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<p>Advance Care Planning for Children</p><p>With Rare Diseases: A Pilot RCT</p><p>Maureen E. Lyon, PhD,a,b Jamie L. Fraser, MD, PhD,a,b Jessica D. Thompkins, BSN, RN, CPN,a Heidi Clark, MSN, CPN,a</p><p>Nicola Brodie, MD,a,b Kathryn Detwiler,a Clarivet Torres, MD,a Michael F. Guerrera, MD,a Tamiko Younge, MD,a</p><p>Samar Aoun, MPH, PhD,c Eduardo A. Trujillo Rivera, PhDa,b</p><p>abstractBACKGROUND AND OBJECTIVE: Pediatric rare diseases are often life-limiting conditions and/or require</p><p>constant caregiving. Investigators assessed the initial efficacy of the FAmily CEntered (FACE)</p><p>pediatric advance care planning (pACP), FACE-Rare, intervention on families’ quality of life.</p><p>METHODS: A pilot-phase, single-blinded, intent-to-treat, randomized controlled clinical trial enrolled</p><p>families from 1 pediatric quaternary hospital between 2021 and 2023. Intervention families</p><p>received 3 weekly 60-minute (FACE-Rare pACP) sessions: (1) Carer Support Needs Assessment</p><p>Tool or Action Plan, (2) Carer Support Needs Assessment Tol Action Plan Review, and (3) Pediatric</p><p>Next Steps: Respecting Choices pACP. Controls received treatment as usual (TAU). Outcome meas-</p><p>ures were Beck Anxiety Inventory, Family Appraisal of Caregiving, Functional Assessment of</p><p>Chronic Illness Therapy (FACIT)-Spirituality, and health care utilization. Generalized mixed effect</p><p>models with g response assessed the intervention effect at 3-month follow-up.</p><p>RESULTS: Children (n5 21) were aged 1 to 10 years, 48% male, 24% Black; and 100% technology</p><p>dependent. Primary family caregivers (n 5 21) were aged 30 to 43 years, 19% male, 19% Black;</p><p>and 27% household income below the Federal poverty level. Dyads underwent 1:1 randomization:</p><p>9 to FACE-Rare and 12 to TAU. TAU caregivers reported statistically lower meaning and peace</p><p>than FACE-Rare caregivers (0.9, P 5 .03, confidence interval [CI]: 0.75–0.99). Black caregivers re-</p><p>ported significantly less caregiver distress (0.7, P 5 .04, CI: 0.47–0.98) than non-Black caregivers.</p><p>Poor families reported more anxiety (3.5, P5 .002, CI: 1.62–7.94), more caregiver strain (1.2, P5</p><p>.006, CI: 1.07–1.42); and less family well-being (0.8, P5 .02, CI: 0.64–0.95).</p><p>CONCLUSIONS: FACEVR -Rare was feasible, acceptable, safe, and demonstrated initial efficacy, pro-</p><p>viding greater feelings of meaning and peace to caregivers. Poverty impacted well-being.</p><p>A multisite trial is needed to determine generalizability.</p><p>aChildren’s National Hospital, Washington, District of Columbia; bDepartment of Pediatrics, George Washington</p><p>University School of Medicine and Health Sciences, Washington, District of Columbia; and cPeron Institute, Palliative</p><p>Care, The Western University of Australia, Perth, Washington, Australia</p><p>Dr Lyon, in consultation with key stakeholders, conceptualized and designed the study, obtained</p><p>funding, drafted the initial manuscript, and reviewed and revised the manuscript; Ms Thompkins</p><p>trained 3 facilitators to certification in the pediatric Next Steps: Respecting Choices interview, and</p><p>critically reviewed the manuscript for important intellectual content; Drs Fraser, Brodie, Torres,</p><p>Guerrera, and Ms Clark facilitated recruitment from their clinics and reviewed study findings and</p><p>contributed to interpretation and revisions of the manuscript; Ms Detwiler is the mother of a child</p><p>with a rare disease who assisted with the conceptualization of the study and assisted in identifying</p><p>eligible patients in the Complex Care Clinic where she is employed; Dr Younge reviewed study</p><p>findings, contributed to interpretation and presentation of results, and reviewed and revised the</p><p>manuscript; Dr Aoun contributed to the conceptualization of the study intervention, and reviewed and</p><p>revised the manuscript; Dr Trujillo Rivera had full access to all the data in this study, analyzed, and</p><p>interpreted the data and takes responsibility for the integrity of the data and conducted the data</p><p>analysis and contributed to interpretation of the findings; and all authors approved the final</p><p>manuscript as submitted and agree to be accountable for all aspects of the work.</p><p>WHAT’S KNOWN ON THIS SUBJECT: Pediatric rare</p><p>diseases are often life-limiting conditions and/or may</p><p>require constant caregiving. These children remain</p><p>understudied and underserved. Few empirically validated</p><p>interventions exist to support their family caregivers with</p><p>respect to palliative care needs or end-of-life decision-</p><p>making.</p><p>WHAT THIS STUDY ADDS: We pilot tested an intervention</p><p>designed to support the palliative care needs of families</p><p>of children with rare disorders. Families randomized to</p><p>the FACE-Rare pediatric advance care planning intervention</p><p>reported significantly greater meaning and peace, a measure</p><p>of spiritual well-being, than controls.</p><p>To cite: Lyon ME, Fraser JL, Thompkins JD, et al. Advance</p><p>Care Planning for Children With Rare Diseases: A Pilot RCT.</p><p>Pediatrics. 2024;153(6):e2023064557</p><p>PEDIATRICS Volume 153, number 6, June 2024:e2023064557 ARTICLE</p><p>Downloaded from http://publications.aap.org/pediatrics/article-pdf/153/6/e2023064557/1659069/peds.2023-064557.pdf</p><p>by Universidade de São Paulo user</p><p>on 24 September 2024</p><p>A rare disease is defined as a condition affecting fewer</p><p>than 200000 persons in the United States.1 Rare diseases</p><p>are actually common with an estimated 10000 diseases af-</p><p>fecting 350 million people globally.2 Pediatric rare diseases</p><p>are often life-limiting conditions and/or may require cons-</p><p>tant caregiving.3–5 Eighty-two percent of hospitalized pa-</p><p>tients in tertiary pediatric hospitals have rare diseases.6</p><p>Yet, these children remain understudied and underserved.7</p><p>Thus, pediatric advanced care planning (pACP) is ap-</p><p>propriate for these children and their families.8–11 pACP</p><p>involves preparation and skill development to facilitate</p><p>discussions about future medical care choices, but pACP</p><p>is not yet a routine part of care.12 Because of prognostic</p><p>uncertainty, heavy care demands, and social isolation, rare</p><p>diseases can exact a severe emotional toll on families.13,14</p><p>Advanced care planning (ACP) has proven to significantly in-</p><p>crease communication and spiritual and emotional well-</p><p>being for patients and their families compared with controls.15–</p><p>22 To extend the benefits of pACP to families of children</p><p>with rare disorders and to fill the gap in evidence based in-</p><p>terventions,8 we pilot tested an adaptation of 2 evidence-</p><p>based interventions to meet the palliative care needs of chil-</p><p>dren with rare diseases and their families.