The Transition to Adulthood for Youth Living with Rare Diseases.

More children with rare diseases survive into adulthood. The transition period to adult healthcare presents many challenges for pediatric rare diseases. Few adolescents or their families receive any transitional support for the transition to adult healthcare or for their maturing psychosocial needs. Understanding the challenges in the transition process is critical to ensure that interventions designed to improve the transition are holistic and meet the needs of the youth and their families. Few transition programs are in place to meet the needs of those youth with rare diseases who cannot participate in medical decision making or who live independently because of severe disabilities and comorbidities. We searched the literature on preparation and outcomes for youth living with rare diseases in PubMed, CINAHL, and PsychInfo, excluding publications before 2010. The results revealed seven studies specific to rare diseases, special needs, or chronic conditions. Next, we discussed transition with experts in the field, GotTransition.org, and citation chaining, yielding a total of 14 sources. The barriers and challenges to transition were identified. Articles discussing solutions and interventions for transition in medically complex children were categorized care coordination or transition readiness. A large portion of children with rare disease are underserved and experience health disparities in transition.

Shared spiritual beliefs between adolescents with cancer and their families.

BACKGROUND: FAmily CEntered (FACE) Advance Care Planning helps family decision makers to understand and honor patients' preferences for future health care, if patients cannot communicate. Spiritual well-being is a key domain of pediatric oncology care and an integral dimension of pediatric advance care planning. PROCEDURE: As part of four-site randomized controlled trial of FACE for teens with cancer, the functional assessment of chronic illness therapy-spiritual well-being- version 4 (FACIT-Sp-EX-4) was completed independently by 126 adolescents with cancer/family dyads. The prevalence-adjusted and bias-adjusted kappa (PABAK) measured congruence on FACIT-Sp-EX-4. RESULTS: Adolescents (126) had mean age of 16.9 years, were 57% female and 79% White. Religious/spiritual classifications were: Catholic (n = 18), Protestant (n = 76), Mormon (n = 3), none/atheist (n = 22), other (n = 5), and unknown (n = 2). Agreement at item level between spiritual well-being of adolescents and families was assessed. Three items had ≥90% agreement and Excellent PABAK: "I have a reason for living," "I feel loved," "I feel compassion for others in the difficulties they are facing." Three items had <61% agreement and Poor PABAK: "I feel a sense of harmony within myself," "My illness has strengthened my faith or spiritual beliefs," "I feel connected to a higher power (or God)." Dyadic congruence was compared by social-demographics using median one-way analysis. Male family members (median = 72%) were less likely to share spiritual beliefs with their adolescent than female family members (median = 83%), P = .0194. CONCLUSIONS: Family members may not share spiritual beliefs with adolescents and may be unaware of the importance of spiritual well-being for adolescents.

Association of Religious and Spiritual Factors With Patient-Reported Outcomes of Anxiety, Depressive Symptoms, Fatigue, and Pain Interference Among Adolescents and Young Adults With Cancer.

