[in Japanese]

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This study aimed to investigate the long-term clinical features following surgery for intractable aspiration in patients with severe motor and intellectual disabilities (SMID). Methods: We reviewed the inpatient chart until December 2016, and enrolled 25 patients between the ages of 22 and 57 years. The observation period was anywhere between 5 to 23 years, following surgery. Results: The origins of aspiration of the patients were perinatal (n=17), congenital (n=4), and acquired (n=4). Patients underwent tracheoesophageal diversion (n=18), total laryngectomy (n=6), or laryngotracheal separation (n=1). The frequencies of pneumonia were found to have markedly decreased after surgery. However, 4 patients later required mechanical ventilation, due to an advancement in the occurrence of thorax deformity with aging. Total of 18 patients restarted eating orally, but could not wean them off tube feeding. Aerophagia developed in 12 patients who started eating after surgery. Recurrent ileus, abdominal distension, and gastroesophageal reflux disease induced by aerophagia became considerable complications. Conclusions: Aspiration prevention surgery for SMID was effective to control respiratory infection. Additionally, some patients restarted oral nutrition. However, during the long-term clinical course, we must pay attention to both complications that advances with age and development of aerophagia following surgery.

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There are no reports on the use of denosumab to treat osteoporosis patients with severe motor intellectual disabilities (SMID). We report our experience with seven patients with SMID. These patients had bone fractures or bone density less than 70% of the young adult mean (YAM) using the digital image processing method. Denosumab was administered subcutaneously every 6 months. The patients’ age ranged from 9 to 68 years and there were 6 males and 1 female. The underlying SMID was neonatal asphyxia in three patients, trisomy 21 in two patients, a meningoencephalitis in one patient and dentatorubral-pallidoluysian atrophy in one patient. One patient had six bone fractures, five patients had one fracture, and two patients had no history of fracture. History of therapy included alendronate in three patients, vitamin D only in one patient, alendronate and vitamin D in one patient. No therapy had been administered in two patients. The observation period from the first denosumab administration was from 1year 11 months to 2 years 9 months. Fracture of the distal end of the right thigh occurred in one patient 4 months after the first administration. Six patients had no bone fracture after denosumab administration. Bone mineral density in seven patients was 1.37±0.40 mm Al (mean±SD) before treatment, 1.40±0.42 mm Al after 6 months and1.41±0.36 mm Al after 1 year. There were no significant differences. We measured TRACP-5b as the bone absorption marker (670.7±428.9 mU/dl before treatment), which significantly decreased to 210.2±149.7 mU/dl (p=0.028) 3 month after the first treatment. BAP as the bone formation marker was 46.0±32.0 μg/L before treatment and it significantly decreased to 28.0±18.6μg/L 1 year after the first administration(p=0.043). Denosumab is thought to be safe, effective and easily administered in patients with SMID.

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The use of the mechanical in-exsufflator (MI-E) has recently increased with efficacy being reported in patients with severe motor and intellectual disabilities (SMID). It is possible to measure peak cough flow (PCF) and tidal volume intake (Vti) using the new MI-E CoughAssist E70® (E70). Here we evaluated the safety of using E70 in patients with SMID for 6 months from June 2015 to November 2015. Subjects included nine patients admitted to our hospital. We determined the SpO2 level, pulse rate, respiratory rate, PCF, and Vti as well as assessed actual facial expressions, tension changes, respiratory depression, and frequency of vomiting. Patients were aged 7–39 years (mean, 24.2 years). Eight (bedridden; IQ < 20) and one (bedridden; IQ 20–35) patients were grouped into classes 1 and 4, respectively, as per Ooshima’s classification. All patients had undergone tracheotomies; six patients had received ventilator management. In this study, the intake-expiratory pressure used was 10–50 cm H2O, maximum PCF was 59–222 l/min, and maximum Vti was 961–4,300 ml. Maximum PCF was <100 l/min in four of nine patients, and three of the four had muscle diseases. There was no change in Vti when the intake-expiratory pressure was >35 cm H2O. No significant changes in vital signs were observed. Thus, we concluded that it is safe to use E70 in patients with SMID while monitoring PCF and Vti.