</p><p>We hypothesized that, compared with treatment as</p><p>usual (TAU), family centered pediatric advance care plan-</p><p>ning for families of children living with a rare disorder</p><p>(FACE-Rare) family caregivers would report significantly</p><p>better emotional, spiritual, and family caregiving apprais-</p><p>als compared with controls. We also explored the impact</p><p>of FACE-Rare on child healthcare utilization.</p><p>METHODS</p><p>Study Design and Participants</p><p>We conducted a 2-armed, single-blinded, randomized</p><p>controlled clinical trial. Child eligibility criteria included</p><p>(1) $1.0 years and <18.0 years; (2) lack of capacity to</p><p>participate in health care decision making; (3) diagnosis</p><p>of a rare disorder; (4) no do-not-resuscitate order in</p><p>place; (5) not in the ICU; and (6) not in foster care. One</p><p>purpose of this study was to include children who have</p><p>rare diseases previously excluded from condition-specific</p><p>research or who have small studies of low quality.23–36</p><p>Family caregiver inclusion criteria were: (1) $18.0 years;</p><p>(2) legal guardian of child and child’s caregiver; (3) can</p><p>speak and understand English; (4) not known to be de-</p><p>velopmentally delayed; and (5) upon secondary screen-</p><p>ing following enrollment, not actively homicidal, suicidal,</p><p>or psychotic. If 2 caregivers of a child were enrolled,</p><p>then both completed assessments, but 1 was identified as</p><p>the primary caregiver for analysis.</p><p>Families were recruited from Children’s National Hos-</p><p>pital, a quaternary pediatric hospital. Researchers com-</p><p>pleted 2 days of training on the protocol. Standardized</p><p>procedures were enacted for validation of, implementation</p><p>of, and fidelity to the protocol. The Respecting Choices facil-</p><p>itator (research nurse) achieved certification based on pro-</p><p>gram competency-based criteria, which included 6.5 hours</p><p>of on-line training with professional continuing education</p><p>credits and 1 day of classroom skills-based education, fol-</p><p>lowed by a practice video-based demonstration of compe-</p><p>tency. Supervision was provided based on a review of</p><p>video recordings and a competency criteria checklist by</p><p>M.L. The facilitator or researcher was not involved in the</p><p>clinical care of any of the participants, so there was no risk</p><p>of contamination.</p><p>The institutional review board approved the protocol.</p><p>Participants provided written informed e-consent or waiver</p><p>of assent and were compensated. An external Safety Moni-</p><p>toring Committee monitored the trial on an annual basis. We</p><p>followed the Consolidated Standards of Reporting Trials</p><p>guidelines.</p><p>Procedures</p><p>Participants were recruited between July 6, 2021, and</p><p>May 31, 2023. To minimize gatekeeper bias, before study</p><p>initiation, investigator (M.L.) conducted educational fo-</p><p>rums to discuss the study and inclusion or exclusion cri-</p><p>teria. Hospital policy stopped recruitment during the</p><p>coronavirus disease 2019 (COVID-19) pandemic. We later</p><p>received IRB approval to use remote recruitment proce-</p><p>dures, including mailed recruitment letters, follow up by</p><p>e-mail or telephone, e-consents, e-waivers of assent, re-</p><p>mote assessments, and intervention sessions. Recruit-</p><p>ment was further delayed by staff illness and turnover.</p><p>For these reasons recruitment was extended.</p><p>The random allocation sequence was created by the</p><p>original study statistician. Randomization was computer</p><p>generated following completion of a baseline assessment</p><p>using a REDCap database. Employing an intent-to-treat</p><p>paradigm, dyads were randomly assigned to FACE-Rare</p><p>versus TAU at a 1:1 ratio. The FACE-Rare facilitator noti-</p><p>fied intervention or control families the day of random</p><p>assignment and scheduled the study visits. Allocation</p><p>was concealed from the Research Assistant-Assessor to</p><p>prevent bias during the administration of outcome ques-</p><p>tionnaires and process measures. Chart abstraction from</p><p>the electronic health record was conducted by the nurse-</p><p>facilitator who was not blinded to assignment.</p><p>Intervention and Treatment as Usual Control</p><p>FACE-Rare was developed and b-tested with family caregivers</p><p>of children with rare diseases.37 As part of a community-</p><p>based participatory process, families and the National Or-</p><p>ganization for Rare Disorders were interviewed by M.L.</p><p>and shared their preference that palliative care needs be as-</p><p>sessed before pACP. The intervention integrated 2 evidence-</p><p>based interventions37: (1) the pediatric Carer Support Needs</p><p>2 LYON et al</p><p>Downloaded from http://publications.aap.org/pediatrics/article-pdf/153/6/e2023064557/1659069/peds.2023-064557.pdf</p><p>by Universidade de São Paulo user</p><p>on 24 September 2024</p><p>Assessment Tool (pCSNAT-I),©38–42 adapted for use in pedi-</p><p>atrics during preliminary research7,37,42 and (2) the Respect-</p><p>ing Choices Next Steps Pediatric ACP conversation.43–45</p><p>Families randomized to the intervention engaged in 3 to 4</p><p>study sessions of approximately 45 minutes each, scheduled</p><p>weekly.</p><p>Session 1 was a facilitated conversation using the</p><p>pCSNAT process, during which the facilitator assessed</p><p>the prioritized palliative care needs of both the child</p><p>and the family and together developed a Shared Action</p><p>Plan. The pCSNAT is structured around 16 domains that</p><p>fall into 2 groups: those enabling the caregiver to care</p><p>and those that enable more direct support for the care-</p><p>giver. Four response options indicated the extent of the</p><p>support requirements, from “no more” to “very much</p><p>more” were recorded during the intervention and en-</p><p>tered into the REDCap database by the facilitator. See</p><p>Supplemental Table 3 for additional details.