Severe Motor And Intellectual Disabilities Research Articles

Social support needs of caregivers rearing children with severe motor and intellectual disabilities at home in Japan.

In Japan, recently, the number of children with severe motor and intellectual disabilities (SMID) is steadily increasing. Caregivers such as parents and family members are struggling with how to live with their children at home and in the community after discharge. The current study aimed to explore the social support needs faced by caregivers while rearing children with SMID in order to identify effective means of social support in Japan. We conducted a cross-sectional survey of the primary caregivers of children with SMID at home through special-needs elementary, junior high, and senior high schools nationwide, using a self-administered, anonymous questionnaire to investigate the actual social support needs of the caregivers. All statements of social support need were coded using Krippendorff content analysis. Questionnaire returns were obtained from 1,176 families, and the descriptions of 1,173 families were included in the analysis. The results of the analysis showed that the needs of the caregivers consisted of seven categories. The social support needs expressed by the caregivers are necessary findings for Japan today, both for the soft side, such as the development of local systems and regulations to support these families, and for the hard side, such as the increase and improvement of facilities and equipment.

Vitamin D Supplementation at a Dose of 10 µg/Day in Institutionalized Children with Severe Motor and Intellectual Disabilities.

Vitamin D (VD) deficiency can lead to health-related consequences. This study determined the effects of VD administration in VD-deficient children with severe motor and intellectual disabilities (SMID). Twenty-eight subjects were included. Among them, 25 subjects with parental consent for VD administration were given 10 µg/day (400 IU/day) of VD in April 2021. Serum 25-hydroxyvitamin D [25(OH)D] levels were measured at least 30 days after the start of VD administration. The total VD intake, serum 25(OH)D levels, and ultraviolet (UV) exposure before the blood tests were investigated. The results showed that the median serum 25(OH)D levels were 8.7 ng/mL (4.3-17.2) and 24.0 ng/mL (7.8-39 ng/mL) from March to May in 2020 and 2021, respectively. Among the 25 subjects, 22 with UV exposure had >20 ng/mL serum 25(OH)D level, and 2 without UV exposure had <20 ng/mL serum 25(OH)D level. Three subjects who did not receive VD supplementation had <20 ng/mL serum 25(OH)D level. Taken together, VD supplementation (10 µg/day) is effective in children with SMID in institutional care. Moreover, it may be sufficient for children with UV exposure, but not for those without.

The Effect of Vitamin K2 Supplementation on PIVKA-II Levels in Patients with Severe Motor and Intellectual Disabilities Undergoing Long-Term Tube Feeding.

Nutritional support is essential for patients with severe motor and intellectual disabilities (SMID) to ensure the smooth provision of medical care. These patients often require long-term tube feeding with enteral formulas, potentially leading to deficiencies in vitamins and trace elements. Additionally, frequent antibiotic use for infections often disrupts gut microbiota, inhibiting vitamin K2 production by intestinal bacteria. We assessed the serum protein induced by vitamin K absence or antagonists-II (PIVKA-II) and undercarboxylated osteocalcin (ucOC) levels to assess the vitamin K status in 20 patients with SMID (median age: 44.1 years, 11 men and 9 women) undergoing long-term tube feeding for durations ranging from 3 to 31 years. Thirteen (65%) and nine (45%) patients had elevated PIVKA-II (<40 mAU/mL) and serum ucOC levels (reference value < 4.50 ng/mL), respectively. Dietary vitamin K1 intake did not differ between patients with and without elevated PIVKA-II levels. Vitamin K2 supplementation for 3 months decreased serum PIVKA-II levels near those within the reference range. Approximately half of the patients with SMID on tube feeding had subclinical vitamin K deficiency. Further studies are needed to ascertain if long-term vitamin K2 supplementation effectively prevents vitamin K deficiency-induced hypercoagulation, osteoporosis, and vascular calcification in patients with SMID.

Changes in Fecal Gut Microbiome of Home Healthcare Patients with Disabilities through Consumption of Malted Rice Amazake.

