[in Japanese]

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This study aimed to identify the kinds of feeling that siblings of a child with severe motor and intellectual disabilities (SMID) living at home have towards SMID children, themselves, and their parents and grandparents while living with their family, and to examine how nurses can support these siblings. We conducted semi-structured interviews with eleven siblings aged 12 years or over of an SMID child. Our results showed that siblings were ambivalent in recognizing the confusion and annoyance of situations caused by disabilities, while taking their brother/sister with SMID as ordinary siblings. They showed their gratitude to his parents and grandparents, envisaging own future apart from their brother/sister with SMID. Our study suggests the need for support the siblings' own life course selection while understanding their siblings' feelings and conflicts and coordinating their feelings with their families.

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We investigated characteristics of scoliosis in patients with severe cerebral palsy and discussed factors which might prevent progression of the condition. The subjects included 34 patients with severe cerebral palsy aged 18 or over. We investigated presence or absence of scoliosis, association between the curve pattern and the convexity side as well as correlation between the Cobb angle and SPO, L4PO or L4 tilt. For the association between the curve pattern and the convexity side, it was revealed that left convexity was observed significantly more in the lumbar curvature (p<0.01). For the correlation between the Cobb angle and SPO, L4PO or L4 tilt, a significantly-strong positive correlation was observed between the Cobb angle and SPO, and a significantly-moderate positive correlations observed between the Cobb angle and L4PO or L4 tilt (p<0.01). It was considered that, in patients with severe cerebral palsy, there might be a particular direction toward which scoliosis curvature develops. It was also suggested that the larger the angle of scoliosis, the worse the balance between the pelvis and the spinal column, and that the larger the L4 tilt or L4PO, the greater the progression in scoliosis.

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The objective of this study was to reveal what kind of assessment perspectives the visiting nurses have to support mothers who manage physical conditions of their children requiring medical care during the initial stage of transition from NICU to home-based care. A semi-structured interview was conducted with 15 visiting nurses who had had at least 5 years of experiences in pediatric nursing visits, followed by a qualitative inductive analysis. From the analysis, 5 categories were extracted; the visiting nurses had assessment perspectives in “ability of mothers to manage life support of children", “ability of mothers to incorporate management of physical conditions of children requiring medical care into their lives", “ability of mothers to arrange children's care according to their physical conditions", “establishment of devotion to their children" and “ability of mothers to manage their stress". These appeared to be the perspectives which should not be the goals to be completely achieved at the initial stage of transition to home-based care but to learn how to manage physical conditions of children for mothers without feeling stressed while re-establishing their lives at this stage and to continue home-based life activities including parent-child relationship as well as management of physical conditions of their own; in other words, these are the perspectives to recognize abilities of mothers to be involved in management of physical conditions of their children.

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The residents of children(adults) with severe motor and intellectual disabilities (SMID) are limited from social contact for long years. Chickenpox infection from a single patient spread in one of the dormitories and there were twice outbreak of chickenpox infection in the residents in 2016. Varicella antibodies of EIA IgG and EIA IgM were measured at the time of fever or a blister appearance in 8 chickenpox infected patients (6 males and 2 females, median age of 46 (36 to 64 years old)) and then we measured varicella EIA IgG antibody more than 2 weeks thereafter. Three patients showed the first infection pattern, and 5 patients showed the infection history pattern. At the first outbreak, chickenpox infected patients were quarantined and treated by Valaciclovir Hydrochloride (VACV). Other non-infected residents were administrated with preventive dose of VACV. Since any chickenpox patients did not become severe, VACV prevention were effective to some extent. However, we experienced the second outbreak of chickenpox infection after VACV prevention, that might need more sufficient prevention strategy for chickenpox infection. Therefor we suggest that residents of children(adults)with SMID may require repetitive chickenpox vaccination because of the weak booster effect or the lack.

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In the case of children with difficulty in verbalizing pain due to severe motor and intellectual disabilities (SMID), surrogate pain reporting is important. This study examined the practical usefulness of the Japanese-version Paediatric Pain Profile, a scale to assess pain in SMID children, by analyzing the impact of nurses' characteristics on pain assessment using it. On assessing pain at rest, primary nurses scored higher than other nurses (p=0.001). There was no significant correlation between the length of nursing experience and pain score (r=0.213, p=0.277). Although the Japanese-version Paediatric Pain Profile was usable, regardless of the length of nursing experience, the results highlighted the importance of baseline-setting by those who know the target children well and pain assessment by other observers based on such scores.

