Health-related quality of life in paediatric patients with intoxication-type inborn errors of metabolism: Analysis of an international data set.

Acute intoxication-type inborn errors of metabolism (IT-IEM) such as urea cycle disorders and non-acute IT-IEM such as phenylketonuria have a major impact on paediatric patients' life. Patients have to adhere to a strict diet but may face neurocognitive impairment and - in acute diseases - metabolic decompensations nevertheless. Research on the subjective burden of IT-IEM remains sparse. Studies with appropriate sample sizes are needed to make valid statements about health-related quality of life (HrQoL) in children and adolescents with IT-IEM. Six international metabolic centres contributed self-reports and proxy reports of HrQoL (assessed with the Paediatric Quality of Life Inventory) to the final data set (n = 251 patients; age range 2.3-18.8 years). To compare HrQoL of the patient sample with norm data and between acute and non-acute IT-IEM, t tests were conducted. To examine the influence of child age, sex, diagnosis and current dietary treatment on HrQoL, multiple linear regression analyses were conducted. Self-reports and proxy reporst showed significantly lower HrQoL total scores for children with IT-IEM compared to healthy children. Current dietary treatment significantly predicted lower proxy reported total HrQoL. Children with non-acute IT-IEM reported significantly lower psychosocial health and emotional functioning than children with acute IT-IEM. The patient sample showed significantly impaired HrQoL and a diet regimen remains a risk factor for lower HrQoL. Differences in HrQoL between acute and non-acute IT-IEM subgroups indicate that factors beyond symptom severity determine the perception of disease burden. Identifying these factors is of crucial importance to develop and implement appropriate interventions for those in need.

Self-Compassion, Social Constraints, and Psychosocial Outcomes in a Pet Bereavement Sample.

This study investigated self-compassion in the context of grief following the death of a companion animal in a recently bereaved sample (N = 431). We addressed social contexts and individual differences focusing on how psychosocial outcomes vary as a function of social constraints, as well as individual differences in self-compassion and use of continuing bonds (CB). We observed that self-compassion related to the frequency of engagement in CB. Self-compassion also moderated relationships between grief severity and depression as well as social constraints and depression. We recommend future research on self-compassion training and psychosocial outcomes, especially for those experiencing social constraints or disenfranchised grief.

Quality of life and psychological functioning of pediatric and young adult patients with Gaucher disease, type 1.

This study examined the health-related quality of life (HRQoL) and psychological functioning of children and young adults with Gaucher disease, type 1 (GD1). Thirty-two (17 pediatric, 15 young adult) patients with GD1 and one parent completed age-appropriate assessments of HRQoL, emotional, and behavioral health. The HRQoL of children with GD1 was compared with a healthy sample and to children diagnosed with Fabry disease (FD; another lysosomal storage disease), while young adults were compared to a healthy sample and to patients with self-reported chronic illnesses. Children with GD1 reported significantly lower HRQoL across all domains relative to healthy counterparts yet comparable HRQoL compared to children with FD. Young adults reported mildly lower physical functioning than healthy peers, but no differences in HRQoL relative to the chronic illness sample. Parent-reported symptom severity was associated with poorer HRQOL in children but not young adults. Few group differences in psychological functioning were observed, except young children showed more school problems than the normative average and there was a trend toward internalizing symptoms. Overall, results consistently identified younger patients with GD1 as more affected than older patients in HRQoL and psychological domains. Implementation of psychosocial interventions may be particularly beneficial during early childhood.

Pet loss and continuing bonds in children and adolescents.

There is little research available regarding the impact of pet loss on children. In the current mixed-methods study, we explored the different ways that children use continuing bonds (CB) to cope following the death of a pet. We studied 32 children (5-18 years) and their parents. Children answered four questionnaires and the Continuing Bonds Interview. Parents answered a demographic questionnaire. Results suggest that all children utilize CB while grieving the loss of a pet, although CB expression varies depending on the age of the child, the level of grief following the loss, and the strength of attachment to the pet.

The need to support caregivers during pediatric bone marrow transplantation (BMT): A case report.

