CL 1991 Paediatrics

  5. Risk factors for OSA in DS1 • Snoring, previous adenotonsillectomy and history of PHT could not predict OSA • Risk factors for OSAS were age and BMI • 1. Riekstins A. ERS 2009 (abstract)

  6. Pathophysiology of SDB in DS Obstruction Airway collapse Circumferential pharyngeal Lateral pharyngeal Laryngeal Tongue base • Adenoidal hypertrophy • Tonsillar hypertrophy • Facial anatomy • Obesity • Deviated nasal septum • Chronic rhinitis

  7. Treatment for SDB in DS • 30-50% have persistent OSA following adenotonsillectomy • 27 patients with DS post AT with persistent OSA evaluated1 • Cine MR studies performed, mean age = 9.9 years • Glossoptosis in 17/27 (63%) • Hypopharyngeal collapse 6/27 (22%) • Enlarged lingular tonsils 8/28 (30%) • Macroglossia 20/27 ( 74%) • Adenoidal regrowth • Images utilised to plan surgical interventions • Outcomes of further surgical intervention not known and many DS children require NIPPV 1. Donnelly AJR Am J Roentgenol. 2004

  8. Down Syndrome and SDB: Summary Difficult to predict OSA in DS Traditional surgery does not cure OSA in DS Advanced imaging and surgical techniques may change the future management Many children with DS will require NIPPV- challenging! All DS children should have sleep surveillance Baseline PSG in all 3-4 year old children even if no history suggestive of SDB 1, 2 1. Weijerman M, Eur J Paed 2. Shott S, 2006, Arch Oto Head & Neck Surg

  9. Sickle Cell Disease (SCD) Inherited hemoglobinopathies associated with vasoocclusive disease, cardiovascular and neurovascular complications Sleep Problems: OSA - Prevalence reported to be as high as 70% 1 More severe nocturnal oxygen desaturations and higher CO2 in those with SCD and OSA compared with no-SCD OSA groups 1 Periodic leg movements reported to be 29%- more prevalent in SCD group with NO OSA 2 Rogers et al, J Clin Sleep Med 2010 Kaleyias J, J PaediatrcHematolOncol 2008

  10. Sickle Cell Disease and SDB • Sick Kids data 1 • 41 patients (24 females) were referred with history of snoring • median age 6.4 yrs • 44% patients had OSA • mean OAHI 18.4 • History of snoring or BMI could not predict OSA • But OSA was significantly associated with age < 7 years • ??? Unclear if an association between OSA and VOC • F/U SCD patients had resolution of OSA with with AT • 1. Al-Saleh Sleep 2009 (abstract)

  11. Pathophysiology for OSA in SCD • Anatomical changes 2O bone marrow hyperplasia • Compensatory adenotonsillar hypertrophy after splenic infarction • Racial differences in upper airway • More severe oxygen desaturations and hypercapnia: • Anaemia • V/Q mismatch • Lung disease/abnormal lung function • Unclear as to the reason for high PLMs

  12. Cardiac disease and SDB • Adult data suggests that SDB in the context of heart failure associated with increased morbidity and mortality Prospective study: • Cardiomyopathy patients without other morbidity known to contribute to SDB eg DMD • PSG, Echocardiogram and ENT assessment –all within 24 hours of PSG

  13. SDB and Cardiomyopathy

  14. Cardiac disease and SDB: Summary • Higher prevalence of SDB than expected in CM • But…...not related to snoring • No individual had an adenotonsillectomy • One patient had a heart transplant • Resolution of SDB on PSG post transplant • Mechanisms • Rostral fluid shift • Altered respiratory control due to increased fluid in pulmonary bed • Increased upper airway resistance

  15. Prader Willi Syndrome and SDB Recognised SDB in PWS: Increased prevalence of OSA, as high as 100% 3 Central sleep apnea/hypoventilation syndrome 4 Increased sleepiness Lindgren A, 1998, ActaPed. 2. Myers S, J Paediatr 2000 O’Donoghue FJ, J Paediatr 2005. 4. Festen D. J ClinEndocrinolMetab 2006

  16. PWS and Sleepiness • 37 PWS children with PWS1 • 20/37 had MSLT • 5/20 had MSLT s consistent with narcolepsy • None had cataplexy • But no association between obstructive AHI and MSLT findings 1. Williams K, J Clin Sleep Med

