[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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[in Japanese]

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Purpose: This study aimed to reveal the mothers' experiences of transitional medicine for their young and adult children having rare diseases. Methods: The date obtained from interviews with twenty-three mothers belong to society of the patients and the families were qualitatively and inductively analyzed. Results: The mothers' experiences of transitional medicine from pediatric to adult health care for their children with rare diseases were classified into seven categories. The mothers group following the transitional medicine showed [reluctance to accept the transition of medicine] at the initial stage of transition, and the mothers not following the transition to adult health care had [an understanding that the children could receive the pediatrician's treatment throughout the children's life]. On the other hand, the mothers showed [reluctance to accept the transition of medicine], but felt relieved to know that [the cure and the care given by the pediatrician remained unchanged] except for the doctor. These mothers recognized the changes of their children's social life, so that they changed their mind [shift from treatment of children to caring for children], and always expected to get [the latest medical information and the opportunities to receive the latest treatment]. Conclusion: These parents were afraid that their children might be short-lived, so that they have not taken measures against adult-onset diseases for their children. In consideration of this, it is necessary to prepare the adult health care system that can provide proper care and cure for the child-onset rare diseases in compliance with wishes of the patients and their families, and offer useful information about adult health management to those patients with child-onset rare diseases after they come of age.

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The purpose of this study was to reveal visual functions of children (patients) with Rett syndrome as well as current circumstances regarding utilization of gaze in rehabilitation and education for these children (patients). In the year 2016, we conducted a questionnaire survey using postal services in a total of 1,220 institutions with principals of 1,016 schools for special needs and managers of rehabilitation departments of 130 long-term medical care facilities for disabled children, 73 wards for patients with severe motor and intellectual disabilities in the national hospital organization, and one facility of national center of neurology and psychiatry. Information of a total of 216 children (patients) with Rett syndrome (ages between 3-53 years old, Yokochi's classification A1-B6, C6, D6 and E6) was obtained. Refractive anomaly, squint or nystagmus was not detected in 79 % of the cases and ability of gazing at and tracking an object was observed in 80%. Gaze had been utilized in rehabilitation and education in 60% of the cases, and the purposes of utilization the most commonly reported were to provide opportunities to present their intentions through selection of toys, cards or activities and to encourage them to gaze at and track an object, etc. The results of this survey suggested that visual functions of children (patients) with Rett syndrome were satisfactory and that there were needs of visual function assessments followed by interventions according to individual functions.

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There are quite a few children (individuals) with severe motor and intellectual disabilities (SMID) who are transferred to a critical care medical center during respite care, although there is recently an increasing need for respite care services provided at facilities for children (individuals) with SMID. Quality of Life (QOL) of facility users decreases due to worsening of physical conditions; the purpose of this study was to reveal backgrounds of the users in association with worsening of physical conditions during respite care as well as whether or not the changes in heart rate could be indexes for worsening of physical conditions. The subjects included 14 patients (17 cases) who were transferred to a critical care medical center during respite care at our facility within two years from January 2017, and worsening of physical conditions was investigated on their medical records. According to the results, physical conditions were medically or sub-medically dependent in all these patients, and 12 patients (15 cases) were under 18 years of age. Either dysphagia, irregular breathing, or history of apnea was detected in all patients. Meanwhile, the change in heart rate at the time of worsening physical conditions varied among these patients. These findings suggested that brainstem dysfunction and autonomic nerve dysfunction had been associated with their physical conditions in the context of young patients with medically or sub-medically dependent conditions. There was however no consistent tendency observed in heart rate change, suggesting that change in heart rate is not necessarily an index for worsening of physical conditions.