The aim of this study was to investigate changes in gut microbiome both during and after the consumption of malted rice amazake (MR-Amazake), a fermented food from Japan, in home healthcare patients with disabilities including patients with severe motor and intellectual disabilities (SMID). We monitored 12 patients who consumed MR-Amazake for six weeks, investigating them before and after the intervention as well as six weeks after the end of intake to compare their physical condition, diet, the type of their medication, Constipation Assessment Scale (CAS), and an analysis of their comprehensive fecal microbiome using 16S rRNA sequencing. Their constipation symptom significantly alleviated and principal coordinates analysis revealed that 30% of patients showed significant changes in gut microbiome after MR-Amazake ingestion. Furthermore, Bifidobacterium was strongly associated with these changes. These changes were observed only during MR-Amazake intake; the original gut microbiome was restored when MR-Amazake intake was discontinued. These results suggest that six weeks is a reasonable period of time for MR-Amazake to change human gut microbiome and that continuous consumption of MR-Amazake is required to sustain such changes.

Relationship between energy intake and changes in BMI over time in persons with severe motor and intellectual disabilities(SMID)

Relationship between energy intake and changes in BMI over time in persons with severe motor and intellectual disabilities(SMID)

Surgery for acute abdomen in adult patients with severe motor and intellectual disabilities.

The present study aimed to examine the characteristics, outcomes, and problems related to surgery for acute abdomen in adult patients with severe motor and intellectual disabilities (MID). The clinical records of 35 adult patients with severe MID who received emergency surgery for acute abdomen between 2011 and 2020 were reviewed. The median duration from onset to surgery was 48 hours. There were 2 cases of in-hospital mortality (5.7%), and all the patients underwent surgery more than 72 hours after onset. The in-hospital mortality rate was significantly higher in patients who received surgery later than 72 hours after onset. Bowel obstruction was the most common disease among the acute abdomen cases (71.4%) and most often involved volvulus of the small bowel and cecum. Of the patients with bowel obstruction with severe MID, 72.0% had abdominal distention, 16.0% had abdominal pain, and 4.0% had vomiting. The median duration from onset to surgery was significantly longer in the patients with bowel obstruction with severe MID than in those without severe MID (24 hours vs. 16 hours). Acute abdomen in patients with severe MID was often due to bowel obstruction caused by volvulus. Because patients with severe MID have few symptoms, they are susceptible to adverse surgical outcomes associated with a prolonged duration from onset to surgery.

Consideration for Positive and Negative Effect of Multi-Sensory Environment Interventions on Disabled Patients through Electrocardiography

Many studies have supported the efficacy of multi-sensory environment (MSE) interventions in reducing behavioral and psychological symptoms and improving the quality of life for disabled patients. However, it is difficult to identify the groups that are helped and those who are harmed. This study verified the effect of multi-sensory environment interventions on disabled patients by using thermal images, then addressed the precaution using electrocardiography (ECG). Twenty disabled patients participated in experiments for 12 min: ten with muscular dystrophy (MD) and ten with severe motor and intellectual disabilities (SMID). The continuous measurement of nasal temperature evaluated the emotional arousal after facial detection. The QT-RR relation was used to assess the risk degree. It was found that the continuous measurement of nasal temperature enabled us to evaluate the emotional arousal of disabled patients in MSE with the comparison of ECG. Through the QT-RR relation, it was found that the risk assessment for the patient with SMID was 11 times higher than those with MD because the QT was below 300 ms. Therefore, it was concluded that the specification for the risky group was related to the kind of prescribed medication through continuous measurement.

Tendency and risk factors of acute pancreatitis in children with severe motor and intellectual disabilities: A single-center study

