[in Japanese]

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Current situation and factors of hypozincemia in residents of a facility for children (adults) with severe motor and intellectual disabilities were studied from the nutritional point of view. A total of 84 patients (age 34 - 70 years old) with whom the serum zinc level was measured between April 2018 and March 2019 were included in this study to compare the serum zinc level, the amount of zinc intake, factors associated with zinc deficiency, and the amount of nutrition intake for each nutrition method. For the nutrition methods, subjects were divided into 3 groups: A group receiving oral nutritional supplements (ONS) including high density liquid diet, a group receiving both ONS and meals, and a group receiving meals. The median of the serum zinc level of 84 subjects was 67 (44 - 128) μg/dl and that of the amount of zinc intake was 12.1 (5.7 - 70.6) mg/day. The serum zinc level of 46 out of 58 subjects (79%), whose zinc intake had been greater than the recommended dose (10 mg/day for males, 8 mg/day for females), was lower than the reference value (80 - 130μg/dl), suggesting that there are currently many residents who present zinc deficiency even though the amount of zinc intake have been adequate. A multivariate analysis using the serum zinc level and the amount of zinc intake as objective variables revealed that there were significant associations with the subjects' age, sex, serum copper level, concomitant use of zinc preparations, serum albumin level, nutrition methods and energy. In particular, concomitant use of zinc preparations and differences in nutrition methods had strongly been associated with the amount of zinc intake (increased with ONS compared with meals). When comparisons were made on the amount of nutrient intake between the nutrition methods, it was revealed that the amount of copper, calcium, dietary fiber or vitamin c intake as well as the ratio of zinc to copper intake were not associated with the differences in the amount of zinc required between ONS and meals. It was suggested that hypozincemia could be treated, while preventing zinc excess, by increasing the amount of zinc intake from meals as much as possible.

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When treating tracheostomized patients with a neuromuscular disorder, problems can occur with inappropriate fitting of the tracheostomy tube associated with a progressive tracheal deformity, a condition occurring secondary to scoliosis and neck contracture. In such cases, it is important to select a tracheostomy tube that closely corresponds to the anatomy of the trachea. Recently, we have utilized custom-made tracheostomy tubes when such problems remain unsolved by changing the type of ready-made tube. In this study, we retrospectively evaluated the effects of custom-made tracheostomy tubes in 7 tracheostomized patients receiving treatments for neuromuscular disorders. Six were long-term tracheostomy patients, in whom various ready-made tracheostomy tubes did not fit appropriately, causing pain and compression to the tracheal wall, which predisposed them to tracheal injury. Use of custom-made tracheostomy tubes in those cases resulted in an improved fit, and decreased pain and risk of complications. The other patient, whose tracheostomy experience was short-term, stated a desire to have an ability to speak while on mechanical ventilation. That was not possible with ready-made fenestration tubes, whereas a custom-made tube allowed for comprehensible communication. In the present cohort, use of custom-made tracheostomy tubes resulted in a decrease in subsequent risk of complications as well as improvement in quality of life including the ability to communicate verbally. We consider that a custom-made tracheostomy tube is an effective option for tracheostomized patients who have unresolved problems with ready-made tubes.

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As of the end of March 2017, a total of 449 nurses completed training sessions offered at 7 educational centers in the institution for nurses specialized in sever motor and intellectual disabilities authorized by Japanese Association on Welfare for Severe Motor and Intellectual Disabilities established in 2011. Upon this opportunity of nationwide promotions of educational centers, we conducted a survey of nurses who participated in the training sessions (trainings) held at certified educational centers in order to assess achievements of their goals and issues raised in the trainings. According to the results of the survey for “quality of nursing care/improvement in the specialty field", it was revealed that over 70% of participants had recognized improvement in the specialty field in 10 out of 18 items compared to before the trainings. For “performance of leading roles" at their facilities, expansion in their roles to be in charge of study/training sessions was recognized. In addition, an advantage of trainings which went beyond the boundaries of facilities was observed through communications and networks with staff members of other facilities. Meanwhile, it was revealed as an issue that there would be a need to improve the training contents as well as to establish a supporting system by a nursing manager.