</p><p>Session 2 was a shared review of the pCSNAT Action</p><p>Plan with the facilitator of what the family accomplished or</p><p>obstacles encountered. The facilitator shared additional re-</p><p>sources and recorded questions the family should ask the</p><p>treating clinician at their child’s next medical visit.</p><p>Session 3 was a structured conversation using the Re-</p><p>specting Choices Next Steps Pediatric ACP conversation.</p><p>This session explored the families understanding of the</p><p>disorder, symptoms, fears, and hopes and then transi-</p><p>tioned into a process of exploring possible future medical</p><p>decisions consistent with their goals and values. Families</p><p>could complete an Advance Care Plan during this session</p><p>or schedule an additional visit. See Supplemental Table 3</p><p>for additional details. This session was videotaped for fi-</p><p>delity purposes.</p><p>Treatment as Usual Control</p><p>To minimize the burden to families, we chose a treat-</p><p>ment as usual (TAU) comparison condition. Both groups</p><p>were provided with an ACP handbook and a booklet of re-</p><p>sources provided by the National Alliance for Caregiving.</p><p>Data Source and Measures</p><p>All families completed questionnaires via telemedicine at</p><p>baseline, 4-weeks post intervention, and 4 months post-</p><p>baseline. Questionnaires were administered by trained,</p><p>blinded research assessors who were not the interven-</p><p>tion facilitator. To control for literacy and to ensure data</p><p>completeness, the blinded assessors read the questions</p><p>aloud on a shared screen and entered the family’s re-</p><p>sponses directly into the REDCap database.</p><p>The Demographic Data Form was administered at baseline</p><p>to obtain family-reported socio-demographic information, in-</p><p>cluding age, gender, race and ethnicity, education, employ-</p><p>ment status, and household income.</p><p>The Beck Anxiety Inventory46 (BAI) is a 21-item mea-</p><p>sure of anxiety rated on a 4-point Likert scale of symp-</p><p>toms over the past week. The BAI has demonstrated</p><p>reliability and validity to assess anxiety in individuals</p><p>aged 17 to 80 years. The BAI has good reliability and va-</p><p>lidity for family caregivers of seriously ill children.47</p><p>The Family Appraisal of Caregiving Questionnaire for</p><p>Palliative Care48 shows changes over time in caregivers’</p><p>impacts from caregiving. There are 25 items with 4 theo-</p><p>retically driven subscales: strain, distress, positive ap-</p><p>praisals, and family wellbeing. Scores are from 5 5 strongly</p><p>agree to 1 5 strongly disagree. It has good internal consis-</p><p>tency reliability and construct validity.48 The Family Ap-</p><p>praisal of Caregiving Questionnaire for Palliative Care has</p><p>been used in pediatric studies and demonstrated sensitivity</p><p>to change over time and intervention effects using the FACE</p><p>model for teens with cancer.49</p><p>The Functional Assessment of Chronic Illness Therapy -</p><p>Spiritual Well-being Scale - Expanded Version-450 (FACIT-Sp)</p><p>is a 23-item scale, focusing on the existential aspects of spir-</p><p>ituality. There are 2 subscales: meaning and peace and faith.</p><p>The higher the score, the better the spiritual well-being.</p><p>FACIT-Sp is culturally sensitive to those with nontheistic</p><p>beliefs. There is evidence of validity with adolescents51 and</p><p>parents of children with cancer.52 The FACIT-Sp has also</p><p>been used to study the spiritual well-being of adolescents</p><p>living with HIV and their families.53</p><p>Child healthcare utilization was defined54 as: initiation</p><p>of palliative care consultations, number of days in pallia-</p><p>tive care before death, hospitalizations, emergency de-</p><p>partment visits, ICU use, surgeries, and place of death.</p><p>Covariates of final models were child age, child gender,</p><p>child race, Federal poverty level, indicator treatment</p><p>group, and time of measurement.</p><p>Statistical Analysis</p><p>Sample Size and Power</p><p>A sample size of 32 dyads was proposed, based upon our</p><p>experience with previous pilot trials.16,18 We estimated</p><p>15% attrition with a final sample of 26 dyads at 3-month</p><p>post intervention.</p><p>Statistical Procedures</p><p>Analysis was by original assigned groups using the intent-</p><p>to-treat design (Fig 1). Data were entered into REDCap soft-</p><p>ware version 8.10.18-©2020 (Vanderbilt University).</p><p>For the primary outcome quality of life, the sample size</p><p>was N 5 21 dyads. Generalized</p><p>linear mixed effect models55</p><p>with random intercept per patient, g response and loga-</p><p>rithmic link function were used to model each of the out-</p><p>comes and to test the effect of the intervention on family</p><p>caregiver anxiety, spirituality subscales meaning and peace,</p><p>and caregiver appraisal subscales at 3 months postinterven-</p><p>tion, controlling for baseline levels. We considered more</p><p>PEDIATRICS Volume 153, number 6, June 2024 3</p><p>Downloaded from http://publications.aap.org/pediatrics/article-pdf/153/6/e2023064557/1659069/peds.2023-064557.pdf</p><p>by Universidade de São Paulo user</p><p>on 24 September 2024</p><p>than 65 variables as covariates for each of the models</p><p>(Table 1), but we only included the most significant from</p><p>a practical, statistical, and standardized point of view.56 All</p><p>variables in Table 1 were considered for each outcome in</p><p>the final model. Model variable selection was accomplished</p><p>using L1-penalized estimation and cross validation.57 We</p><p>kept the covariates treatment, age, race, indicator for being</p><p>under the poverty line, and indicator for initial visit or</p><p>3 months follow up in all our models regardless of statistical</p><p>significance. Final models are the results of the described</p><p>procedure.</p><p>Statistical significance was set to a 5 .05. The R (4.3.0</p><p>“Already Tomorrow”) statistical software was used for</p><p>data analysis.58 The main R packages used for analysis,</p><p>for model development and validation, table and plots</p><p>generations are tidyverse,59 glmmTMB,60 doParallel,61</p><p>glmmLasso, flextable, and table-1.62</p><p>RESULTS</p><p>Participant Characteristics</p><p>Children (N 5 21) were median aged 3 years (Quartile [Q]</p><p>1 5 1 year, Q3 5 6 years); 53% were female; 24% were</p><p>Black/African American; 62% were white; 14% were His-</p><p>panic or Latino; and 100% were technology dependent, de-</p><p>fined as use of a wheelchair, breathing machine, feeding</p><p>tube, or central line. (Table 1). Children had 17 unique rare</p><p>diagnoses, as well as multiple morbidities, described in Table 1.