Importance: The associations of spiritual and religious factors with patient-reported outcomes among adolescents with cancer are unknown. Objective: To model the association of spiritual and religious constructs with patient-reported outcomes of anxiety, depressive symptoms, fatigue, and pain interference. Design, Setting, and Participants: This cross-sectional study used baseline data, collected from 2016 to 2019, from an ongoing 5-year randomized clinical trial being conducted at 4 tertiary-referral pediatric medical centers in the US. A total of 366 adolescents were eligible for the clinical trial, and 126 were randomized; participants had to be aged 14 to 21 years at enrollment and be diagnosed with any form of cancer. Exclusion criteria included developmental delay, scoring greater than 26 on the Beck Depression Inventory II, non-English speaking, or unaware of cancer diagnosis. Exposures: Spiritual experiences, values, and beliefs; religious practices; and overall self-ranking of spirituality's importance. Main Outcomes and Measures: Variables were taken from the Brief Multidimensional Measurement of Religiousness/Spirituality (ie, feeling God's presence, daily prayer, religious service attendance, being very religious, and being very spiritual) and the spiritual well-being subscales of the Functional Assessment of Chronic Illness Therapy (meaning/peace and faith). Predefined outcome variables were anxiety, depressive symptoms, fatigue, and pain interference from Patient-Reported Outcomes Measurement Information System pediatric measures. Results: A total of 126 individuals participated (72 [57.1%] female participants; 100 [79.4%] white participants; mean [SD] age, 16.9 [1.9] years). Structural equation modeling showed that meaning and peace were inversely associated with anxiety (ß = -7.94; 95% CI, -12.88 to -4.12), depressive symptoms (ß = -10.49; 95% CI, -15.92 to -6.50), and fatigue (ß = -8.90; 95% CI, -15.34 to -3.61). Feeling God's presence daily was indirectly associated with anxiety (ß = -3.37; 95% CI, -6.82 to -0.95), depressive symptoms (ß = -4.50; 95% CI, -8.51 to -1.40), and fatigue (ß = -3.73; 95% CI, -8.03 to -0.90) through meaning and peace. Considering oneself very religious was indirectly associated with anxiety (ß = -2.81; 95% CI, -6.06 to -0.45), depressive symptoms (ß = -3.787; 95% CI, -7.68 to -0.61), and fatigue (ß = -3.11, 95% CI, -7.31 to -0.40) through meaning and peace. Considering oneself very spiritual was indirectly associated with anxiety (ß = 2.11; 95% CI, 0.05 to 4.95) and depression (ß = 2.8, 95% CI, 0.07 to 6.29) through meaning and peace. No associations were found between spiritual scales and pain interference. Conclusions and Relevance: In this study, multiple facets of spirituality and religiousness were associated with anxiety, depression, and fatigue, all of which were indirectly associated with the participant's sense of meaning and peace, which is a modifiable process. Although these results do not establish a causal direction, they do suggest palliative interventions addressing meaning-making, possibly including a spiritual or religious dimension, as a novel focus for intervention development.

The Role of Religiousness and Spirituality in Health-related Quality of Life of Persons Living with HIV: A Latent Class Analysis.

PURPOSE: Understanding how religion and spirituality influence health-related quality of life (HRQoL) is important for developing holistic, patient-centered treatment. This study determined distinct latent classes of religiousness/spirituality for adult persons living with HIV (PLWH) and associations between latent class membership and HRQoL. METHODS: Baseline data was collected from 223 patients in the FAmily CEntered (FACE) Advance Care Planning (ACP) clinical trial for PLWH. Patients completed the Brief Multidimensional Measurement of Religiousness/Spirituality (BMMRS-adapted), Patient Health Questionnaire (PHQ-9), Medical Outcome Study-HIV Survey (MOS-HIV) and the Palliative care Outcome Scale (POS). The optimal number of latent classes was determined by comparing K-Class model with (K-1)-Class model iteratively. The relationship between latent class membership, HRQoL and demographic characteristics was assessed. RESULTS: Patients were 56.1% male, 86.1% Black/African American, aged 22-77 (Mean=50.83). 75.3% were Christian. LCA identified three classes: traditionally religious (35%), privately religious (47.1%), and spiritual but not religious (17.9%). MOS-HIV mental health (p < 0.001), MOS-HIV quality of life (p = 0.014) and MOS-HIV health transition (p = 0.016) scores were significantly higher among patients who were traditionally religious. These patients were more likely to be 40+ years and Black. Patients in the privately religious group had the lowest levels of mental health and HRQoL. CONCLUSIONS: Understanding how religiousness and spirituality influence HRQoL can help the medical community develop holistic, patient-centered and culturally-sensitive interventions that could improve outcomes for PLWH and potentially mitigate the impact of health disparities within the Black and LGBTQ communities.

The influence of religious beliefs and practices on health care decision-making among HIV positive adolescents.

