In order to reveal Quality of Life (QOL) and relevant factors for parents who are involved the most in the home care of their children with severe motor and intellectual disabilities (SMID) and for their partners, we distributed a questionnaire form with questions associated with QOL and relevant factors for both mothers and fathers (the parents who are involved the most in the home care of their children with SMID and of their partners) and received responses from 530 cases. About 90% of the parents who are in charge of their children's care were mothers. Many of them work part-time rather than full-time, or they are unemployed. They sleep less, wake up more frequently during night time, feel more burden of care for their children, and have lower QOL. For both the parents who are involved the most in children's care and their partners, the less frequently they wake up during night time, the higher the level of their physical health status. In addition, the less they feel burden of care and the more they are employed, the higher the level of their mental health status. These findings suggest that the strategy to support them would include helping them have an adequate amount of sleep and quality lifestyles in terms of eating and doing exercise to maintain the physical health status and providing advice for relieving the burden of care and for being employed to maintain the mental health status. The factors relevant to QOL of both the parents who are involved the most in their children's care and their partners included variables associated with themselves as well as with their families including their partners and children. This suggests that it is important to provide support for the entire families including the partners, in addition to the intense support for the parents who are involved the most in their children's care.
We developed a fracture risk assessment scale with which facture risk for children (adults) with severe motor and intellectual disabilities (SMID) can be predicted. A significance test was conducted in patients with fractures and patients without fractures for factors which might be associated with fractures observed at our hospital. According to the results, the following 7 factors were extracted as the fracture risk factors: Age of 50 or older, Female patients aged 50 or older, Mobility function levels 2, 3 and 4 of Yokochi classification, Intellectual development levels B and C of Yokochi classification, Past history of having a fracture, Oral administration of enzyme-induced antiepileptic drugs, and Bone density of 40% or less. We distributed scores of 1-3 to these risk factors according to the level of incidence of fractures associated with these factors and then developed a fracture assessment scale with which we could predict presence or absence of fracture risks from the total score of each one of the risk factors. Consequently, we were able to predict that a patient with a total score of 6 or above for these factors might have the risk of fracture. In a hospital with high percentage of such children (adults) with SMID as in our case, it is beneficial to use this scale to prevent fracture as it enables staff members of the team to make standardized evaluations on users, leading to sharing of users' information and improvement in a sense of professionalism.
There are still few day service such as after school of children with severe motor and intellectual disabilities (SMID), and there are many cases that it is not clarified such as ingenuity and consideration to accept children with SMID. Therefore, in order to clarify the actual situation and examine the necessary support, we carried out anonymous self-written questionnaire survey to managers / function training officials / guardians. The subjects were day service places, such as after school for 202 children with SMID, 91 cases collected, 79 persons in charge of function training, and 128 cases for parents. According to the survey of the administrator, it is clear that the balance of day service such as after school of children with SMID is severe, and from the survey of parents, the fact that there is no day service such as after school where medical care children can use is an issue, the severity of income was considered as a factor. Since the Ministry of Health, Labor and Welfare sets goals, it seems that day service such as after school for children with SMID will increase, but because the current system has severe management, business establishment. It is necessary to review the remuneration system that can survive.
Active management for hyperglycemia is crucial for disabled children (adults) who are likely to develop abnormalities in blood glucose fluctuation along with hyperglycemia. In this study, we conducted a retrospective investigation on presence or absence of abnormalities associated with blood glucose fluctuation as well as blood glucose level using a portable blood glucose meter in 35 disabled children (adults) aged between 7 and 55 years (23 males and 12 females with the median age of 21 years) who had been taken care at Facility A. Abnormal values were observed in association with blood glucose in 2 out of 7 subjects in the oral-intake group but not for the results obtained with the portable blood glucose meter. While in the enteral nutrition group, abnormalities were detected for blood-glucose related values in 13 out of 28 subjects (46.4%) and for values obtained with the portable blood glucose meter in 4 subjects (14.3%). Moreover, in 2 subjects with hyperglycemia who had been receiving enteral nutrition, the blood glucose fluctuation range was reduced with low GI/GL liquid diet or low GL liquid diet. It was suggested that, for disabled children (adults), presence of blood-glucose fluctuation abnormalities should be suspected, and it would be crucial to obtain active diagnoses and provide interventions in these patients.
The objective of this study was to reveal factors affecting subjective QOL in mothers of children (adults) with severe motor and intellectual disabilities (SMID) from both positive and negative aspects according to the life stage of their children as well as to obtain suggestions on how to support these mothers. The subjects included 6 mothers who had been living with children (adults) with SMID after adolescence. We conducted semi-structured interviews using the life-line interview method. The subjective QOL of mothers tend to be the lowest after being notified of their children's disabilities or receiving diagnose of their diseases. Thereafter, their QOL repeatedly went up and down; some mothers attempted to move forward by positively accepting the past events while others went into depression. The factors which improved their subjective QOL included realization of development of their children, social connections, regaining their social roles, and supports from families. While factors which lowered their subjective QOL included worsening of their children's health status, making a decision on a treatment for the secondary disability, the burden of "double care" of both parents and children, and decreased caring ability of the mothers themselves. These factors revealed perspectives on approaches to help mothers recovering from difficult situations at each life-stage of their children.