</p><p>Primary caregivers (N 5 21) were median aged 36 years</p><p>(Q1 5 33, Q3 5 42); 81% were female; 19% were Black;</p><p>Completed Baseline Assessment and Randomized: 21</p><p>Families (27 Caregivers)</p><p>Completed Process Measures:</p><p>12 Families (15 Caregivers)</p><p>Completed Process Measures:</p><p>8 Families (9 Caregivers)</p><p>Completed 3 Month Follow Up:</p><p>8 Families (9 Caregivers)</p><p>Completed Session 3:</p><p>8 Families (10 Caregivers)</p><p>Randomized to Treatment as Usual: 12</p><p>Families (16 Caregivers)</p><p>Screen Failure: 1 Family (1 Caregiver)</p><p>Completed 3 Month Follow Up: 11</p><p>Families (13 Caregivers)</p><p>Completed Session 2:</p><p>8 Families (10 Caregivers)</p><p>Completed Session 1:</p><p>8 Families (10 Caregivers)</p><p>Randomized to Intervention:</p><p>9 Families (11 Caregivers)</p><p>Consented and underwent secondary</p><p>screening: 22 Families (28 Caregivers)</p><p>Excluded: 9 Families</p><p>Ineligible: 2</p><p>Declined: 7</p><p>Time constraints: 2 Topic</p><p>Discomfort:1</p><p>Concerns about confidentiality:1</p><p>No reason given: 3</p><p>Responded, Assessed for Initial Eligibility: 32 Families</p><p>No Response: 52 Families</p><p>Total FACE-Rare Outreach: 84 Families</p><p>FIGURE 1</p><p>Consort diagram. Flow of participants through the trial.</p><p>4 LYON et al</p><p>Downloaded from http://publications.aap.org/pediatrics/article-pdf/153/6/e2023064557/1659069/peds.2023-064557.pdf</p><p>by Universidade de São Paulo user</p><p>on 24 September 2024</p><p>TABLE 1 Child, Caregiver, and Clinical Characteristics by Randomization Group</p><p>FACE-Rare Interventiona</p><p>(N 5 9 dyads)</p><p>Treatment As Usuala</p><p>(N 5 12 dyads)</p><p>Study Populationa</p><p>(N 5 21 dyads) P</p><p>Child characteristics</p><p>Age, years (median, [Q1, Q3]) 2 (1, 3) 5 (1, 10) 3 (1, 6) .16</p><p>Gender</p><p>Female 5 (56) 6 (50) 11 (52) 1</p><p>Male 4 (44) 6 (50) 10 (48)</p><p>Race</p><p>Black or African 4 (44) 1 (8) 5 (24) .05</p><p>White 3 (33) 10 (83) 13 (62)</p><p>More than 1 race 2 (22) 1 (8) 3 (14)</p><p>Ethnicity</p><p>Hispanic or Latino 2 (22) 1 (8) 3 (14) .75</p><p>Not Hispanic or Latino 7 (78) 10 (83) 17 (81)</p><p>Declined 0 (0) 1 (8) 1 (5)</p><p>Medical technology use</p><p>Any 7 (78) 11 (92) 18 (86) .78</p><p>Wheelchair 1 (11) 5 (42) 6 (29) .30</p><p>Nasal canula, BiPAP, or tracheostomy 1 (5) 4 (33) 5 (24) .51</p><p>Surgical feeding tube 7 (78) 7 (58) 14 (67) .64</p><p>Central line 2 (22) 1 (8) 3 (14) .79</p><p>Otherb 1 (11) 4 (33) 5 (24) .24</p><p>Caregiver characteristics</p><p>Age, years (median [Q1, Q3]) 36 (30, 37) 39 (35, 43) 36 (33, 42) .27</p><p>Race</p><p>Black or African 3 (33) 1 (8) 4 (19) .49</p><p>White or Caucasian 5 (56) 10 (83) 15 (71)</p><p>More than 1 race 1 (11) 1 (8) 2 (10)</p><p>Ethnicity</p><p>Hispanic or Latino 1 (11) 1 (8) 2 (10) 1</p><p>Not Hispanic or Latino 8 (89) 10 (83) 18 (86)</p><p>Declined 0 (0) 1 (8) 1 (5)</p><p>Religiousness</p><p>Slightly or not religious 4 (44) 6 (50) 10 (48) 1</p><p>Very or moderately religious 5 (56) 6 (50) 11 (52)</p><p>Education</p><p>High school diploma or GED 2 (22) 2 (17) 4 (19) .29</p><p>Some college; no degree 4 (44) 4 (33) 8 (38)</p><p>Bachelor degree 0 (0) 3 (25) 3 (14)</p><p>Graduate degree 2 (22) 2 (17) 4 (19)</p><p>Employment</p><p>Employed (full or parttime) 4 (44) 6 (50) 10 (48) .22</p><p>Student (Full or parttime) 2 (22) 0 (0) 2 (10)</p><p>Homemaker or other 1 (11) 5 (42) 6 (29)</p><p>Unemployed 2 (22) 0 (0) 2 (10)</p><p>Retired 0 (0) 1 (8) 1 (5)</p><p>Family income under 2020 FPL 3 (33) 3 (25) 6 (29) 1</p><p>Unstable housing 1 (11) 1 (8) 2 (10) 1</p><p>Partner status</p><p>Married or partnered 5 (56) 10 (83) 15 (71) .15</p><p>Divorced or single 4 (44) 2 (17) 6 (29)</p><p>Time caregiver has known diagnosis, months (median [Q1, Q3]) 15 (12, 36) 39 (23, 74) 24 (15, 64) .1</p><p>Clinical characteristics</p><p>Hospitalized during study period 2 (22) 5 (42) 7 (33) .65</p><p>Length of hospitalization, days (median [Q1, Q3]) 29 (28, 29) 14 (14, 15) 15 (14, 24) 1</p><p>Rare disease diagnosis</p><p>ANKRD11-related KBG syndromec 1 (5) 0 (0) 1 (5)</p><p>Arthrogryposis congenita with intestinal atresiad 1 (5) 0 (0) 1 (5)</p><p>Asparagine synthetase deficiencye 1 (5) 0 (0) 1 (5)</p><p>CHARGE syndromef,g 1 (5) 1 (5) 2 (10)</p><p>Chromosome 14q12 microdeletion FOXG1 syndromeh 1 (5) 0 (0) 1 (5)</p><p>PEDIATRICS Volume 153, number 6, June 2024 5</p><p>Downloaded from http://publications.aap.org/pediatrics/article-pdf/153/6/e2023064557/1659069/peds.2023-064557.pdf</p><p>by Universidade de São Paulo user</p><p>on 24 September 2024</p><p>TABLE 1 Continued</p><p>FACE-Rare Interventiona</p><p>(N 5 9 dyads)</p><p>Treatment As Usuala</p><p>(N 5 12 dyads)</p><p>Study Populationa</p><p>(N 5 21 dyads) P</p><p>Chromosome Xp11.1q11.2 deletion syndrome including WTX and</p><p>ARHGEF9, causing osteopathia striata with cranial sclerosis and</p><p>early infantile epileptic encephalopathyi</p><p>1 (5) 0 (0) 1 (5)</p><p>PTPN11 Noonan syndromej 1 (5) 0 (0) 1 (5)</p><p>Severe hemophilia Aj,k 1 (5) 1 (5) 2 (10)</p><p>Short bowel syndromel,m 1 (5) 1 (5) 2 (10)</p><p>b-propeller protein-associated neurodegenerationn 0 (0) 1 (5) 1 (5)</p><p>Brain injury of prematurity with periventricular leukomalaciao 0 (0) 1 (5) 1 (5)</p><p>Chromosome 17p13.3 microdeletion syndrome including the</p><p>PAFAH1B1 genep</p><p>0 (0) 1 (5) 1 (5)</p><p>Chromosome 1q43–1q44 deletionq 0 (0) 1 (5) 1 (5)</p><p>Cockayne syndromer 0 (0) 1 (5) 1 (5)</p><p>Congenital diaphragmatic hernias 0 (0) 1 (5) 1 (5)</p><p>Polycystic renal disease with Cloacal anomalyt 0 (0) 1 (5) 1 (5)</p><p>TWNK-associated mitochondrial depletion disorderu 0 (0) 1 (5) 1 (5)</p><p>Severe factor VII deficiencyv 0 (0) 1 (5) 1 (5)</p><p>BiPAP, bilevel positive pressure; CHARGE, coloboma, heart defects, atresia choanae, restricted growth; pACP, pediatric advance care plan.</p><p>a Data shown indicate n (%) unless otherwise indicated.</p><p>b Other medical technology includes communication devices, gastric pacemaker, hearing aids, hemodialysis catheter, ventriculoperitoneal shunt, and ventriculopleural shunt.</p><p>c Secondary diagnoses include history of refractory infantile spasms, Lennox-Gastaut epilepsy syndrome, global developmental delay, intraventricular hemorrhage, mild hearing</p><p>loss, mild aortic root dilation, hyperopia with Astigmatism, dysphagia, and chronic ear infection.