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Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: a case example in developmental disabilities.

Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency is a rare autosomal recessive disorder with varied expression, from severe hypoglycemia and possible sudden infant death to neurosensory deficits secondary to the acute onset. The neurosensory deficits can include clinical features such as seizure disorders, mental retardation, neuropathy, and retinopathy. The basic defect is the lack of the LCHAD enzyme in the liver, which is necessary for fatty acid metabolism. The condition is usually precipitated by infection and dehydration. A case example of a preschooler with LCHAD deficiency is presented to show the complexity of this disorder and resultant developmental disabilities. Implications for nursing practice, education, and research are discussed in relation to the needs of families with complex, developmental disabilities.

3-Hydroxyacyl CoA Dehydrogenases↗

Relation between polysomnographic measures during nocturnal sleep and a quotient of behavioral development in infants with developmental disabilities.

Polysomnographic features during nocturnal sleep were investigated in 27 infants with developmental disabilities. With the use of a multiple regression analysis, 78% of the variance of a Development Quotient (DQ) measured by a questionnaire on behavioral development for infants was explained by sleep measures. Of 14 sleep measures employed in the study, (1) cumulative awakening time during a nocturnal sleep time, (2) duration of REM stage, and (3) percentage of REM to total sleep time were important in association with the DQ. The findings are consistent with the sleep-cognition hypothesis proposed by Espie, et al.

Child Development↗

Life events as correlates of problem behavior and mental health in a residential population of adults with developmental disabilities.

Very few existing empirical studies have explored the putative association between exposure to negative life events and psychological well-being in adults with developmental disabilities. In the present study, data on exposure to life events in the previous 12 months, adaptive behavior, problem behavior, and psychiatric problems were provided by care staff for 93 adults with developmental disabilities living in a residential hospital setting. Residents had typically been exposed to between three and four negative life events mainly relating to staffing and residence changes, conflict, family bereavements and relationships, and illness or injury. Those exposed to more recent life events were also rated as displaying more frequent aggressive/destructive behavior, and were at increased risk for affective/neurotic disorder. The need for replication of these data, especially using designs allowing causality to be inferred, is emphasized.

Adaptation, Psychological↗

Governmental spending for mental retardation and developmental disabilities, 1977-1984.

A study of public spending for mental retardation and developmental disabilities in fiscal years 1977 through 1984 reveals that combined state and federal government spending grew by 23 percent despite diminished growth in federal spending after passage of the Omnibus Budget Reconciliation Act of 1981. Combined state and federal expenditures for community services grew by 40 percent, primarily because of an unprecedented rise in state spending. Total state and federal spending for institutional services plateaued, as a 26 percent drop in state appropriations was offset by an infusion of federal dollars, mostly through the Intermediate Care Facilities for the Mentally Retarded program. Federal, state, and local expenditures in fiscal 1984, estimated to total $16.49 billion, are assessed, and future trends in the financing of institutional and community services in the U.S. are discussed.

Child↗

The relationship of play behavior to cognitive ability in developmentally disabled preschoolers.

The relationship of play behavior to cognitive functioning in preschool developmentally disabled children was studied by comparing performance on Lowe and Costello's Symbolic Play Test (SPT) with that on the Bayley Scales and Stanford-Binet Scale. Subjects were 247 children referred for evaluation to a hospital-based child development clinic. Subjects were classified as mildly retarded, moderately retarded, atypical, borderline, and language disordered. Correlations between the Bayley/Binet and SPT for the whole sample were significant and in the low to moderate range. Correlations between cognitive and play measures for each clinical group were in the low to moderate range, except for atypical children where the correlations were negligible. The retarded and borderline groups achieved similar mental ages on the Bayley/Binet and SPT, but the language-disordered and atypical groups demonstrated marked differences in their Bayley/Binet and SPT functioning. Implications for using the SPT in clinical practice were discussed.

Child Development Disorders, Pervasive↗

Friendships between persons with and without developmental disabilities.

