Robert Skomro

Home mechanical ventilation: a Canadian Thoracic Society clinical practice guideline.

Increasing numbers of patients are surviving episodes of prolonged mechanical ventilation or benefitting from the recent availability of userfriendly noninvasive ventilators. Although many publications pertaining to specific aspects of home mechanical ventilation (HMV) exist, very few comprehensive guidelines that bring together all of the current literature on patients at risk for or using mechanical ventilatory support are available. The Canadian Thoracic Society HMV Guideline Committee has reviewed the available English literature on topics related to HMV in adults, and completed a detailed guideline that will help standardize and improve the assessment and management of individuals requiring noninvasive or invasive HMV. The guideline provides a disease-specific review of illnesses including amyotrophic lateral sclerosis, spinal cord injury, muscular dystrophies, myotonic dystrophy, kyphoscoliosis, post-polio syndrome, central hypoventilation syndrome, obesity hypoventilation syndrome, and chronic obstructive pulmonary disease as well as important common themes such as airway clearance and the process of transition to home. The guidelines have been extensively reviewed by international experts, allied health professionals and target audiences. They will be updated on a regular basis to incorporate any new information.

Outcomes of Home-Based Diagnosis and Treatment of Obstructive Sleep Apnea

BACKGROUND Home diagnosis and therapy for obstructive sleep apnea (OSA) may improve access to testing and continuous positive airway pressure (CPAP) treatment. We compared subjective sleepiness, sleep quality, quality of life, BP, and CPAP adherence after 4 weeks of CPAP therapy in subjects in whom OSA was diagnosed and treated at home and in those evaluated in the sleep laboratory. METHODS A randomized trial was performed consisting of home-based level 3 testing followed by 1 week of auto-CPAP and fixed-pressure CPAP based on the 95% pressure derived from the auto-CPAP device, and in-laboratory polysomnography (PSG) (using mostly split-night protocol) with CPAP titration; 102 subjects were randomized (age, 47.4 +/- 11.4 years; 63 men; BMI, 32.3 +/- 6.3 kg/m(2); Epworth Sleepiness Scale [ESS]: 12.5 +/- 4.3). The outcome measures were daytime sleepiness (ESS), sleep quality (Pittsburgh Sleep Quality Index [PSQI]), quality of life (Calgary Sleep Apnea Quality of Life Index [SAQLI], 36-Item Short-Form Health Survey [SF-36], BP, and CPAP adherence after 4 weeks. RESULTS After 4 weeks of CPAP therapy, there were no significant differences in ESS (PSG 6.4 +/- 3.8 vs home monitoring [HM] 6.5 +/- 3.8, P = .71), PSQI (PSG 5.4 +/- 3.1 vs HM 6.2 +/- 3.4, P = .30), SAQLI (PSG 4.5 +/- 1.1 vs HM 4.6 +/- 1.1, P = .85), SF-36 vitality (PSG 62.2 +/- 23.3 vs HM 64.1 +/- 18.4, P = .79), SF-36 HM (PSG 84.0 +/- 10.4 vs HM 81.3 +/- 14.9, P = .39), and BP (PSG 129/84 +/- 11/0 vs HM 125/81 +/- 13/9, P = .121). There was no difference in CPAP adherence (PSG 5.6 +/- 1.7 h/night vs HM 5.4 +/- 1.0 h/night, P = .49). CONCLUSIONS Compared with the home-based protocol, diagnosis and treatment of OSA in the sleep laboratory does not lead to superior 4-week outcomes in sleepiness scores, sleep quality, quality of life, BP, and CPAP adherence. TRIAL REGISTRATION clinicaltrials.gov; Identifier: NCT00139022.

Nonspecific interstitial pneumonia and usual interstitial pneumonia with mutation in surfactant protein C in familial pulmonary fibrosis

Nonspecific interstitial pneumonia, a recently described form of idiopathic interstitial pneumonia, is characterized by uniform involvement of the alveolar septae with interstitial inflammation and variable amounts of fibrosis. Histological observations differentiate nonspecific interstitial pneumonia from usual interstitial pneumonia and clinically, patients with a nonspecific interstitial pneumonia pattern show better prognosis than those with usual interstitial pneumonia. We have genetically analyzed a family with a history of usual interstitial pneumonia. Most of the patients presented as adults and their biopsies showed a pattern consistent with usual interstitial pneumonia. However, three family members presented in early childhood and their biopsies revealed a nonspecific interstitial pneumonia pattern. The inheritance pattern of usual interstitial pneumonia is consistent with autosomal dominant inheritance with variable expression. DNA sequence analyses of the surfactant protein C gene in children with nonspecific interstitial pneumonia and adults with usual interstitial pneumonia exhibit a common heterozygous mutation located in exon 5. The mutation causes a Leu188 to Gln188 change in the carboxy-terminal region of prosurfactant protein C, possibly affecting peptide processing. These observations suggest that individuals with this particular mutation in surfactant protein C gene might be at increased risk of interstitial lung disease of variety of types.

