[in Japanese]

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We conducted a survey on the actual situation of multiple medical care requirements in persons suffering from severe motor and intellectual disabilities (SMID) in the day-care service centers of five Japanese hospitals or institutes consisting of four type A centers (15 beds with transportation) and one type B center (five beds without transportation) in 2009 and 2010. In the type A centers, more than 90% of SMID persons had markedly severe disabilities with Oshima scores of 1–4. Twenty percent of these persons required extremely intensive (or extensive) medical care; this included tube feeding assistance and/or frequent body position changes in approximately 70%, and ventilator care through tracheal tubes or the nasal airway in 12%. Disabled persons who were cared for in type B centers had less severe disabilities compared with type A centers. All the centers expressed difficulties in coordinating transportation of disabled persons to and from the centers as well as in manpower and facility equipment for accepting more SMID persons requiring extremely extensive medical care. It was also highlighted how a newly devised score for assessing the extremely intensive (or extensive) medical care group among SMID persons may not precisely reflect the level of dependence on care and assistance. These medical day-care services are very important for the welfare of SMID persons living at home, and therefore the government should prioritize the establishment of legislation to facilitate reliable and comprehensive day-care services.

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The Kumamoto Pediatric Association conducted a survey by mailing questionnaires to the medical institutions of Kumamoto Prefecture in May 2009. Its goal was to determine the present state of pediatric home health care in Kumamoto and to collect fundamental data for investigating how to support patients with severe motor and intellectual disabilities (SMID) who live at home. We found that the medical institutions that participated in pediatric home health care included 13 hospitals, 3 institutions for SMID and 15 pediatric offices. The number of patients that were supported by medical institutions was 185 in hospitals, 84 in institutions for SMID and 40 in pediatric offices. A total of 309 patients were counted. However, 57 patients visited multiple medical institutions, and therefore, the actual number was 238. For treatment at home, 51 patients used artificial ventilation, 79 used tracheotomy, 124 used oxygen therapy and 163 had tube feeding. Pediatric offices supported half of the patients with artificial ventilation who lived at home. Clinics that supported SMID patients who lived at home were located in urban areas; in rural areas, almost all the patients were supported by hospitals and institutions for SMID. It is expected that pediatric home health care might increase in the future. There is an urgent need to determine how to construct a pediatric home health care system in Kumamoto prefecture using finite medical resources.

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The aim of the present investigation was to clarify social functioning abilities and effects of support in children with disabilities. The subjects were 162 patients aged from 13 to 17 years in Japanese residential institutions for children with physical disabilities. The survey comprised the following seven items indicative of social functioning abilities: decision-making, carrying out daily routine tasks, using communication devices, basic interpersonal interaction, carrying out basic economic transactions, and looking after one's health. The 1st and 4th qualifier presented in the International Classification of Functioning, Disability and Health (ICF) was used to assess performance in the subject's current environment and performance without support. The level of social functioning abilities was highest in (1) the mild or moderate disability group, followed by (2) the severe physical disability group, then (3) the severe intellectual disability group, and finally (4) the severe motor and intellectual disability group. In addition, similar differences in the effects of support were found among the disability groups. The limitations in social functioning abilities and effects of support are not only closely related to intellectual disabilities, but also are associated to some extent with motor disabilities.

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The present investigation assessed the participation of 157 children with disabilities in Japanese residential institutions for children with physical disabilities. The following four participation items were employed in a survey using the International Classification of Functioning, Disability and Heath (ICF): recreation and leisure, acquisition of goods and services, apprenticeship and non-remunerative employment. The first qualifier was used to assess performance of participation in a subject's current environment. All the items were assigned to a scale below 1 (mild difficulty) in more than half the children in both the mild or moderate and the severe physical disability groups. A scale above 2 (moderate difficulty) accounted for more than 70% of children in the severe intellectual disability and severe motor and intellectual disability groups. These results suggest that participation restriction predominantly increases with severity of intellectual disabilities and is also associated to some extent with motor disabilities.