Interpersonal connections, friendships, and belonging play important roles in a person's emotional and physical well-being. However, recent studies reveal that persons with developmental disabilities often live with few connections and friendships within unwelcoming communities. Through this qualitative study, we enter this relatively unexplored area of friendships between persons with and without developmental disabilities by interviewing four existing friendship dyads in the intentional community of L'Arche. In this research these friendships are described, factors that foster or inhibit their development are identified, and communal influences on the relationships are discussed.

Adult↗

Problem behavior and psychiatric impairment within a developmentally disabled population I: behavior frequency.

This report summarizes information about problem behavior and psychiatric impairment in a population of over 30,000 individuals receiving developmental disabilities services. Teenage and adult individuals displayed more problem behaviors than young children or the elderly, more markedly mentally retarded individuals were more likely to display problem behavior, individuals in less restrictive residential settings displayed less problem behavior than those in more restrictive settings, and persons with psychiatric impairment displayed more frequent problems than persons without psychiatric impairment. The proportion of developmentally disabled persons with a psychiatric impairment was positively associated with presence of problem behaviors classified as affective or cognitive. Implications for community placement and further research are addressed.

Adolescent↗

Maltreatment in psychiatrically hospitalized children and adolescents with developmental disabilities: prevalence and correlates.

OBJECTIVE: The goals of this study were (1) to determine the prevalence of child maltreatment in hospitalized children and adolescents with developmental disabilities and concomitant psychiatric disorders, and (2) to identify the contribution of specific mother and child characteristics to the use of more severe disciplinary practices by mothers. METHOD: One hundred thirty-eight psychiatrically hospitalized children and adolescents with developmental disabilities and mothers were assessed using a semistructured interview (Child Abuse and Neglect Interview Schedule) examining factors associated with risk of child maltreatment, and questionnaires measuring maternal and child functioning. The Child Abuse and Neglect Interview Schedule and hospital charts were then used to derive ratings on the prevalence and severity of child maltreatment. RESULTS: Findings revealed that 61% of children had experienced some form of severe maltreatment by a care provider in their lifetime. Regression analysis indicated that interactions between high levels of social functioning and disruptive/oppositional behaviors and younger age in children, and low levels of social support and increased anger reactivity in mothers, were most predictive of mothers' use of severe disciplinary practices. CONCLUSIONS: Maltreatment in psychiatrically hospitalized children and adolescents with disabilities is very prevalent, and it warrants careful clinical assessment. In the psychiatric setting, families in which the child is younger, higher functioning, and behaviorally disruptive, and where mothers have little social support and exhibit increased anger reactivity, are at especially elevated risk.

Adolescent↗

Assessment of needs of adults with developmental disabilities in skilled nursing and intermediate care facilities in Illinois.

Findings are reported from a statewide assessment of the habilitation, medical, and behavioral training needs of adults with developmental disabilities in Illinois general nursing homes. Only 10% were determined to be appropriately placed in medical settings, and only 27% were enrolled in developmental training programs. A large proportion of those recommended for alternative residential settings had significantly more intense medical, adaptive behavior, self-care, and self-preservation needs than did residents who typically reside in residential facilities for persons with developmental disabilities. Historical barriers to addressing the residential and active treatment needs of the assessed residents were discussed as were recent program developments in Illinois and implications of this study for other states.

Activities of Daily Living↗

Addressing the need for education: curriculum development for nurses about intellectual and developmental disabilities.

People with disabilities and national and international agencies are voicing their views, forcing health care providers to look at how people with disabilities are treated in the health care system and to find ways to help them achieve equal access to quality care. Education about nursing care of patients with I/DD is limited in basic nursing education programs and for nurses who are in practice. A number of developmental disabilities nursing projects are addressing this need with curriculum development that has validated the need for education and has begun testing the various methods of instruction. As the curriculum is disseminated in written or Internet-accessible formats, nurses in education and practice will be able to find resources that target a specific topic area or a set of comprehensive instructions to acquire a better understanding of the comprehensive needs of people with I/DD and better ways to provide care. There is a need for a greater integration of curriculum about nursing care of people with developmental disabilities into basic nursing education at all levels and further evaluation of the impact of this curriculum on nursing care for people with I/DD. The hope is that these efforts can improve the education of nurses and other health care providers for the direct benefit of individuals with intellectual disabilities.