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We experienced two cases of epilepsy in elderly patients with severe motor and intellectual disabilities (SMID). The first case was a male of 72 years old with a score 15 in Oshima's classification at the time of onset. He developed tonic-clonic seizure for 20 seconds without any particular cause. After 9 months of follow-up, he often presented symptoms such as automatism (moving the month with lip sounds), and focal impaired awareness seizure which lasted for a few minutes along with postictal confusion. A treatment with levetiracetam was started at 500 mg/day, and the seizure could be controlled at 1000 mg/day. The second case was a female of 73 years old with a score 1 in Oshima's classification at the time of onset. Movements such as sticking the tongue out as well as regular twitches of the upper limbs and upper eyelids along with tachycardia were observed intermittently. A treatment with clobazam was started. The seizure remission was once achieved at a dose of 15 mg/day. However, after two months, twitches of the upper limbs/eyelids lasting for a few minutes started to appear frequently, sometimes along with tachycardia and fixation of eyeballs. The dose of clobazam was increased, but it did not seem effective. Hence, the treatment was replaced by levetiracetam. The dose was started at 500 mg/day, and seizure control was achieved at 1000 mg/day. No adverse reactions such as decrease in the alertness level or cognitive functions were observed in either case. It should be noted that epilepsy in the elderly might develop in patients with SMID. In order to treat such patients, it is desirable to use novel antiepileptic drugs which cause less adverse reactions or drug interactions.

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Background: Patients with severe cerebral palsy tend to experience loss of muscle mass and strength with age. This negatively impacts performance of activities of daily living (ADL) and often causes patients to become bedridden. Purpose: To determine whether a strength training program could improve ADL performance in a patient with severe cerebral palsy. Patient: A 56-year-old man with severe cerebral palsy who had previously been able to walk with a walker but had become almost completely bedridden. Methods: A multidisciplinary team conducted twice-weekly strength training sessions with the patient for 20 consecutive weeks. Exercise intensity was adjusted according to the patient’s vital signs and feedback from an original face scale for evaluating exercise intensity to ensure safety and appropriate loading. Muscle mass, muscle strength, calf raise hold time, continuous walking distance, movement monitoring findings, ADL, and spasticity were evaluated. Results: Muscle mass and strength increased with no corresponding increase in spasticity. Both gait and continuous walking distance improved, which led to improvement in ADL performance. Conclusions: Strength training increased muscle mass and strength and improved ADL performance in a patient with severe cerebral palsy.

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The patient was a 7-year-old girl who developed severe motor and intellectual disabilities (SMID) associated with acute encephalopathy of unknown cause and had been receiving enteral nutrition. She was admitted to our hospital for introduction of food paste, and anaphylaxis occurred after injection of heat-processed egg custard. It is desirable to take into consideration the possibility of developing allergic reaction when introducing food paste to a child with SMID.

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Wolf-Hirschhorn syndrome is characterized by severe intellectual disability and refractory epilepsy; the prognosis depends on the severity of epilepsy and the presence of heart disease. Recently, the frequency of malignant tumors has increased as a cause of death in aging patients with severe motor and intellectual disabilities (SMID). In the present case, the patient was a 40-year-old Japanese male who suddenly displayed extreme displeasure and cried frequently. Because he also had a fever, he visited our hospital. An upper gastrointestinal endoscopy revealed neuroendocrine cancer, which has a historically poor prognosis, at the elevated lesion of the esophagus. We discussed his treatment with a gastroenterologist and his parents. As a result, his family chose palliative care. A multidisciplinary care team was launched quickly and careful treatment was performed after discussing the patient's pain assessment with his parents. Several multidisciplinary meetings were held to prepare for a smooth transition to home palliative care. Eventually, the patient was able to spend time at home. It is difficult for pediatricians alone to provide appropriate palliative care for older patients with SMID and organize the transition to home. Therefore, collaboration with multidisciplinary colleagues is needed.