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To provide safe and effective pharmacotherapy for outpatients with epilepsy or developmental disorder, we have configured the "Outpatient medication history" data base (henceforth "Outpatient medication history") that is made of records of adverse effects, individually appropriate packaging form and personal information as well as outpatient's drug history. "Outpatient medication history" dose not have to trust one's memory and enables pharmacists to correspond with needs-oriented dispensing for outpatients. We have continued to understand outpatient's pharmacotherapy and instructed outpatients on pharmaceutical treatment by using "Outpatient medication history", so that the outpatients can conveniently communicate their information to pharmacists and outpatients can consult easily pharmacists for advice effectively. Adverse effect reports have been compiled into the description of medicine. We have been informing the physician of each outpatient's progress record that is constituted with the drug history, characteristic symptom and seizure frequency based on the outpatient's information. Sharing of medical information by "Outpatient medication history" prevents transfer mistakes, insufficient understandings and omissions which might cause any medical incidents. In conclusion, by using "Outpatient medication history", safe medical treatments would be provided for the outpatients.

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There are approximately 600 so-called "mobile SMID" patients such as children (persons) with severe/profound mental retardation and (severe) behavioral disorders, children (persons) with severe mental retardation and physical complications who are able to move by themselves, in the "mobile SMID" wards of nine National Hospital Organization psychiatric hospitals. In this study, we conducted a long-term inpatient survey in the Hizen Psychiatric Center "mobile SMID" ward and analyzed the patient's admission route, the patient's condition and their reason for admission. The results showed that a total of 73% of long-term inpatients had been admitted to our "mobile SMID" ward after difficulties in treatment at other SMID facilities, facilities for the mentally retarded, and psychiatric hospitals. In addition, there were also cases of inpatients coming directly to the Hizen Psychiatric Center from their home without being accepted by another facilities or hospitals. We believe that the "mobile SMID" wards will continue to play a specialist role in the future collaboration with facilities for mentally retarded children (persons) and other psychiatric hospitals as a place where patients can receive (1) developmental age-appropriate professional medical rehabilitation, (2) psychiatric care of emotional/behavioral disorders, and (3) treatment of physical complications.

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We reported clinical courses, treatments and complications in acute respiratory distress syndrome (ADRS) in three patients with severe intellectual and motor disabilities (SMID). One patient had sepsis from pyelonephritis and developed ARDS. The two other patients developed ARDS from respiratory tract infections. All patients were treated with antibiotics, mechanical ventilation and vasoconstrictors (dopamine and dobutamine). Two of the three patients had severe circulatory failure and multiple organ failure. Their conditions improved only slightly with steroid pulse therapy. They also received peritoneal dialysis (PD) for renal failure. After PD, one patient rapidly recovered from renal failure and ARDS. PD facilitated the removal of inflammatory cytokines, which were contributing to the patients' ARDS. One patient showed flaccid paralysis of the extrimities after the onset of ARDS. From the clinical evaluation and neuroelectrophysiological examination, the etiology of paralysis was neuropathy caused by their critical illness. Persons with SMID have a relatively high risk of infection. Therefore we need to be aware of the occurrence of ARDS.

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Severe motor and intellectual disabilities (SMID) secondary to central nervous system damage of any cause during childhood are associated with various complex problems including chronic pyuria and constipation. These problems are often difficult to control. Given that the preventive effects of cranberry juice (CJ) on recurrent urinary tract infection have been reported and CJ, as a source of antioxidants, has been suggested to have various beneficial effects, we hypothesized that CJ may have potential in ameliorating the complex health problems in SMID. The aim of the study was to investigate the possible beneficial effects of 100% CJ on SMID patients. Hospitalized SMID patients (male = 9; female = 4; mean age = 46.0±16.1 years) with chronic pyuria and bacteriuria were enrolled in the study. Cranberry juice (300 ml; 100%) was administered daily for 8 weeks between January 2010 and March 2010. We analyzed the effect of CJ on pyuria, bowel movements, and serum oxidative/antioxidative status. The primary outcome was reduction of pyuria. All 13 patients completed the study. At entry, proteinuria, hematuria, urinary lithiasis were found in 61.5%, 23.0%, and 46.2% of the patients, respectively. Daily ingestion of 100% CJ did not reduce pyuria or bacteriuria during the intervention period of 8 weeks. However, the number of defecations during the study period was significantly increased from the pre-intervention period without increasing the frequency of diarrhea. Serum diacron-reactive oxygen metabolites, as a marker of oxidative stress, were decreased at 8, 12, and 16 weeks after the administration of CJ compared with pre-intervention levels. CJ did not have any effect on serum biological antioxidant potential levels. Adverse events such as weight gain, hyperglycemia, hypercholesterolemia, hypertriglycemia, bleeding tendency, or urinary lithiasis were not observed. The conclusions were that daily ingestion of 300 ml of 100% CJ did not ameliorate pyuria in patients with SMID, however, dietary CJ might be a safe alternative remedy for constipation and high oxidative stress in patients with SMID.

