The Role of Home. Pediatric Liver and Small Bowel Transplant. Laurie Reyen, RN, MN. UCLA Health System, University of California, Los Angeles

Transcription

1 The Role of Home Infusion Therapy in Pediatric Liver and Small Bowel Transplant Laurie Reyen, RN, MN Clinical i l Nurse Specialist Parenteral/Enteral t l Nutrition UCLA Health System, University of California, Los Angeles

2 Top 5 Things to Know for CE: Make sure your BADGE IS SCANNED each time you enter a session, to record your attendance. Carry the Evaluation Packet you received on registration with you to EVERY session. If you re not applying for CE, we still want to hear from you! Your opinions about our conference are very valuable. Pharmacists, Pharmacy Technicians and Nurses need to track their hours on the Statement of Continuing Education Certificate form as they go. FOR CE: At your last session, total the hours and sign both pages of your Statement of Continuing Education Certificate form. Keep the PINK copies for your records. Place the YELLOW and WHITE copies in your Evaluation packet. Make sure an evaluation form from each session you attended is completed and in your Evaluation packet (forgot to pick up an evaluation form at a session? (Extras are available in an accordion file near the registration desk.) Put your name and unique member ID number (six digit number on the bottom of your badge) on the outside of the packet, seal it, and drop it in the drop boxes in the NHIA registration area at the convention center.

3 Disclosures Laurie Reyen declares no conflicts of interest or financial interest in any service or product mentioned in this program. Cli i l t i l d ff l b l ill tb Clinical trials and off-label uses will not be discussed.

4 Learning Objectives Explain the underlying medical conditions and disease comorbidities most frequently necessitating liver, small bowel or multivisceral transplant. List the most common home infusion needs of the pediatric i patient awaiting i liver, small bowel or multivisceral transplant Describe the infusion therapy needs of the Describe the infusion therapy needs of the pediatric patient awaiting liver, small bowel or multivisceral transplant post transplant.

5 Types of GI Solid Organ Transplants Isolated liver transplant Isolated small bowel transplant Liver and small bowel transplant Multivisceral transplant

6 Pediatric Liver Transplant : Historical Perspective st attempt at LTx in a human by Dr. Starzl on a 3 y/o w/ biliary atresia First successful LTx 1.5 y/o with a malignant liver tumor. Survived for 400 days before succumbing to disseminated malignancy FDA approves cyclosporine 8 NIH C C f d l li 1983 NIH Consensus Conference declares liver transplantation a valid therapy for ESLD

7 Pediatric Liver Transplant : Historical Perspective 1984 Reduced size LTx technique is established 1988 First split liver transplant is performed 1989 Tacrolimus introduced into clinical trials st f l li i l d LT st successful living related LTx performed

8 Historical Perspective Peds LTx Significant improvements over the past 30 years. 1970s 1 yr pt survival = 25 30% 1990s= 80 90% 2005 Multi center (SPLIT) Data= 89% Advances in immunosuppression, i organ preservation, surgical techniques, referral, medical/nutritional management pre & post LTx have accounted for this success. By 2002, approximately 15,000 children had undergone LTx worldwide. McDiarmid S Pediatric Transplantation 1998; 2: SPLIT Annual Report.

9 Indications for Pediatric diti LTx Intractable Cholestasis Portal HTN with variceal bleeding Multiple episodes of ascending cholangitis Failure of synthetic function Intractable ascites Encephalopathy *Failure to thrive & malnutrition *Unacceptable QOL (intractable pruritus) *Metabolic defects for which LTx will reverse life threatening complications *= Pediatric Specific McDiarmid SV et al. Pediatric Transplantation. 1998; 2:

10 Common Disease States in Pediatric LTx Candidates dd Cholestatic diseases Biliary Atresia Choledocal Cyst Neonatal Hepatitis Fulminant hepatic failure Tumor Hepatoblastoma

11 Indications for Pediatric LTx: The UCLA Experience Farmer, Venick, McDiarmid et al. J Am Coll Surg (5):904 14

12 PELD: The Pediatric End Stage Liver Disease Score

13 PELD Score Determines pediatric liver allocation Derived from a population of children from the Studies of Pediatric Liver Transplant (SPLIT) Similar principles & statistical methods to MELD score Objective, verifiable data

14 The PELD Score From multivariate analyses of SPLIT population 5 factors accurately predicted death at 3 months on the waiting list: Total bilirubin INR Serum Albumin Age <1 year Growth failure (<2 SD below mean for age & Growth failure (<2 SD below mean for age & gender)

15 Pediatric urgent status listing Status 1A & 1B Status 1A Fulminant hepatic failure Onset of hepatic encephalopathy within 8 weeks of first symptoms Located in ICU with Vent dependence Dialysis, CVVH, or CVVHD INR >2 Status 1 B Located in ICU with PELD > 25 and: Ventilator Bleeding Dialysis, CVVH, or CVVHD

16 Candidates for home infusion therapy in the pediatric patient awaiting liver transplant Infants with cholestatic liver disease High risk for growth failure, malnutrition related to: Increased resting energy requirements Fat, fat soluble vitamin malabsorption Impaired oral intake due to ascites, anorexia Malnutrition, growth failure increase risk of poor outcome pre and post transplant Nasogastric feeds Patients with inadequate oral intake, failure to thrive Parenteral Nutrition Patients with failure to thrive despite optimal enteral support Lipid only Partial TPN

17 Candidates for home infusion therapy in the pediatric patient awaiting liver transplant Hepatic malignancy unresectable Hepatoblasotma Hepatocellular carcinoma May require home infusion support while undergoing chemo in the pre transplant period Support needs may include: Hydration fluid Parenteral nutrition Colony stimulating factors

