[in Japanese]

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The number of patients who died in the ward of severe motor and intellectual disabilities (SMID) of Kofu National Hospital during 12 years from 1994 to 2005 was 16 (11.5% of the total number of inpatients during this period), and it was less than the number reported in the previous survey (during 12 years from 1982 to 1993), which was 32 (21.1% as above), conducted at the previous facility, Nishi Kofu National Hospital. As for backgrounds of the hospitalized patients, their mean age had risen from 30.9 to 41.6 years old, and the percentage of those categorized as Oshima’s Classification 1 had increased from 38.3% to 47.9%, suggesting that the age of inpatients had become more advanced and their conditions had been worse. According to the previous survey, only one patient had received tracheostomy, and no death was reported associated with the procedure. While in this survey, 18 patients went through tracheostomy, and 3 of them died. Of these, two of them died of complications associated with the procedure. Tracheostomy reduced the incidence of respiratory infections and contributed to prolong the lives of patients in severe conditions who could have died in the earlier stage. On the other hand, death had occurred due to complications of tracheostomy itself. It is thought necessary to pay special attentions on methods of selection / fixation of the tracheostomy tube as well as on management of nutrition, body movement / positions when performing tracheostomy in patients with SMID.

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Of all patients who use our short-term stay service, percentages of patients with severe motor and intellectual disabilities in the medical care dependent group (SMID-MCDG) and those of sub SMID-MCDG have been increasing. In order to find out if the short-term stay service has been effectively used by the patients with SMID-MCDG and sub SMID-MCDG, a survey was conducted on their use of the service for the past 10 years. The subjects included 61 patients who have used the facility; they were categorized for the number of days they stayed per year, the age when they started using the facility, and the reasons for stay according to the cause of disabilities they have (prenatal, perinatal, or postnatal disability). As a result, it was found that SMID-MCDG patients spent more days at the facility and started using the short-stay service sooner than those with sub SMID-MCDG. The commonest reason of their stay was difficulty of care. Furthermore, some differences were observed according to the cause of disabilities they have; the major reasons for the patients with postnatal disabilities were for caregivers to rest and difficulty of care, and the number of days they spent at the facility was longer. While in patients with prenatal disabilities, they had been using our service since they were small, and the reasons for stay were less likely to be difficulty of care. These findings seem to be greatly associated with severity of their disabilities as well as the caregiver’s stress level in care and acceptance of their disabilities. It is thought necessary to come up with supports or measures by considering not only the severity of disorders but also characteristics by the cause of disabilities so that SMID-MCDG and sub SMID-MCDG patients will be able to use the short-stay service more effectively.

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There is an increase in the number of cases where siblings / relatives of patients with severe motor and intellectual disabilities (hereinafter, referred to as SMID) become guardians because these patients with SMID get older in their facilities. The following points were studied based on the previous cases where decision making process for treatment of SMID patients who had been predicted to have poor prognoses were determined by siblings: 1) therapeutic decision making for patients without the capacity to consent for themselves, 2) the roles of guardians, 3) the rationale for siblings to become guardians, and 4) the differences in decision making for treatment by different status. When receiving informed consent from SMID patients, the decision has to be made by their guardians from the aspect of patients’ protection. The guardians should follow the “optimal benefit criteria” as the judgment criteria. In addition, the guardians are family members in actual cases; considering their roles, it seems necessary to ask the siblings, who would be the future guardians, to be present when we discuss the future plans or make treatment decisions at the time of the patients’ admission. Furthermore, it is preferable for them to spend more time together; it is important to support them so that the patients can stay home over night or their family can visit them. It is also desirable to respect the guardians’ right for decision making, and at the same time, for the medical practitioners themselves to consider the optimal benefits for the patients when they eventually make decisions together.

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This is a report of patients with severe motor and intellectual disability showing hypozincemia, with whom hypozincemia was improved by high dose vitamin C administration. In Nakagawa-no-sato hospital for the disabled children, hypozincemia was observed in 47 out of 67 inpatients in 4 years approximately from the year 2004. Of these, 35 patients showed improvements by treatments, namely, by changing them from zinc-fortified soya protein to a concomitant use of vitamin C-fortified zinc powder and milk protein in 4 patients, increasing vitamin C intake significantly from 100 mg/day to 500 mg /day approximately in 6 patients, treating infections in 5 patients, administering thyroid drugs in 6 patients, and simply by increasing nutrition intake including zinc in 5 patients. In addition, 10 patients received multiple treatments described above. As a whole, a large dose of vitamin C was given to 16 patients, thyroid drugs were administered to 13 patients, infections were treated in 9 patients, and nutrition was improved in 16 patients. There is no report available, as far as we know, regarding the treatment of hypozincemia by high-dose vitamin C administration. Hence, it is necessary to continue investigations on this aspect in the future.