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Objective: To assess the presence of carnitine deficiency in individuals with severe motor and intellectual disability (SMID) in our center and to analyze the correlation of carnitine deficiency with sodium valproate (VPA) and nutrition. Methods: We determined blood carnitine levels in 73 patients (43 males; age, 1–58 years old; median age, 32 years old) in our center, considering blood free carnitine (FC) < 20 μmol as carnitine deficiency and FC 20–36 μmol or an acylcarnitine/FC ratio > 0.4 as risk of carnitine deficiency. The relationships of the data with VPA and nutrition were analyzed. Results: Of the 73 patients, 7 (9.6%) had carnitine deficiency, 14 (19.2%) had risk of carnitine deficiency, and 52 (4.1%) had excess carnitine. In the 66 patients not taking an L-carnitine preparation, blood FC levels were significantly lower in patients with tube feeding with carnitine-free enteral nutrition than in patients with oral feeding and those with tube feeding with addition of carnitine (both p<0.01). There was no significant difference in blood FC levels according to use of VPA. However, there was a negative correlation between VPA doses per body weight and blood FC levels (r=−0.839, p<0.01). Conclusions: Tube feeding with carnitine-free enteral nutrition was associated with high risk of carnitine deficiency and VPA was a dose-dependent risk factor for carnitine deficiency in individuals with SMID.

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It is known that physical activity in adults with cerebral palsy decreases by exacerbation of motor dysfunction. This study investigated the correlation between adults with cerebral palsy who show sedentary behavior (SB) and their physical activity, gross motor function, body composition, and bone mineral density in adults with cerebral palsy. The study participants were 23 adults with cerebral palsy who were classified using the Gross Motor Function Classification System (GMFCS) levels I, II, III, and IV. The factors taken into consideration included sitting time, light physical activity (LPA) time, moderate-to-vigorous physical activity (MVPA) time per day, and body fat percentage, site-specific skeletal muscle mass, and bone mineral density. The participants in the GMFCS levels III and IV had a longer sitting time and lower LPA and MVPA time than the GMFCS levels I and II. The GMFCS levels III and IV had lower limb skeletal muscle mass and bone mineral density. In addition, there was a significant correlation between SB and the skeletal muscle of the upper limb, while SB, LPA, and MVPA time were significantly correlated with bone mineral density. The results of this study suggested that lifestyle guidance that decreasing sitting time and increasing LPA and MVPA time contribute to the maintenance and improvement of bone mineral density in adults with cerebral palsy.

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The purpose of this study is to consider the treatment for hypozincemia on children (persons) with severe motor and intellectual disabilities who are long-term residents in a facility. Regarding the target and method, 22 persons who began first-time treatment for hypozincemia between April 2018 and November 2019 were selected as subjects. For 15 cases with serum zinc levels of 60-79μg/dl, 14g of cocoa (containing 1mg zinc, cocoa group) were administered and for 7 cases with serum zinc levels under 60μg/dl, 75mg/day of Polaprezinc® (containing 17mg zinc, low-dose zinc preparation group) were administered to consider the effects thereof. As a result, the serum zinc median before and after treatment for both the cocoa group and the low-dose zinc preparation group significantly increased from 70μg/dl to 73μg/dl and from 57μg/dl to 97μg/dl, respectively. Post-treatment serum zinc levels for the low-dose zinc preparation group were significantly higher than the cocoa group. While the ratio of effective cases within the reference value for the cocoa group was 33.3%, the ratio was 71.4% for the low-dose zinc preparation group. In conclusion, while supplementing zinc through diet from cocoa was effective, the effects were limited. More case studies on the supplement dosage of zinc preparations are desired.

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We report the case of a two-year-old boy born at 35 gestational weeks, with a birth weight of 2506 g. He suffered from neonatal asphyxia with severe motor and intellectual disabilities (SMID) due to abruptio placentae. At the age of 1 year and 8 months, he was hospitalized in our hospital facility. At 2 years and 9 months of age, he suffered from a urinary tract infection and was administered cefaclor. On 3rd day after administration, he developed anemia, and on 5th day, he developed severe anemia. We stopped administering the drug due to drug-induced anemia but his anemia still progressed. We examined the cause of the anemia with the help of a positive direct Coombs test and we further diagnosed drug-introduced immune hemolytic anemia that was caused by the administration of cefaclor and started giving him prednisolone. On 5th day after prednisolone administration, the progression of anemia was stopped, and the child's condition began to improve. Cephem antibiotics are used frequently in clinical practice, particularly in the case of children with SMID at high risk of urinary tract infection; therefore, drug-introduced immune hemolytic anemia is a disease that requires special attention.