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In order to determine the optimal amount of nutritional support for children (persons) with severe motor and intellectual disabilities (hereinafter referred as "children with SMID"), the amount of energy consumed at rest, resting energy expenditure(REE), was measured using the exhaled gas of persons admitted to this facility for children with SMID. For the group with high muscle tone, REE was significantly higher than the basal energy expenditure (BEE) calculated by Harris-Benedict formula, and for the group with low muscle tone, REE was significantly lower than BEE. There was no significant difference in the group with normal muscle tone. Regarding the amount of nutritional support for children with SMID, the appropriate method is thought to be to multiply REE by the activity index when REE is measured, or multiply BEE by the activity and stress index when REE is not measured, and adjust this amount by watching for body weight change each month. The activity index of children with severe disabilities was determined as 1.0 when bedridden according to Oshima's classification, 1.1 when the patient could sit on his/her own, and 1.2 when there are walking disability, and the stress index was determined as 0.9 with a tracheotomy, and 0.6 when on an artificial respirator all day. Multiplication of 1.0 or more was considered appropriate for persons who have high muscle tone, less than 1.0 for those with lower muscle tone, and 1.1 or more for those capable of oral intake. For children with SMID, the characteristics of the disability vary according to each case, but useful information regarding nutrition prescription could be obtained by indirect calorimetry which measured the actual consumed energy by the individual.

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Patients with severe motor and intellectual disabilities have a high risk of bone fracture and their bones are fragile. One patient who was admitted to our ward had bone fractures of the lower extremities twice in 1 year. These findings suggest that our movement assistance skills are not sufficient to prevent bone fracture. Reexamination of movement assistance skills was carried out by cooperation with individual physical therapists. They demonstrated movement assistance skills that were suitable for the physical status of individual patients, and we recorded these on DVD. The entire staff watched the DVD and acquired and standardized the skills. The experience of to take posture change that caused bone fracture easily is useful to recognize the importance of the skills those were useful to prevent bone fracture.These visual aids to try and implement education about movement assistance skills to prevent bone fracture enabled us to understand these skills precisely and reconfirm these methods easily.

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When a patient with severe motor and intellectual disabilities (SMID) is in the same position for a long time, the temperature of the area supporting the body increases to the point of causing discomfort, and this is thought to result in the loss of posture. The purpose of this study is to obtain basic data regarding this phenomenon. The backs of 18 healthy adult men were rapidly heated by infrared heater. Discomfort was subjectively felt when skin temperature of the back reached an average 37.1°C in winter and 35.5°C in summer. Furthermore some persons felt discomfort at approximately 32°C and could not tolerant approximately 37°C. On the other hand, the average temperature of clothes on the back of 23 patients with SMID taken at different positions were higher in the following order: reclining 36.8°C, supine 36.4°C, and sitting 36.3°C, and according to seat shape: mold type 36.9°C, modular type 36.4°C, and sling type 36.2°C. When a mesh spinal brace was used, the temperature was low at 34.0°C, and during injection through the gastrostomy, the temperature was high at 36.8°C. As the contact surface temperature of the back was higher than axillary temperature at reclining position, mold type seat shape, and gastrostomy injection by scene, considerations such as allowing fresh air to flow over the back are desired when the patient is in the reclining position for a long period of time.

