Screening for Obstructive Sleep Apnea in Children With Sickle Cell Disease: A Pilot Study

2020 ◽  
Author(s):  
Andrew J. Maroda ◽  
Matthew N. Spence ◽  
Stephen R. Larson ◽  
Jeremie H. Estepp ◽  
M. Boyd Gillespie ◽  
...  
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Pilot Study ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Obstructive Sleep

Sickle Cell Disease Is a Risk Factor for Obstructive Sleep Apnea

CHEST Journal ◽  
2010 ◽  
Vol 138 (4) ◽  
pp. 322A
Author(s):  
JoAnn Eng ◽  
George A. Apergis ◽  
Samir Fahmy
Keyword(s):  
Obstructive Sleep Apnea ◽  
Risk Factor ◽  
Sleep Apnea ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cell Disease ◽  
Obstructive Sleep

Adverse Events in Sickle Cell Patients with Nocturnal Hypoxia.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3793-3793
Author(s):  
Andrew D. Campbell ◽  
Megumi Okumura ◽  
Ndidi Unaka ◽  
Sally Hutchinson ◽  
Onyinye Onyekwere
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Sickle Cell Disease ◽  
Adverse Events ◽  
Sickle Cell ◽  
Case Series ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Obstructive Sleep ◽  
The Relationship

Abstract The relationship between hemoglobin dexoygenation and sickling is well known. However, the relationship between hypoxia and severity of disease in sickle cell patients has not been well established. Recently, nocturnal hypoxemia has been associated with higher incidence of CNS events including strokes, and elevated TCDs. We present our case series on 13 patients(12 SS, 1 SC) with sickle cell disease (SCD) who have nocturnal hypoxia. Approximately 75 patients were screened at the University of Michigan Sickle Cell clinic for nocturnal hypoxia either by symptoms of obstructive sleep apnea or by longitudinal baseline clinic 02 saturations (02 Sat <92%). Of the 13 hypoxic pts, median baseline O2 Sat 90%(n=13, mean 90) and the median nocturnal O2sat (Nctnl 02 sat) 84%(n=13, mean 80%) with 10/13 with moderate-severe nocturnal hypoxia (O2sats<85%)based on sleep studies. Multiple adverse events noted in the cohort were pulmonary hypertension (PHTN TRJV>2.5, n=9, median 2.74/mean 2.74,) frequent pain episodes(>3visits to ER or hospitalizations/year, n=7, with 5 pts >5/year ), recurrent acute chest syndrome( ≥ 3 episodes, n= 10), CNS events (n=3 silent infarcts, vascular stenosis), priapism( (n=4, among 6 males ). Also reported were possible causes of the underlying hypoxia including obstructive sleep apnea(OSA)(n=7 of 11 pts), asthma(n= 10 of 13 pts), and chronic lung disease( n=8). In conclusion, the persistence of nocturnal hypoxia in pediatric sickle cell disease could possibly contribute to the development of severe complications of sickle cell disease. Treatment of underlying hypoxia (ie nighttime oxygen, maximize asthma treatment, T&A for OSA)may help prevent complications and lead to the improvement clinical symptoms. Further, chronic nocturnal hypoxia may complicate pulmonary disease and accelerated the development of PHTN. More studies are needed to clarify the mechanism of hypoxia in SCD. Table I. Clinical &Demographic Data of 13 SCD Patients with Nocturnal Hypoxia. Age:(6–22y/o, mean 15) Sex: M=6 F=7 Clinical: Total Mild Mod Sev. Genotype: SS=12, SC=1 Mean Range Nctnl Hypoxia(<%) 13 3 5(<85) 5(<80) Baseline O2 Sat(%) 90 +3.0 86–97 Obstr Sleep Apnea 7 3 3 1 Nctnl 02 sat (%) 80+8.4 59–87 Pulm HTN 9 4 4 1 Total #Apneic Events(11) 65.6+80 6–256 Rest. Lung Ds. 8 2 5 1 Obstr. Apneic Events(7) 27+68.5 0–221 # of Episodes <3 3–4 >4 Hypopneic Events(9) 32.5+38 0–132 ACS 2 5 5 TRJet Velocity 2.74+.42 2–3.5 Severe Pain Crises/yr 1 2 5