ObjectiveAcute pancreatitis (AP) is a life-threatening disease in children with severe motor and intellectual disabilities (SMID). This study aimed to determine the clinical characteristics and causes of AP in children with SMID. MethodsWe enrolled 29 children with AP admitted to our hospital. Patients were divided into children with SMID (AP with SMID group) and the remaining patients (AP without SMID group). All patients in the AP without SMID group had no pre-existing neurological disabilities. We obtained data on the clinical background of patients, severity of AP, treatments, and outcomes. We enrolled 34 children with SMID without a history of AP (SMID control group) to compare their clinical characteristics with the AP with SMID group. We statistically compared the data between the AP with SMID and AP without SMID groups and between the AP with SMID and SMID control groups. ResultCompared to the AP without SMID group, the AP with SMID group showed significantly more severe pancreatitis (50% vs 12%) and recurrent pancreatitis (58% vs 18%). Moreover, compared with the SMID control group, the AP with SMID group showed significantly more frequent absence of voluntary movement (83% vs 26%), requirement of respiratory devices (75% vs 35%), panhypopituitarism (33% vs 6%), thermoregulatory dysfunction (58% vs 18%), and low serum albumin levels (3.6 vs 4.1 g/dL). ConclusionAP in children with SMID tended to be associated with severe and recurrent pancreatitis. Children with SMID who had a history of AP had more severe neurological impairment among children with SMID. Hypothalamic-pituitary system dysfunction caused by severe cerebral damage may contribute to the development of AP.

Dysbiosis of the gut microbiota in children with severe motor and intellectual disabilities receiving enteral nutrition: A pilot study.

Children with severe motor and intellectual disabilities (SMIDs) frequently and continuously receive enteral nutrition and medications and lack adequate exercise, which may lead to dysbiosis, an imbalance in the composition of the gut microbiota. However, studies on the composition of gut microbiota in children with SMIDs are limited. Therefore, we aimed to examine the characteristics of the gut microbiota in children with SMIDs. 16S rRNA gene sequencing was performed using fecal samples of 10 children with SMIDs, who received enteral nutrition through a gastric fistula or gastric tube (SMID group: median age, 10.0 years), and 19 healthy children (healthy control [HC] group: median age, 9.0 years). Microbial diversity, microbial composition, and abundance of butyric acid-producing bacteria were compared between the groups. Daily dietary fiber intake in the SMID group was evaluated using questionnaires. The Shannon and Simpson indices (alpha diversity indices) were significantly lower in the SMID group than those in the HC group. Beta diversity analysis identified different clusters. Compared with the HC group, Clostridiales and butyric acid-producing bacteria were less abundant and Bacteroidales were more abundant in the SMID group. Dietary fiber intake in the SMID group was approximately two-thirds of the estimated average requirement for healthy Japanese children. Children with SMIDs showed dysbiosis with alteration in the microbial diversity, which could partly be attributed to their low dietary fiber intake. Further studies, with the intervention of prebiotics, probiotics, and synbiotics, are warranted to improve dysbiosis in children with SMIDs.

Healthcare workers’ presenteeism causing an outbreak of respiratory infections in a facility for patients with severe motor and intellectual disabilities

BackgroundThis study aimed to identify the source of infection and medical costs for a respiratory infection outbreak in a facility for patients with severe motor and intellectual disabilities (SMID). Presenteeism refers to a situation wherein a person continues going to work despite being ill. MethodsThe cohort included 1 healthcare worker and 17 patients who developed a fever of ≥37.5°C with respiratory symptoms for nearly a month. An outbreak investigation was conducted, which determined the initial case of the outbreak to be a single healthcare worker. We performed a univariate analysis to determine the association of the healthcare worker. From the medical records, we evaluated the costs of addition treatment and laboratory tests for the respiratory infection. ResultsThe source of infection was a healthcare worker at the facility (Odds ratio, 17.5; 95% confidential interval, 3.0–101.8). The total medical cost for hospitalized patients due to this outbreak was $12,324. DiscussionThe source of a respiratory infection outbreak in a facility for SMID was suggested to be a healthcare worker's presenteeism. ConclusionsThe cause of this outbreak was healthcare workers’ presenteeism. To prevent outbreaks, such facilities should address the causative factors.

Clinical Usefulness of Blenderized Food for Selenium Deficiency in Pediatric Patients with Severe Motor and Intellectual Disabilities

Introduction: This study aimed to assess the preventive or therapeutic effects of blenderized food (BF) on selenium deficiency in pediatric patients with severe motor and intellectual disabilities (SMID). Methods: The medical records of all 40 consecutive pediatric patients with SMID who underwent nutritional assessment were retrospectively reviewed and compared between two groups: the enteral formula (EF) group and the BF group fed with BF providing more than 10% of total caloric intake. Next, for the selenium-deficient patients who were newly started on blenderized tube feeds after the first nutritional assessment, improvement of selenium deficiency and change of dietary contents were assessed. Results: The BF group patients had a significantly lower prevalence of selenium deficiency and higher serum selenium levels than the EF group patients. In all 7 selenium-deficient patients who started blenderized tube feeds after the first nutritional assessment, serum selenium levels were significantly increased at the second nutritional assessment, even though total selenium intake, selenium intake by EF, and total caloric intake did not differ significantly, and, in fact, caloric intake was significantly decreased by EF. Conclusion: Combined feeding of BFs can be useful for prevention and therapy of selenium deficiency in pediatric SMID patients.