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Thoracic deformity in patients with severe motor and intellectual disabilities (SMID) was measured by CT imaging, and the following two points were considered, 1) Is there any relation between the sevelity of thoracic deformity and of clinical state?, and 2) How does the thoracic deformity change according to the measuring level? 30 patients with SMID (average age 41.1±9.3 years, 17 men and 13 women) were targeted. The severity was classified into two groups, level IV and level V, by Gross Motor Function Classification System-Expanded and Revised-Japanese edition (GMFCS-ER). CT imaging at three different thoracic level were set as the target images. Each parameter, 1) spinal rotation, 2) thoracic rotation, and 3) thoracic flattening, which consists of thoracic deformity, was measured in each image. According to the results, in the group with GMFCS-ER level V, the degree of thoracic deformity is more severe. And for both level IV and V groups, as the measuring level becomes lower, the deformity gets more severe, but it also is suggested that the changing pattern of parameters in each group differs. From this, characteristics of three-dimensional twist of thoracic deformity differ depending on severity, and evaluation of thoracic deformity at multiple level is thought to be important in determining the cause of the clinical symptoms as well.

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An ABAB design was performed using a speaker to play music and vibroacoustic (VA) equipment to provide sound with vibrations, to two children who had disabilities in the perinatal period. One child had severe motor and intellectual disabilities (SMID) and profound deafness conjectured from auditory brainstem responses, and the other had SMID and healthy hearing. Spectrum analysis was performed on heart rate variability while music was played. As a result, we found that using VA equipment to play music tended to produce a greater high frequency, which reflects parasympathetic system activity, before playing music to the child with SMID who has profound deafness, but during and after playing music to the child with SMID who has healthy hearing. These findings suggest that for a child with SMID, it is easier to receive music played with vibration by VA equipment compared with only playing music via the auditory senses. This may promote relaxation as an effect on the function of the parasympathetic system. Periodically and continuously playing music by VA equipment, while considering the physical condition, could also contribute to improving the physical condition of a child with SMID, and enhancing the quality of life.

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Respiratory disorders are closely related to the prognosis of children with severe motor and intellectual disabilities, and affect quality of life. In recent years, intrapulmonary percussive ventilation(IPV) has been used in the medical treatment of acute respiratory infection, and its effects have been reported. However, there are few reports considering the effectiveness of mid- and long-term IPV use during the chronic phase. At this time, we carried out treatment two to four times a month on 13 children with severe motor and intellectual disabilities suffering from severe chronic respiratory disorders, who were admitted to or regularly visited our hospital from May 2008 to May 2011. In seven cases, treatment continued, and in six cases, treatment was discontinued. For the cases where treatment continued, frequency of infection decreased, oxygenation improved,and frequency of suction decreased. Significant improvement was observed in two cases, and these two cases are mainly reported here. Improvement of respiratory function and decreased suctions are considerd to benefit not only the patient ,but also lighten the burden for those assisting the patient.

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Phenobarbital (PB) high dose therapy was carried out for four years on a boy with juvenile Huntington's disease. Onset of the disease was at age five and the patient died at age 16. At age 11, the patient received an operation for volvulus. Due to increased hypertonia and convulsive seizures, multi-drug treatment, including PB high dose therapy was initiated, and respiratory care began at age 12. At around 13 years old, the patient developed bradycardia and low blood pressure during sleep and suffered myoclonic status epilepticus. PB high dose therapy (20mg/kg/day) was intensified and continued, resulting in the suppression of myoclonic seizures, and pulse and blood pressure were stabilized. Although the long-term effects of PB high dose therapy on respiratory function were not clear since respiratory care was required due to advancement of the primary disease, no abnormalities were observed in the blood test except for an increase in γGTP. Furthermore, autonomic function was stable, consciousness could be maintained at a level where communication was possible, and no significant adverse effects were observed. In this case, to achieve suppression of myoclonic seizures, blood level had to be maintained between 120 – 150μg/ml. Long-term administration of non-intravenous PB high dose therapy is considered to be a potential supplementary measure for refractory convulsions and involuntary movement caused by serious neurodegenerative diseases.

