Many times throughout the year we will receive a request to accept a 15-17 year old weighing more than 200 pounds with gallstones who needs to be transferred so that he/she can be cared for in a pediatric facility. The really crazy part is that some of these ‘kids’ need to transferred back to an adult facility to have an ERCP to remove the gallstones if they are lodged in the common bile duct (CBD). Very few pediatric gastroenterologists are adequately trained in ERCP.
A recent retrospective study (PC Bonasso et al JPGN 2019; 68: 64-7) shows some of the consequences of this problem –longer hospitalizations and delays in treatment. The authors compared 79 (48%) pediatric patients who required transfer compared to 85 (52%) who were managed at the tertiary care pediatric hospital. The median age was 15 years with 42% obese and 23% overweight.
- Transfer group patients had longer length of stay, median 7 days vs 5 days for non-transfer group (P< 0.0001) and more days between ERCP and surgery.
- Transfer patients were more likely to have an MRCP (34% vs 8% for non-transfer).
- Transfer patients were more likely to have a stent placement, 9% vs 5% (which would require a subsequent anesthetic to remove).
- Transfer patients were more likely to have a non-therapeutic ERCP; stone/sludge removal was 70% in transfer group vs 86% in non-transfer group. This could be related to the delay (eg. more time for stone to pass) or due to the evaluation by team not responsible for ERCP.
The authors note that there are fewer than 20 pediatric gastroenterologists trained in ERCP; this is not likely to change much in the near term due to the large number of ERCPs needed to become proficient and few options for pediatric training. Their study notes that 46% had adult gastroenterologist management for non-transfer group.
My take: This is clearly an area in need of collaboration. More pediatric hospitals need to have adult gastroenterologists available and adult hospitals need to consider keeping some of these young adults to improve both the care and costs for these individuals.
Related blog posts:
This article was referenced previously on this blog
…to resolve the problems of sickle cell anemia. The effects of transfusions for sickle cell patients’ hepatobiliary function are poorly understood. By lowering the level of hemoglobin S and reducing sickling, can this lead to improvement in organs damaged by sinusoidal congestion and infarction? Well probably not (J Pediatr 2012; 160: 281-85).
Strokes are known to occur in 5-10% of sickle cell patients by 20 years of age and if untreated, >50% have recurrence. This has led to transfusion programs. The ‘Stroke with Transfusions Changing to Hydrdoxyurea’ (SWiTCH) study is a multi-center randomized trial trying to determine how current treatment (transfusions and chelation) compares with hydroxyurea/phlebotomy for preventing stroke and managing iron overload. As part of this study, a baseline assessment with ultrasound showed widespread problems -despite an average of 7 years of transfusions. In this cohort of 149 patients, the following findings were identified:
- Spleen volumes were increased in more than 1/3rd of patients leading to hypersplenism (low platelet counts). 12 subjects had nonvisible spleens due to autoinfarction.
- Nephromegaly was present. This finding is known to occur with sickle cell disease and is a marker of glomerular hyperfiltration.
- Hepatobiliary disease was nearly ubiquitous. 37 of 148 had previous cholecytectomy; of the remaining, 46 of 111 (41%) had gallstones and 14% had gallbladder sludge. Liver lengths were significantly longer as well.
Conclusions: Transfusion therapy was insufficient to reverse or prevent organ damage in children with sickle cell anemia. An important limitation– the severity of the underlying prevalence of organ dysfunction prior to initiation of transfusion therapy was not known.
- -Blood 2011; 117: 772-9. Silent cerebral infarcts occur despite regular blood transfusions.
- -Clin Gastro & Hep 2007; 5: 1469. Reviews types of sickle cell associated liver disease.
- -Pediatric Hematology and Oncology. 2006 Mar;23(2): 95-102(8). Sickle cell intrahepatic cholestasis (SCIC), which is related to intrahepatic sinusoidal RBC sickling (due to relative hypoxia) and can be associated with progressive hepatomegaly, mild transaminitis, extreme hyperbilirubinemia
- -JPGN 2004; 39: 200. Review of sickle cell hepatic crisis. Cholestasis resolves over 3 months. Acute crisis treated with hyperhydration & transfusion. Cohort of 350; 6 developed hepatic crisis.
- -J Pediatr 2001; 139: 785-789 & 790-796. Transfusions and hydroxyurea for SS dz.