Adolescent↗

Preference assessment procedures for individuals with developmental disabilities.

Significant advancements have been made in the development of procedures to systematically identify preferred stimuli that may function as reinforcers for persons with developmental disabilities. Indirect assessment procedures include care provider and client interviews, whereas direct assessment procedures involve systematically exposing participants to stimuli while recording their responses. These types of direct assessment procedures can be categorized as either approach-based or engagement-based. Approach-based procedures involve recording the individuals' approach responses to stimuli presented singly or concurrently with other stimuli, whereas engagement-based procedures involve recording duration of engagement with stimuli. Although the predictive validity of indirect preference assessment procedures has yet to be established, using them in combination with direct measures of preference may be most efficacious for identifying potential reinforcers. Recent research on preference assessment procedures used with persons with developmental disabilities is reviewed and the variables that one might consider prior to selecting which procedure to use in a given situation are discussed.

Attention↗

Redefinition of school nursing practice: integrating the developmentally disabled.

Results of three needs assessment studies which had the purpose of providing direction for school nursing practices which would address some of the unmet health needs of today's children and adolescents are reported. Results provide specific examples of how school nurses have not been meeting school health needs through an analysis of one specific need -- integrating the developmentally disabled into the regular school setting. Data were collected from parents, teachers and other supportive personnel in schools by interview and survey methods. Environmental assessments were conducted and the policies and procedures governing health-related activities required by special need students were reviewed. The data indicated that for developmentally disabled students, nurses were not performing the commonly accepted functions of nursing and were not transposing their knowledge of the components of health care ordinarily provided in health care settings to care provided in nontraditional health care settings. Examples of nursing functions and the activities needed to fulfill those functions are provided.

Adolescent↗

Medical and health care needs of families providing in-home care for relatives with developmental disabilities.

The purpose of this investigation was to attempt to describe the health care needs of families providing in-home care to members with developmental disabilities as well as the characteristics and demographics of families providing in-home care. The survey included 761 families who participated in a federal demonstration project in rural southern Georgia. The results indicated that impoverished families need increased assistance to provide adequate medical and health care when providing in-home care to relatives experiencing developmental disabilities. Implications for administration of programs and development of policies are discussed.

Caregivers↗

Vagus Nerve Stimulation in the Developmentally Disabled.

Vagus nerve stimulation (VNS) with the neuro cybernetic prosthesis (NCP) is an approved treatment of partial seizures for patients 12 years and older. Developmentally disabled or mentally retarded patients with epilepsy may also benefit from VNS; however, their evaluation and management pose greater problems. A retrospective chart review was conducted on all patients diagnosed with mild to severe mental retardation who had an NCP implanted. Records of these 21 patients, ranging in age from 3 to 56 years, were reviewed regarding VNS efficacy, side effects, behavioral changes, and alterations in antiepileptic drugs (AEDs). Seizure types included partial onset and generalized. Sixteen patients had clearly evaluable seizures both pre- and postimplant, with a greater than 50% reduction in seizures noted in 68% (11/16) after 6 months of implant. There were no adverse events that prevented chronic stimulation. Institutional staff and family members were provided with both pre- and postoperative education on VNS and magnet use. VNS appeared to be an effective and well-tolerated therapy in this group of developmentally disabled patients with refractory epilepsy.

Journal Article↗

Therapeutic effects and long-term efficacy of antidepressant medication for persons with developmental disabilities. Behavioral assessment in two cases of treatment-resistant aggression and self-injury.