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We reported a case who had abnormal calcification in the pelvic floor since 2004 which were confirmed vaginal stones upon close examination carried out to investigate a shape of an artifact being displayed in 2010 x-ray examination. The patient was a 42 year old female. Tonic quadriplegia was exhibited as a result of her severe motor and intellectual disabilities since the age of two and she had been bedridden. Regular tetragon-shaped abnormal calcification was observed in x-ray of the abdominal and pelvic region and diagnosed as a foreign body in the vagina with ultrasound and CT scans. The removal of the vaginal foreign body was performed in the Obstetrics and Gynecology Department. The foreign body was diagnosed as an ammonium magnesium phosphate stone upon analysis. No fistula such as vesicovaginal fistula seen in the healthy patients with vaginal stones was observed and the long-term discharge of urine into the vagina resulting from the patient's bedridden state was thought to be the mechanism.

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A survey interview of the "thoughts" and "life" of a mother for 11 years after the loss of her child with severe motor and intellectual disabilities was carried out. As a result, the following points could be ascertained: 1) The mother experienced unstable conditions for 10 years, and depression for three years. 2) Three years after the death of her child, she started to work as a caregiver and participated in volunteer activities, but could not recover. 3) After the death of her child, she began to think of herself separate from her husband. 4) She began to think about her own future. 5) She decided to live happily with her husband. 6) The death of her child was becoming a past event. 7) She began to enjoy life with her husband. 8) Although interaction with former associates (mothers) became rare, increased interaction with neighbors did not occur. Although the mother made efforts to recover from the grief of losing her child, it was not easy and took her a long 10 years. The need to support a mother after losing a child is indicated.

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Mild zinc deficiency is a well-known disorder that affects the sense of taste; however, management of this disorder in patients with severe motor and intellectual disabilities (SMID) is difficult since these patients cannot talk and may not be able to report the decrease in taste sensitivity. In this study, polaprezinc (zinc content: 34 mg/g) was administered for zinc supplementation to more than 100 patients with SMID. Polaprezinc administration was found to increase the zinc levels and decrease the recurrence of infection. Further, polaprezinc administration prevented the recurrence of infection in 3 patients with SMID, one of whom showed a decreased serum copper level. In patients with SMID, recurrent infection is suspected to be a symptom of zinc deficiency, and this deficiency could be the contributing factor to the development of recurrent infections. Zinc supplementation with polaprezinc is a useful method for treating zinc deficiency. In the Dietary Reference Intake 2010 recommendations, the estimated average requirement of zinc is 10 mg, but this amount is not adequate for patients with SMID. Further, SMID patients may show decrease in serum copper levels, and therefore, monitoring of serum copper level is necessary.

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The purpose of this study is to consider fluctuations in autonomic functions of children (persons) with severe motor and intellectual disabilities (SMID) in one day, and to assess the response to stimulation and events occurring during sensory activities, by heart rate variability analysis. Two subjects with Oshima’s classification of Class 1, and who were admitted to a medical facility were targeted. Data measurement was comprised of electrocardiogram and video recordings during sensory activities once a day for two days, and heart rate variability analysis as well as stimulation and events occurring during sensory activities were analyzed and examined. As a result, in a day with stable physical conditions, a higher value of high-frequency component power (HF) was observed at night than during the day. Therefore, parasympathetic nerve function of many patients with SMID is considered to be dominant at night when physical conditions are stable. Furthermore, from the characteristic changes in heart rate variability, stimulation and events occurring during sensory activities could be identified into three patterns: (1) sympathetic nerve activity increases due to uncomfortable stimulations/events or enjoyable stimulations/events, (2) parasympathetic nerve activity increases due to comfortable stimulations/events, and (3) both parasympathetic and sympathetic nerve activities simultaneously increase when comfortable stimulations/events coincide with enjoyable stimulations/events and the discontinuation of comfortable stimulations/events.

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