Getting from here to there: future planning as reported by adult siblings of individuals with disabilities.

BACKGROUND: Although they will often serve as caregivers for their brothers-sisters with intellectual and developmental disabilities (IDD), adult siblings are rarely included in future planning. METHOD: This study examined 495 American siblings who completed a web-based questionnaire about themselves, their brother-sister with IDD, parents and whether their families completed 11 future planning activities. RESULTS: Although virtually all families completed some future planning, on average, families completed slightly over half of the 11 activities (75% completed eight or fewer). Families more frequently identified a successor to current caregivers and engaged in planning discussions with one another and with the brother-sister; least often, families completed a letter of intent or began securing residential placements. Future planning activities comprised three domains: (1) legal activities, (2) residential activities and (3) family discussions about the future. Variables relating to one or more domains included whether the brother-sister lived in or outside of family home; brother-sister independent living abilities; presence of an intellectual disability; parent caregiving ability; and current sibling caregiving and involvement with the brother-sister with IDD. CONCLUSIONS: Although most families engage in some future planning, performance varies widely within and across future planning domains. Future planning involves different considerations and interventions depending on whether one is considering legal, residential or family discussions.

A pilot evaluation of an advocacy programme on knowledge, empowerment, family-school partnership and parent well-being.

BACKGROUND: Internationally, it has been recognised that parents need to advocate for their children with disabilities to receive services. However, many parents find advocacy difficult because of systemic and logistical barriers. As such, parents of children with disabilities may seek a special education advocate to help them understand their child's rights and secure services. Yet little research has been conducted about programmes to develop special education advocates. METHODS: In this study, we conducted a comparison study to determine the association of an advocacy programme (i.e. the Volunteer Advocacy Project) on a primary outcome (i.e. special education knowledge) and other outcomes (i.e. family-school partnership, empowerment and parent well-being). Specifically, in 2017, 34 participants, all mothers of children with disabilities, were recruited from disability organisations in the USA. Seventeen mothers participated in the intervention group (i.e. the advocacy training), while 17 mothers participated in the wait list control group. The Volunteer Advocacy Project is a 36 hr advocacy training for individuals to gain instrumental and affective knowledge to advocate for their own children with disabilities and for other families. All participants completed a pre-survey and post-survey; only intervention group participants completed a 6-month follow-up survey. RESULTS: Compared with 17 wait list control group participants, the 17 intervention group participants demonstrated improvements in special education knowledge, P = 0.002, η2  = 0.32, and self-mastery, P = 0.04, η2  = 0.15, and decreases in the quality of family-school partnerships, P = 0.002, η2  = 0.32. At the follow-up survey, intervention group participants demonstrated increases in empowerment, P = 0.04, η2  = 0.29, and special education knowledge, P = 0.02, η2  = 0.38. CONCLUSIONS: Implications for research including the need for a randomised controlled trial are discussed; also, practitioners need to evaluate advocacy training programmes regarding their effectiveness.

Getting by with a little help from my friends: siblings report on the amount of informal support received by adults with disabilities.

BACKGROUND: Given decreased formal supports for adults with intellectual and developmental disabilities (IDDs) in many industrialised countries, we need to know more about informal, or natural, supports. METHOD: Adult siblings (N = 632) responded to a web-based survey about the informal supports received by their brothers/sisters with IDDs. RESULTS: Informal support was organised by the life domains of recreation, employment and housing. Adults with IDDs received the most extensive informal support in recreation and the least extensive in housing; low levels characterised all domains. Individuals with greater numbers of supporters in a domain experienced higher levels of support, as did those residing with family and who received more state-supported, formal benefits. CONCLUSIONS: Unpaid, informal supports supplement the support needs of adults with IDDs. Connections between formal and informal supports for adults with IDDs need to be examined further.

Inflammatory cell burden and phenotype in endomyocardial biopsies with antibody-mediated rejection (AMR): a multicenter pilot study from the AECVP.