It is unknown if religiousness/spirituality influences end-of-life treatment preferences among adolescents. Investigators assessed whether religiousness/spirituality moderates the relationship between an advance care planning intervention and end-of-life treatment preferences among 85 primarily African-American adolescents living with HIV/AIDS in outpatient-hospital-based HIV-specialty clinics in the United States. Adolescents aged 14-21 years living with HIV/AIDS and their families were randomized to three-weekly-60-minute sessions either: advance care planning (survey, goals of care conversation, advance directive); or control (developmental history, safety tips, nutrition/exercise). At 3-months post-intervention the intervention effect (decreasing the likelihood of choosing to continue treatments in all situations) was significantly moderated by religiousness/spirituality. Highly religious/spiritual adolescents were four times more likely to choose to continue treatments in all situations. Thus, intensive treatments at end-of-life may represent health equity, rather than health disparity. The belief believed that HIV is a punishment from God at baseline (15%, 14/94) was not associated with end-of-life treatment preferences. Twelve percent (11/94) reported they had stopped taking HIV medications for more than 3 days because of the belief in a miracle. Religiousness moderates adolescent's medical decision-making. Adolescents who believe in miracles should receive chaplaincy referrals to help maintain medication adherence.

Interdisciplinary Communication: Documentation of Advance Care Planning and End-of-Life Care in Adolescents and Young Adults With Cancer.

Advance care planning is being increasingly recognized as a component of quality in end-of-life care, but standardized documentation in the electronic health record has not yet been achieved, undermining interdisciplinary communication about care needs and limiting research opportunities.We examined the electronic health records of nine adolescent and young adults with cancer who died after participation in an advance care planning clinical trial (N = 30). In this secondary analysis of this subgroup, disease trajectory and end-of-life information were abstracted from the electronic health record, and treatment preferences from the original study were obtained.All deceased participants older than 18 years had a surrogate decision maker identified in the electronic health record, and all deceased participants had limitations placed on their care, varying from 1.5 hours up to 2 months before death. However, assessment of relations between treatment preferences and end-of-life care was difficult and revealed the presence of circumstances that advance care planning is designed to avoid, such as family conflict. Lack of an integrated health care record regarding advance care planning and end-of-life care makes both care coordination and examination of the association between planning and goal concordant care more difficult.

The Role of Religiousness/Spirituality in Health-Related Quality of Life Among Adolescents with HIV: A Latent Profile Analysis.

The purpose of this study was to determine whether distinct latent profiles of religiousness/spirituality exist for ALWH, and if so, are latent profile memberships associated with health-related quality of life (HRQoL). Latent profile analysis of religiosity identified four profiles/groups. Compared to the other three groups, higher levels of emotional well-being were found among young perinatally infected adolescents who attended religious services, but who did not pray privately, feel God's presence or identify as religious or spiritual. Social HRQoL was significantly higher among the highest overall religious/spiritual group. Understanding adolescent profiles of religiousness/spirituality on HRQoL could inform faith-based interventions. Trial registration NCT01289444.

A longitudinal, randomized, controlled trial of advance care planning for teens with cancer: anxiety, depression, quality of life, advance directives, spirituality.