</p><p>d Secondary diagnoses include short bowel syndrome, total parenteral nutrition dependence, gastrostomy tube dependence, bacterial overgrowth, severe obstructive sleep apnea, and</p><p>metabolic bone disease.</p><p>e Secondary diagnoses include global developmental delay, delayed myelination, infantile spasm, microcephaly, noisy breathing, seizure disorder, truncal hypotonia, and vision impairment.</p><p>f Secondary diagnoses for one patient include adenotonsillar hypertrophy, aspiration pneumonia, bilateral sensorineural hearing loss, chronic lung disease, gastrostomy jejunostomy tube</p><p>dependence, gastroesophageal reflux disease, global developmental delay, pulmonary valve stenosis, retching, stricture esophagus, tracheoesophageal fistula s/p repair, and tracheomalacia.</p><p>g Secondary diagnoses for 1 patient include alternating esotropia, bilateral sensorineural hearing loss, congenital chorioretinal coloboma of both eyes, congenital ptosis, constipation, tracheos-</p><p>tomy dependence, Duane anomaly of the eye, excessive oral secretions, gastroesophageal reflux disease, gastrostomy tube dependence, global developmental delay, monocular esotropia, atopic</p><p>dermatitis, premature birth at 35 to 36 wk completed weeks gestation and birth weight 2000 to 2499 g, reactive airway disease, sleep disturbance, strabismic amblyopia, and wheezing.</p><p>h Secondary diagnoses include agenesis of corpus callosum, developmental delay, epilepsy, bilateral vocal cord paresis, tracheostomy dependence, gastrostomy tube dependence,</p><p>eosinophilic esophagitis, cerebral palsy, premature ventricular contractions, and vagal bradycardia.</p><p>i Secondary diagnoses include anorectal malformation, cerebral venous thrombosis of sigmoid sinus, chronic constipation, chronic gastroesophageal reflux disease status post</p><p>Nissen fundoplication, chronic otitis media with effusion, communicating hydrocephalus status post ventriculopleural shunt, deep vein thrombosis of right upper extremity, ele-</p><p>vated lipoprotein A level, functional incontinence, gastrostomy tube dependent, global developmental delay, cleft palate, stroke, hypotonia, intermittent alternating esotropia, mac-</p><p>rocephaly, malrotation of intestine status post Ladd procedure, mixed receptive-expressive language disorder, multiple anomalies of brain, oropharyngeal dysphagia, Osteopathia</p><p>striata with cranial sclerosis, seizures, patent foramen ovale, Pierre Robin sequence, postural kyphosis, rampant dental caries, reactive airway disease, retraction nystagmus,</p><p>tracheostomy dependent, cleft palate, gastrostomy tube dependence, and ventricular septal defect.</p><p>j Secondary diagnoses include atrial septal defect, cardiac hypertrophy, chronic constipation, feeding difficulty, flexural eczema, gastrostomy tube dependence, global developmental delay,</p><p>inguinal testis of both sides, lagophthalmos of both upper eyelids, severe obstructive sleep apnea, slow wt gain in child, tonsillar hypertrophy, and undescended testicle of both sides.</p><p>k Secondary diagnoses for 1 patient include moderate persistent asthma, autosomal dominant interferon regulatory factor 8 deficiency, and von Willebrand disease.</p><p>l Secondary diagnoses for 1 patient include chronic constipation.</p><p>m Secondary diagnoses of 1 patient with this diagnosis include total parenteral nutrition dependence, gastrostomy tube dependence, and bacterial overgrowth.</p><p>n Secondary diagnoses of 1 patient with this diagnosis include total parenteral nutrition dependence, gastrostomy tube dependence, bacterial overgrowth, and intestinal failure-</p><p>associated liver disease.</p><p>o Secondary diagnoses include global developmental delay, Lennox-Gastaut syndrome, Autism, chronic constipation with recurrent urinary trach infections, dysphagia, gastro-</p><p>esophageal reflux disease, spasticity, asthma, food allergies, environmental allergies, cortical vision impairment, premature adrenarche, and hypercholesteremia.</p><p>p Secondary diagnoses include central visual impairment, chronic constipation, cortical visual impairment, lower extremity complex regional pain syndrome, epilepsy with contin-</p><p>uous spike wave during slow-wave sleep, feeding intolerance, focal epilepsy with impairment of consciousness, gastroesophageal reflux disease, gastrointestinal dysmotility, gas-</p><p>trojejunostomy tube dependence, global developmental delay, hip subluxation, incontinence, intermittent exotropia of left eye, neurogenic dysphagia, neuromuscular scoliosis of</p><p>the thoracolumbar region, spastic quadriplegic cerebral palsy, strabismic amblyopia, tonic clonic seizures, and varus deformity of hip.</p><p>p Secondary diagnoses include Klinefelter syndrome, lissencephaly, chronic constipation, chronic bilateral otitis media with effusion, delayed visual maturation, oropharyngeal</p><p>dysphagia, gastrostomy tube dependence, gastroesophageal reflux disease, global developmental impairment, hypotonia, history of intractable infantile spasms, intractable Len-</p><p>nox-Gastaut epilepsy syndrome with status epilepticus, mild reactive airways disease, monocular exotropia with other concomitances, and subglottic stenosis.</p><p>q Secondary diagnoses include chronic constipation, feeding difficulty, gastrostomy dependence, global developmental delay, hypotonia, intermittent monocular esotropia, intracta-</p><p>ble Lennox-Gastaut epilepsy syndrome, monocular exotropia, myopia of both eyes, obstructive sleep apnea, ketogenic diet therapy, regular astigmatism of both eyes, sialorrhea,</p><p>sleep disorder, snoring, strabismic amblyopia, and polymicrogyria.</p><p>r Secondary diagnoses include cerebellar ataxia, global developmental delay, muscle spasticity, localization-related epilepsy, gastrostomy tube dependence, gastroesophageal re-</p><p>flux disease, tremors, sensorineural hearing loss, and cataracts.</p><p>s Secondary diagnoses include chronic respiratory failure, gastrojejunostomy tube dependence, oropharyngeal dysphagia, recurrent acute otitis media, secundum atrial septal</p><p>defect, and tracheostomy dependence.</p><p>t Secondary diagnoses include end-stage renal disease status post renal transplant, autism, central sleep apnea, obstructive sleep apnea, and developmental dysplasia of the hip.</p><p>u Secondary diagnoses include global developmental delay, hypotonia, abnormal involuntary movements, seizures, oropharyngeal dysphagia, and gastrostomy dependence.