Tumor Screening, Incidence, and Treatment for Patients with Severe Motor and Intellectual Disabilities.

The increasing age of patients with severe motor and intellectual disabilities (SMID) has become a serious concern. Few studies have investigated tumor treatment in this population. Tumor treatments for 12 SMID patients were examined. Blood tests and ultrasonography were useful for screening. With regard to treatment, surgery for SMID patients was performed in the same manner as for patients without SMID, and the results were generally satisfactory, without major complications. Typically, cancer was diagnosed at an advanced stage, and many metachronous double cancers were observed. Treatment yielded satisfactory results for patients with SMID and their families. Future studies should examine the clinical significance of screening and tumor operative method for patients with SMID.

Gallstones in patients with severe motor and intellectual disability.

Asymptomatic gallbladder stones may be detected with ultrasound; some gallstones produce symptomatic diseases, such as cholecystitis, cholangitis, or pancreatitis. Identifying the clinical features of symptomatic gallstones may help prevent severe complications by providing intervention for asymptomatic gallstones. We aimed to investigate risk factors associated with developing symptomatic disease in pediatric patients with severe motor and intellectual disabilities (SMID). This retrospective study enrolled 30 patients with SMID who were treated at the Nara Medical University between March 2016 and March 2019. We examined the prevalence of gallstones, and the rate at which associated symptoms of gallstones were observed in patients. Furthermore, we compared the clinical features of patients with and without gallstones. Among 30 patients with SMID, 7 (23%) had gallstones, with 6 (86%) of them being symptomatic: 3 patients had acute pancreatitis and 3 had cholecystitis or cholangitis. Among 23 patients without gallstones, 2 had acute pancreatitis and 2 had biliary sludge. Patients with gallstones had significantly lower daily calorie intake than those without gallstones (P = 0.042). Furthermore, the incidence of gallstones was higher in patients who received total parenteral nutrition than in those who did not (P = 0.031). Comparative analysis between symptomatic and asymptomatic patients was not performed because almost all cases were symptomatic. Gallstones were detected in 23% of patients with SMID. The gallstones were symptomatic at a very high rate. Considering this fact, patients with SMID should be surveyed for gallstones; careful management may be needed in such patients.

Oral support for patients with severe motor and intellectual disabilities.

Oral support during feeding was clinically provided to patients with severe motor and intellectual disabilities (SMID) for the prevention of dysphasia. The present study investigated the advantages of oral support anatomically and functionally. A videofluoroscopic swallowing study was conducted on nine patients with SMID (age = 5-41 years; mean age = 15.0; four males, five females) and 24 healthy adults (age = 26-67 years; mean age = 44.3; 16 males, eight females). The movements of the hyoid bone and mandible during pharyngeal swallowing were tracked, and the pharyngeal residues were evaluated. The temporal and spatial features of the movements were compared between patients with and without oral support as well as healthy adults. The mandible moved downward earlier and showed larger displacement in the patients with SMID. The patients also had insufficient anterior displacement of the hyoid, which was associated with the pharyngeal residue. This displacement was enhanced, and the pharyngeal residue decreased with oral support. Oral support to hold the mandible successfully improved hyoid excursion and reduced pharyngeal residue during swallowing, which is a simple and reliable strategy for the prevention of dysphasia in patients with SMID.

