[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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We studied management plans and outcomes of surgical treatments in patients with severe motor and intellectual disabilities (SMID) at a pediatric surgical unit of a general hospital situated in the west of Hyogo prefecture. Thirteen patients with SMID presenting gastroenterological or respiratory symptoms with difficulty controlling with medical treatments at the hospital or at home were tested and treated surgically between January 2011 and March 2015. The patients aged between 11 and 37 years, and 7 were males and 6 were females. For all patients, the chief complaint was recurrent aspiration pneumonitis. As for their living environment, 12 were living at home, and 1 was staying at a special facility. The management plans included gastrostomy in 7 patients, cardioplasty in 2 patients, and laryngotracheal separation in 11 patients. The post-operational course had been satisfactory in terms of nutritional control and prevention of gastroesophageal reflux in patients who received gastrostomy and cardioplasty. In patients who received laryngotracheal separation, their quality of life was improved as they no longer developed aspiration pneumonitis and less suction was needed.

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The objectives of this study were to reveal the living environment maintenance status and actual nursing burdens with severe cerebral palsy patients (hereinafter, severe CP patients) and to discuss the relationship between them. A questionnaire survey was conducted in caregivers of severe CP patients. For use of welfare equipment, 38.0% of the severe CP patients were using futon (bedding); the caregivers felt strong physical discomfort when the patients were using futon. Of all patients, 47.6% were being assisted without using welfare equipment when moving inside their residences, and 84%, when being transported. As for their residences, 64.2% had been reformed or newly built, taking their physical disabilities into consideration; 49.2% had been reformed or built after the patients graduated from high schools. Hence, it appeared necessary for physical therapists and occupational therapists, who are expected to be involved in the living environment maintenance, to consider the patients’ long life-styles from infancy to advanced age and to actively provide information anticipating their long-term prognoses from childhood and school age.

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Drawing on our experiences of daily life-support activities for severely disabled children, we developed the contents of a hiragana script study as a tool for developing their communication skills. The participants were 19 children, 3 with severe mental and physical disorders and 16 with intellectual disability. We investigated the efficacy of these contents through visualization of the participants’ respective sightlines. Children who used our contents ranged from 10 months old to second-year high school students. Study periods ranged from a minimum of 3 minutes and 25 seconds (terminated due to mucous plugging) to a maximum of 35 minutes and 4 seconds with a mean of 18 minutes and 11 seconds—the 10-month-old child surpassed the mean at 21 minutes and 24 seconds. The children watched a video without sleeping, crying, or becoming upset and were engaged in the quiz. Although three children with mental disorders were, in addition to their primary disease, diagnosed with irreversible coma (brain death) due to a catastrophic event, scattered gaze points and attention points were also confirmed for these children. These points were also confirmed for the 10-month-old child, who has spinal muscular atrophy (SMA) type I. From our results, we ascertained four uses (positive effects) for our contents: (1) as an assessment tool, (2) as a study (learning) tool, (3) as a visual-functions assessment and training tool, and (4) for assessment and learning while having fun with a sense of play. However, as our study involves eye movements, we will need to use an even more accurate eye tracker in future research. Our results suggest that our contents can easily be used for line-of-sight detection and assessment and can serve as a tool that can be an enjoyable experience for children.

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Nebulized tobramycin is effective for treating Pseudomonas aeruginosa infection in patients with cystic fibrosis, and Nebulized tobramycin may be effective in neurologically impaired children. We attempted to treat three cases of recurrent Pseudomonas aeruginoa pneumonia with nebulized tobramycin. Case 1 was a girl aged 4 years and 6 months with trisomy 18. She was treated by tracheal resection and occlusion of vocalfold. After nebulized tobramycin treatment, she had no pneumonia. Case 2 had Kugelberg–Welander disease. He was asphyxiated at 21 years old when he had bronchitis. He underwent tracheotomy and tracheal separation, but he had recurrent Pseudomonas pneumonia and sputum spurted from part of the opening of windpipe. Nebulized tobramycin decreased the sputum and prevented him from pneumonia. Case 3 was an extremely low birth weight infant who was bed ridden from the neonatal period. He was 27years old . He had severe scoliosis and recurrent Pseudomonas pneumonia resulted in CO2 narcosis. Tracheotomy was performed and nebulized tobramycin decreased the number of times of pneumonia. Nebulized tobramycin may be useful for treatment of Pseudomonas aeruginosa pneumonia in severely disabled patients.

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A boy with Down syndrome presented transient myoclonus after hypoxia with acute pneumonia. He had fever, cough and wheezing, and admitted to our hospital with hypoxia and drowsiness on the day 4. Myoclonus first appeared on peripheral extremities, and spreaded into whole body in accordance with improvement of consciousness, and it was seen almost continuously during wakefulness. No additional abnormality was found in brain MRI and EEG. 5-HIAA level in CSF was slightly increased. Myoclonus spontaneously decreased gradually without any additional treatment after the day 9. The mechanism like Lance-Adams syndrome caused by hypoxia and his background weakness in serotonergic system with Down syndrome was suspected.

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[in Japanese]

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