This multicenter case-controlled pilot study evaluated myocardial inflammatory burden (IB) and phenotype in endomyocardial biopsies (EMBs) with and without pathologic antibody-mediated rejection (pAMR). Sixty-five EMBs from five European heart transplant centers were centrally reviewed as positive (grade 2, n = 28), suspicious (grade 1, n = 7) or negative (n = 30) for pAMR. Absolute counts of total, intravascular (IV) and extravascular (EV) immunophenotyped mononuclear cells were correlated with pAMR grade, capillary C4d deposition, donor specific antibody (DSA) status and acute cellular rejection (ACR). In pAMR+ biopsies, equivalent number of IV CD3+ T lymphocytes (23 ± 4/0.225 mm(2) ) and CD68+ macrophages (21 ± 4/0.225 mm(2) ) were seen. IB and cell phenotype correlated with pAMR grade, C4d positivity and DSA positivity (p < 0.0001). High numbers of IV T lymphocytes were associated with low grade ACR (p = 0.002). In late-occurring AMR EV plasma cells occurring in 34% of pAMR+ EMBs were associated with higher IB. The IB in AMR correlated with pAMR+, C4d positivity and DSA positivity. In pAMR+ equivalent numbers of IV T lymphocytes and macrophages were found. The presence of plasma cells was associated with a higher IB and occurrence of pAMR late after transplantation.

A Qualitative Study Exploring Parental Perceptions of Telehealth in Early Intervention.

Using telehealth as a mode of service delivery has the potential to address some long-standing challenges in early intervention (EI) services such as waiting lists to access services. Yet, little is known about parent perceptions of telehealth in EI based on their lived experiences partnering with EI practitioners. The purpose of this study was to explore parent perceptions on using telehealth, especially on family-professional partnerships and coaching. Interviews were conducted with 15 parents of children receiving EI services via telehealth from June to August of 2021. Almost half of the participants reflected under-represented racial and ethnic backgrounds. Constant comparative analysis and emergent coding were used for data analysis. The findings showed that the advantages outnumbered the disadvantages regarding telehealth. Participants reported that telehealth provided a safe and flexible option and eliminated the wait to access EI services. However, participants identified some disadvantages to telehealth including telehealth precluded substantive interactions with therapists and limited access to technology. The findings also indicated that telehealth enhanced family-professional partnerships. Nearly all participants valued coaching during telehealth. Participants suggested initial supports to facilitate EI via telehealth, including stable internet access, telehealth training, and an initial in-person visit. Implications for research and practice are discussed. Supplementary Information: The online version contains supplementary material available at 10.1007/s10882-022-09853-w.

Defendants with intellectual disabilities and mental health diagnoses: faring in a mental health court.

BACKGROUND: Begun in the late 1990s, mental health courts are specialty criminal courts developed to address the needs of persons with mental illness. METHODS: As many persons with intellectual disabilities (IDs) may overlap in the mental health court system, we used mental health court records to examine the phenomenology and outcomes of 224 defendants with and without co-occurring IDs in the mental health court. This study had two goals: (1) to examine the prevalence of defendants with IDs in the court and (2) to compare defendants with dual diagnoses with defendants with lone mental health disorders. RESULTS: Approximately 11% of defendants in the mental health court also had IDs. Compared with individuals with mental health disorders alone, individuals with dual diagnoses were more likely to be younger, male, African-American and less well-educated; these defendants were also more likely to show externalising, 'turning-against-others' symptoms, less likely to show internalising, 'turning-against-self' symptoms. Defendants with IDs (vs. those without) more often received behavioural, vocational rehabilitation and other services, although the two groups did not differ on most outcome variables. CONCLUSION: Directions for future research are discussed.

De novo HLA sensitization and antibody mediated rejection following pregnancy in a heart transplant recipient.

Here we report a case wherein both donor-specific and third-party, paternal, HLA class II specific antibodies developed following a spontaneous miscarriage resulting in antibody-mediated rejection in a patient who had undergone an orthotopic cardiac transplant six years earlier.

Heart transplantation for homozygous familial transthyretin (TTR) V122I cardiac amyloidosis.