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The purpose of this study was to identify the process through which mothers of disabled children adapt to their child's disability while they engage in rehabilitative care. Thus, semi-structured interviews were conducted with 11 mothers of children with severe motor and intellectual disabilities (SMID), and their verbatim statements were analyzed using the Modified Grounded Theory Approach. The mothers appeared to repeatedly grapple with the question of how they should engage during their child's rehabilitation, and experienced conflict in reconciling their fatigue with their desire to improve the child's capacities. At the same time, the mothers came across people who accepted their child as he/she is, started to recognize the dignity and courage of their child, and adopted a defiant attitude to societal pressure. This helped the mothers recognize that their values and perspective were changing and they could internalize their own emotional stance. While the mothers appeared to struggle initially with the dichotomy of striving to improve the child's capacities and abandoning any such effort, their attitude eventually changed as they adopted a more stable approach, wherein they kept encouraging their child while maintaining their own equilibrium. In light of these findings, rehabilitation experts should bear in mind that a mother's acceptance of their child's disability constantly fluctuates as she undergoes a trial-and-error process.

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In recent years, advances in medical technology in the field of pediatrics and the promotion of home medical care have led to the transition to home care for children who are highly dependent on medical care, such as the use of ventilators. It is important for children to live in home and in the community from the viewpoint of their growth and development and QOL. However, families raising children requiring medical care at home are saddled with mental, physical, social, economic, and time burdens, such as providing not only care for severe complex disorders and their complications but also advanced medical care at home. According to a survey of children with severe motor and intellectual disabilities, 97% of medical care such as suction and tube feeding is provided by family members, and 93% of these are done by mothers. Under these circumstances, mothers who raise children requiring medical care at home are living under around-the-clock pressure due to medical care and child-rearing, as well as oppressive feeling and childcare anxiety, complicated thoughts, and stress, but no specific details of such stress have been clarified. The purpose of this study was to clarify what kind of child-rearing stress mothers who raise children requiring medical care at home have.

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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 reduces the risk of disconnections between medical devices for different medical applications. This prevents medical mistakes and ensures a stable supply of products through international coordination. Healthcare workers who provide care to patients with severe motor and intellectual disabilities (SMID) use enteric tubes not only for feeding but also for administering medications and venting multiple times a day. The syringes are attached to and detached from the connector so frequently that clinicians have concerns as to whether these new connectors will be able to meet the needs of populations with SMID. New connectors have a screw thread design, whereas the existing connectors have funnel-type designs that are easy to attach and detach. Thus, we examined the number of attachment/detachment actions performed in the management 156 patients with SMID by 140 clinicians during the dayshift at 3 facilities. The median number of attachment/detachment actions needed for a patient with SMID was 11, the median number of attachment/detachment actions that a healthcare worker performed was 32, and the average number of patients that a healthcare worker was responsible for was 2.86. Moreover, the maximum values for these parameters were 65, 187, and 9, respectively, due to implementation of blenderized tube feeding and the contents of medication. Transitioning to new connectors may cost time and impose a physical burden on clinicians.

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Previous trials have measured the flow rate and force of new connectors (International Organization for Standardization [ISO] 80369-3 compliant connectors) versus existing connectors (legacy feeding devices); however, we were not able to find data on time-lag and differences in the subjective usability by caregivers or the burden of syringe feeding. Thus, we performed a trial of syringe feeding using a RACOL-NF (RACOL-NF Semi-Solid for Enteral Use). The study population included 40 clinicians at 5 facilities. We measured the time clinicians spent on all steps of feeding 300 kcal-RACOL-NF with both the existing and new devices, and asked them to evaluate the subjective usability and burden using a visual analogue scale (VAS). To analyze the subjective burden, we divided feeding actions into 4 steps (filling [1st], attaching the syringe to the connector [2nd], administration [3rd], and detaching the syringe from the connector [4th]) for existing devices. For new devices included 2 additional steps (attachment and detachment of the adaptor for semi-solid nutrient filling). The median time for all feeding steps (all values shown as existing vs. new) was 282 s vs. 365 s (increased by 1.30±0.20 times, p<0.01). The median VAS value for usability was 91.5 mm vs. 42.0 mm (p<0.01). The median VAS values for the burden of using the respective devices were as follows: 1st: 20.5 mm vs. 50.0mm (p<0.01); 2nd: 6.0 mm vs. 67.5mm (p<0.01); 3rd: 14.5 mm vs. 20.0 mm (p<0.05); 4th: 7.5 mm vs. 68.0 mm (p<0.01); total: 16.5 mm vs. 66.0 mm (p<0.01), respectively. Limited to the burden of the new devices, the VAS values of the 4th and 2nd steps were higher, while the VAS value of the 3rd step was lower. Transitioning to new connectors may cost time and impose a burden on clinicians.