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An interview study was carried out to better understand home care conditions for patients with severe motor and intellectual disabilities (SMID), identify factors in the management of those who are engaged in home care for SMID, and to propose suggestions for better and safer home care management. The principal caregivers of five patients with SMID who used resources of the Tokyo SMID Home Visit Care Services (Tama region) participated in this study and all of the care recipients were younger than 20 years old. Results demonstrated that most of the factors in the management of home care for SMID could be classified into the following ten categories: existence of good consultants in a household; availability of family members who can provide care similar to that of principal caregivers; availability of public agency consultants; resourcefulness in daily life; understanding from the workplace of the caregiver’s spouse; ongoing personal development of principal caregivers; good environments for home care; care support from relatives who live separately; life stability; and sibling relationships. Life stability and principal caregivers’ personal growth during the home care tenure were identified as primary factors in the long-term maintenance of home care for SMID.

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Non-structured interviews with 11 mothers of home-care patients with severe motor and intellectual disabilities under 30 years of age were carried out in order to clarify in what situation they recognize their ongoing problem as a stress and remove their stress. As a result, the following three categories were extracted: “the life with her own child who needs care”, “recognition of her own physical state”, and “management of the everyday life by encouraging herself mentally and physically”. In addition, the following two points were obtained as new findings; the difficulty of “recognition of her own physical status” in “the life with her own child who needs care” and the fact that the daily actions taken by the mothers were not the use of support but mainly their own self-supporting efforts. Therefore, it is suggested that some sort of support is necessary for the mothers by developing an index with which they can easily recognize their physical state.

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A questionnaire survey for 58 mothers caring for a cerebral palsy child at home was carried to clarify the causes of mothers’ low back pain and to ascertain home care conditions. The mothers were divided into two groups; mild and severe low back pain groups. As a result, we confirmed the statistical significance (p < 0.05) of 6 items between the two groups: (a) face scale for cervical-brachial pain, (b) total hours of care, (c) going to hospital regularly or not at present, (d) care giving burden, (e) will to continue with home care, and (f) the necessity of position changes at nighttime. The items of physical burden that many mothers rated the highest were holding the child and bath care. The conditions they labored under were also related to the child’s growth, their own aging, and various mental burdens. In this research, we clarified that mothers caring at home for a child with cerebral palsy have to adapt to physically burdensome conditions such as low back pain, as well as mental stresses. It is important that efforts be made to alleviate the physical and mental burdens suffered by mothers caring for a child with cerebral palsy at home.

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The body mass index (BMI) is an essential measure of body size in terms of nutrition control. However, the BMI value does not seem to reflect the true figure in many patients with severe motor and intellectual disabilities (SMID) and the elderly, especially in those with advanced extremity contracture or severe scoliosis caused by bed-ridden condition. The BMI is defined as the individual's body weight (kg) divided by the square of his or her height (m) and precise measurement of height is difficult in these patients, a new index, which is easily calculated without using height and can replace the BMI, was considered necessary. Therefore, we thought about quantification of appearance evaluation (thin, normal or obese) for measuring body size; an overall judgment was made by evaluating selected factors such as the amount of fat on the face, upper arms (instead of triceps skinfold thickness, TSF), and in the sacral area, which is the risk factor of bed sore. The new index includes this with amount of muscles, the primary element of body size, as a supportive evaluation. The index obtained from these factors showed less variability in evaluations made by examiners and reflected actual figures most of the time. Hence, it was thought that this could be used as an index instead of the BMI.

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In recent years, substantial education / treatment is required for patients with severe motor and intellectual disabilities, medical care dependent group (hereinafter, referred to as “SMID-MCDG”) who need intensive medical care. For many of them, it is difficult to observe their response to the provided care. Therefore, it has been an issue to find the way to evaluate their response and to provide the practical care based on the response. In this study, the patients’ response to the cares provided by care givers was assessed in patients with SMID-MCDG. After making preliminary observations on the patients’ response to the stimuli, we gave them the name-call stimulation and the combined stimulation. For the response to these stimuli, their heart rate reactions responses and video images of their conditions were analyzed and discussed. As a result, high response rate was observed with the name-call stimulation in two patients; one of them showed the long-term effect in the response rate to the stimuli. The analyses on the video images enabled us to study on their small movements which are likely to be overlooked in daily care activities. Based on these findings, it was suggested that evaluations would be possible on patients with SMID-MCDG, with whom it is difficult to observe their response, by combining physiological indexes and video image analyses.

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This study examined issues surrounding the selection of appropriate tasks for the rehabilitation of patients suffering from severe motor and intellectual disabilities (SMID). We investigated various aspects of handcraft activities, including the skills required by for the tasks, the impaired upper-limb function involved, and the cognitive developmental stages associated with the activities. From the results of 92 patients, we found that there was a need to fit the self-help devices to the upper-limb function levels of SMID patients. In addition, we found that an appropriate aim of rehabilitative activities is to include sensory stimulation activities, encourage tool manipulation, and develop alternative handcraft skills according to the cognitive developmental stages of patients. Moreover, the results suggest that when planning handcraft activities for patients with SMID, determining the cognitive developmental stage of patients could aid in selecting and adapting tasks to the most appropriate arrangement and presentation format.

