Increased frequency of restless legs syndrome in chronic obstructive pulmonary disease patients.

BACKGROUND: Despite complaints of poor sleep being very common in people with chronic obstructive pulmonary disease (COPD), restless legs syndrome (RLS) symptoms have not been extensively investigated in these patients. OBJECTIVE: To assess the prevalence and severity of RLS in patients with COPD and to investigate the factors potentially associated with RLS. METHODS: A total of 87 patients with COPD and 110 controls, matched for age and sex, were evaluated regarding the presence and severity of RLS symptoms. A diagnosis of RLS was made according to the criteria of the International RLS Study Group (IRSLSSG), and severity was assessed by the IRLSSG severity scale. Excessive daytime somnolence was assessed using the Epworth sleepiness scale (ESS). RESULTS: RLS was significantly more frequent in COPD patients than in controls (36.8% vs. 11%; p < 0.001). Compared to controls, COPD patients with RLS showed higher disease severity (mean IRLSSG severity scale score: 20.5 +/- 2.8 for COPD, and 18 +/- 3.5 for controls; p = 0.016) and more pronounced daytime somnolence (mean ESS score: 11.8 +/- 1.1 for COPD, and 8.6 +/- 3.6 for controls; p = 0.009). Moreover, compared to those without RLS, COPD patients with RLS showed increased daytime sleepiness (mean ESS score: 11.8 +/- 1.1 for COPD/RLS, and 7.3 +/- 4 for COPD/non-RLS; p < 0.001) and longer disease duration (11.9 +/- 7 years for COPD/RLS, and 8.7 +/- 6.9 years for COPD/non-RLS; p = 0.045). Multivariate analysis showed that ESS score was the only factor significantly associated with RLS in COPD patients. CONCLUSIONS: RLS is a frequent cause of disabling sleep disturbance in patients with COPD and should be specifically investigated in these patients.

Outcome and attitudes toward home tracheostomy ventilation of consecutive patients: a 10-year experience.

OBJECTIVES: To describe survival, predictors of long-term outcome and attitudes in patients treated at home by tracheostomy-intermittent positive-pressure ventilation (TIPPV) for respiratory failure during a 10-year period (1995-2004). METHODS: Seventy-seven consecutive patients were treated by TIPPV at home. Patients were divided into three groups: neuromuscular, pulmonary, and non-pulmonary patients. Effects of TIPPV on survival, factors influencing outcome after TIPPV, and attitudes of patients and caregivers regarding mechanical ventilation were studied. RESULTS: Forty-one patients (53%) were neuromuscular, 19 (25%) were affected by pulmonary diseases, and 17 (22%) by non-pulmonary diseases. The median survival time after TIPPV in the group was 49 months (range 3-149 months). There was statistically significant longer survival in neuromuscular compared to pulmonary patients (p=0.006), and a trend toward longer survival for non-pulmonary when compared to pulmonary patients (p=0.048). Chronic obstructive pulmonary disease (COPD) patients (n=14) showed the poorest outlook (median survival 26 months, range 3-45 months) and the highest number of emergency readmissions to hospital. The median survival in amyotrophic lateral sclerosis (ALS) patients was 49 months (range 30-61), lower than the whole group of neuromuscular patients. Major tracheostomy complications were low: 2.6%. Multivariate analysis showed that COPD and ALS patients had a three-fold higher risk of death than patients with other diagnoses. Lastly, 64 patients (83%) were pleased they had chosen TIPPV and 69 (90%) would choose it again. Forty-two caregivers (55%) were pleased the patients had chosen home ventilation, but 29 (38%) reported major burdens. CONCLUSIONS: TIPPV is well-received by the patients, is safe, and provides survival for long periods of time. Underlying conditions (COPD and ALS) might represent important prognostic factors for survival.

The amyotrophic lateral sclerosis functional rating scale predicts survival time in amyotrophic lateral sclerosis patients on invasive mechanical ventilation.

OBJECTIVE: To determine whether the amyotrophic lateral sclerosis functional rating scale (ALSFRS), which is a validated instrument that assesses the functional status and the disease progression in patients with amyotrophic lateral sclerosis (ALS), predicts hospital length of stay and survival time in ALS patients treated with tracheostomy-intermittent positive-pressure ventilation (TIPPV). METHODS: Thirty-three consecutive ALS patients with acute respiratory failure who received therapy with TIPPV were prospectively followed up from their admission to the hospital until death. The association of ALSFRS score at hospital admission with length of hospital stay and survival after TIPPV were examined using Cox proportional hazard models, adjusting for age at baseline, sex, and symptom duration. RESULTS: The median ALSFRS score of the ALS patients at hospital admission was 11 (range, 4 to 22). The median length of hospital stay was 55 days (range, 7 to 124 days), with a hospital mortality rate of 9%. For the 30 patients (91%) discharged from the hospital, the median survival time was 37 months (range, 2 to 64 months). The total ALSFRS score (above or below the median score) was a significant predictor of length of hospital stay (hazard ratio [HR], 2.86; 95% confidence interval [CI], 1.2 to 6.5; p = 0.003) and survival after TIPPV (HR, 3.76; 95% CI, 1.4 to 9.7; p = 0.002). The total ALSFRS score at hospital admission was also associated with length of hospital stay (HR, 2.1; 95% CI, 1.1 to 5.1; p = 0.005) and survival (HR, 0.52; 95% CI, 0.1 to 0.8; p = 0.002) when included in a Cox multivariable model together with the other demographic and clinical variables. CONCLUSION: In ALS patients with acute respiratory failure who have been treated with TIPPV, the total ALSFRS score may predict length of hospital stay and long-term survival after invasive mechanical ventilation.

Development of chronic hypoventilation in amyotrophic lateral sclerosis patients.

Early prediction of respiratory muscle involvement and chronic hypoventilation (CH) in amyotrophic lateral sclerosis (ALS) patients can help to plan mechanical ventilatory aids and palliative care interventions well before respiratory failure occurs. To describe the natural history of the progressive pulmonary dysfunction leading to CH, and to identify potential parameters associated with its development in ALS, we prospectively followed 38 ALS patients up to 26 months, starting from their first presentation at our Clinic. At study entry, median FVC was 87% (interquartile range: 72-104%) and declined by 10% after 6 months (range: 2-49%), showing a very high inter-patient variability. Over the 26-months follow-up, 19 patients (50%) presented CH in the first 12 months, and eight patients (21%) developed CH in the remaining 14 months of the study. The remaining 29% of patients did not show signs of CH during the whole period of observation. In the Cox model, the category of disease progression (rapid vs. intermediate and slow), assessed using the Appel ALS Rating Scale (AARS) in the first 3 months after presentation, was the only variable associated with a significantly increased likelihood of CH. We conclude that CH can occur within 1 year from presentation in a great proportion of patients, independently from their initial respiratory status. Including the patients in specific categories of early disease progression, as assessed with the AARS, could be a sensitive method to identify patients with different risk of developing CH, and may help physicians to more efficiently plan the frequencies of respiratory evaluations, initiate mechanical ventilation and discuss advance directives with the patients and their caregivers.