Obstructive Sleep Apnea Syndrome in Sickle Cell Disease

1989 ◽  
Vol 98 (3) ◽  
pp. 174-178 ◽  
Author(s):  
Bruce R. Maddern ◽  
Kwaku Ohene-Frempong ◽  
Hollis T. Reed ◽  
Robert C. Beckerman
Keyword(s):  
Obstructive Sleep Apnea ◽  
Sleep Apnea ◽  
Sickle Cell Disease ◽  
Obstructive Sleep Apnea Syndrome ◽  
Sickle Cell ◽  
Sleep Apnea Syndrome ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Obstructive Sleep ◽  
Apnea Syndrome

We present a series of 21 children with obstructive sleep apnea syndrome and sickle cell disease (SCD). Thirteen patients underwent adenotonsillectomy with resolution of symptoms and improvement in alveolar hypoventilation. Adenotonsillectomy involves significantly increased risks in patients with SCD. We present our protocol for management of these difficult surgical patients, including vigorous hydration and transfusion therapy. Polysomnography has proven valuable both in diagnosis and in identifying severely affected patients. Increased end tidal alveolar carbon dioxide during sleep has proven to be a significant predictor of disease, while oxygen saturation monitoring was shown to be unreliable. The incidence of adenotonsillar hypertrophy (ATH) in SCD appears increased and not related to infectious diseases. We suggest that ATH represents a part of the natural course of compensatory lymphoid tissue enlargement in children with SCD.

scholarly journals Nocturnal Hypoxemia Rather Than Obstructive Sleep Apnea Is Associated With Decreased Red Blood Cell Deformability and Enhanced Hemolysis in Patients With Sickle Cell Disease

2021 ◽  
Vol 12 ◽  
Author(s):  
Emeric Stauffer ◽  
Solène Poutrel ◽  
Giovanna Cannas ◽  
Alexandra Gauthier ◽  
Romain Fort ◽  
...  
Keyword(s):  
Obstructive Sleep Apnea ◽  
Pulmonary Hypertension ◽  
Sleep Apnea ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Severity ◽  
Cell Disease ◽  
Nocturnal Hypoxemia ◽  
Obstructive Sleep ◽  
Rbc Deformability

Background: Although obstructive sleep apnea (OSA) could act as a modulator of clinical severity in sickle cell disease (SCD), few studies focused on the associations between the two diseases.Research Question: The aims of this study were: (1) to explore the associations between OSA, nocturnal oxyhemoglobin saturation (SpO2) and the history of several acute/chronic complications, (2) to investigate the impact of OSA and nocturnal SpO2 on several biomarkers (hematological, blood rheological, and coagulation) in patients with SCD.Study Design and Methods: Forty-three homozygous SCD patients underwent a complete polysomnography recording followed by blood sampling.Results: The proportion of patients suffering from nocturnal hypoxemia did not differ between those with and those without OSA. No association between OSA and clinical severity was found. Nocturnal hypoxemia was associated with a higher proportion of patients with hemolytic complications (glomerulopathy, leg ulcer, priapism, or pulmonary hypertension). In addition, nocturnal hypoxemia was accompanied by a decrease in RBC deformability, enhanced hemolysis and more severe anemia.Interpretation: Nocturnal hypoxemia in SCD patients could be responsible for changes in RBC deformability resulting in enhanced hemolysis leading to the development of complications such as leg ulcers, priapism, pulmonary hypertension or glomerulopathy.Clinical Trial Registration:www.ClinicalTrials.gov, identifier: NCT03753854.

Sign in / Sign up

Export Citation Format

Share Document