Canadian Thoracic Society 2011 guideline update: Diagnosis and treatment of sleep disordered breathing

The Canadian Thoracic Society (CTS) published an executive summary of guidelines for the diagnosis and treatment of sleep disordered breathing in 2006⁄2007. These guidelines were developed during several meetings by a group of experts with evidence grading based on committee consensus. These guidelines were well received and the majority of the recommendations remain unchanged. The CTS embarked on a more rigorous process for the 2011 guideline update, and addressed eight areas that were believed to be controversial or in which new data emerged. The CTS Sleep Disordered Breathing Committee posed specific questions for each area. The recommendations regarding maximum assessment wait times, portable monitoring, treatment of asymptomatic adult obstructive sleep apnea patients, treatment with conventional continuous positive airway pressure compared with automatic continuous positive airway pressure, and treatment of central sleep apnea syndrome in heart failure patients replace the recommendations in the 2006⁄2007 guidelines. The recommendations on bariatric surgery, complex sleep apnea and optimum positive airway pressure technologies are new topics, which were not covered in the 2006⁄2007 guidelines.

Pregnant women with gestational hypertension may have a high frequency of sleep disordered breathing.

BACKGROUND Gestational hypertension is a common complication of pregnancy. Recent evidence suggests that women with gestational hypertension have a high rate of sleep disordered breathing (SDB). Using laboratory-based polysomnography, we evaluated for the frequency of SDB in women with gestational hypertension compared to healthy women with uncomplicated pregnancies. METHODS In this single-center cross-sectional study, women with the diagnosis of gestational hypertension were screened in the Fetal Assessment Unit and Antepartum ward. Healthy subjects were recruited by local advertising. Subjects completed a series of questionnaires addressing sleep quality and daytime sleepiness, followed by full night polysomnography. The primary outcome was frequency of SDB (defined as a respiratory disturbance index ≥ 5) in the gestational hypertension and healthy groups. RESULTS A total of 34 women with gestational hypertension and singleton pregnancies and 26 healthy women with uncomplicated singleton pregnancies consented to participate in the study. The mean ages and gestational ages, but not the body mass indices, of the 2 groups were similar. The frequencies of SDB in the more obese gestational hypertension group and the healthy group were 53% and 12%, respectively (P < 0.001). INTERPRETATION Women with gestational hypertension may have a significantly higher frequency of SDB than do healthy women with uncomplicated pregnancies of similar gestational age. The relative causal contributions, if any, of SDB and obesity remain to be determined.

Restless legs syndrome in a rheumatoid arthritis patient cohort.

Objective:To use the 2003 International Restless Legs Syndrome Study Group (IRLSSG) diagnostic criteria and to evaluate restless legs syndrome (RLS) prevalence in a rheumatoid arthritis (RA) and osteoarthritis (OA) population. Further, we wished to evaluate physician awareness of this disorder by as reflected in prevalence of preexisting diagnoses of RLS in these populations. Methods:This was a questionnaire study of Saskatchewan RA and OA patients enrolled in a longitudinal database study. A data collection instrument, including the 2003 IRLSSG criteria for RLS was distributed to the patients enrolled. Results:Of the 193 respondents, 158 (81.9%) were women. The population consisted of 148 RA and 45 OA patients. RA patients were younger (mean age, 65.8 years) in comparison with those in the OA group (mean age, 72.8 years; P < 0.001). All criteria for RLS were met by 27.7% of RA patients and by 24.4% of OA patients. A previous diagnosis of RLS was reported by 2.6% of patients. Conclusions:A quarter of all our patients met the 2003 IRLSSG criteria, in both RA and OA groups; however, only 2.6% of study patients reported a previous diagnosis of RLS. As RLS can significantly affect quality of life, increased awareness with improvement in surveillance, recognition, and treatment would be beneficial to patient care. We advocate screening for symptoms of sleep disorders to be incorporated into the routine rheumatologic history for all patients with RA and OA.

Obstructive sleep apnea is common among patients referred for coronary artery bypass grafting and can be diagnosed by portable monitoring.