In this study, we identified the causes of swallowing disorder objectively by performing quantitative analyses using videofluoroscopic (VF) examination of swallowing animation in patients with severe motor and intellectual disabilities (SMID). The subjects were 23 healthy adults (healthy group), 10 patients with SMID (disability group Safe) who did not have aspiration and residual bolus in the pharynx after swallowing (residual after swallowing), and 13 patients with SMID (disability group Dysphagia) who had aspiration or residual after swallowing. We recorded a video using the VF as they swallowed 3–5 ml of paste food. Using the VF recordings, we measured the dwell time of the bolus at the pharynx before swallowing, time of the movement of the swallow-related organ, and movement distance of the hyoid bone on the coordinate plane with the second and fourth cervical spines as a reference line. We found that both the disability groups Safe and Dysphagia had smaller hyoid bone movement distances than the healthy group. In the disability group Dysphagia, the hyoid bone elevation started after the bolus reached the deep part compared to that in the healthy group. Therefore, a high proportion of patients with SMID exhibit problematic swallowing movement, and it becomes a serious swallowing disorder when combined with the problem of the perception of swallowing.
The objective of this study is to reveal actual attempts to reduce stereotypic movements of hands observed in children (persons) with Rett syndrome. A questionnaire survey was conducted nationwide in 2016 using postal service in subjects of a total of 1,220 institutions including principals of 1,016 special support schools as well as persons in charge of rehabilitation division of 130 residential healthcare facilities for disabled children, 73 wards for children with severe motor and intellectual disabilities at independent institutions of national hospital organization, and 1 facility of National Center of Neurology and Psychiatry. As a consequence, information of a total of 216 children (persons) (aged 3-53 years, Yokochi classification A1-E6) with Rett syndrome was obtained. Attempts were made to reduce stereotypic movements of hands in 104 subjects, while in other 104 subjects, no attempt was made. There was no difference in terms of conditions of both groups. The attempts included more efforts to encourage using hands rather than to suppress stereotypic movements. For children (persons) of Yokochi classification A1-A6, sensory stimulation was used in majority of the attempts. Various attempts had been made according to individual subjects; further investigation will be needed to reveal what kinds of attempts would be effective for what types of children (persons).
Characteristics of epilepsy including underlying diseases, complication with epileptic encephalopathy, seizure types and epilepsy classifications were investigated in our 81 residents with severe motor and intellectual disabilities (young and elderly adults) of 20 years of age or older, who had developed epilepsy in their childhood. The subjects were divided into two groups (the frequent-attack group and non-frequent attack group). The frequent-attack group included more patients with prenatal etiology as well as those complicated with epileptic encephalopathy compared to the non-frequent attack group. The commonest seizure type was myoclonus, and many of the patients had experienced multiple types of seizures in various combinations (e.g. “tonic + clonic", “tonic + myoclonus", “clonic + myoclonus", “tonic + focal-motor", “myoclonus + focal-motor", “focal-motor + from focal to bilateral tonic-clonic"). In terms of epilepsy classification, many patients appeared to have both generalized and focal seizures. Old anti-epileptic drugs had been mainly used in both groups. Many of the patients in both groups had been treated with hepatic enzyme-inducing dugs (EIAED: enzyme-inducing anti-epileptic drugs). In the group using EIAED, the serumγ-GTP or ALP level was higher and more patients had history of fractures compared to the other group not treated with EIAPD.
In order to recognize issues on epilepsy managements for institutionalized persons with severe motor and intellectual disabilities (SMID), a questionnaire survey was performed in 29 institutions for SMID in Kinki area. The answers were sent back from 24 institutions housing 2602 people with SMID in total (1450 males, 1152 females). Those under 20 years old were counted 354 (13.6%). Eighteen hundred and one (69.2%) had a complication of epilepsy. Among those under 20 years old the comorbidity rate of epilepsy was 77.1%, significantly higher than among the adults. One attack and more in a month were seen in 39.6% and 36.5% took three antiepileptic drugs or more. VTR EEGs were studied in 7 out of 24 institutions, although ordinary EEGs were recorded in all but one. Full time pediatric neurologists worked for 18 institutions, but EEG technicians were positioned in 19 institutions, adult neurologists and/or neurosurgeons in 7, epileptologists in 6, respectively. Epilepsy is quite common and hard to control among persons with SMID. Newly marketed antiepileptic drugs and diagnostic tools are needed to be introduced much more in addition to medical professionals trained in epilepsy management.
Neurogenic bladder is frequently recognized as a common comorbidity in patients with severe motor and intellectual disabilities (SMID). The residual urine volume retained in the neurogenic bladder often amounts to the several hundred milliliters. Before the present study, we experienced a rare case of spontaneous rupture of the bladder due to the excessive urinary retention by the neurogenic bladder. To prevent this critical complication, an ultrasound scanning device (Bladder Scan BVI6100), which enables us to measure the urine volume of the bladder by a non-invasive manner, was newly introduced into our residential hospital. We use this device according to the pre-determined criteria. In the present study, we examined the usability of this device to the patients with neurogenic bladder, by regular measuring the urine volume and appropriate catheterization in the excessive urinary retention as needed. As the consequence of the use of this scanning device, the incidence of urinary tract infection including pyelonephritis was prominently decreased, resulting in the reduction of the antibiotic administration. We suggest that the measurement of the residual urine volume by scanning bladder ultrasound is useful for neurogenic bladder to prevent the serious complication such as the rupture of a bladder and systemic urinary tract infection.