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Due to the mandatory transition from the existing connectors (legacy feeding devices) to new connectors (International Organization for Standardization 80369-3) from December 2019 to November 2021, we surveyed the characteristics of facilities' inpatients with severe motor and intellectual disabilities (SMID) who required tube feeding, and the physical burden on clinicians' wrists using the existing connectors. We sent 2 types of questionnaire to the chief managers of 39 facilities in western Japan by e-mail: one for the chief manager, inquiring about the characteristics of SMID inpatients who required tube feeding, and another for clinicians concerning the physical burden on their wrists. We received responses from 29 chief managers (response rate: 76.3%) and 1,082 clinicians out of 2,039 total clinicians in 29 facilities (response rate: 53.1%). The surveyed inpatients included 252 (21%) under 20 years old, 402 (34%) receiving blenderized tube feeding, and 304 (26%) requiring venting with syringes, resulting in a total of 1,194 inpatients with SMID who required tube feeding. These characteristics were found to be factors increasing the number of attachment/detachment actions performed in our previous research. Regarding clinicians' burden, 538 (50%) had experienced wrist pain with syringe feeding, and 20% of these (110 clinicians) had consulted the doctor for their wrist pain. Administration in 412 (77%) and filling in 335 (62%) were the top actions with which clinicians felt wrist pain during syringe feeding. The existing connectors have funnel-type designs that are easy to attach and detach, whereas the new connectors have a screw lock design that are hard to attach and detach. Thus, transitioning to new connectors may impose more physical burden on clinicians.

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Patients with severe motor and intellectual disabilities (SMID) have a high risk of thrombosis due to long-term bed rest and paralysis of the lower limbs. However, sometimes it is difficult to diagnose thrombosis for patients with SMID, because they cannot complain of their symptoms properly. We experienced a case of SMID with treatment-resistant edema, and we diagnosed the patient with pulmonary thromboembolism (PTE). The patient is a 33-year-old woman, Oshima classification 1, Yokochi classification A1. When she had aspiration pneumonia, systemic edema was observed on her body. The systemic edema was treatment resistant, even after the recovering of aspiration pneumonia. Three months after the onset, we performed detailed examination for her edema, and finally diagnosed PTE. The cause of edema was right heart dysfunction due to PTE. Because her symptoms except edema were scarce, the diagnosis was difficult. However, D-dimer blood level and echocardiographic findings of right heart load were useful for diagnosis. The patient had multiple risk factors for thrombosis, including central venous catheter placement, antiphospholipid antibody syndrome, and use of dysmenorrhea therapeutic medicine. Most of patients with SMID, like this patient, are at high risk of PTE. PTE does not show specific symptoms and may be difficult to diagnose. While taking PTE into consideration, we should carefully examine patients with SMID.

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We report a case of 53-year-old man with secondary tracheomalacia successfully treated by tracheal resection and reconstruction. He suffered a traumatic brain injury and had a tracheostomy from the age of 2 years to 8 years. At 51 years, he had repeated dying spells, exemplified by choking with cyanosis. We diagnosed secondary tracheomalacia using rigid bronchoscopy. The lesion was limited to the second and third tracheal cartilages. We performed tracheal resection and reconstruction via a cervical approach with a collar incision of the neck. The sternum and the innominate artery lay at the lesion's caudal side; therefore, we did not interfere with those structures. Annular excision and end-to-end anastomosis of the first and fourth tracheal cartilages were performed. He had been under sedation for 5 days, and 6 days after the operation, his endotracheal tube was removed. The postoperative course was uneventful, and his dying spell disappeared. Tracheal resection and reconstruction are useful in cases of secondary tracheomalacia, especially in cases in which the lesion is limited to the upper trachea.