BackgroundObstructive sleep apnea (OSA) is common among patients with coronary artery disease. However, OSA remains largely under recognized. The lack of clinical suspicion and difficulties to access full polysomnography (PSG) are limiting factors. The aim of this study was to evaluate, among patients referred to coronary artery bypass grafting (CABG): (i) the prevalence of OSA, (ii) the association of OSA with clinical symptoms, (iii) the performance of overnight unattended portable monitoring (PM) as an alternative method for the diagnosis of OSA. MethodsConsecutive patients referred for CABG were evaluated by standard physical evaluation and validated questionnaires (Berlin questionnaire and Epworth Sleepiness Scale) and underwent full PSG and PM (Stardust II). ResultsWe studied 70 consecutive patients (76% men), age 58±7 years (mean±SD), BMI [median (interquartile range)] 27.6 kg/m2 (25.8–31.1). The prevalence of OSA (full PSG) using an apnea–hypopnea index of at least 5 events/h was 87%. Commonly used clinical traits for the screening of OSA such as the Epworth Sleepiness Scale and neck circumference had low sensitivities to detect OSA. In contrast, the Berlin questionnaire showed a good sensitivity (72%) to detect OSA. PM showed good sensitivity (92%) and specificity (67%) for the diagnosis of OSA. ConclusionOSA is strikingly common among patients referred for CABG. The Berlin questionnaire, but not symptom of excessive daytime sleepiness is a useful tool to screen OSA. PM is useful for the diagnosis of OSA and therefore is an attractive tool for widespread use among patients with coronary artery disease.

Sleep and neuromuscular disorders in children

Children suffering from neuromuscular diseases are at an increased risk of sleep-related breathing disorders (SRBD) such as obstructive sleep apnea syndrome (OSAS) and hypoventilation as well as central sleep apnea, which is frequent in these patients due to diaphragmatic weakness. They are at higher risk for developing complications of nocturnal hypoxemia, including pulmonary hypertension, cor pulmonale and neurocognitive dysfunction. Neuromuscular disorders and OSAS are both prevalent disorders and frequently overlap. Sleep-related hypoventilation/hypoxemia due to neuromuscular diseases may be exacerbated in the presence of OSAS; these children are likely to experience greater severity and duration of sleep-related hypoxemia than are children with either disorder alone. Additionally, some of these children have reduced central neural chemoresponsiveness. The development of SRBD in these patients further impairs their quality of life and worsens their respiratory status. We review the literature on the diagnosis and treatment of SRBD in children with a variety of neuromuscular disorders.

Canadian Sleep Society/Canadian Thoracic Society position paper on the use of portable monitoring for the diagnosis of obstructive sleep apnea/hypopnea in adults

The present position paper on the use of portable monitoring (PM) as a diagnostic tool for obstructive sleep apnea⁄hypopnea (OSAH) in adults was based on consensus and expert opinion regarding best practice standards from stakeholders across Canada. These recommendations were prepared to guide appropriate clinical use of this new technology and to ensure that quality assurance standards are adhered to. Clinical guidelines for the use of PM for the diagnosis and management of OSAH as an alternative to in-laboratory polysomnography published by the American Academy of Sleep Medicine Portable Monitoring Task Force were used to tailor our recommendations to address the following: indications; methodology including physician involvement, physician and technical staff qualifications, and follow-up requirements; technical considerations; quality assurance; and conflict of interest guidelines. When used appropriately under the supervision of a physician with training in sleep medicine, and in conjunction with a comprehensive sleep evaluation, PM may expedite treatment when there is a high clinical suspicion of OSAH.

Hypersomnolence and sleep disorders in a rheumatic disease patient population.

Objectives:There is increasing awareness of the importance of sleep in health maintenance. Our primary objective was to evaluate prevalence of excess daytime sleepiness in a rheumatic disease patient population. Secondary objectives included evaluation of prevalence of abnormal sleep quality and primary sleep disorders. Methods:Consecutive Rheumatology clinic patients were invited to participate in a self-administered questionnaire study. Included were measures for pain, fatigue, and global functioning, modified Health Assessment Questionnaire, Epworth Sleepiness Score (ESS), Pittsburgh Sleep Quality Index (PSQI), Berlin Score, diagnostic criteria for restless legs syndrome (RLS), Centre for Epidemiologic Studies Depression score (CES-D), stress scores, and the short form-36 quality of life instrument. Results:Of 507 consecutive patients invited to participate, 423 agreed. Mean age was 52.1 years; 26% were male. Prevalence of excessive sleepiness (ESS >10) was 25.7%, abnormal sleep quality (PSQI >5) was 67.3%, high risk for obstructive sleep apnea Berlin scores were present in 35.2% and 24% of participants met criteria for RLS. Significantly worse pain, fatigue, global function, short form-36 summary scores, modified Health Assessment Questionnaire, depression, and stress scores were present in patients with higher ESS and PSQI scores. No significant differences in sleep assessment scores were observed between specific rheumatic disease groups. Conclusions:Our findings suggest a high prevalence of unrecognized hypersomnolence, poor sleep quality, and primary sleep disorders in rheumatology patients. We suggest evaluation of sleep health be incorporated into standard clinical assessments of all rheumatology patients. We would recommend this evaluation include the ESS and the criteria for RLS.