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We report a case of a 27-year-old woman. At 10 months of age, she had contracted bacterial meningitis by Streptococcus pneumoniae, which resulted in hydrocephalus and severe neurological sequelae. She contracted recurrent aspiration pneumonia during growth and underwent gastrostomy at the age of 19 and tracheotomy at the age of 22 years. At the age of 25, she was hospitalized but contracted pneumonia by aspiration of salvia. She underwent laryngotracheal separation when she was 26 years old. 10 months after surgery, she presented with excessive bleeding from the trachea. Contrast computed tomography of the neck revealed bleeding from the granulation tissue in the trachea along with collateral circulation in the bronchial arteries. The bleeding was stopped using a tracheal cuff but it resumed the next day. The patient died due to blood retention in the trachea.

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In a patient with severe motor and intellectual disabilities who had increasing respiratory failure resulting from tracheal stenosis and tracheomalacia, respiratory symptoms were resolved after sternum partial resection and brachiocephalic artery transection. The patient was a 25-year-old man with cerebral palsy at the surgery. He had been treated with non-invasive positive-pressure ventilation for hyper CO2 at 17 years of age. Hypertonicity had caused severe scoliosis, so he had been treated with intrathecal baclofen therapy since the age of 19 years. And he was taking several muscle relaxants. Respiratory infections repeatedly occurred within the 10 months before the surgery, causing repeated paroxysmal cyanotic episodes. After recovery from the respiratory infections, his muscle tone was further enhanced, and his oxygen requirement increased. A chest computed tomography scan revealed that the trachea was compressed by the sternum and the brachiocephalic artery, as a consequence, was narrowed. In consideration of the patient's quality of life, a tracheotomy was avoided, and a partial resection of the sternum and transection of the brachiocephalic artery were performed. After surgery, oxygen supply during the day was no longer required and the cyanotic attacks no longer occurred. Sternum partial resection and brachiocephalic artery transection is considered to be an effective choice in patients with respiratory failure due to tracheal stenosis and tracheomalacia, without increasing social constraints.

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We report two patients with severe motor and intellectual disabilities (SMID) who exhibited aggravate paroxysmal discharges on electroencephalogram (EEG) during metabolic alkalosis. Case 1 was a 52-year-old male with cerebral palsy and epilepsy. He had frequent vomiting due to gastroesophageal regurgitation and had his gastric juice drained from the gastrostomy to prevent vomiting. Then his venous blood gas analysis showed metabolic alkalosis : pH. 7.59, HCO3-. 62.8 mmol/L, K+ 2.81 meq/L and Cl-. 81 meq/L. Though he had no seizure, his EEG showed frequent paroxysmal discharges in the right frontal area. Case 2 had bleeding from arteriovenous malformation in his brain stem and had convulsions at neonatal period. His seizures were controlled by phenobarbital. But he had frequent vomiting and had also his gastric juice drained from the gastrostomy to relieve vomiting. At the age of 4 years and 11 months, EEG showed frequent continuous paroxysmal discharges at his right posterior head area. His venous blood gas analysis showed metabolic alkalosis : pH.7.54,HCO3-38mmol/L,K+ 2.82 meq/L, Cl- 95 meq/L, In both cases, treatment of metabolic alkalosis reduced the frequency of paroxysmal discharges. When patient with SMID showed aggravated paroxysmal discharges on EEG, attention should be paid to conditions that induce metabolic alkalosis, such as frequent vomiting.

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The purpose of this study is to investigate how to support patients with severe motor and intellectual disabilities who have been persistently presenting with spitting behaviors since childhood. The study was conducted using applied behavior analysis. There is always something to trigger a behavior before it happens. Hence, we extracted two scenes where a spitting behavior is likely to occur, “when getting into a wheelchair before breakfast" and “when getting into a wheelchair before dinner", and conducted an ABC analysis using a scoring system to assess situations associated with the spitting behavior. According to the results of the ABC analysis, we considered that a spitting behavior was associated with an expression of attention-seeking desire, which is a function of behavioral disorder. Therefore, we conducted interventions such as verbal as well as visual communications and physical contacts through massage and compared the scores before and after these interventions. According to the results, it was revealed that the number of days that they did not show a spitting behavior increased, suggesting that it is important to support patients with behavioral disorder, utilizing the applied behavior analysis.