ABSTRACTObjective:Pediatric bone marrow transplants represent a medically stressful, potentially traumatic experience for children and caregivers, and psychological support for parental caregivers is paramount to their long-term well-being. However, many medical centers do not have protocols in place to sustain caregiver well-being during these distressing experiences. METHOD: We report on a case of a 10-month-old infant with Wiskott Aldrich Syndrome who was hospitalized for bone marrow transplantation. RESULT: We describe the significant burden that fell upon caregivers during and after a bone marrow transplantation. SIGNIFICANCE OF RESULTS: This case helped guide our suggestions to improve care for caregivers. Several logistical hurdles could be overcome to alleviate some of these burdens. We suggest that a child psychologist or psychiatrist should be on patient care teams and be attentive to parental stress, impairments, or impediments to self-care, and signs of emergency of mental illness in this setting of medical trauma. Additionally, promotion of sleep hygiene and linkage to support systems can maximize resiliency. Finally, we believe that hospital administrators should partner with clinicians to facilitate routine support during highly stressful transitions of care.

Disorder in the Court: Cluster B Personality Disorders in United States Case Law.

Previous research has shown that antisocial, borderline, narcissistic and histrionic personality disorders, also known as the Cluster B personality disorders in the Diagnostic and Statistical Manual of Mental Disorders-Fifth Edition (DSM-5), are commonly raised in lawsuits. Cluster B disorders are characterized by problems with emotion regulation, impulsivity and interpersonal conflicts. Without question, individuals diagnosed with a Cluster B disorder possess traits that make them more susceptible to becoming involved in litigation; however, to date there has been no research on how the disorders interact with the judicial system. This study surveyed litigant success of Cluster B personality disorders in United States federal and state case law. Results showed that both criminal and civil litigants tended to be unsuccessful in their cases. Overall, this study demonstrated that court opinions can provide a window into the psychology of trial litigants and how personality can affect trial outcomes.

Posttraumatic Growth Following the Loss of a Pet.

The current study examined posttraumatic growth (PTG) experienced by bereaved pet owners following the death of their pet. Using qualitative methodology, we analyzed responses of 308 participants who answered yes to a question about experiencing PTG. Within the five factors model of PTG, the most endorsed included the following: Relating to Others ( n = 76), Appreciation of Life ( n = 52), Personal Strength ( n = 51), Spiritual Change ( n = 32), and New Possibilities ( n = 29). Other themes not captured by the PTG included as follows: relating to animals ( n = 70), continuing bonds ( n = 53), attachment relationship ( n = 44), and unconditional love ( n = 13). Our findings support the notion that PTG occurs for people who have experienced pet loss, with new emergent themes.

Posttraumatic Growth Following the Loss of a Pet.

The current study examined Posttraumatic Growth (PTG) experienced by bereaved pet owners in the United States, French-Canada, Japan, and Hong Kong following the death of their pet. Using qualitative methodology, we analyzed responses of participants who answered "yes" to a question about experiencing PTG and explored to what extent the cross-cultural responses mapped onto the five factors of the Posttraumatic Growth Inventory (PTGI). For the U.S. sample, 58% of responses mapped onto the PTGI. For French-Canada, 72% of responses mapped onto the PTGI. For Japan, 50% of responses mapped onto the PTGI and for Hong Kong, 39% of responses mapped onto the PTGI. We also explored emergent categories related to PTG for individuals who have lost a pet and discerned the unique aspects for PTG across cultures.

Health-Related Quality of Life among Pediatric Hematopoietic Stem Cell Donors.

OBJECTIVES: To examine health-related quality of life (HRQoL) among sibling pediatric hematopoietic stem cell donors from predonation through 1 year postdonation, to compare donor-reported HRQoL scores with proxy-reports by parents/guardians and those of healthy norms, and to identify predonation factors (including donor age) potentially associated with postdonation HRQoL, to better understand the physical and psychosocial effects of pediatric hematopoietic stem cell donation. STUDY DESIGN: A random sample of 105 pediatric donors from US centers and a parent/guardian were interviewed by telephone predonation and 4 weeks and 1 year postdonation. The interview included sociodemographic, psychosocial, and HRQoL items. A sample of healthy controls matched to donors by age, gender, and race/ethnicity was generated. RESULTS: Key findings included (1) approximately 20% of donors at each time point had very poor HRQoL; (2) child self-reported HRQoL was significantly lower than parent proxy-reported HRQoL at all 3 time points and significantly lower than that of norms at predonation and 4 weeks postdonation; and (3) younger children were at particular risk of poor HRQoL. CONCLUSIONS: Additional research to identify the specific sources of poorer HRQoL among at-risk donors (eg, the donation experience vs having a chronically ill sibling) and the reasons that parents may be overestimating HRQoL in their donor children is critical and should lead to interventions and policy changes that ensure positive experiences for these minor donors.

