[in Japanese]

View full abstract

We conducted an experiment to determine whether the emotions of individuals with severe motor and intellectual disabilities (SMID) can be assessed using the blink indices. The participants included four individuals with SMID, hospitalized in the ward. Pleasant and unpleasant environmental sound sequences were created and presented continuously on the bed of the participants in the hospital room. Blinks were recorded with a micro video camera. The blink rate was higher under unpleasant conditions than under the baseline and pleasant conditions. In addition, the blink rate under favorable conditions tended to decrease compared to baseline conditions. From these results, it is suggested that blinking may be an index that reflects the emotions of individuals with SMID. However, since the blink rate of individuals with SMID tends to be lower than the average blink rate of people with typical development, and individual differences are large, it is important to assess the baseline and emotional movement of each individual with SMID.

View full abstract

There are very few studies reporting the safety and efficacy of the novel coronavirusdisease 2019 (COVID-19) vaccine in patients with severe motor and intellectualdisabilities (SMID). This has led to anxiety within the families of these patients. We conducted a prospective study of the adverse effects of the COVID-19 vaccination in 124 patients with SMID (13 to 85 years old) who were vaccinated twice at our facility. The levels of SARS-CoV-2 spike proteins and immunoglobulin G antibodies were determined in 30 of them. Systemic adverse reactions such as fever and local reactions were more prominent after the second inoculation compared to after the first one. No cases of death or anaphylaxis after vaccination were recorded, indicating that the vaccine was safe. However, careful observation of changes in physical condition after vaccination was necessary. The antibody titer against spike protein considerably increased after two vaccinations, with a more notable increase observed with in the younger age group (10 to 20s). Thus, this study confirmed the safety and efficacy of COVID-19 vaccines for patients with SMID.

View full abstract

We report our experience with 2 cases in which 2 children with severe motor and intellectual disabilities had complicating chronic hepatitis C that was successfully treated with direct antiviral drug. In Case 1, the patient's condition had progressed to hepatic cirrhosis. As antiseizure drugs are contraindicated or should be administered with caution when co-administered with antiviral drugs for hepatitis C, medication change was performed in both the patients. In Case 1, when the drug was changed, the patient had status epilepticus, which made the condition refractory to the treatment. Besides, the patient had ascites, which required a long time to resolve even after the treatment. In both the patients, sustained virological response was achieved without occurrence of adverse reaction. As hepatitis C frequently progresses to hepatic cirrhosis or hepatic cancer, it was considered necessary to consider initiation of the treatment in appropriate timing to prevent occurrence of cancer and hepatic disease-related death. Achievement of virological response led to removal of restrictions to living activities, which enables infection prevention of these patients to be implemented within the basic infection prevention measures. In these patients, opportunity to be infected with hepatitis C virus could be removed, which gave facility staff a sense of security.

View full abstract

A case of a 29-year-old male with cerebral palsy, congenital aplasia of the right kidney, and rare chromosomal abnormalities. The patient suffered from a persistent fever which did not respond to antimicrobial agents, followed by urinary retention on Day 12 of the fever. Increased white blood cell count, elevated CRP, and decreased renal function was observed by blood test. An enlarged left renal pelvis and cloudy suspended particles in the left renal pelvis, and a mass with equiluminance in the left ureter were observed by abdominal ultrasound. In addition, an obstruction in the ureter at a site consistent with the aforementioned mass was observed by contrast-enhanced CT scan. Urinary retention could be alleviated by nephrostomy and numerous white floating particles were observed in the urine. C. albicans was detected by blood and urine culture tests, and the white suspended particles in the urine was considered to be urinary tract mycetoma caused by C. albicans. in cases of delayed diagnosis, fungal urinary tract infections can form mycetoma, leading to urinary tract obstruction and acute kidney injury. Consequently, prompt diagnosis is needed. For persons (children) with severe mental and/or physical disabilities with a history of recurrent urinary tract infections, there is a risk of mycotic urinary tract infection. In cases of fever, mycotic urinary tract infection should be listed as a differential diagnosis, and a general urinalysis, Gram stain, and bacteriological examination should be performed.

View full abstract

In order to clarify the decision-making process of the primary caregiver when introducing multiple types of medical care which are progressively needed in the various stages of the growth process of a child with severe motor and intellectual disabilities, a case study was carried out targeting one case. As a result, 6 concepts were extracted, namely “Accepting what one is told without understanding,” “Accepting alternative suggestions due to a fear of treatment,” “Recognizing changes in the child, and actively requesting treatment,” “Recognizing changes in the child, and accepting the inevitable,” “Having feelings of conflict while recognizing the need,” “Change from being someone else's problem to being one's own problem.” Decision-making was influenced by the presence/absence of someone to consult, with/without knowledge, access to positive information through interaction with other parents of severely ill children, and changes in the physical condition of the severely ill children experienced in one's daily life. Reasons for the continued conflict and lack of action included: Aware of changes in physical condition but the conditions do not seem urgent, doctors only suggesting treatment when urgent and leaving the decision to the parents, resistance to the medical care needed, resistance to proxy decision-making, and receiving both positive and negative information from other parents of severely ill children.