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The purpose of this paper is to reveal the expertise of nurses engaging in the care of seriously ill children through researching how they observe the physical conditions of serious ill children and how they exercise clinical judgements. A semi-structured interview was first conducted with ten nurses familiar with seriously ill children and then a qualitative and descriptive analysis was performed. Consequently, it was revealed that the clinical judgements made by the nurses were the process in which they【made a substitute leading to the change in medical examinations and treatments】 by based on 【their observation considering the characteristics of seriously ill children】【evaluating changes in seriously ill children's physical conditions requiring treatments】with paying attention also to their unique pathological conditions, poor physical reserved capacity, etc. for the purpose of linking these efforts to【the nursing care towards the sound maintenance of good physical conditions】for those seriously ill children who would never make a full recovery. In short, such clinical judgements were based on their basic approach to taking care of seriously ill children with【giving top priority to the QOL of seriously ill children】. It was also found that the nurses made the clinical judgements on the necessity of a therapeutic intervention by evaluating seriously ill children's deviation from their normal everyday life pattern with focusing on changes in their physical conditions which made an impact on their everyday life. It is concluded that the expertise in the nursing care of seriously ill children is the pursuit of their everyday life maintenance under good physical conditions by repeatedly exercising clinical judgements while giving considerations not only to their mere disease conditions but also to their everyday life conditions themselves.

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An accurate understanding of people with disabilities is important for normalizing their lives. Accordingly, we investigated the public's level of accurate knowledge about child development and rehabilitation subjects. We recruited a total of 74 participants: 40 first-year nursing students and 34 first-year speech-language and hearing therapy students. The sample comprised 14 male and 60 female participants, ranging in age from 18 to 43 years. We asked them whether they knew certain words; in cases where they responded affirmatively, we further asked them to explain the meanings of each word in 2-3 sentences. The response rate was 100%. The words related to children with disabilities were not well known. In particular, “children with severe impairment," “children with severe multiple impairments," and “medical care" were not known to 93%, 73%, and 69% of the students, respectively. The words related to developmental disorders were well known but not understood accurately. About 34% participants confused the term “developmental disorder" with “intellectual disability," while 20% confused the term “learning disability" with “intellectual disability." These findings suggest that even students regarded as being interested in child development and rehabilitation do not have sufficient knowledge about people with disabilities. Therefore, we need to extend the public's accurate knowledge.

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Upon introduction of enteral feeding connector with interconnection-prevention (ISO80369-3), a questionnaire survey was conducted online in caregivers for children receiving home-based medical care in order to extract issues in the field of pediatric developmental stage. Six hundred and sixty-seven responses were valid. The number of attachment/removal of the connector per day was 21-40 times in 42%, 41-60 times in 19%, and more than 61 times in 12%, suggesting that it occurred very frequently. For the change of the connecting part to a lock system the numbers of responses for “inconvenient if it cannot be removed" (19%) and “better if it can be removed" (42%) were remarkably higher than “better if it cannot be removed" (13%) and “inconvenient if it can be removed" (1.8%). The rationales included 118 responses regarding possibility of developing serious incidences such as “accidental removal of gastrostoma itself/nasogastric tube" and “damage to the gastrostoma". For the change to a screw type, 89% of the subjects responded “apprehensive"; most of them raised issues regarding increased burdens of caregivers as well as hygienic concerns with nutrient stuck in a ditch. It appeared that the connector with a novel standard, the screw-type lock system, might be disadvantage for children receiving home-based care in terms of safety and burdens for caregivers, suggesting that we should continue handling conventional connectors until the above-mentioned issues would be investigated.

