Comprehensive Care and Treatment Options for Children with Pancreatic Disease

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Comprehensive Care and Treatment Options for Children with Pancreatic Disease Stephen Mark Ogg, MSN, RN, FNP-C Cincinnati Children's Hospital Medical Center Cincinnati, Ohio Disclosure Information No disclosures
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Comprehensive Care and Treatment Options for Children with Pancreatic Disease Stephen Mark Ogg, MSN, RN, FNP-C Cincinnati Children's Hospital Medical Center Cincinnati, Ohio Disclosure Information No disclosures to declare Objectives 1. Recognize common causes of chronic pancreatitis in the pediatric population. 2. Understand some treatment options for pancreatitis patients and components involved in TPIAT. 3. Learn the benefits of utilizing a multidisciplinary team approach evaluation for management of a complex medical condition. 1 Pediatric problems of the pancreas Acute pancreatitis (AP), Acute reoccurring pancreatitis (ARP), chronic pancreatitis (CP) Autoimmune pancreatitis Pancreatic congenital anomalies Pancreatic cysts and pseudocysts Pancreatic tumors and mass Pancreatic exocrine insufficiency Diagnosed when: Acute Pancreatitis An insult to the pancreas that leads to the presence of acute inflammatory cells, edema and necrosis that may result in organ damage or fibrosis At least 2 episodes of pancreatitis with: Complete resolution of pain AND 1 month pain free Or complete normalization of pancreatic enzymes and complete resolution of pain Morinville VD, et al. Definitions of pediatric pancreatitis and survey of present clinical practices. J Pediatr Gastroenterol Nutr 2012;55(3): Work up of ARP - what s going on? Inflammatory Bowel Disease, Celiac Mitochondrial disease, Cystic Fibrosis? Metabolic disease Anatomic Genetics (PRSS1, SPINK1, CTRC, CFTR) 2 Chronic pancreatitis Diagnosed when: Abnormal findings in pancreatic duct, atrophy or organ or calcifications present In addition to one of the following: Abdominal pain consistent with pancreatitis Exocrine pancreatic insufficiency Endocrine pancreatic insufficiency Morinville VD. JPGN Sep;55(3):261-5 JAMA Pediatr. Published online April 11, doi: /jamapediatrics Managing CP Nutrition Maintain weight gain/ growth Assess for vitamin deficiencies Endocrine Assess insulin function Magnetic resonance cholangiopancreatograpy (MRCP), Endoscopic retrograde cholangiopancreatography (ERCP), Endoscopic ultrasound (EUS) Assess and treat anatomic abnormality or obstruction Pain management Medication, Psychology 3 Imaging in AP and CP Ultrasound can confirm inflammation and thus diagnose pancreatitis CT can be utilized in severe cases to assess necrosis, fluid collections or masses MRCP is best imagine study to examine for abnormalities, i.e. divisum, stenosis of duct Abu-El-Haija et al. JPGN 2012; 58(6):703-8 ERCP Sphincterotomy Stent placement Pseudocyst drainage Stone removal Total Pancreatectomy Islet Cell Auto Transplant (TPIAT) NOT a cure Goal of pancreatectomy (TP) = relieve incapacitating pain and debilitation Goal of Islet Cell Transplant (IAT) = preserve islet cell and insulin secretory function 4 Criteria for TPIAT Impaired quality of life - chronic pain, daily opioid use, inability to attend school, improvement failure through Pain Team Diagnosis of ARP or CP - genetic testing, imagining, pancreatic insufficiency No reversible cause for pancreatitis No improvement with ERCP or other medical management Adequate b-cell function (criteria for IAT) No physiologic or psychosocial contraindication TPIAT Operation Surgery involves removing the entire pancreas along with parts of the surrounding organs (duodenum, spleen) Sending the