View full abstract

We encountered a case of valproic acid (VPA)-induced Fanconi syndrome (VFS) in a child with severe motor and intellectual disabilities (hereinafter referred to as a child with severe disabilities) that required a long time for the diagnosis to be made and resulted in two episodes of fracture. The patient was a three-year-old male child who started to orally receive VPA at the age of seven months for cerebral palsy and West syndrome. Four months before the presentation to our hospital, pyrexia and right femur fracture of unknown cause occurred. As pyrexia of unknown cause repeatedly occurred even after that, the patient presented to our hospital. Left femur fracture and hypophosphatemia were also identified, and based on additional urine test result, Fanconi syndrome was diagnosed. After tapering and completion of VPA administration, no recurrence of pyrexia or newly formed fracture was observed and laboratory test findings improved, and therefore, VFS was diagnosed. In the present case, if early diagnosis had been made based on the presence of pyrexia and fracture, the second fracture might have been prevented. However, it is not always easy to make a diagnosis of VFS in children with severe disabilities unless VFS is suspected. Among previously reported cases of VFS in children with severe disabilities, there is a certain number of cases suggesting that it took a long time from the onset of symptoms to the diagnosis. In children with severe disabilities who orally receive VPA, VFS should be thought of at the time point when pyrexia or fracture of unknown cause is identified.

View full abstract

Where a home caregiver who provides care to a child with severe motor and intellectual disabilities (SMID) is infected with novel coronavirus (coronavirus) and cannot provide relevant care, who should provide care including medical care to the child, who is identified as having close contact to the infected person, has become a significant issue. We had a difficulty in handling a patient who was found to have had close contact to a coronavirus infected person after being admitted to our center for short stay (SS) and thus herein report this case. A patient was a 55-year-old male with SMID. The patient had cerebral palsy with epilepsy secondary to severe asphyxia and was receiving care at home. As the patient's father aged 83 years (dementia) and mother felt sick, emergency SS was requested. After admission to our center, the patient was identified as having close contact to a coronavirus infected person because a home-care worker was found to be infected with coronavirus. After that, the patient's mother was found to have coronavirus pneumonia. We asked the administrative agency and public health center of Osaka City for advice and requested them for transfer of the patient to medical institution, but the transfer was difficult because the patient' s PCR test was negative. We used a private room with negative-pressure equipment in our center and set up temporary zoning to continue monitoring of the patient. Eventually, isolation was released after 11-day isolation, and the patient was uneventfully discharged. The handling of similar cases under the coronavirus crisis should be discussed with the administrative agency in the future.

View full abstract

Respiratory failure occurs due to various causes in patients with severe motor and intellectual disabilities (SMID). Our patient developed dyspnea and expiratory wheezing at night. Treatment for bronchial asthma temporarily improved his condition, however it worsened again and led to respiratory failure. A foreign body on the left main bronchus was suspected on chest radiography, and an attempt was made to remove it with a bronchoscope under general anesthesia, but the foreign body was incarcerated and difficult to remove. A few days later, it was confirmed that the foreign body had moved into the right main bronchus by physiotherapy. After retrying with the bronchoscope, the foreign body with a size of 2.5-cm was removed and was identified as tartar containing calcium phosphate. After removing the foreign body, his symptoms were improved. In our patient, difficulty in opening his mouth by muscle hypertonia may have led to insufficient daily oral hygiene, resulting in the formation of tartar. Thereafter, deciduous tartar was aspirated and became a foreign body in the airway, and consequently, the patient experienced respiratory failure. This case suggests the importance of oral hygiene in patients with SMID.

View full abstract

This study sheds light on the thoughts and experiences of the siblings of medical care children and examines the kind of support they require. Semistructured interviews were conducted with adolescent brothers and sisters of children requiring medical care. From the interview data, 4 categories (14 subcategories) were extracted with regard to the thought of having a sibling that needs medical care: gratitude and dilemma for family, expectation for social resources, love and disgust for siblings, and responsibility and anxiety for the future. Meanwhile, 5 categories (21 subcategories) were obtained with respect to the experience of having a sibling requiring medical care: consideration and conflict with family, desire and concern for the mother, joy and stress of having a compatriot, gratitude and sympathy for those around us, and everyday awareness of surroundings. Siblings of medical care children have different experiences in terms of their relationships with family, friends, and local people, and they have positive and negative thoughts and aspirations for their siblings, family, and society. They need direct support to encourage the expression of thoughts by listening to the pain and dissatisfaction, and relation to their siblings who need medical care as well as their entire family.

View full abstract

A prospective observational study was conducted for examining the effect and adverse events of polyethylene glycol preparation (PEG; MOVICOL®) for patients with severe motor and intellectual disabilities (SMID) who have intractable chronic constipation. By administration of PEG, the number of spontaneous defecation was significantly increased (p= 0.04), and the number of usage of cure medicines (e.g. glycerin enema) was decreased (p= 0.03). Stool hardness did not differ before and after PEG administration. No significant adverse events were observed. PEG can be a new treatment option for patients with SMID who do not respond to conventional therapies for constipation.

View full abstract

In this study, we conducted an anonymous self-recording questionnaire survey in guardians of children with spinal muscular atrophy (SMA) type I who use mechanical ventilator to identify the actual situations and issues of bathing practices at home in these children from the viewpoints of the caregivers (response rate of 80% and valid response rate of 80%). The bathing practices of children with SMA type I in 44 responders showed high individuality, and the responses indicated that these caregivers have provided bathing care while facing difficulties that increase with growth of the children. Although bathing care involves life-threatening and difficult situations, 90% of the family caregivers provided bathing care three times per week or more and 50% provided every day. They regard the bathing practices as a matter having diverse aspects that is more than the care provided simply to keep the body clean, and 90% of the guardians thought that they want to try hard to give bathing care although it causes difficulties. The most common caregiver who assisted the bathing care except for the family members was home visiting nurse accounting for 80% of these caregivers. In addition, among the professionals who provided advice concerning the bathing care, home visiting nurse was also predominantly common, accounting for 70% or higher of these professionals. Home visiting nurse serves as a key person to realize and continue the bathing practices desired by the children and their family. There is an urgent need for information sharing on the efforts made by the family caregivers and professionals who support these family caregivers, promotion of collaboration between the family caregivers and the professionals, and enhancement of social resources such as home bathing care services.

View full abstract