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As the number of disabled children with severe respiratory disorder under home-based care increased, we launched a multidisciplinary outpatient clinic for respiratory-rehabilitation (resp-rehab) in 2000 for the purpose of improving patients' QOL. The results of this study which revealed psychological conditions of patients' family members with whom we shared this pioneering attempt would be crucial when we establish a future approach. The purpose of this study was (1) to understand psychological conditions of family members who had been living with their children with respiratory disorder while providing care as instructed, (2) to know their feelings that they would like to share with medical professionals as well as the next generation, and (3) to reveal what we would need to consider in order to continue the resp-rehab clinic. We conducted semi-structured interviews with 4 families which lost their children and performed a verbatim qualitative-inductive analysis. We extracted three categories "anxiety regarding information provided","distress due to various restrictions" and "actual feelings and current thoughts regarding difficulties that they confronted at that time" along with 9 subcategories. Then we clarified the issues on the method, timing and setting when we provide medical information. Furthermore, feelings of the patients and families which were not likely to be realized by medical professionals such as that "feeling lonely when returning the ventilator to the agent, as if it was a part of the patient" were also extracted. The results of this study suggested that we would need to establish a setting where medical professionals could adequately understand social psychological conditions involved with the patients, and to incorporate novel including videos while providing information to the patients, their families and supporters.

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There, in the first stage of this study, we administered a smoking status questionnaire survey to our company staff and devised measures for improving not only the future health of our staff but also the environment of inpatients by establishing smoking cessation campaigns, anti-smoking movements, and smoking cessation outpatient services. As the result, the smoking rate of our staff dropped from 22.7% to 13.3% over ten years. While the rates in women in particular have decreased steadily, a recent survey showed that the decline in smoking rate among men has not showed down. In addition to the measures mentioned above, currently, smoking-related financial assistance is being provided. However, the effects are still insufficient and future measures should be planned in the future.

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Severe motor and intellectual disabilities (SMID) need a medical care for a long time. Especially for epilepsy therapy, antiepileptic drugs therapy is taken for a long time. A diagram of clinical course with medical treatment is highly useful for evaluating the clinical effects for a long treatment. Drawing the diagram requires a great effort and time. Therefore, we developed software that transfers numerical data of electronic medical record to Excel in a short time. We measured time to transfer numerical data of electronic medical record to Excel in 2 and 5 years for 12 patients using this software. It took an average of 124.8 seconds for 2 years and 168.5 seconds for 5 years. It took up to about 10 minutes to draw the diagram from the start of Tetrapod. In conclusion, Tetrapod can draw a diagram of clinical course with medical care in a short time, and it will be useful for deciding the treatment plan of SMID with epilepsy.

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Current situations of Kanagawa Prefectural Special Support Schools which receive students requiring significant care at home were speculated based on analyses made on near miss accidents reported from the schools and counted at the department of special support education of Kanagawa prefecture, followed by discussion made on medical care. According to the results, it was revealed that medical care had been provided mainly by teachers in charge at these schools; accidents often occurred at lunch time; the most commonly reported accidents were associated with infusion; and the primary concern was whether or not the accidents were handled according to the manuals. However, it was also revealed that near miss accidents not associated with medical care were not reported to the prefecture and that only a few near miss cases occurred at the schools had been counted at the department of special support education. The special support schools receive various types of students who require medical care in their daily lives. However, there is no medical professional who can capture the whole picture of the situations at these schools. As the number of students requiring medical care has been increasing and the types of medical care provided at schools have been more complicated, an investigation should be made on medical care provided at these special support schools. It was considered that the investigation should be started by sharing problematic issues with medical professionals.

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[in Japanese]

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[in Japanese]

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