Systemic corticosteroid as an adjunctive treatment for lower respiratory tract infection in children with severe motor and intellectual disabilities

IntroductionChildren with severe motor and intellectual disabilities (SMID) are susceptible to severe lower respiratory tract infection (LTRI). As SMID patients are prone to develop recurrent wheezing and are often diagnosed with bronchial asthma, they frequently receive systemic corticosteroids as an adjunctive treatment for LRTIs. However, the efficacy of corticosteroid therapy for LTRIs in SMID children is unclear. We investigated whether or not corticosteroid therapy was associated with better clinical outcomes for SMID children with LRTIs. MethodsOur retrospective study enrolled 217 SMID children 1–15 years old hospitalized for LTRIs. We compared the clinical characteristics and outcomes between patients with and without corticosteroid therapy. ResultsOf the 217 patients, 29 (13.3%) received corticosteroid therapy. The proportion of patients with a history of bronchial asthma was higher and LRTI was more severe in patients with corticosteroid therapy than in those without the therapy. The length of hospital stay (LOHS) was significantly longer in patients with corticosteroid therapy (median 13 days) than in those without corticosteroid therapy (median 9 days) (P = 0.02). The same tendency was shown for the LOHS in patients with severe or moderate LRTI, although not to a significant extent. ConclusionSystemic corticosteroid therapy was not associated with better clinical outcomes in SMID children with LRTIs, even if the patients suffer from severe LRTIs. Corticosteroids should be used cautiously for LRTIs in SMID children because bronchial asthma is likely to be overdiagnosed in these children.

Creatinine-to-cystatin C ratio estimates muscle mass correlating the markers of the patients with severe motor and intellectual disabilities

AimThe creatinine-to-cystatin C ratio (CCR) has been acknowledged as a potential marker of muscle mass. The purpose of the present study was to evaluate the relationship between CCR and nutritional status in a bioelectrical impedance analysis (BIA) of patients with severe motor and intellectual disabilities (SMID). MethodsThis study included 39 patients with SMID (17 males, 22 females) over 16 years of age were included retrospectively. CCR was calculated as serum creatinine (mg/dL)/cystatin C (mg/L) × 10. The BIA parameters such as the phase angle (PhA), fat free mass (FFM), appendicular skeletal muscle mass (ASM) and appendicular skeletal muscle mass index (ASMI) values were measured using BIA. Correlation analyses between CCR and the BIA parameters were conducted. ResultsThe mean CCR is 4.47 ± 1.34. Significant positive relationships between CCR and FFM, PhA, ASM, ASMI were identified (r = 0.3373, p = 0.0357. r = 0.4273, p = 0.0093. r = 0.5008, p = 0.0012. r = 0.4706, p = 0.0025 and r = 0.4751, p = 0.0022, respectively). ConclusionsThe study indicated that CCR in the patients with SMID is a useful parameter that allows for the muscle mass to be estimated easily and accurately. This means that evaluating CCR could be used as a simple and important screening tool for PhA, FFM and muscle mass.

The Assessments of Psychological Dynamics in Daily Life of Individual With Severe Motor and Intellectual Disabilities (SMID): Relationship Between the Inter-Stimulus Interval on Communications and the SMID’s Heart Rate

Central fatigue in Parkinson’s disease (PD) is a common and disabling symptom that further worsens the patients’ quality of life. A deficit in the serotonergic system may be implicated in the occurrence of fatigue in patients with PD as well as in those with other chronic conditions characterized by fatigue. The loudness dependence of auditory evoked potentials (LDAEP) is a neurophysiological tool that has proved to be effective in measuring the serotonergic central function in vivo. The aim of the present study was to assess central serotonergic activity in PD patients and to explore its possible association with the presence of fatigue. LDAEP was recorded in 38 PD patients (26 without fatigue – PDnF and 12 with fatigue – PDF) and 34 healthy controls. A significant difference between parkinsonian patients and controls emerged, with patients displaying stronger LDAEP values (which reflect a lower serotonergic central tone) than controls. By contrast, no differences in LDAEP emerged between PDF and PDnF. Our electrophysiological data confirmed the presence of a deficit in serotonergic central transmission in PD. An association between this deficit and fatigue was not demonstrated. It is likely that an altered dopamine/serotonin balance, rather than a serotonin deficit alone, is involved in the genesis of central fatigue. This complex and multifaceted symptom is related above all to a dysfunction in the striato-thalamo-cortical loop that connects the neostriatum to the frontal lobe and is strongly affected by motivation.