Heart failure is the usual cause of death in patients with amyloid cardiomyopathy. The commonest form of hereditary cardiac amyloidosis is associated with the Val122Ile variant of transthyretin (TTR), which is carried by 3-4% of the African American population. Here, we report the outcome of the first cardiac transplantation in a patient with TTR V122I. A 59-year-old Caribbean man presented with biventricular failure. Other than previous bilateral carpel tunnel syndrome, he had been well and had no evidence of extracardiac amyloidosis. An endomyocardial biopsy demonstrated amyloid of TTR type. Sequencing of TTR gene indicated homozygosity for V122I. He underwent cardiac transplantation and 3 years later, remains well with no evidence of allograft or systemic amyloid deposition.

Overlapping conditions among patients with chronic fatigue syndrome, fibromyalgia, and temporomandibular disorder.

BACKGROUND: Patients with chronic fatigue syndrome (CFS), fibromyalgia (FM), and temporomandibular disorder (TMD) share many clinical illness features such as myalgia, fatigue, sleep disturbances, and impairment in ability to perform activities of daily living as a consequence of these symptoms. A growing literature suggests that a variety of comorbid illnesses also may commonly coexist in these patients, including irritable bowel syndrome, chronic tension-type headache, and interstitial cystitis. OBJECTIVE: To describe the frequency of 10 clinical conditions among patients with CFS, FM, and TMD compared with healthy controls with respect to past diagnoses, degree to which they manifested symptoms for each condition as determined by expert-based criteria, and published diagnostic criteria. METHODS: Patients diagnosed as having CFS, FM, and TMD by their physicians were recruited from hospital-based clinics. Healthy control subjects from a dermatology clinic were enrolled as a comparison group. All subjects completed a 138-item symptom checklist and underwent a brief physical examination performed by the project physicians. RESULTS: With little exception, patients reported few past diagnoses of the 10 clinical conditions beyond their referring diagnosis of CFS, FM, or TMD. In contrast, patients were more likely than controls to meet lifetime symptom and diagnostic criteria for many of the conditions, including CFS, FM, irritable bowel syndrome, multiple chemical sensitivities, and headache. Lifetime rates of irritable bowel syndrome were particularly striking in the patient groups (CFS, 92%; FM, 77%; TMD, 64%) compared with controls (18%) (P<.001). Individual symptom analysis revealed that patients with CFS, FM, and TMD share common symptoms, including generalized pain sensitivity, sleep and concentration difficulties, bowel complaints, and headache. However, several symptoms also distinguished the patient groups. CONCLUSIONS: This study provides preliminary evidence that patients with CFS, FM, and TMD share key symptoms. It also is apparent that other localized and systemic conditions may frequently co-occur with CFS, FM, and TMD. Future research that seeks to identify the temporal relationships and other pathophysiologic mechanism(s) linking CFS, FM, and TMD will likely advance our understanding and treatment of these chronic, recurrent conditions.

Proliferative myositis. An immunohistochemical and ultrastructural study.

We studied four cases of proliferative myositis by the avidin-biotin-peroxidase complex technique, using a panel of 12 antibodies, and by electron microscopy. The aim was to clarify the nature of their constituent cells, specifically the giant ganglion-like cells and spindle cells, and to discuss the implications for histogenesis. In all cases, both cell types showed positive cytoplasmic staining with antibodies to vimentin, actin (C4), and alpha-smooth muscle actin-1, but in only one was there positive staining with desmin. No staining was obtained with factor XIIIa, muramidase, alpha-1-antitrypsin, myoglobin, S-100 protein, CAM 5.2, factor VIII-related antigen, or neuron-specific enolase. By electron microscopy, both types of cells were seen to contain numerous thin filaments, dense bodies, coated and pinocytotic vesicles, active and dilated rough endoplasmic reticulum, few microvilli, and incomplete desmosomal junctions. Our findings imply a myofibroblastic nature for the giant ganglion-like cells and spindle cells. Our observations also support the hypothesis that they are derived from a pericytic cell.

Risk of lymphoid neoplasia after cardiothoracic transplantation. a cohort study of the relation to Epstein-Barr virus.