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The patient was 38-year-old male with a degenerative disease of unknown cause who had been receiving long-term tube-feeding. As he developed proteinuria and hypoalbuminemia along with cellulitis associated with zinc deficiency, administration of polaprezinc was performed. Four months later, however, polaprezinc was discontinued due to development of copper deficiency anemia associated with zinc supplementation. Therefore, 10 g of pure cocoa, which is rich in copper, was added to the treatment, and copper deficiency anemia was resolved in 4 weeks. Although it has been suggested that copper deficiency may occur with zinc supplementation if the serum zinc level is higher than 200μg/dl, this patient developed copper deficiency anemia at the serum zinc level less than 100μg/dl. The patient did not have a high serum zinc level due to promotion of urinary excretion of zinc combined with amino acid due to hypoalbuminemia as well as urinary excretion of zinc combined with protein along with urinary protein. Furthermore, it was assumed that copper absorption disorder as well as promotion of copper GI excretion occurred due to relative excess in zinc in terms of zinc-copper ratio. Attention is required for development of copper deficiency anemia even at a serum zinc level less than 100μg/dl with zinc supplementation for a child (patient) with severe motor and intellectual disabilities with hypoalbuminemia.

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Although various studies have been conducted on school-going children who require long-term medical care, several problems persist especially in those with sub severe motor and intellectual disabilities (SMID) who require intratracheal suction. One of these is the use of school buses. Since carrying out medical care on the school bus is difficult, support for transportation is required from the guardians. In this study, a multidisciplinary team investigated the possibility of taking the school bus for a child with tracheotomy who was using our daycare facility, by examining the effects of his posture before and while riding the daycare bus. He could ride on a bus without intratracheal suctions in a prone posture. However, his mother decided to take him to school by her car as she was concerned about his safety and anticipated him to travel in a sitting position on a bus, enjoying the view from the window like other children. Before attending school, which is one of the life events, mothers of disable children often have to confront their disabilities multiple times. Therefore, a multidisciplinary approach in accordance with the mother's wishes was important to support her decision-making from the child's perspective.

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Infection control in facilities for patients with severe motor and intellectual disabilities (SMID) can be challenging due to the special circumstances of the care environment and clinical condition of SMID patients that limits the applicability of existing textbooks and guidelines on nosocomial infection control measures. Recently, we experienced an outbreak of infection with bacteria producing extended-spectrum beta-lactamase (ESBL) in the hospital ward for SMID patients. The total number of inpatients in this ward was 42 at that time, with the average age being 46 years. Twenty-one of the inpatients received tube feeding, and the remaining 21 were capable of oral food intake. We suspected an outbreak and ordered a stool culture for all patients hospitalized in the ward. The results obtained revealed 60% (25/42) of the patients to be carriers of ESBL-producing bacteria. The carriage rate was 90% in a group or patients receiving tube feeding, and 29% in the oral food intake group (p < 0.001). The environmental swabbing of the tube feeding preparation room located in the ward detected intestinal bacteria, which suggests that the contaminated hands of our staff and contaminated tube feeding equipment could be responsible for the outbreak of bacterial infection. Our review of the equipment cleaning/disinfection procedure revealed inadequate disinfection of the equipment. For example, tubes used for feeding were replaced only once a week, which actually need to be replaced after each use. Careful cleaning and disinfection of tube feeding equipment and replacement of tubes after each use are demanding on both human and financial resources, which is a major issue in infection control.

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The 2019 coronavirus strain (COVID-19) has resulted in serious and abrupt outbreaks in pandemic proportions. At current, most institutions geared towards persons with severe motor and intellectual disabilities (SMID) have restricted their usual face-to-face visitations so as to prevent viral transmissions; most have subsequently launched online visitations via the use of information and communication technology (ICT) as an alternative. This study aimed to examine the status of online visitations among institutions for persons with SMID. A questionnaire survey was administered to 135 public and private institutions (for persons with SMID) nationwide. The results indicated that online visitations were performed from the home, the institutions, or either through free choice. Online visitations were scheduled for either weekdays or every day of the week (in 64% of institutions), and lasted for less than 2 hours at a time (in 56% of cases). Meanwhile, the time limit for the online visitation of a family was less than 15 minutes (in 80% of institutions). Video communication applications - such as LINE, Zoom, and Skype - were frequently used. At most institutions, fewer than 30% of families received online visitations. Based on these findings, we have proposed a new concept and associated methodology for appropriate online visitations. Additionally, online visitations should not only be used as alternatives, but should be coupled with face-to-face sessions on a daily basis.