Health-Related Quality of Life in Patients with MPS II.

Mucopolysaccharidosis type II (MPS II), also known as Hunter syndrome, is a chronic and progressive X-linked lysosomal disease that mainly affects males. The National MPS Society (2013) reports that MPS II affects 1 in 100,000 to 1 in 150,000 males worldwide. Two distinct forms of the disease are based on age of onset and clinical course: attenuated and severe. MPS II affects many organ systems including the nervous, cardiovascular, gastrointestinal and respiratory systems. Clinical manifestations can include progressive hearing loss, mental impairment, and enlarged liver and spleen. This study focuses on the health-related quality of life of individuals (HRQOL) with MPS II as measured by the parent and self-report versions of the Pediatric Quality of Life Inventory (PedsQL™). Both parents of patients with MPS II as well as patients themselves reported lower scores on all domains of the PedsQL™ (physical, emotional, social and school functioning) indicating that children with MPS II have an overall lower HRQOL when compared to a healthy sample. When compared with patients with other chronic illnesses (cancer, MSUD, galactosemia,), the MPS II sample had significantly lower scores on a number of PedsQL™ scales, suggesting an overall lower HRQOL. No significant relationships were found using scores from parent or self report PedsQL™ measures and length of time on ERT.

The neurocognitive impact of Fabry disease on pediatric patients.

Fabry disease (FD) is an X-linked lysosomal storage disorder that results in progressive multisystemic organ complications. Several studies have examined neurocognitive impairments in adults; however, there is a paucity of research examining neurocognitive functioning in children with FD. This is the first exploratory study to examine the neurocognitive functioning of pediatric patients with FD and to evaluate the effects of enzyme replacement therapy (ERT) on neurocognitive functioning within this population. Families attending a national conference with at least one child with FD and one parent affected by FD comprised the sample (n = 48; 24 pediatric patients, 24 parents). Pediatric participants (10 males, 14 females) between the ages of 6 and 18 years and their parent(s) were involved in the study. Data from a demographic questionnaire and two neurocognitive self-report and parent-report measures were analyzed. Parent reports of neurocognitive functioning were also compared to a sample of children with and without head injury and to a sample of children who had undergone liver transplant (LT). Children with FD had poorer cognitive and executive functioning than healthy peers, and were comparable to children with head injury and LT. In addition, children using ERT had higher scores on measures of overall cognitive functioning, as well as fewer problems with attention/working memory and executive functioning. Results of this study suggest that children with FD may exhibit poorer cognitive and executive functioning relative to healthy peers. The use of ERT may mitigate the negative impact of FD on neurocognitive functioning in pediatric patients.

MPS II: adaptive behavior of patients and impact on the family system.

Mucopolysaccharidosis type II (MPS II), also known as Hunter syndrome, is a chronic and progressive X-linked lysosomal disease that mainly affects males. It occurs in 1 in every 65,000 to 1 in 132,000 births. There are two distinct forms of the disease based on age of onset and clinical course: mild and severe. MPS II affects many organ systems including the nervous, cardiovascular, gastrointestinal and respiratory systems. Complications can include vision problems, progressive hearing loss, thickened and elastic skin, mental impairment, and enlarged liver and spleen. We herein focus on the adaptive behavior of individuals with MPS II, and the impact of MPS II on the family system. Outcomes from the Vineland-II Adaptive Behavior Scales showed that the MPS II patient sample experienced significantly lower functioning in communication, daily living skills, socialization, and motor skills compared to normative data. Patients with severe MPS II were found to have significantly lower adaptive functioning in all domains, as compared to those with mild MPS II. Length of time on ERT had no significant relationship to adaptive functioning. Results from the Peds QL Family Impact Module indicated that families of patients with MPS II experienced a lower overall health-related quality of life and overall lower family functioning (including lower emotional and cognitive functioning) than those with chronic illnesses residing in an inpatient setting.

