Abstract

Patients with hypermobility syndromes often have difficulty using Positive Airway Pressure (PAP) Therapy for treatment of their Sleep Disordered Breathing. To investigate this, the CPAP titrations of patients with hypermobility were studied in detail. The attended PAP titrations of 19 consecutive patients with a Beighton hypermobility score of 5 or greater at a neurology institute were retrospectively reviewed. Of 19 patients, 6 had Sleep Apnea - Unspecified (Upper Airway Resistance Syndrome) (G47.30) and 13 had Obstructive Sleep Apnea (G47.33). All ± values reflect the standard error of the mean. During titrations, after an initial nadir for scorable hypopneas was achieved, 11 of 19 patients experienced an average increase of 10.24 ± 2.5 in their hypopnea index as pressure was increased. At their hypopnea nadir, 15 of 19 patients’ AHI was 0 and an average Respiratory Effort Related Arousal Index of 38.4 ± 6.7 remained. Example titrations and images of typical airways in hypermobile patients will be presented. 1. In typical CPAP titrations hypopneas and RERAs should generally decrease and eventually plateau as PAP is increased. However, the pattern observed in the titration of these patients with hypermobility is somewhat atypical. 2. In hypermobile patients, the high RERA index at the hypopnea nadir, with subsequent increases in both with increasing pressure, could be explained by the following mechanism: In hypermobile patients, the epiglottis is more flexible than usual, and even though the upper portion of the lower airway can be expanded by PAP, increasing pressure in hypermobile patients could lead to epiglottal closure. 3. Auto-titrating CPAPs currently do not measure RERA’s well, because of their inability to assess EEG arousals, despite having formidable algorithms which can measure changes in airway resistance. Thus, the use of auto-titrating PAP devices may not be effective in the relief of sleep complaints in patients with hypermobility and SDB. 4. This lack of effectiveness could lead to poor compliance with PAP therapy in patients with hypermobility. 5. Thus, patients with hypermobility probably need an attended PAP titration to achieve optional airway patency, and resolution of their complaints related to SDB.

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