PURPOSE: To test the feasibility, acceptability and safety of a pediatric advance care planning intervention, Family-Centered Advance Care Planning for Teens With Cancer (FACE-TC). METHODS: Adolescent (age 14-20 years)/family dyads (N = 30) with a cancer diagnosis participated in a two-armed, randomized, controlled trial. Exclusion criteria included severe depression and impaired mental status. Acceptability was measured by the Satisfaction Questionnaire. General Estimating Equations models assessed the impact of FACE-TC on 3-month post-intervention outcomes as measured by the Pediatric Quality of Life Inventory 4.0 Generic Core Scale, the Pediatric Quality of Life Inventory 4.0 Cancer-Specific Module, the Beck Depression and Anxiety Inventories, the Spiritual Well-Being Scale of the Functional Assessment of Chronic Illness Therapy-IV, and advance directive completion. RESULTS: Acceptability was demonstrated with enrollment of 72% of eligible families, 100% attendance at all three sessions, 93% retention at 3-month post-intervention, and 100% data completion. Intervention families rated FACE-TC worthwhile (100%), whereas adolescents' ratings increased over time (65%-82%). Adolescents' anxiety decreased significantly from baseline to 3 months post-intervention in both groups (ß = -5.6; p = .0212). Low depressive symptom scores and high quality of life scores were maintained by adolescents in both groups. Advance directives were located easily in medical records (100% of FACE-TC adolescents vs. no controls). Oncologists received electronic copies. Total Spirituality scores (ß = 8.1; p = .0296) were significantly higher among FACE-TC adolescents versus controls. The FACE-TC adolescents endorsed the best time to bring up end-of-life decisions: 19% before being sick, 19% at diagnosis, none when first ill or hospitalized, 25% when dying, and 38% for all of the above. CONCLUSIONS: Family-Centered Advance Care Planning for Teens With Cancer demonstrated feasibility and acceptability. Courageous adolescents willingly participated in highly structured, in-depth pediatric advance care planning conversations safely.

Spiritual well-being among HIV-infected adolescents and their families.

Congruence in spirituality between HIV+ adolescent (n = 40)/family (n = 40) dyads and psychological adjustment and quality of life were assessed, using the Spiritual Well-Being Scale of the Functional Assessment of Chronic Illness Therapy, Beck Depression Inventory-II, Beck Anxiety Inventory and Pediatric Quality of Life Inventory at baseline and 3-month post-intervention. Adolescents were 60 % female and 92 % African American. Congruence in spirituality between adolescent/surrogate dyads remained unchanged at 3 months. High congruence existed for "having a reason for living"; rejection of "life lacks meaning/purpose" and "HIV is a punishment from God." Adolescents were less likely to forgive the harm others caused them than their families.

A profile of children with complex chronic conditions at end of life among Medicaid beneficiaries: implications for health care reform.

BACKGROUND: As the United States braces for full implementation of health care reform, the eyes of the nation are on Medicaid. The large number of newly eligible Medicaid beneficiaries may challenge health care resources and ultimately impact quality of care. This is a special concern among current Medicaid beneficiaries such as children with complex chronic conditions (CCCs) who have significant health care needs, especially at end of life (EOL). Yet, a comprehensive profile of these children is lacking. OBJECTIVE: To understand the demographic and health characteristics, health care utilization, and expenditures among Medicaid children with CCCs at EOL. METHODS: Our study used a retrospective cohort design with data from the 2007 and 2008 California Medicaid data files. Descriptive statistics were used to profile children in the last year of life. RESULTS: We found a diverse group of children who suffered with serious, multiple chronic conditions, and who accessed comprehensive, multidisciplinary care. Most children had neuromuscular conditions (54%), cardiovascular conditions (46%), and cancer (30%). A majority (56%) had multiple CCCs. Children with CCCs received comprehensive care including hospital inpatient (67%), primary (82%), ancillary (87%), and other acute care services (83%); however, few children utilized hospice and home health care services (26%). Significant age differences existed among the children. CONCLUSIONS: The current California Medicaid system appears to provide comprehensive care for children at EOL. The underutilization of hospice and home health services, however, represents an opportunity to improve the quality of EOL care while potentially reducing or remaining budget neutral.

An exploratory study of HIV+ adolescents' spirituality: will you pray with me?