</p><p>v Secondary diagnoses include global developmental delay, hydrocephalus status post ventriculoperitoneal shunt, dystonic cerebral palsy, encephalopathy, equinovarus deformity</p><p>of foot, spasticity, and visual impairment.</p><p>6 LYON et al</p><p>Downloaded from http://publications.aap.org/pediatrics/article-pdf/153/6/e2023064557/1659069/peds.2023-064557.pdf</p><p>by Universidade de São Paulo user</p><p>on 24 September 2024</p><p>71% were white; 10% were Hispanic/Latino; 29% had</p><p>household income less than the 2021 Federal poverty level</p><p>(FPL) guidelines63; 27% were full-time employed; and 19%</p><p>had a high school diploma or less.</p><p>Initial eligibility criteria were met by 32 dyads. (Fig 1). Of</p><p>these, 7 declined and 3 were ineligible. Of those who gave</p><p>reasons for declining, 2 families cited lack of time; 1 sited</p><p>topic discomfort; and 1 sited privacy concerns. One family af-</p><p>ter enrollment and randomization was determined to be ineli-</p><p>gible and withdrawn from the study, leaving a participation</p><p>rate of 72% (21 of 29). The predetermined sample size of 32</p><p>enrolled and eligible dyads was not achieved. Of those ran-</p><p>domized to the intervention arm, 100% attended sessions 1</p><p>through 3. Two families choose to split the Respecting</p><p>Choices interview into 2 parts: (1) goals of care conversa-</p><p>tion; (2) completion of an advance care plan. The overall re-</p><p>tention rate at 3-month follow-up was 90% (19 of 21),</p><p>exceeding our benchmark of 85% retention.</p><p>Intervention Effects on Meaning and Peace, Healthcare</p><p>Utilization, and Safety</p><p>Our models revealed that after adjusting for significant cova-</p><p>riates (Table 1 and Fig 2), TAU caregivers experienced on</p><p>average, statistically lower meaning and peace subscale score</p><p>than FACE-Rare caregivers (0.9 P 5 .032, confidence interval</p><p>[CI]: 0.8–1.0) (Table 2). See Supplemental Tables 4–12 for</p><p>detailed findings for other study outcomes and exact 2-sided</p><p>P values. No other intervention effects, nor age, sex, or eth-</p><p>nicity effects of children or caregivers, employment, or reli-</p><p>giousness were identified for study outcomes. No adverse</p><p>events were reported.</p><p>Racial Differences Between Groups Independent of</p><p>Intervention Effects</p><p>Regardless of intervention</p><p>assignment, Black compared with</p><p>non-Black caregivers were less likely to report, on average,</p><p>caregiver distress (0.7, P 5 .04, CI: 0.5–1.0). (Table 2).</p><p>Persons Experiencing Poverty Independent of</p><p>Intervention Effects</p><p>Regardless of intervention assignment, families with house-</p><p>hold incomes below the 2021 FPL, compared with those</p><p>with incomes above the 2021 FPL, were significantly more</p><p>likely to report anxiety (3.5, P 5 .002, CI: 1.6–7.9) and care-</p><p>giver strain (1.2, P 5 .006, CI: 1.1–1.4); less likely to report</p><p>family well-being (0.8, P 5 .02, CI: 0.6–1.0). (Table 2).</p><p>FIGURE 2</p><p>Estimated mean score ratios between indicated covariate level and baselines, N5 21.</p><p>PEDIATRICS Volume 153, number 6, June 2024 7</p><p>Downloaded from http://publications.aap.org/pediatrics/article-pdf/153/6/e2023064557/1659069/peds.2023-064557.pdf</p><p>by Universidade de São Paulo user</p><p>on 24 September 2024</p><p>Self-Identified Palliative Care Needs of Family Caregivers</p><p>The top 3 palliative care needs prioritized by families in the</p><p>intervention arm (N 5 11, 2 families had 2 caregivers partic-</p><p>ipate) were (1) having time for yourself in the day (n 5 8,</p><p>73%), (2) knowing what to expect in the future when caring</p><p>for your child (n 5 5, 46%), and (3) financial, legal, or work</p><p>issues (n 5 4, 36%). Traditional palliative care needs such</p><p>as managing your child’s symptoms or giving medicines and</p><p>equipment to help care for your child were not prioritized.</p><p>See Fig 3 for a complete list of the identified needs.</p><p>Child Healthcare Utilization</p><p>No deaths occurred during the study, nor was there initi-</p><p>ation of palliative care consultations or ICU use, surger-</p><p>ies, or emergency department visits. The number of</p><p>hospitalizations were few and can be found in Table 1.</p><p>The Impact of Coronavirus Disease 2019 and Use of</p><p>Telemedicine</p><p>To inform the transition during the COVID-19 pandemic</p><p>to the use of telemedicine and to understand how</p><p>COVID-19 may have influenced the results with respect</p><p>to decision making, we consulted with our Community</p><p>Advisory Board about their experience and how to pro-</p><p>ceed with the study. Here are 2 exemplary responses:</p><p>“My son has thrived medically because of COVID-19.</p><p>Mask wearing, hand-washing, [sic] and greatly reduced</p><p>exposure to other people and kids who might be sick</p><p>with any sort of contagious illness have kept him healthy</p><p>in ways we had not previously experienced… .”</p><p>“Although COVID-19 changed a lot with regards to work and</p><p>school or school programs for the children-which impacted</p><p>our schedules and daily life, we are very well versed in living</p><p>in ‘isolation.’ Our lives have often involved being isolated to</p><p>protect our child’s health or because of inaccessibility of the</p><p>world, etc. The world because of COVID-19 was stepping a</p><p>bit into the life we have lived for 16 years. It did not change</p><p>how we make health care decisions for our child. We still</p><p>hold our decision-making criteria as our true north.”</p><p>DISCUSSION</p><p>FACE-Rare was feasible, safe, and acceptable. FACE-Rare</p><p>families, on average, reported significantly higher feelings</p><p>of meaning and peace compared with TAU families. Con-</p><p>sistent with Lazarus and Folkman’s model of transactional</p><p>coping with stress through problem solving,64–67 actively</p><p>coping with the palliative care needs of their child and</p><p>their own needs, and a representational approach to pa-</p><p>tient education,68 enhanced feelings of peace and meaning.