Jacquelyn Baskin

and 6 more

Background: Children treated for cancer are at risk of developing iron toxicity due to receiving red cell transfusions and myelosuppressive chemotherapy. Transfusions administered during prolonged episodes of marrow suppression may increase exposure to toxic, reactive forms of iron and thereby increase risk of extrahepatic iron accumulation and long-term organ damage. Objective: This study aimed to evaluate the severity and organ distribution of clinically significant iron overload through measurement of hepatic, cardiac, pancreatic, and pituitary iron deposition in an at-risk cohort of children and young adults treated for cancer. Methods: This retrospective study evaluated patients treated for any type of cancer who underwent an MRI due to clinical concern for evaluation of iron overload (n=103, 73 post-treatment). Data regarding cancer type and treatment, MRI and laboratory results, and treatment for iron overload were analyzed. Results: Over half (53%) of this sample had moderate or greater hepatic siderosis, 80% had pancreatic siderosis, and nearly half (45%) had pituitary siderosis and/or volume loss. Pancreatic iron was associated with both cardiac (p=0.0043) and pituitary iron (p=0.0101). Patients treated for acute myeloid leukemia or high-risk acute lymphoblastic leukemia had higher liver iron concentration (LIC) compared to other cancer types (median LIC 8.5 vs. 2.9 mg/g DLW, p=0.0011). Conclusion: Pediatric cancer patients are at risk for transfusional iron overload, with significant exposure to toxic forms of iron indicated by extrahepatic iron deposition (pancreas, heart and pituitary). Further studies should examine the effect of exposure to reactive iron on long-term outcomes and develop management recommendations.

Jacquelyn Baskin

and 6 more

Introduction: Transition from pediatric to adult care for emerging adults with sickle cell disease (SCD) has been challenging due to limited availability of experienced adult providers and patient difficulty navigating the adult health care system. The purpose of this study was to determine among adults with SCD, healthcare utilization and their trust and satisfaction with their health care provider. Methods: We surveyed adult patients greater than 21 years old with SCD previously transitioned from Children’s Hospital Los Angeles. Assessments of provider trust and satisfaction were conducted along with health care utilization and the transition experience. Results: Of 31 participants, 61% and 68% identified having an adult primary care provider (PCP) and adult hematologist respectively. Increased satisfaction with care was associated with increased trust in the adult hematologist (r=0.72 p<0.001) and PCP (r=0.76 p=0.001) and improved communication (p< 0.001). Trust in their hematologist was greater than PCP (76.5 vs 64.2, p = 0.058). For SCD complications, 65% of participants visited the ED, 80% of whom had negative experiences including sub-optimal pain management. Regarding transition experience, 55% felt unprepared for adult care. Discussion: More than 30% of adult SCD patients transferred out of pediatric care are not receiving regular hematology care for their SCD, resulting in fragmented medical care. Increased trust in their adult hematologist and clear communication are associated with higher levels of satisfaction with care. These findings will be utilized to develop a transition program to improve patient preparation and build on partnerships with adult providers to improve long-term outcomes.