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In Japan, the Ministry of Health, Labor and Welfare has mandated hospitals and clinicians from December 2019 to November 2021 to make the transition from the existing connectors (legacy feeding devices) to new connectors (International Organization for Standardization (ISO) 80369-3 compliant connectors), which reduce the risk linked to misconnections between medical devices for different medical applications in order to prevent medical mistakes and ensure a stable supply of products through international coordination. However, in the United States, the adoption of the international standard is less than 20 %, even though three years have passed since the start of their availability. Concerns about the international standard include infection potential from the narrow and deep moat area on the patient side of the new connector, its complexity of use with adapters, dosing inaccuracy and inadvertent gastrostomy tube removal as a result of tugging due to the positive-lock system, and thus there are worries these new connectors will not be able to meet the needs of the home enteral nutrition population. Caregivers are more likely to provide blenderized tube feeding, gravity or syringe feeding, or use the syringes for venting at home. Therefore, it is necessary to confirm the performance of new connectors not only for use by healthcare workers, but also for use by caregivers outside the hospital. We therefore would like to propose that the expiration date of the existing connectors be extended until various concerns about the new connectors can be eliminated by thoroughly examining the current problems associated with their usage.

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At many facilities for children (patients) with severe motor and intellectual disabilities (SMID), a small number of clinical laboratory technicians are in charge of conducting laboratory and physiological tests as well as playing significant roles in various committees such as infection control. In a workshop for staff members of facilities for children (patients) with SMID, it was revealed in a subcommittee meeting for laboratory technicians, which had been held since 2008, that technicians of each facility had been conducting tests while dealing on individual basis with various issues/problems in clinical laboratory studies specifically associated with children (patients) with SMID. In the subcommittee meeting for laboratory technicians held in 2018, a questionnaire survey was conducted on “Roles and Motivation for Clinical Laboratory Technicians in Facilities for Children (Patients) with SMID" at 134 facilities nationwide with regard to how they feel about their tasks in such circumstances. Although the response rate was low at 20.2%, 92.9% of them felt “being involved in their roles" and 89.3% felt “being motivated". We consider that in the future clinical lab technicians will feel even more involved in their roles and motivated if we could provide better-organized activities including subcommittee meetings for laboratory technicians as well as information regarding systematic measures for various issues so that clinical laboratory technicians at many of the facilities for children (patients) with SMID will be able to share and utilize the information in the tests in general, contributing to the medical care for the children (patients) with SMID.

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We conducted body measurements needed in designing clothes for children (patients) with severe motor and intellectual disabilities (SMID) and made clothes for them for the purpose of improving their QOL and relieving burdens of their caregivers.We measured bodies of 25 inpatients/outpatients of Todaiji Ryoiku Hospital for Children and summarized information/reference sizes needed for designing clothes for the children (patients) with SMID on newly-designed “measurement records". Then we compared the measurement results with pre-existing data and analyzed them, revealing that more than 80 percent of the subjects were small in height and thin/skinny in body size. In particular, their legs were extremely thin; there seemed to be an imbalance in terms of thickness between their body trunks and limbs. Considering that it is difficult to put on or remove clothes especially around the thin areas due to severe paralysis, we suggest that it is necessary to select elastic materials and design openings appropriate for changing clothes in clothing design for children (patients) with SMID.

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Recently, the incidence of tracheal cannula-free has been increasing because it is a useful approach to prevent complications, such as endotracheal granulation and a trachea-brachial artery fistula. However, one issue is that endotracheal suction can be performed only by medical staff. In our day care center for severe motor and intellectual disabilities (SMID), there are four SMID with cannula-free tracheostoma. Occasionally, a nurse may not be able to accompany the patient during transfer, and endotracheal suction by care workers or nursery workers during an emergency is unavoidable. Therefore, we established a working group (WG) for the safety of SMID with tracheal cannula-free. The activities of our WG were as follows: (1) conduct lectures on cannula-free tracheostoma; (2) evaluate the current care of two SMID with cannula-free tracheostoma and consider the approach for their care; (3) create a personal checklist card; (4) create flowcharts for emergency situations in the manuals of shuttle buses; (5) conduct training sessions on suction techniques and emergency situations for care workers using simulators; and (6) start shuttle buses that have care workers. It is necessary to establish the system, as identified by our WG, so that the quality of life of SMID patients with tracheal cannula-free does not deteriorate owing to social restrictions.