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In order to clarify daily life issues and circumstances of persons with severe motor and intellectual disabilities (SMID) upon graduating from special schools, we implemented a questionnaire survey of families of persons with SMID attending 22 daily life care facilities, primarily in the Tama area. The results of our research were divided into three different time periods and analyzed as follows: less than ten years after graduation, 10-14 years after graduation, and fifteen-plus years after graduation. Our results shows that some 90% of the families of persons with SMID experienced difficulties in day-to-day life, while approximately 70% provided long hours of care. Compared to parents/guardians ten or more years after graduation, families of individuals who had graduated from school less than ten years prior had greater needs for help services, respite services, assistance with outings, etc. Differences in awareness levels were observed amongst parents/guardians of these two groups regarding social welfare services and/or regional assistance programs. For all three periods of time, both lower and upper limb function drills were considered most important in the future, for all three categories. Parents/guardians indicated the services/assistance they would want in the future, as follows: information on social welfare, learning assistance, medical institutions, and regional facilities. Differences were also observed depending on the age of the parents/guardians. Additionally, approximately 30% of families wanted the persons with disabilities to be placed in special facilities for children with severe disabilities following the eventual death of their parents, while 10% wanted group-home.

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The purpose of this study is to clarify the difficulties experienced with regard to stereotyped hand movements in children (persons) with Rett Syndrome from the perspective of guardians. A questionnaire survey by mail was conducted on 131 families who are members of the Japan Rett Syndrome Association and 63 families who are members of Rett Syndrome support organization, totaling 194 families. 71 families submitted their responses and information on 72 Rett Syndrome children (persons), including 1 set of twins, was obtained. 87% of the guardians of the children (persons) responded that they are experiencing difficulties with stereotyped hand movements or have experienced such problems in the past, with 81% giving specific details of their difficulties. Included in the difficulties experienced were: skin damage to the hand, fingers and chin, putting of hands in the mouth, difficulties in providing meal assistance, joint contracture or deformation, concern about hygiene and infectious diseases, inability to use their hands, difficulties in providing dressing, grooming and tooth-brushing assistance, concern about what other people think, muscle rigidity, poor teeth alignment, slanted posture, and chewing on clothing. Based on the results of the survey, the necessity of providing intervention for Rett syndrome children (persons) in accordance with the state of stereotyped hand movement while giving support to the guardians of the children (persons) with regard to the difficulties they face in their daily lives was indicated.

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Based on the Rett Syndrome Behaviour Questionnaire (RSBQ), the total score for all items and the total points for each area can be obtained for the 45 question items given to caregivers who responded either “existent," “occasionally" or “absent." The goal of this study was to clarify the characteristics of the results of RSBQ for Rett Syndrome children (persons) in Japan and to define the correlation between their age, intellectual development and motor functions. The subjects were a total of 194 families of Japan Rett Syndrome Association members and Rett Syndrome support organization members and a self-administered questionnaire was used. Information from 72 persons was collected and 70 or more persons responded to each of the question items in the RSBQ. 70% or more of the children (persons) responded either “existent" or “occasionally" to all of the 6 items under the “hand movement" area. There was found to be significant correlation between the total points in the “hand movement" area and intellectual development, accurately reflecting the symptoms and characteristics of Rett Syndrome. Thus, the potential for this method to be employed in the evaluation for rehabilitation and educational support as well as to be used as one indicator for efficacy determination was suggested. On the other hand, 60% or more of the children (persons) responded primarily “absent" to the “nighttime behavior" area, indicating that there are some items and areas requiring a review with regard to their connection with Rett Syndrome symptoms.

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The objective of this study was to obtain opinions of facility staff regarding advance care planning (ACP) before introducing this system. A questionnaire survey on ACP was conducted in the facility staff (e.g. physicians, nurses, social workers, and rehabilitation workers) who had been directly involved in the care for children (patients) with severe motor and intellectual disabilities (SMID). It was revealed that ACP had been recognized only by 7% of the staff. However, once they were told what it was about, 22% responded that they had already been practicing ACP. According to the survey, the most frequently reported time to start talking about ACP was when patients were approaching death, and the topics of ACP that they discussed were more on symptoms and treatments rather than on their concerns, preferences or values. As for issues on practicing ACP, it appeared necessary to consider how to share the details of discussions or how to provide an adequate consultation system for patients and their families as well as how to educate/train the facility staff. It is difficult to obtain opinions from children (patients) with SMID. Hence, it is important to collect objective information in order to predict their opinions as accurate as possible. It is a crucial role for a facility which is responsible for providing end-of-life treatments/care for children (patients) with SMID to work on recording/sharing information as well as on early start of ACP between families and medical care teams.

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