BACKGROUND: Organ transplantation is associated with a greatly increased risk of Epstein-Barr virus (EBV)-associated lymphoproliferative disease (LPD), which is often fatal. There has been little epidemiological analysis, however, of the risk factors for LPD in transplant patients and none on whether the risks of non-EBV-associated lymphoid neoplasms are also increased. METHODS: The risk of lymphoid neoplasia was assessed in a cohort of 1563 patients who underwent cardiothoracic transplantation at Harefield Hospital, UK from 1980 to 1994 and were followed until December 1995. EBV antibody was assessed in the patients before transplantation, and lymphoid neoplasms were assessed for EBV RNA and latent EBV gene expression. RESULTS: Thirty cases of LPD occurred during follow-up. One lymphoma of unknown EBV status occurred. There were also six cases of EBV-negative non-Hodgkin's lymphoma (EBV-negative NHL), a highly significant excess over expectations from the general population rates of NHL (standardized incidence ratio 10.2 [95% confidence interval, 4.6-22.8]). The risk of LPD was significantly 10-fold raised in individuals who were EBV seronegative before transplantation; independently of this, it decreased steeply with age at transplantation and was greatest in the first year after transplantation. The risk was significantly raised in young seronegative recipients if the donor was older than the recipient. EBV-negative NHL occurred entirely in men 45 years old and older who were EBV seropositive before transplantation, and risk was not related to duration since transplantation. CONCLUSIONS: The risk factors found for LPD accord with EBV etiology and with greater hazard from primary infection than from reactivation. A second non-Hodgkin's lymphoid neoplasm, not related to EBV, seems also to be a consequence of transplantation and immunosuppression but is unlikely to be due to first infection by a ubiquitous agent. Its etiology and prevention need investigation separately from LPD.

Noninvasive monitoring of human cardiac allograft rejection.

The frequency of donor-reactive cytolytic T lymphocytes was measured in the peripheral blood mononuclear cell population of a group of 12 cardiac allograft recipients immediately before and at various time points after transplantation. At each of the time points after transplantation the donor heart was biopsied and the rejection status of the graft was determined by applying standard histological criteria. The results of this study showed that the preoperative frequency of donor-reactive cytolytic T lymphocytes in the blood was not predictive of a future tendency toward graft rejection. However, when all the data were examined it was apparent that the frequency of donor-reactive cytolytic T lymphocytes was significantly higher (P less than 0.05) in blood samples from patients whose simultaneous biopsy showed histological evidence of acute cardiac allograft rejection than in blood samples from transplant patients showing no evidence of rejection.

Flow cytometric study of nodular fasciitis, proliferative fasciitis, and proliferative myositis.

DNA ploidy was assessed retrospectively, using flow cytometry, in 13 nodular fasciitis (NF) lesions, three proliferative myositis (PM) lesions, one proliferative fasciitis lesion, and 12 other benign fibrous lesions (BFLs). All were diploid. In view of the large amounts of cellular debris, cell cycle analysis was possible in only seven NF lesions, three PM lesions, and six BFLs. The mean percentage of S phase did not differ markedly between the combination of NF and PM lesions (6.6%) and BFLs (7.1%); the mean percentage of G2 + M phase (5.4%) of the NF/PM lesion combination was twice as large as that of the BFLs (2.5%). No correlation was detected between the cell cycle analysis and the mitotic count, the predominant histologic type in NF lesions, or the predominant stroma in PM lesions.

Transbronchial biopsy in heart and lung transplantation: clinicopathologic correlations.