The aims of the study were (1) to determine whether adolescents find it acceptable to have physicians explore their spiritual beliefs as part of their medical care, (2) to characterize the role of spirituality and religious beliefs in adolescents with and without HIV, and (3) to examine associations between spirituality/religion and quality of life. Adolescents receiving their medical care at an urban Adolescent Health Clinic completed a study-specific questionnaire about spiritual inquiry by their physician, the Brief Multidimensional Measurement of Religiousness/Spirituality, and the Pediatric Quality of Life Inventory 4.0. Chi-squared analysis, Fischer's exact test, and t tests were used to assess associations. A total of 45 participants enrolled: 19 HIV+ (53% vertical transmission) and 26 HIV-; mean age 17.2 years; 80% African American. Four out of 45 (9%) had ever been asked by their doctor about their spiritual/religious beliefs, and only 8 (18%) had ever shared these beliefs with their healthcare provider. Most teens wanted their provider to ask them about their spiritual beliefs during some visits, especially when dealing with death/dying or chronic illness (67%). Those with HIV were more likely to endorse wanting their doctors to pray with them (42% vs. 15%), feeling "God's presence" (Mean = 3.95 vs. 2.83), being "part of a larger force" (Mean = 2.58 vs. 1.69), and feeling "God had abandoned them" (Mean = 1.63 vs. 1.15). There are certain circumstances in which healthcare providers should include a spiritual history with teenage patients. Few differences emerged in the teens studied with and without HIV.

Longitudinal Pediatric Palliative Care: Quality of Life & Spiritual Struggle (FACE): design and methods.

As life expectancy increases for adolescents ever diagnosed with AIDS due to treatment advances, the optimum timing of advance care planning is unclear. Left unprepared for end-of-life (EOL) decisions, families may encounter miscommunication and disagreements, resulting in families being charged with neglect, court battles and even legislative intervention. Advanced care planning (ACP) is a valuable tool rarely used with adolescents. The Longitudinal Pediatric Palliative Care: Quality of Life & Spiritual Struggle study is a two-arm, randomized controlled trial assessing the effectiveness of a disease specific FAmily CEntered (FACE) advanced care planning intervention model among adolescents diagnosed with AIDS, aimed at relieving psychological, spiritual, and physical suffering, while maximizing quality of life through facilitated conversations about ACP. Participants will include 130 eligible dyads (adolescent and family decision-maker) from four urban cities in the United States, randomized to either the FACE intervention or a Healthy Living Control. Three 60-minute sessions will be conducted at weekly intervals. The dyads will be assessed at baseline as well as 3-, 6-, 12-, and 18-month post-intervention. The primary outcome measures will be in congruence with EOL treatment preferences, decisional conflict, and quality of communication. The mediating and moderating effects of threat appraisal, HAART adherence, and spiritual struggle on the relationships among FACE and quality of life and hospitalization/dialysis use will also be assessed. This study will be the first longitudinal study of an AIDS-specific model of ACP with adolescents. If successful, this intervention could quickly translate into clinical practice.

Spirituality in HIV-infected adolescents and their families: FAmily CEntered (FACE) Advance Care Planning and medication adherence.

PURPOSE: To explore the effect of spirituality and religious beliefs on FAmily CEntered (FACE) Advance Care Planning and medication adherence among human immunodeficiency virus (HIV) positive adolescents and their surrogate decision-makers. METHODS: A sample of HIV-positive adolescents (n = 40) and their surrogates, aged ≥ 21 years, (n = 40), was randomized to an active Healthy Living Control group or the FACE Advance Care Planning intervention, guided by transactional stress and coping theory. Adolescents' spirituality and their belief that HIV is a punishment from God were assessed at baseline and 3 months after the intervention, using the Functional Assessment of Chronic Illness Therapy-Spiritual Well Being Scale, Expanded, Version 4. RESULTS: Control adolescents increased faith and meaning/purpose more than FACE adolescents (p = .02). At baseline, more behaviorally infected adolescents (16%) believed that HIV was a punishment from God as compared with those who were infected perinatally (8%). Adolescents endorsing that HIV was a punishment scored lower on spirituality (p = .05) and adherence to Highly Active Antiretroviral Therapy (HAART) (p = .04). Surrogates were more spiritual than adolescents (p ≤ .0001). CONCLUSION: Providing family support in a friendly, facilitated environment enhanced spirituality among adolescents. Facilitated family conversations had an especially positive effect on medication adherence and spiritual beliefs among behaviorally infected adolescents.