  17. Pathophysiology of SDB in PWS Obesity Adenotonsillar hypertrophy Hypotonia Blunted arousal responses to hypoxia and hypercapnia Decreased Hypocretin in CSF hypocretin synthesised in hypothalamus and promotes wakefullness and supresses REM sleep ? GH therapy

  18. PWS, SDB and GH Therapy • Several case reports that GH related to sudden death • ? Linked to co-existing URTI • 6 months after GH, ? decrease in apneic events 1 • Increased ventilatory response to CO2 2 • Worse AHI after 6 weeks GH 3 • No change in apneas/AHI after 6 months of GH 4 1. Haqq A. J Clin Endocrinol Metab 2003. 2. Lindgren A. Eur J Paediatr 1999 3. Miller J. J Clin Endocrinol Metab 2005. 4. Festen D. J Clin Endocrinol Metab 2006

  19. Treatment of SDB in PWS • Adenotonsillectomy • Weight loss (faciliated by GH) • NIPPV • Oxygen • Modafinil for excessive daytime sleepiness • ? Growth hormone therapy

  20. Hypnogram: 7 month old PWS child

  21. PSG: 2 minute screen

  22. Oxygen - PSG: 2 minute screen

  23. Craniofacial Malformations

  24. Craniofacial Malformations • High prevalence of OSA due to anatomical variations • High prevalence (74%) of moderate to severe OSA in children with craniosynostosis • Complete resolution of OSA not observed in the majority following cranial, palate or adenotonsillectomy surgeries 1 • Many children will require NIPPV 1. Al-Saleh S. J Craniomaxillofac Surg. 2011

  25. Central sleep apnea/hypoventilation (CSAH) DISCRETE CENTRAL APNEAS NOCTURNAL CENTRAL HYPOVENTILATION CSAH DISORDERS

  26. Primary CSAH Disorders • Congenital central hypoventilation syndrome (CCHS) • Late onset CCHS • Congenital form of severe hypoventilation • Mutation in PHOX2B gene identified in 2003 • Intact voluntary but not automatic control of ventilation • Impaired ventilatory responses to hypoxia and hypercarbia • During sleep and even wakefulness, CCHS patients will have significant hypoventilation requiring ventilation • Official ATS Statement on CCHS • Weese-Mayer DE et al, Am J Respir Crit Care Med 2010

  27. Secondary CSAH Disorders

  28. Management of 20 CSAH

  29. Case KR 6 year old boy, no current known health problems Referral: Non-urgent Snoring’ Tired, irritable and sleepy during the day, developmentally normal Please assess

  30. Case KR Night-time Bath, book, bed Falls asleep easily in his own room and bed at 8 pm Snoring, pauses in breathing, gasping Not restless at night, does not wake up Father sleeps in his son’s room and describes many pauses during the night

  31. Case KR Daytime Tired and difficult to wake up in the morning Tired and sleepy during the day Irritable and poor concentration at school Increasing developmental concerns over the last year Physical Examination CVS, RS, AS and CNS examination normal Tonsillar size 2+

  32. Case KR: PSG (3 minutes)

  33. Case KR: PSG Summary Central apnea index 55/hour Central apneas, average duration 16 seconds associated with mild oxygen desaturations to 85% Respiratory rate 4 – 12/minute OAHI 4.3/hour Sleep efficiency 90%, baseline SaO2 was 95% Sleep reasonably well consolidated Arousal index 20.6 Normal CO2 during night

  34. Case KR: Investigations Blood work – all normal Capillary blood gas – normal EKG – normal, Echo – normal PHOX 2B – no evidence for CCHS Neurology opinion – no abnormal findings Urgent referral for MRI brain Arnold Chiari malformation Decompression surgery performed 2 days later

  35. Case KR: PSG 6 months later

  36. SDB and Morbidity • Sleep disordered breathing associated with • Increased risk of cardiovascular disease • Increased risk of glucose intolerance/diabetes • Increased risk for neurcognitive deficits/behaviourial problems • Poorer quality of life • Risk magnified in the context of underlying disease

  37. Summary Very limited data exist for these specific high risk groups Clinically, maintain a high index of suspicion for identifying those at risk of SDB even in absence of reported symptoms High risk children should NOT be treated with the same paradigm as otherwise healthy children High risk children should be evaluated pre and post intervention

  38. When is PSG indicated in high risk groups? Wise MS et al. SLEEP 2011;34(3):389-398 Aurora RN, et al. Sleep 2011; 34(3):379-388

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