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Regarding the use of a chair during bathing of children (persons) with severe motor and intellectual disabilities (hereinafter referred to as “children (persons) with SMID”), a design that would reduce the burden on the caregiver was considered. A questionnaire survey targeting parents of children (persons) with SMID was carried out, and needs were identified. From the results of a qualitative analysis of the obtained responses, major burdens in assistance were “the bathroom is small” and “growth of the children,” indicating inconvenience and difficulty. Issues which cannot be solved even by using current support chairs included, “burden getting in and out of the bathtub,” and “burden of assistance in a stopped position,” and functions for which improvement or addition were requested included “easier to take care of after use,” “better seat material such as texture,” and “adjustable height.” Regarding requests in function and structure of a new bathing chair, not only was a reduction of burden in assistance movement by the caregivers given, but a structure that considered storage after use, such as “compact” or “foldable,” and ease in maintenance such as “mold resistant,” “quick-drying” were also given.

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There is a tremendous amount of burden on families who take care of home-care patients (children) requiring medical care. We investigated influence of patient mothers’ conducting medical care at nighttime on their autonomic nerve activities during sleep. The subjects of this study included mothers who had been taking care of patients (children) with physical handicaps and multiple severe disabilities aged between 6 - 24 with Type 1 and Level 1 physically disabled persons’ certificate. Using an activity measurement device with a built-in photoelectric plethysmograph, we measured pulse intervals and the amount of activities of subjects between 8:00 p.m. and 8:00 a.m. for 3 days, and calculated the time when they fell asleep/woke up, hours of sleep, and the number of times and duration of awakening during sleep. In addition, we calculated low frequency (LF) and high frequency (HF) as well as LF/HF during sleep from the time when they fell asleep/woke up and investigated relationships between the autonomic nerve activities and sleep conditions, presence or absence of medical care, and feeling of burden in the home-care setting. Positive correlations were observed between LF/HF and the number of times/duration of awakening during sleep. The number of times and duration of awakening and LF/HF were significantly high in mothers who had been conducting intratracheal aspiration. Moreover, there was a tendency that the higher the number of aspiration, the more LF/HF was elevated. Awakening for intratracheal aspiration after they fell asleep and elevation of LF/HF were observed in family members who had been conducting intratracheal aspiration, suggesting that they might not have been resting well physically and physiologically during sleep.

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Serum cystatin C (Cys-C) is more useful than creatinine to evaluate renal function in patients with severe motor and intellectual disabilities. However, it is not appropriate to be used as a screening test. Hence, we created a prediction formula for renal dysfunction using patient’s background and routine blood test data, and investigated clinical validity of this formula. During the period between April and November 2015, we measured Cys-C in 327 disabled patients aged 5 to 80 years and evaluated this renal function by means of Cys-C levels was high. We created a prediction formula using a model-preparation group of 261 patients, who had been randomly extracted from the 327 subjects, and verified the prediction formula using a verification group which consisted of the remaining 66 subjects. The prediction formula was created by multivariate logistic regression analysis, and explanatory variables selected included motor function (bed-ridden), sex, albumin, blood urea nitrogen and creatinine. The AUC of the prediction formula was 0.8759 in the model-preparation group and 0.9125 in the verification group. The sensitivity and specificity of the entire subjects were 75.0% and 85.8%, respectively, using probability of 0.21 as a cut-off value. This prediction formula was satisfactory in terms of both internal and external validities, suggesting that it would be useful for screening the renal function of disabled people.

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It is necessary to introduce a seating system for children with severe motor and intellectual disabilities (SMID) to continuously maintain an optimal sitting position. However, any instability in their posture after the introduction of the seating system in the clinical setting is regarded as a problem; moreover, it has been shown that the main cause of this is posterior inclination and sliding of the pelvis. Therefore, in this study, we prepared a kneeblock corresponding to backward inclination and sliding of the pelvis, set the tilt angle of the seating system at 5 degrees, which is the tilt/reclining angle normally used, for 7 severely affected children. The sagittal plane pelvic tilt angle in the presence/absence of the kneeblock under the 2 conditions was measured. The results of this study indicate that it was possible to suppress posterior pelvic incline and slippage of children with SMID by introducing the prototype kneeblock into their seating system. It was also suggested that the use of the kneeblock even in the active posture could reduce the probability that the posture collapses.

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An interview survey was conducted to reveal how mothers of patients with severe motor and intellectual disabilities (SMID) perceive subtle reactions of their children. Interviews were performed with 8 mothers and the data were analyzed qualitatively. The Krippendorff’s method was used in the analysis. The following were extracted from the result: core-categories including “changes in expressions of patients with SMID perceived by their mothers”, “expression of patients with SMID not perceived by their mothers”, and “patterns of expressions of patients with SMID perceived by their mothers” as well as 16 subcategories which constituted the core categories, and 75 contextual symbols. From this result, the following nursing suggestions were obtained: (1) their attitudes toward understanding meanings of expressions made by the patients with SMID allow them to perceive the patients’ expressions, (2) mothers pay the most attention to facial expressions of the patients with SMID and understand meanings of the perceived expressions as patterns, (3) they must constantly pay attention to new expressions made by the patients as their expressions may change over time.