</p><p>Findings are consistent with our previous research using</p><p>the FACE model, which found that adolescents in the</p><p>TABLE 2 Ratio Estimate and 95% Confidence Interval of Effect of Each of the Variables on Respective Outcomes</p><p>Variable</p><p>Emergency</p><p>Department</p><p>Visit</p><p>Faith</p><p>Subscale</p><p>Meaning or</p><p>Peace</p><p>Subscale</p><p>Family</p><p>Wellbeing</p><p>Subscale</p><p>Caregiver</p><p>Distress</p><p>Subscale</p><p>Positive</p><p>Caregiving</p><p>Appraisal</p><p>Subscale</p><p>Caregiver</p><p>Strain</p><p>Subscale BAI Total FACIT Total</p><p>Treatment as usuala 1.7 (0.1–29) 0.8 (0.5–1) 0.9 (0.8–1)** 1.0 (0.8–1) 1.0 (0.7–1) 1.0 (0.9–1) 1.0 (0.9–1) 0.7 (0.3–1) 0.9 (0.7–1)</p><p>Follow up 3 monthsb 1.8 (0.4–9) 1.0 (0.8–1) 1.0 (1–1) 1.0 (1–1) 0.9 (1–1) 1.0 (1–1) 1.1 (1–1) 1.0 (1–1) 1.0 (1–1)</p><p>Age patient (years) 0.9 (1–1) 1.0 (1–1) 1.0 (1–1)* 1.0 (1–1) 1.0 (1–1) 1.0 (1–1) 1.0 (1–1) 1.0 (1–1) 1.0 (1–1)*</p><p>Malec 0.8 (0–16) 0.9 (1–2) 1.1 (1–1) 1.0 (1–1) 1.0 (1–1) 1.0 (1–1) 0.9 (1–1) 1.0 (0.5–2) 1.1 (1–1)</p><p>Under poverty lined 12.3 (1–1189)* — 0.8 (1–1)*** 0.8 (1–1)** 1.3 (1–2)* — 1.2 (1–1)*** 3.5 (2–8)*** —</p><p>Black or African</p><p>Americane</p><p>— 1.6 (1–3)* 1.1 (1–1) 1.0 (1–1) 0.7 (1–1)** 1.1 (1–1) 1.0 (1–) 0.5 (0.2–1) 1.2 (1–1)</p><p>As a carer: not able to</p><p>do job as I would</p><p>likef</p><p>— 0.7 (1–1)** 0.9 (1–1)** — — 1.0 (1–1)** 1.3 (1–1)*** — 0.9 (1–1)**</p><p>Employedg — — — — — 1.0 (1–1)* — — —</p><p>Consider myself very</p><p>or moderately</p><p>religioush</p><p>— — — — — — 0.9 (1–1) — —</p><p>Each column summarizes the exponentiated population fixed effect of a generalized linear mixed effect model describing the respective outcome. Not statistically significant vari-</p><p>ables are not included in the models, and their associated ratio estimates are not shown because they are not present in the respective model. Only the variables “treatment as</p><p>usual,” “Follow up 3 months,” patient age, and male indicator were included in all models regardless of statistical significance.</p><p>a Indicator of patient assigned to usual treatment.</p><p>b Indicator of observation occurring during the three months follow up.</p><p>c Indicator of sex male.</p><p>d Indicator of family being under the poverty line.</p><p>e Indicator of black race.</p><p>f Indicator of response “yes” to the associated question.</p><p>g Indicator of being employed.</p><p>h Indicator of considering themselves very or moderately religious.</p><p>*Associated P 5 .05–.10; **P value 5 .01–.50; ***P value <.01.</p><p>8 LYON et al</p><p>Downloaded from http://publications.aap.org/pediatrics/article-pdf/153/6/e2023064557/1659069/peds.2023-064557.pdf</p><p>by Universidade de São Paulo user</p><p>on 24 September 2024</p><p>FACE for teens with cancer (FACE-TC) group reported sig-</p><p>nificantly higher feelings of meaning and peace,15 than</p><p>controls, likewise with teens living with HIV where higher</p><p>spirituality was associated with better clinical outcomes</p><p>and psychosocial adjustment.69 Addressing spirituality in</p><p>pediatric palliative care is important,70,71 as meaning and</p><p>purpose consistently predict lower mortality risk across</p><p>the lifespan.72,73 Findings support the American Academy</p><p>of Pediatric guidelines that spiritual support be offered</p><p>throughout the trajectory of care.74</p><p>In our small sample, Black caregivers reported less care-</p><p>giver distress than non-Black caregivers. This finding con-</p><p>tradicts our FACE-TC trial where more caregiver distress</p><p>was reported among Black caregivers.49 However, a study</p><p>of caregivers of patients with cancer also found greater re-</p><p>silience reported among Black caregivers.75 These contra-</p><p>dictory findings may represent a paradox in the context of</p><p>exposure to sociocultural and institutional adversity and</p><p>racism. Our study took place during high activity in the</p><p>Black Lives Matter movement. Black caregivers may have</p><p>felt they had less leave to report negative emotions or dis-</p><p>tress or may appraise caregiving distress less negatively in</p><p>the context of greater societal distress.76–78</p><p>Alternatively, this may be because of supportive social net-</p><p>works that shield families from carer distress. The impact of</p><p>improved social connectedness by Compassionate Communi-</p><p>ties’ model of care raises the question as to whether a high dis-</p><p>tress score is a marker of lack of community networks, rather</p><p>than the increased need for professional services.79,80 Addition-</p><p>ally, some rare disorders may be less stigmatized than others</p><p>and, thereby, generate increased understanding from the public</p><p>and receive more support.81 Future research should examine if</p><p>there are racial differences in supportive social networks and</p><p>stigma shield families from caregiver distress.</p><p>Poverty significantly determined outcomes and reaf-</p><p>firmed the support needed for family caregivers living</p><p>below the 2021 FPL. These findings confirm Healthy Peo-</p><p>ple 2030’s call to action,</p><p>providing further evidence of</p><p>the need to address social determinants of health, partic-</p><p>ularly social isolation and poverty.82 It is also noteworthy</p><p>that traditional palliative needs such as symptom manage-</p><p>ment and equipment needs appeared to be met, whereas</p><p>respite care, prognostic information (likely unknown to</p><p>physicians as well), and financial, legal, or work issues</p><p>were prioritized. Future research should determine if Med-</p><p>icaid is successfully meeting the needs of family caregivers</p><p>compared with those with private insurance with respect</p><p>to symptom management and medical equipment.</p><p>Limitations</p><p>We do not know what effects the use of telehealth had on</p><p>our findings as we have no comparator, since everyone re-</p><p>ceived the intervention via telemedicine. Perhaps the high</p><p>rate of recruitment, 74% of those who were reached agreed</p><p>to participate, was caused by telemedicine, as this is higher</p><p>than the in-person rate or recruitment from our previous tri-</p><p>als that ranged from 39% to 50%.15–22 One mother re-</p><p>ported a preference for in-person sessions because of the</p><p>intense emotions experienced when discussing the possibil-</p><p>ity that her child would die before her. Some families pre-</p><p>ferred the convenience of telemedicine because it saved</p><p>time and protected their children from the risk of infec-</p><p>tion posed by coming into the hospital. Others reported</p><p>preferring telehealth because it overcame the challenges</p><p>of transportation and the need to arrange for skilled</p><p>nursing care for their child. Two surveys directly exam-</p><p>ined the psychosocial situation of families caring for chil-</p><p>dren during the COVID-19 pandemic and found lower</p><p>FIGURE 3</p><p>Carer Support Needs Assessment Tool from session 1 of intervention arm, N5 9.