Intussusception Caused by Percutaneous Endoscopic Gastrostomy With Jejunal Extension in Patients With Severe Motor and Intellectual Disabilities

The risk of intussusception related to percutaneous endoscopic gastrostomy with jejunal extension (PEG-J) in patients with severe motor and intellectual disabilities (SMID) remains unknown. In a cross-sectional study, a review of 26 patients (mean age, 11.6 ± 6.4 years) with SMID who underwent PEG-J was performed. During the follow-up period, 6 of 26 (23%) patients developed intussusception. The median period from PEG-J to the onset of intussusception was 364 (range, 8–1344) days. No significant difference was observed in the Cobb angle between the intussusception and nonintussusception groups; however, body mass index at the time of PEG-J was significantly lower in the intussusception group. Intussusception related to PEG-J occurs relatively frequently in patients, and it is possibly attributable to factors such as deformity caused by undernutrition and weight loss. If enteral nutrition via PEG-J has been established, earlier enterostomy can be recommended because of the high risk of intussusception in patients with SMID.

Integrating Behavior of Children with Profound Intellectual, Multiple, or Severe Motor Disabilities With Location and Environment Data Sensors for Independent Communication and Mobility: App Development and Pilot Testing.

BackgroundChildren with profound intellectual and multiple disabilities (PIMD) or severe motor and intellectual disabilities (SMID) only communicate through movements, vocalizations, body postures, muscle tensions, or facial expressions on a pre- or protosymbolic level. Yet, to the best of our knowledge, there are few systems developed to specifically aid in categorizing and interpreting behaviors of children with PIMD or SMID to facilitate independent communication and mobility. Further, environmental data such as weather variables were found to have associations with human affects and behaviors among typically developing children; however, studies involving children with neurological functioning impairments that affect communication or those who have physical and/or motor disabilities are unexpectedly scarce.ObjectiveThis paper describes the design and development of the ChildSIDE app, which collects and transmits data associated with children’s behaviors, and linked location and environment information collected from data sources (GPS, iBeacon device, ALPS Sensor, and OpenWeatherMap application programming interface [API]) to the database. The aims of this study were to measure and compare the server/API performance of the app in detecting and transmitting environment data from the data sources to the database, and to categorize the movements associated with each behavior data as the basis for future development and analyses.MethodsThis study utilized a cross-sectional observational design by performing multiple single-subject face-to-face and video-recorded sessions among purposively sampled child-caregiver dyads (children diagnosed with PIMD/SMID, or severe or profound intellectual disability and their primary caregivers) from September 2019 to February 2020. To measure the server/API performance of the app in detecting and transmitting data from data sources to the database, frequency distribution and percentages of 31 location and environment data parameters were computed and compared. To categorize which body parts or movements were involved in each behavior, the interrater agreement κ statistic was used.ResultsThe study comprised 150 sessions involving 20 child-caregiver dyads. The app collected 371 individual behavior data, 327 of which had associated location and environment data from data collection sources. The analyses revealed that ChildSIDE had a server/API performance >93% in detecting and transmitting outdoor location (GPS) and environment data (ALPS sensors, OpenWeatherMap API), whereas the performance with iBeacon data was lower (82.3%). Behaviors were manifested mainly through hand (22.8%) and body movements (27.7%), and vocalizations (21.6%).ConclusionsThe ChildSIDE app is an effective tool in collecting the behavior data of children with PIMD/SMID. The app showed high server/API performance in detecting outdoor location and environment data from sensors and an online API to the database with a performance rate above 93%. The results of the analysis and categorization of behaviors suggest a need for a system that uses motion capture and trajectory analyses for developing machine- or deep-learning algorithms to predict the needs of children with PIMD/SMID in the future.

A Case Report of a Patient With Severe Motor and Intellectual Disabilities (SMIDs) With Severe Physique Deformity, Who Underwent Cholecystectomy for Cholangitis and Cholecystolithiasis

A Case Report of a Patient With Severe Motor and Intellectual Disabilities (SMIDs) With Severe Physique Deformity, Who Underwent Cholecystectomy for Cholangitis and Cholecystolithiasis