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Upon preparation of a utilization plan for all users of welfare services for disabled persons, a questionnaire survey was conducted to reveal current situations at service offices, namely, whether or not consultation support specialists had been able to provide consultations to adequately meet the users' needs. The subjects included 20 offices in "a list of specialized consultation support offices designated by Hachioji city", and the response rate was 85%. Out of all offices, 10 had been accepting children (adults) with severe conditions, and of these, 3 had been accepting more than 10 users. For working style of the consultation support specialists, they were working "full-time in multiple positions" at 50% of the offices. For "motivation", they were feeling motivated at 90% of the offices, yet feeling difficult at 90% of the offices. They felt difficult primarily due to "low income" followed by "a large amount of work", "coordination with other relevant agencies" and "lack of social resources". This suggests that they are highly motivated yet have many issues at the same time at most of the offices. The consultation support specialists are expected to play some roles in supporting disabled persons so that they can live independently. We consider that their current issues are (1) to connect and expand medical collaboration and social resources, and (2) to revise the remuneration policy.

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A retrospective survey was conducted in a total of 2,069 children who used a short-term stay program at our medical-care facility for disabled children during the past two years between April 2014 and March 2016, for cases which required additional treatments during the stay according to their medical records. Out of 2,069 children, 429 (21%) had received some sort of treatments during the stay. The most common reasons included eye/skin symptoms in 176 children (41%) followed by fever in 119 (28%), respiratory symptoms in 78 (18%), convulsion in 52 (12%), gastrointestinal symptoms in 45 (10%), and muscle hypertonia in 29 (7%). Thirty-one children withdrew from the program in the middle and 11 transferred to other facilities. Out of all, 97.7% completed the program as scheduled and left the facility. It was revealed that not many children experienced significantly-poor physical conditions during the short-stay program at our facility. However, serious cases, such as respiratory arrest due to choking on phlegm or fracture, were also reported in some children.

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Semi-structured interviews were performed with employees who had been in charge of supporting persons with severe motor and intellectual disabilities (SMID), on positive feelings such as delightful feeling when they were working with these persons. The results obtained were roughly categorized into the following groups: "positive feelings toward favorable facial expressions or reactions of persons with severe conditions", "direct feeling of support; positive feelings on improvement of physical conditions achieved by treatments", "positive feelings in relationship with families of persons with severe conditions" and "how the employees feel about themselves". Further investigations on these categories revealed that some sort of favorable reactions of the persons with severe conditions helped the employees to have positive feelings and perspectives toward the persons with severe conditions and reinforced their motivations on supporting these persons. We considered in this paper that such situations would contribute to the support for persons with severe conditions, and proposed "a sheet for evaluating positive aspects of daily lives of users in facilities for persons with SMID".

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The present study aimed to establish an Internet telephone call setting using the Skype communications application, and clarify its utility as an information and communications technology (ICT)-based support tool for facilitating interaction among family members of homebound persons with severe motor and intellectual disabilities (SMID). The special web camera built-in the Skype application was used because this form of call, by connecting a web camera via high-definition multimedia interface (HDMI) cable with a television set, was best suited to those with limited proficiency using personal computers. Using this setup, three families of persons with SMID participated in an ICT-based family interaction program once a month for 8 months. The results suggested that this form of group communication was helpful in reducing anxiety and stress and averting feelings of isolation for both persons with SMID and their families.

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Children (adults) with severe motor and intellectual disabilities (SMID) tend to develop abnormalities in respiratory, circulatory and immune systems; once they become infected their conditions can be easily aggravated. In recent years, the number of drug-resistant bacteria carriers has increased, and infection has been controlled through cohorting. It is however difficult to identify the carriers once they leave their beds. In order to make sure that all individuals, including multi-disciplinary personnel and volunteers, involved in children (adults) with SMID to practice infection control, they must share information on these carriers. Hence, we labeled their wheel chairs with an icon so that they can be immediately identified. For volunteers, we provided instructions on leaflets and installed hand sanitizer on wheel chairs so that they can conduct appropriate infection control.

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