</p><p>PEDIATRICS Volume 153, number 6, June 2024 9</p><p>Downloaded from http://publications.aap.org/pediatrics/article-pdf/153/6/e2023064557/1659069/peds.2023-064557.pdf</p><p>by Universidade de São Paulo user</p><p>on 24 September 2024</p><p>health related quality of life and mental health for moth-</p><p>ers (N 5 210)83 and higher distress levels among family</p><p>caregivers (N 5 149).84</p><p>The enrollment goal of 32 families was not achieved be-</p><p>cause of delays and changes in recruitment procedures be-</p><p>cause of the COVID-19 pandemic, eg, time demands on</p><p>providers. The time-consuming process of identifying eligible</p><p>children also delayed recruitment. The small sample size in-</p><p>creased the risk of Type II error or false negatives. We did</p><p>not have enough power to detect differences in most of the</p><p>outcome measures. The effects of siblings in the family or sin-</p><p>gle parent households were not considered in the models.</p><p>The higher proportion of married caregivers and those with</p><p>higher education in the TAU group compared with the inter-</p><p>vention group could have influenced the study findings. The</p><p>single site limits generalizability. Families of children with</p><p>complex care needs could have benefited but were ineligi-</p><p>ble. Social desirability bias could have occurred with remote,</p><p>but face-to-face, administration of study questionnaires.</p><p>However, this approach enabled monitoring of emotional re-</p><p>actions and controlled for issues of literacy, impaired vision,</p><p>item comprehension, and questionnaire completeness. Data</p><p>on the child’s medical condition were abstracted from the</p><p>electronic health record by the nurse facilitator which may</p><p>have introduced bias into the results concerning diagnosis</p><p>and technology dependency. The sample of rare disorders is</p><p>not representative of the rare disorder community in general.</p><p>CONCLUSIONS</p><p>FACE-Rare is feasible, acceptable, safe, and demonstrates</p><p>initial efficacy. This promising intervention improved</p><p>meaning and peace for family caregivers. Poverty impacted</p><p>quality of life. Clinicians caring for children with potentially</p><p>life-limiting illnesses may want to create space for these con-</p><p>versations but may not have adequate training. Further re-</p><p>search is needed to determine where along the continuum</p><p>of care these conversations are most valuable and accessible.</p><p>Future studies should examine if meaning and purpose pre-</p><p>dict health outcomes for family caregivers of children with</p><p>rare diseases. Future research should directly study the in-</p><p>tervention effect on social isolation, a social determinant of</p><p>health that may be amenable to change. Future research</p><p>should determine if children who were on Medicaid, com-</p><p>pared with private insurance, had better health outcomes</p><p>with respect to symptom management and provision of</p><p>equipment and devices.</p><p>ACKNOWLEDGMENTS</p><p>We would like to thank the family caregivers who agreed to</p><p>participate in this pilot trial and the parents on our Community</p><p>Advisory Board. We would also like to thank the following re-</p><p>search staff who made contributions to the study: Salome Kar-</p><p>iuki, Fiyinfolu (Fisayo) Adetunji, Hailey Robyn Moore, Alexandra</p><p>Folleco, Maria Ortega, Lindsey Rae, and Melanie Sandquist.</p><p>ABBREVIATIONS</p><p>CI: confidence interval</p><p>FACEVR Rare: FAmily CEntered pediatric advance care</p><p>planning for families of children living with a rare</p><p>disorder</p><p>pACP: pediatric advance care planning</p><p>TAU: treatment as usual</p><p>This trial has been registered at clinicaltrials.gov (identifier, NCT04855734).</p><p>DOI: https://doi.org/10.1542/peds.2023-064557</p><p>Accepted for publication Mar 5, 2024</p><p>Address correspondence to Maureen E. Lyon, PhD, Children's National Hospital, 111 Michigan Ave NW, Washington, DC 20010-2970. E-mail:</p><p>mlyon@childrensnational.org</p><p>PEDIATRICS (ISSN Numbers: Print, 0031-4005; Online, 1098-4275).</p><p>Copyright© 2024 by the American Academy of Pediatrics</p><p>FUNDING: This study was funded by the United States National Institute of Nursing Research and the National Institutes of Health Award Number R21</p><p>NR019340, No Cost Extension. Resources provided by the NIH National Center for Advancing Translational Sciences-Children’s National UL1TR0000075 and</p><p>UL1RR031988. Content is solely the responsibility of the authors and does not necessarily represent the official views of National Institute of Nursing</p><p>Research nor National Institutes of Health. The funders and sponsors were not involved in the design and conduct of the study; collection, management,</p><p>analysis, and interpretation of the data; or preparation, review, or approval of the manuscript; or decision to submit for publication.</p><p>CONFLICT OF INTEREST DISCLOSURES: The authors have indicated they have no conflicts of interest to disclose.</p><p>Data Sharing Statement: Deidentified individual participant data will be made available, in addition to study protocols, the statistical analysis plan, and</p><p>the informed consent form, upon publication to researchers. Proposals should be submitted to mlyon@childrensnational.org.</p><p>10 LYON et al</p><p>Downloaded from http://publications.aap.org/pediatrics/article-pdf/153/6/e2023064557/1659069/peds.2023-064557.pdf</p><p>by Universidade de São Paulo user</p><p>on 24 September 2024</p><p>https://doi.org/10.1542/peds.2023-064557</p><p>mailto:mlyon@childrensnational.org</p><p>mailto:mlyon@childrensnational.org</p><p>REFERENCES</p><p>1. National Human Genome Research Institute. Rare diseases FAQ.</p><p>Available at: https://www.genome.gov/FAQ/Rare-Diseases. Accessed</p><p>September 20, 2023</p><p>2. Haendel M, Vasilevsky N, Unni D, et al. How many rare diseases</p><p>are there? Nat Rev Drug Discov. 2020;19(2):77–78</p><p>3. The Hastings Center. Family caregiving 2016. Available at: www.</p><p>thehastingscenter.org/briefingbook/family-caregiving/. Accessed</p><p>September 20, 2023</p><p>4. Global Genes. RARE diseases: facts and statistics 2016. 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