pancreas off to a lab to isolate its islets Then transplanting (infusing) those islets into the liver through the portal vein Blondet et al. Surg Clin North Am 2007;87: TPIAT video 5 Surgery Risks Bleeding Blood clots Systemic Inflammatory Response Syndrome (SIRS) Infection Biliary Leak or stenosis Delayed gastric emptying 6 Islet Isolation Goal: to digest pancreas and disrupt exocrine tissue to release relatively pure islets in small tissue volume that can be safely infused into portal vein Collagenase digestion, gentle mechanical dispersion Rocordi method (~4 hours) Pellet assessed for count, viability, purity, endotoxin 1 Ricordi et al. Diabetes 1988;37: Islet Yield Predicting islet yield is difficult Insulin independence is dependent on the number of islet equivalents (IE) transplanted per kg body weight Prior ductal drainage procedures and distal resections compromise islet yield at ~ 50% reduction Chinnakotla et al. Ann Surg 2014;260: Wilson et al. Surgery 2015;158: Outcomes: Improved pain and QOL Significant reduction in opioid use after TPIAT. Reduced prevalence of pancreatitis pain and severity of pain. Nearly all improvement in pancreatitis pain occurred in first 3 months. Effects are sustained over time. Chinnakotla et al. Ann Surg 2014;260: Outcomes: lost school days Percentage of parents reporting lost days at school statistically declined from 87% to a negligible value at 2 years post-tpiat. Chinnakotla et al. Ann Surg 2014;260: Outcomes: Islet function / glucose control Younger children ( 12 yo) were more likely (56%) to achieve insulin independence than older children (40.5%). Insulin independence observed for as long as 10 years after TPIAT. Chinnakotla et al. Ann Surg 2014;260: CCHMC TPIAT Outcomes Surgery TIE IE/kg F/U Opioids Off opioids Insulin Off insulin 12F 4/23/15 243,000 4, mos none 2 wks Basal/Bolus -- 12F 5/21/15 297,000 6, mos none 7 mos none 9 mos 16M 11/12/15 309,000 3, mos none 11 mos Basal/Bolus -- 7F 11/19/15 198, mos none 2 mos Basal/Bolus -- 17F 1/5/16 224,000 4, mos yes -- none 8 mos 9F 3/31/16 423,000 14,242 9 mos none 3 wks none 6 mos 15F 4/28/16 450,000 6,858 8 mos none 6 mos Basal -- 5M 5/26/16 129,000 6,355 7 mos none 5 mos Basal -- 10F 6/14/16 488,000 10,429 7 mos none 3 mos Basal/Bolus -- 14F 8/2/16 525,000 5,529 5 mos none 3 mos Basal/Bolus -- 17M 9/8/16 409,000 4,377 4 mos yes -- Basal/Bolus -- 8M 11/10/16 172,300 5,189 2 mos yes -- Basal/Bolus -- 14M 12/8/16 302,700 5,821 3 mo yes -- Basal/Bolus -- 11F 1/12/17 238,500 6,699 1 mo yes -- Basal/Bolus -- 4F 1/31/17 367,500 8,441 1 wk yes -- Basal/Bolus -- 18M 2/23/17 437,550 6, yes -- Basal/Bolus -- Pancreas Care Center (PCC) Founded within Department of Gastroenterology Specifically addresses the increasing needs of pediatric patients with pancreatic disease Expanded to become a multidisciplinary care team 9 CCHMC PCC Patient Care Multidisciplinary Pediatric transplant surgeon Gastroenterologist Endocrinologist Pain team Psychologist Infectious Disease Hematology Radiologist Geneticist Dietician Social worker Thorough Care Weekly meeting to discuss patients of PCC Weekly meeting with radiologist to review patient images Weekly meeting to review TPIAT patients Pancreas Care Clinic every Thursday Conclusions TPIAT is an important and viable option in treatment of children with CP and ARP Considered when maximal medical therapies and endoscopic approaches fail to relieve pain and to address complications Comprehensive multidisciplinary patient evaluation is critical to ensure optimal outcomes following TPIAT In appropriately selected children, TPIAT achieves durable pain relief and improves QOL with manageable glycemic control 10
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