International genetic counseling students' perspective on their training experience in the United States.

International students face social, psychological and academic challenges upon moving to a foreign country to pursue higher education. Clinical disciplines such as genetic counseling present additional challenges adapting to an unfamiliar health care system and different interactions and expectations with patients and colleagues. This study used semi-structured interviews to identify challenges that international genetic counseling students face during training in the United States. Eight international genetic counseling alumni who graduated from U.S.-accredited programs were interviewed. Participants stated that the U.S. academic system was unfamiliar-class participation and paper-writing required the greatest adjustment. There was a need for help in understanding social norms in academic settings. Clinically, they were unfamiliar with the dynamics and communication style of U.S. families. Non-native English speakers experienced greater difficulty in all areas. Most participants reported that they were uncomfortable asking for help in transitioning to life, study and work. Participants identified mentorship programs for international students as potentially useful in clarifying expectations in academic and clinical settings. These results may assist international students preparing to study genetic counseling in the U.S. and may help genetic counseling training programs identify the academic and clinical challenges faced by international students.

Inborn errors of metabolism: psychosocial challenges and proposed family systems model of intervention.

Inborn errors of metabolism result in psychosocial crises that challenge individual and familial modes of functioning across the life cycle. Increased stress, mood disorders, interpersonal challenges, decreased quality of life, and grief reactions are all common for patients and their families. To effectively care for these patients, a holistic approach to their care, which incorporates their social context, is essential. Patients and their families need support as they focus on immediate practical demands, grieve over illness-related losses, and reorient future expectations. A family systems based model provides a flexible and individualized approach to care that allows for optimal psychosocial adjustment throughout the disease process.

Young adults with MSUD and their transition to adulthood: psychosocial issues.

Maple Syrup Urine Disease (MSUD) is an autosomal recessive condition with an incidence of 1 in 185,000 births worldwide. Regardless of the type of MSUD, treatment includes immediate and lifelong dietary restriction of isoleucine, leucine and valine. There is little known about the psychosocial impact of MSUD on the developmental milestones of emerging adulthood. We used a qualitative case study approach to explore the human experiences of MSUD on young adults (n = 8) and parents (n = 8). All participants were administered a semi-structured, qualitative interview as well as quantitative measures. Six core themes emerged: 1) lifelong strain of dietary management; 2) social isolation from peers and impact on dating; 3) impact of MSUD on academics and employment; 4) medical experiences and transition to adult care; 5) impact on family functioning; and 6) positive effects and growth. The results of this investigation highlight and expand awareness of the psychological and social needs of young adults with MSUD. This study calls for a collaborative, multidisciplinary effort in the treatment of these patients and their families.

Mass Depopulation of Swine during COVID-19: An Exploration of Swine Veterinarians' Perspectives.

This qualitative study (n = 25) was created to better understand the mental health of U.S. swine veterinarians who were involved in the mass depopulation events related to COVID-19. A total of 25 swine veterinarians, participants in a previous larger quantitative study, volunteered to be interviewed about their experiences related to the COVID-19 mass depopulation event. Themes that emerged from these interviews included: (1) the need to be better prepared for crisis events; (2) lack of public understanding; (3) moral distress; (4) empathy for others, especially young veterinarians; (5) sources of support; (6) pride, honor and gratitude; and (7) an overarching theme of emotional distancing and detachment-concerns external to one's own mental health. Based on our results, we recommend additional training and supportive services for those who might be involved in future depopulation efforts. Additionally, we suggest that the veterinary profession prioritize public education campaigns to help the public better understand the need for depopulation.

US Adults' Perceptions of Dog Breed Bans, Dog Aggression and Breed-Specific Laws.

