The present study assessed the relationship between resilience, adherence, and transition readiness in adolescents/young adults with chronic illness. Participants included 50 patients (Mean age, Mage = 17.3 ± 2.1 years) diagnosed with an oncology disorder (n = 7; 12.1%), hematology disorder (n = 5; 8.6%), nephrology disorder (n = 31; 53.4%), or rheumatology disorder (n = 7; 12.1%). Patients were administered questionnaires assessing resilience (Conner–Davidson Resilience Scale 25-item questionnaire, CD-RISC-25), transition readiness (Self-Management and Transition to Adulthood with Rx=Treatment, STARx), and self-reported medication adherence (Medication Adherence Module, MAM). Medical chart reviews were conducted to determine objective medication adherence rates based on pharmacy refill history (medication adherence ratios). A multivariate correlation analysis was used to examine the relationship between resilience, transition readiness, and adherence. There was a moderate relationship (r = 0.34, p ≤ 0.05) between resilience (M = 74.67 ± 13.95) and transition readiness (M = 67.55 ± 8.20), such that more resilient patients reported increased readiness to transition to adult care. There also was a strong relationship (r = 0.80, p ≤ 0.01) between self-reported medication adherence (M = 86.27% ± 15.98) and pharmacy refill history (Mean Medication Adherence Ratio, MMAR = 0.75 ± 0.27), which indicated that self-reported adherence was consistent with prescription refill history across pediatric illness cohorts. Our findings underscore the importance of assessing resilience, transition readiness, and adherence years before transitioning pediatric patients to adult providers to ensure an easier transition to adult care.

Introduction Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy that originates from plasmacytoid dendritic cells. It can involve skin, bone marrow, and/or lymph nodes. There is no consensus recommendation regarding treatment especially in the relapsed setting. Tagraxofusp, a CD123 directed agent, was recently approved by the Food and Drug Administration to treat BPDCN. We report a case of an elderly patient with diagnosis of BPDCN who was treated initially with tagraxofusp followed by azacitidine and venetoclax combination on relapse. Case report A 79 year old male presented with violaceous skin lesions. He had no other symptoms. Biopsy of these lesions was consistent with a diagnosis of BPDCN. Further testing showed no extracutaneous involvement. Management and outcome: Tagraxofusp was started at full dose (12 mcg/kg). This dose was not tolerated well. Patient could only tolerate the lowest dose (5 mcg/kg). Toxicities included elevated liver function tests, hyperglycemia, capillary leak syndrome, and pancreatitis. Dose escalation on progression was not possible due to side effects. Treatment was switched to venetoclax and azacitidine. Combination treatment was tolerated very well and patient showed major cutaneous response after 5 cycles and continues to do well. Discussion Tagraxofusp is a novel therapy that needs more real-world experience. This case describes the clinical course of an elderly patient on tagraxofusp. We also review the literature of azacytidine/venetoclax combination as a potential yet tolerable treatment option for this rare disease entity. This is the fourth case in literature to be treated with this combination.

Despite significant gains in survival rates for pediatric patients and adolescents/young adults (AYA) with chronic illness, patients in this vulnerable age group are also at an increased risk for developing one or more adverse effects related to their disease, treatment, or maladaptive health behaviors. Maladaptive health behaviors ultimately increase the risk for developing adverse effects, including: increased rates of morbidity and mortality, impaired physical functioning, increased fatigue, obesity, increased psychological distress, and poor quality of life. With close attention including participation in preventive and therapeutic health promotion interventions, problematic health behaviors can be mitigated and ultimately prevented over time. It is well known that improved psychological functioning and adaptive coping can result in improved health status. The present paper provides four case examples illustrating various psychological interventions in pediatric chronic illness. As evidenced in the four case examples, pediatric psychologists provide comprehensive interventions for patients with acute and chronic medical conditions through the use of health promotion interventions, adherence and self-management promotion, cognitive behavioral therapy, behavioral therapy, medical coping, parent training, and motivational interviewing. Our case series demonstrates that for the most impactful behavior change to occur, a combination of interventions is often the most effective.