Oral Positive Expiratory Pressure Device for Excessive Dynamic Airway Collapse Caused by Emphysema.

Excessive dynamic airway collapse (EDAC) contributes to breathlessness and reduced quality of life in individuals with emphysema. We tested a novel, portable, oral positive expiratory pressure (o-PEP) device in a patient with emphysema and EDAC. MRI revealed expiratory tracheal narrowing to 80 mm2 that increased to 170 mm2 with the o-PEP device. After 2-weeks use of the o-PEP device for 33% to 66% of activities, breathlessness, quality of life, and exertional dyspnea improved compared with minimal clinically important differences (MCID): University of California-San Diego Shortness of Breath questionnaire score declined 69 to 42 (MCID, ≥5), St. George's Respiratory Questionnaire score decreased 71 to 27 (MCID, ≥4), and before and after the 6-minute walk test Borg score difference improved from Δ3 to Δ2 (MCID, ≥1). During the 6-minute walk test on room air without the use of the o-PEP device, oxyhemoglobin saturation declined 91% to 83%; whereas, with the o-PEP device, the nadir was 90%. Use of the o-PEP device reduced expiratory central airway collapse and improved dyspnea, quality of life, and exertional desaturation in a patient with EDAC and emphysema.

The emerging role of the pediatric physical therapist in evaluation and intervention for individuals with lysosomal storage diseases.

PURPOSE: The purposes of this article are to describe the pathology, medical implications, and typical impairments of individuals with various lysosomal storage diseases (LSDs), summarize results of recent clinical trials on medical interventions relevant to physical therapy practice, report new advances in functional measurement, and suggest a framework for physical therapy management and intervention. SUMMARY OF KEY POINTS: Medical and surgical interventions are enabling individuals with LSDs to not only survive but to improve their daily functioning and quality of life. This is likely to become an increasing area of emphasis in pediatric physical therapy, as the intervention emphasis for some individuals will shift from maintenance to restorative programs. RECOMMENDATIONS: We recommend that pediatric physical therapists become familiar with new LSD therapeutics, play a major role in evaluating impairment and functional limitation changes in individuals with LSDs, and become knowledgeable about the indications and precautions for restorative physical therapy programs.

Reliability and validity of the Portage assessment scale for clinical studies of mentally handicapped populations.

The Finnish version of the Portage Assessment Scale was used with 114 aspartylglucosaminuria (AGU) persons aged between 1 and 56 years. Ninety-three persons with AGU were also given psychological tests, mainly SB (Finnish), WISC-R and WPPSI, but also Merrill-Palmer, Bayley, Leiter and WAIS tests. The data also include for the reliability assessment a sample of 30 normal pre-school children. The internal consistency reliability was calculated for the areas of socialization 0.80, language 0.81, self-help 0.73, cognitive 0.70, motor 0.75 and for the whole scale 0.80. The factor analysis produced one very strong factor. The results of the Portage assessment were compared to the developmental levels provided by the tests. Both methods gave almost identical cross-sectional descriptions of the skills of patients with AGU, which was interpreted as providing a cross-validation for Portage in clinical use and to mean that Portage can be used as a reasonable assessment method. The advantages of Portage include easy use by non-psychologists, concrete descriptions of the skills, the 5 areas, and the close relation to rehabilitation.