BACKGROUND AND METHODS: We reviewed and correlated the histologic and clinical records for the 1027 transbronchial biopsies performed, as clinically indicated, in 313 heart and lung transplant recipients in the Harefield Transplant Unit from 1988 through 1991. Three pieces of lower lobe or radiologically abnormal lung were routinely sent for histologic diagnosis. Clinical diagnoses of rejection and infection were based on symptomatologic, radiologic, and bacteriologic findings and response to appropriate therapy. Standard histopathologic technology and diagnostic criteria were used, including the Working Formulation for the standardization of nomenclature in the diagnosis of heart and lung rejection grading. RESULTS: Rejection was the most common finding (22.2%) and showed good clinicopathologic correlation. With unequivocal histologic features of rejection (Working Formulation grade A1 or above), specificity (clinical agreement with biopsy diagnosis) was 93.1% and sensitivity (clinical rejection confirmed by transbronchial biopsy) was 61%. Sensitivity increased to 77% if unsatisfactory specimens were excluded. Possible/probable rejection only was reported in 83 specimens; there were technically unsatisfactory, showed only minimal perivascular infiltrates, or had infiltrates limited to one vessel; 71% of these did have clinical rejection. Infection, excluding opportunistic, was reported in 18.5% of biopsy specimens; specificity was 70.5% and sensitivity 51.3% (both rising by 9%), with unsatisfactory specimens excluded. Histologic features of both rejection and infection were seen in 47 transbronchial biopsy specimens (4.7%). Where both components appeared definite specificity was 66.7%, but where either had been doubtful the clinical diagnosis was most often rejection. Sensitivity was also 66.7%. Cytomegalovirus inclusions were identified in 12.1% of biopsy specimens, with specificity of 91% and sensitivity of 83.5%. Sensitivity (88%) and specificity (100%) were both high for the 17 cases with pneumocystis infections. Sensitivity for the 25 transbronchial biopsy specimens from fungal infections was only 20%. Sensitivity was also poor (27.7%) in obliterative bronchiolitis, although specificity was 75%. Almost a third of transbronchial biopsy specimens from patients with obliterative bronchiolitis were unsatisfactory. Pneumonitis was the only change noted in 68 biopsy specimens. Most correlated with clinical status, but 26.5% were from patients with active rejection. Nonspecific changes or no significant pathologic condition was seen in 278 transbronchial biopsy specimens; over a third of these were from patients with clinical rejection (17.7%) or infection (18%) and 6.5% were from obliterative bronchiolitis cases. Excluding 78 technically unsatisfactory specimens reduced the proportion of false negative findings in rejection and infection by 6% and 4%, respectively. CONCLUSIONS: We found that transbronchial biopsies consisting of three adequate pieces of lung parenchyma correlated well with clinical rejections and infections other than fungal but was of limited value in confirming a diagnosis of obliterative bronchiolitis or fungal infection.

The linkage that worked: the Catholic University of America/Carroll Manor Teaching Nursing Home Program.

The TNH at the CUA/CM site included a group of people who agreed to direct their mutual commitment to common goals; they operated within a shared belief system, agreed to model a liaison that would result in quality care for the elderly, and hoped to create interest in gerontological nursing by increasing the number of prepared gerontological nursing specialists. Four areas of clinical knowledge enabled the success of this project: practice, education, administration, and research. The elements of this one situation can be translated into criteria that guide the establishment and maintenance of a TNH, namely: Two critical ingredients: a nursing home and a school of nursing with an undergraduate and a graduate nursing program; A formal agreement that specifies; a shared mission statement; responsibilities in the school and in the nursing home for practice, education, administration, research, and consultation; Clearly defined roles for the nursing faculty in the nursing home, and for the professional nursing staff in the school of nursing; Opportunity for faculty and agency input into respective institutions at all levels, including the highest policy making level; and A steering committee with representation from the school of nursing and the nursing home that guides the functions of the teaching nursing home activities; Depending upon the circumstances, the environment, and the people, other persons may or may not be able to replicate this model. Whether or not replication is possible, there may be some aspects of this experience that will suggest positive avenues to explore with the hope of establishing collaboration linkages, between nursing education and nursing service.

A comparison of Single Radial Immunodiffusion and formylated rocket immunoelectrophoresis for routine immunoglobulin measurement.

The technique of formylated rocket immunoelectrophoresis for the measurement of serum immunoglobulins has recently been developed. Advantages have been claimed for this technique when compared to the Single Radial Immunodiffusion technique (SRID). Our results indicate that SRID has advantages in reproducibility over the formylated rocket technique and of the two is the method of choice for routine immunoglobulin measurements.

Normal immunoglobulin G, A and M levels in an Irish population.

Immunoglobulin G, A and M levels were quantitated by Single Radial Immunodiffusion on 216 blood donors aged 18-65 yrs. Units of measurement were mg/100 ml, with reference to WHO Immune Reference Serum 67/97 as a baseline measurement. IgM levels were found to be significantly higher in females than in males. Factors which should be considered when evaluating immunoglobulin levels are discussed.