Recent advances in pharmacological treatment of severe behavior disorders in persons with developmental disabilities suggest the use of antidepressant medication for therapeutic management. This research evaluated two antidepressant medications for treatment-resistant aggression and self-injury exhibited by two persons with developmental disabilities. Behavioral assessment data documented that sertraline (a serotonin selective reuptake inhibitor) was effective in reducing self-injurious behaviors in a 20-year-old man with severe mental retardation and clomipramine (a tricyclic antidepressant) was associated with the elimination of aggressive behavior in a 14-year-old boy with autism. Clinical effects from the medications were measured in relation to and shown to be a function of dosage level. Extended follow-up assessments revealed maintenance of treatment gains with continued medication administration.

Adolescent↗

Comprehensive health services for developmentally disabled Navajo children.

A retrospective study of 60 Navajo children with developmental disabilities was conducted to evaluate the quality and comprehensiveness of health services provided. Descriptive analysis by a multidisciplinary panel included medical record reviews, family interviews, and site visits to local health, educational, and family support services. Findings included timely and appropriate management of "medical" problems but a general neglect of "developmental" issues, such as hearing, speech/language, cognitive, and behavioral functioning, and attention to family understanding and adjustment toward caring for a handicapped child. Primary prevention and screening efforts were judged generally adequate, although not utilized by the majority of mothers of disabled children. Diagnostic assessments, family counseling, and referrals for treatment were incomplete, fragmented, and poorly coordinated. These problems resulted in potentially harmful delays in making referrals to available treatment programs. The majority of families interviewed tended to focus on the "medical" problems, had a poor understanding of the "developmental" components, and rarely participated actively in treatment.

Arizona↗

Trinucleotide repeat expansion in the FRAXE locus is not common among institutionalized individuals with non-specific developmental disabilities.

Expansion of a polymorphic GCC-repeat at the FRAXE locus has been associated with expression of chromosome fragility at this site and cognitive impairment in some individuals previously testing negative for CGG-repeat expansion in the fragile X mental retardation-1 (FMR1) gene. To determine the frequency of FRAXE triplet repeat expansion among persons with developmental disability, 396 individuals from two institutions were studied, all of whom were negative for FMR1 repeat expansion. Clinically, there was a wide range of mental impairment, with the majority (61.1%) being severely to profoundly affected. The distribution of FRAXE GCC-repeat numbers in the study population was 5-38: 28 (5.6%) with 10-14 repeats; 366 (73.8%) with 15-19 repeats; 74 (14.9%) with 20-24 repeats; 20 (4.0%) with 25-29 repeats; and 5 (1.0%) with 30-38 repeats, with no individuals demonstrating repeat expansion. One profoundly retarded male was found to have a deletion of about 40 bp. Southern blots of HindIII-digested DNAs from individuals with > or = 26 repeats all showed normal patterns. These results suggest that FRAXE GCC-repeat expansion is not a common cause of developmental disability in institutionalized persons with mild to profound mental retardation.

Adult↗

Content validity and clinical applicability of the Irena Daily Activity assessment measuring occupational performance in adults with developmental disability.

The purpose of this study was to investigate the validity of the Irena Daily Activity (IDA) assessment which measures occupational performance in adults with developmental disabilities. The instrument's content validity was investigated by elucidating its general appropriateness, its clinical applicability and the feasibility of the measurement procedure. Data were collected from a multidisciplinary panel of clinical experts working in day activity centres. Each expert independently judged and reviewed the IDA instrument's content validity. The results show that the content validity index (CVI) values ranged from 0.8 to 1.0 for the instrument's general appropriateness and clinical applicability, and from 0.7 to 1.0 with respect to the significance of the IDA domains' clinical applicability. The applicability of the IDA items were judged as satisfactory (CVI > 80) with respect to their importance for planning treatment and intervention. The feasibility of the measurement procedure was estimated as satisfactory in relation to the items (CVI > 0.70) and satisfactory with respect to the time needed and material used, with CVI values ranging from 0.8 to 1.0. The results of this study support the use of the IDA as a feasible and time-efficient assessment that provides insights regarding the occupational performance of adults with developmental disabilities. A limitation of this study was that it could not illuminate all-important aspects of validity. In conclusion, the IDA has the potential to become a reliable and valid clinical assessment, but additional research on psychometric properties is still needed.

Activities of Daily Living↗