Dog aggression directed towards people is a leading reason for relinquishment and a major public health hazard. In response to the threat of dog aggression and dog bites, breed-specific legislation has been introduced in numerous cities within the United States and countries throughout the world. There is limited evidence, however, to suggest that such laws are effective. This study explored, through an online, anonymous, cross-sectional survey, US residents' views about the bite risk of common dog breeds, breed-specific legislation, and alternative options for improved public safety. A total of 586 surveys were completed by adult US residents, 48.8% female and 48.6% male. Approximately half of the respondents reported feeling that dog bites are a serious public health issue. Although 70% of respondents were opposed to a breed ban, only 56% felt that banning specific breeds creates an animal welfare issue. Females were less likely to support a ban or agree that specific breed bans improve public safety. When participants were asked to indicate their support of several alternatives to breed-specific legislation, the most frequently endorsed options included public education about animal welfare and animal behavior, and stricter leash laws. Further research pertaining to the most effective public education dissemination methods is warranted.

Disenfranchised Guilt-Pet Owners' Burden.

Guilt refers to an unpleasant emotional state associated with one's behaviors, thoughts, or intentions, and it is based on the possibility that one may be in the wrong or that others may have this perception. Parental guilt is one common subtype and is often associated with work-family conflict (WFC). WFC and related guilt have been found to be associated with depression and anxiety. Through an online anonymous survey, the current study was designed to explore dog owners' guilt surrounding their dogs. Results suggest that dog owners' guilt and WFC associated with their dog are at levels similar to those reported in human family studies. Additionally, the relationship between dog owners' guilt and discrepancy between participants' actual and ideal self, in regard to the role of a dog owner, also mirrored human-only family research. Because pet-related guilt is unrecognized, acknowledged, or supported, we suggest it is disenfranchised. As people return to work, in this period of post-COVID-19 pandemic time, it is paramount that companies and communities acknowledge pet owner guilt and WFC and help owners find practical, effective solutions.

"I Couldn't Have Asked for a Better Quarantine Partner!": Experiences with Companion Dogs during Covid-19.

The Covid-19 pandemic has been found to negatively impact the psychological well-being of significant numbers of people globally. Many individuals have been challenged by social distancing mandates and the resultant social isolation. Humans, in our modern world, have rarely been as isolated and socially restricted. Social connectedness and support are critical protective factors for human survival and well-being. Social isolation can lead to loneliness, boredom, and can become a risk factor for physical and mental health issues such as anxiety and depression. The attachments formed with dogs, however, can be as strong or even stronger than human connections, and has been shown to relate to fewer physical health and mental health problems, as well as decrease isolation and loneliness. The purpose of this qualitative research was to examine the thoughts, experiences and concerns of 4105 adults regarding their companion dog during the initial months of Covid-19. Data were collected between March 31st-April 19th, 2020 via online survey and themes were coded by the researchers using directed content analysis. Results highlighted a strong human-animal appreciation, and that dog ownership during this pandemic diminished participants' sense of isolation and loneliness, as well as supported their mental/physical health.

The Initial Months of COVID-19: Dog Owners' Veterinary-Related Concerns.

Veterinarians, like many other professions, were significantly impacted by the onset of COVID-19 in the spring of 2020. Standard practices were disrupted, and veterinary hospitals had to quickly modify standard protocols to safely serve their clients and patients. The purpose of this study was to better understand dog owners' fears and concerns pertaining to veterinary care and obtainment of pet care products and food during the lock down phase of a pandemic to be better prepared to address these concerns now and in the future. To this end, an online, anonymous, cross-sectional survey was designed and distributed to adult dog owners via social media. The results, from a total of 4,105 participants (the majority from the United States and Canada), indicated substantial areas of concern. The number one concern of dog owners during this time was the availability of emergency veterinary care. Owners under 30 years of age, compared to older owners, were significantly more concerned about both availability and cost of veterinary care (emergency and non-emergency). The ability to care for one's dog if they were to become ill was a concern for many owners, yet only 60% had identified a caretaker for their dog if one was needed. These results suggest that the majority of dog owners remained true steadfast guardians of their dogs, continuing to make them a priority, even during pandemic times. Suggestions to help mitigate dog owners' concerns and improve communication between owners and veterinarian teams are offered.