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Visual localization and gazing abilities were assessed in patients with severe motor and intellectual disabilities (hereinafter, abbreviated as SMID) by using near-infrared spectroscopy. In observational assessments, an increase of oxyhemoglobin concentration was observed in patients with SMID and healthy adults, who showed ability to gaze toward visual stimulation. While in patients who did not gaze toward the visual stimulation, there was hardly any change observed with the oxyhemoglobin concentration. Hence, it was suggested that “gaze” or “no gaze” could be regarded as an increase or no change, respectively, of oxyhemoglobin concentration in the visual function assessment using near-infrared spectroscopy in patients with SMID.

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We developed a quantitative measurement method (Green Village Method: GVM) for thoracic deformity using CT images, an ordinary computer software, and a free software. In this study, inter and intra examiner reliability of GVM was studied. A total of 29 CT images of sternum and vertebral transverse process obtained from 29 patients with severe motor and intellectual disabilities, who had taken CT in the past for diseases such as pneumonia, were measured. There were 5 examiners, and they measured the distances between “the midpoint of sternum” and “the midpoint of transverse process” (GVM-d1), “the midpoint of sternum” and “the tip of spinous process” (GVM-d2), and between “the point of intersection of the sternum perpendicular bisector” and “the point of intersection of the perpendicular bisector of the transverse process and the vertebral anterior margin” (GVM-d3) as well as the angle between the sternum and vertebral torsions (GVM-a). GVM-d1, 2 and 3 showed high reliability with small standard error of less than 7 mm; this could be a highly accurate evaluation method. Since reliability of GVM-a was slightly low, it was thought necessary to reconsider the standard of the vertebral perpendicular bisector and further improve the accuracy.

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An acoustic analysis of breathing sounds was carried out targeting children (adults) with severe motor and intellectual disabilities (SMID) by a sound spectrograph using a lung sound analysis system. As a result, increase in the mean intensity of the frequency spectrum due to the increase of accumulated substances in the airway, and changes such as loss of an intermittent rale, could be observed in the prone position with time. Moreover, the intermittent rale was confirmed to have a characteristic shadow and harmonic structure in the low-frequency band of the sound spectrograph (100-300Hz). Furthermore, the breathing sounds caused by accumulated secretions near the tracheostomy cannula, and the characteristic obstructive breath sounds caused by depression of the tongue root and relaxation of the soft palate, which commonly occur in children (adults) with SMID, were observed on the graph. A sound analysis using a lung sound analysis system which visually and non-invasively grasp changes of breathing conditions with time and characteristics of breathing sounds when the airway is blocked, was shown to be of possible benefit in the evaluation of the breathing conditions of children (adults) with SMID in daily life.

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Copper deficiency is a relatively rare complication of enteral nutrition in patients with severe motor and intellectual disabilities. We report a patient with copper deficiency due to enteral nutrition through jejunostomy. He was found to have macrocytic anemia and neutropenia. Copper supplementation with cocoa was effective for improvement of the haematological abnormalities. Although his daily intake of copper was not particularly low compared with that in other gastric tube-fed patients, the serum copper concentration was significantly low. Since copper is usually absorbed in the stomach and the proximal duodenum under acidic condition, it is suggest that nutrition through the jejunostomy should be considered one of the risk factors for copper deficiency.

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This is a case report of a person with severe motor and intellectual disabilities (SMID) who developed copper deficiency as a result of a change from one enteral diet to another, both of which meet the Japanese dietary intake standars (2005). This case is of a male patient who is suspected of having a spinocerebellar degeneration from family history. From December year x–6, feeding mode of the patient was changed from oral to tubal one due to functional decline. In April year x, the low residue diet was changed from E7® to CZ-Hi®. In December year x+1, leukopenia (1600/μl) and anemia (6.7g/dl) was noticed. The hematologists diagnosed these as aplastic anemia and treatment using anabolic steriod was started. In June year x-2, the serum copper level was 118μg/dl; however, in March year x+2, an abnormally low level of 4μg/dl was seen. Readministration of E7® diet with cocoa to enhance the copper content restored the serum copper level to normal range in approximately 2 months, and an improvement was seen in the hemogram. In this case study, there was no significant deficit in copper intake, nor was there excessive administration of zinc. Thus, the factors leading to copper deficiency could not be identified. Since there are many unknown factors regarding the complication mechanisms in patients with SMID, constant attention should be given to their nutrition and regular checkups should not be ignored.

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[in Japanese]

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[in Japanese]

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