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Providing comfort is an important care activity in nursing for patients with severe motor and intellectual disabilities (SMID). This study conducted interviews and did a content analysis of responses to understand how nurses are aware of the comfort for patients with SMID. The analysis yielded six categories: “using a variety of methods to ensure patient comfort", “going through a process of trial and error to achieve patient comfort", “realizing the comfort created by interacting with child patients with SMID", “feeling pleasure when having been able to provide the appropriate comfort, and wishing to tell other nurses by what method", “feeling conflicted when not being able to communicate well among nursing staff", and “searching for a comfortable environment for staff as well as patients". The comfort for children (patients) with SMID tends to be intricately intertwined with several factors including physical and mental conditions characteristic to this patient population. The experiences and values of nurses are also considered to influence the comfort assessment. Therefore, it is important to continuously try to explore better ways of comfort for each individual child (patient) with SMID, to verbalize the process and the results, and to share activities with the whole ward.

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A survey was conducted among nurses who participated in trainings for nurses specialized in severe motor and intellectual disabilities offered at medical care facilities/nursing care offices for disabled children in Tokyo (professional nursing-training course for severe motor and intellectual disabilities in Tokyo; hereinafter, trainings) as well as nurse managers from corresponding facilities, for the purpose of investigating achievements and issues from the trainings. The results of the survey revealed “theoretical exploration of professional knowledge/skills", which was the purpose of the trainings, and “decrease in employee turnover rate" as the achievement of the trainings. Meanwhile, the issues raised for educational centers offering the trainings included “training methods which reduce burdens of trainees" and “training designs, taking local features into consideration", and those for nurse managers included “role allocation, taking trainees' burdens into consideration" and “establishment of a system which can increase the number of trainees for continuation of the trainings.

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The aims of the present study were to identify the risks to caregivers during nursing care of persons with severe disabilities and to examine preventive methods and countermeasures. We conducted an interview and an online survey questionnaire with caregivers in institutions that provide visiting nursing care to persons with severe disabilities. A total of 83.3% of the respondents replied that they believed it to be the [risk of infection] while 87.3% of the respondents indicated the [risk of lower back pain]. Regarding the [risk of infection], many caregivers did not undergo vaccination due to the cost. Future research should examine the government financial support systems for covering the cost of vaccinations for caregivers. Regarding the [risk of lower back pain], many caregivers were taking countermeasures independently. Although the government conducts training for caregivers providing visiting nursing care to persons with severe disabilities as a countermeasure against such problems, it is necessary that the content and composition of this training reflect the results of the present study.

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This study aimed to explore the feasibility of evacuation from home to other place under the condition of a long-term power failure for children (individuals)with severe motor and intellectual disabilities- medical care dependent group (SMID-MCDG) at home, and the psychology of their mothers on the supposition of this critical situation. The interviews with the mothers showed that the feasibility of evacuation depended on [the presence or absence of a place where the children can live away from their house] and [the presence or absence of means for transporting the children to the designated place]. The supposition of evacuation in such a critical situation brought the mothers [loss of their confidence to survive the disasters together with their children] and [hope of going to the heaven with their children]. To prepare for the long-term power failure, the system of accepting those children (individuals) by the local medical institutions should be arranged on a nationwide scale, and the means for transporting those children (or people) to the evacuation site should be ensured with the cooperation of the community. For those purposes, development of the items for evacuation necessary for those children (individuals) is further required. To support the mothers is also necessary in consideration of the fact that the mothers are apt to be negative about the actions for saving their children's life and their own life in such a critical situation.

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