18 Intestinal Failure (IF) Inability of the small intestine to maintain adequate nutrient, fluid, and electrolyte absorption to support normal growth and development A requirement for continuous parenteral nutrition for at least 60 to 90 days. Goulet, O., et al., JPGN, (3): Kocoshis, S.A., et al., JPGN, (S2): S

19 Causes of Intestinal Failure in Children hld Motility - Other Tumor 2% 1% Aganglionosis/ Hirshsprung's 7% Re-Tx Other 8% 4% Volvulus 17% Pseudo- Obstruction 9% Gastroschisis 21% Malabsorption Other 4% Microvillus Inclusion 6% Short Gut Other 4% Intestinal Atresia 8% Necrotizing Enterocolitis 12% Grant et al; Annals of Surgery. 2005; 241, 4:

20 Common Diagnoses Associated w/ IF Short bowel syndrome secondary to: Gastroschisis NEC Intestinal atresia Volvulus Congenital short bowel Trauma Motility disorders Chronic intestinal psuedoobstruction syndrome Aganglionosis Long Segment Hirshprung s disease Malapsorptive disorders Tufting enteropathy Microvillus inclusion disease

21 Parenteral Nutrition i There are an estimated 30,000 pts with SBS in the U.S. who require PN 80% of SBS pts tolerate PN well with few complications For many PN will serve as a bridge to adaptation

22 Parenteral Nutrition/Intestinal Failure Associated Liver Disease Etiology unknown Proposed causes: deficiencies in carnitine, taurine, choline, Vit E, Zn, EFA; excesses in total PN calories, CHO, AA, fats. Risk Factors: Prematurity, LBW Inability to tolerate any enteral feeds Short bowel/no bowel Recurrent sepsis Accounts for 15 22% of mortality in long term PN pts. Infants w/ advanced PNALD: mortality rates approach 100% w/in 1 yr of onset if unable to wean off PN or receive OLT/ITx. Fryer, J etal. Transplantation 2008.

23 Intestinal Transplant: Historical Perspective Early attempts in universally unsuccessful No attempts between : first successful multivisceral i l transplant t 1989: first successful isolated small bowel 2000: Medicare National Coverage Policy

24 Comparative History st Kidney Tx (Twins) st Kidney Tx (cadaveric) st Pancreas Tx st Liver Tx st Heart Tx st Lung Tx st SBTx 1951 Steroids 1958 TBI MP/AZA 1963 AZA + Pred 1966 ALG 1979 CYA 1981 OKT FK 506

25 Current status of intestinal transplant 185 intestinal transplants performed in North America in patients on the waiting list as of March 2011 Graft survival 1 year (2005) 5 years 10 years Patient survival 1 year 5 year 10 years (optn.transplant.hrsa.gov)

26 Challenges of Intestinal Transplant Why is the intestine so difficult to transplant? Large number of resident leukocytes REJECTION Strong expression of histocompatibility antigens REJECTION Gut bacteria INFECTION

27 Indications i for Intestinal Transplant Irreversible intestinal failure with: Failure of Parenteral Nutrition Impending or overt liver failure due to TPN induced liver injury Thrombosis of 2 or more central veins Subclavian, jugular, femoral Frequent line infections, sepsis Two or more episodes of catheter related systemic sepsis per year that require hospitalization Single episode of line related fungemia, septic shock or ARDS Frequent episodes of severe dehydration despite IV fluid supplement in addition to TPN National Coverage Determination for Intestinal and Multivisceral Transplantation. cms.gov/medicarecoverage database

28 Indications for Liver/intestine, Multivisceral ltransplant Irreversible intestinal failure with: Irreversible liver disease Persistent hyperbilirubinemia Portal hypertension Splenomegaly Thrombocytopenia Impaired synthetic function For multivisceral : impairment of an additional organ Pancreas Spleen Kidney

29 Organ Allocation Intestine only Status 1A Liver function abnormalities No longer with central venous access via major vessels Subclavian, jugular, femoral veins Intestine w/ liver Livers allocated through the liver match run

30 Home Infusion therapy pre intestinal transplant Parenteral nutrition Enteral nutrition IV hydration Anti infectives Other: Pain management Colony stimulating factors Proton pump inhibitors Octreotide Omegaven

31 Key aspects of care in the pre transplant period Promote optimal growth Frequent adjustment of nutritional support Avoid underfeeding, overfeeding Manage fluid and electrolytes Ensure adequate replacement of GI tract losses Diarrhea, ostomy, gastrostomy Manage fluid/sodium restrictions as needed in presence of ascites

32 Fluid and electrolyte l challenges hll Managing need for additional IV hydration cc:cc replacement not always feasible in the home setting Proactive liberalization of TPN fluid volume to compensate for anticipated losses Parent/caregiver instruction re: indications for administration i i of supplemental l fluid bolus

33 Fluid and electrolyte l challenges hll The child with ascites Basic principles p of management Sodium restriction : 1 2 meq/kg/day Includes oral and IV sources Water restriction 50 75% maintenance Diuretics Spironolactone Furosemide Albumin, IV lasix for more intractable ascites

34 Key aspects of care in the pre transplant period Manage hypoglycemia As liver function deteriorates, pt s may be less able to tolerate t fasting state tt PN infusions may be extended up to 24 hr/day Manage coagulopathy Monitor PT/INR Supplemental Vitamin K may be indicated Oral ESLD pt IV IF pt

35 Key aspects of care in the pre transplant period Infection control Prevention and early identification Ensure consistent, evidence based approach to catheter care Ensure thorough education of parents/caregivers on catheter care and TPN administration procedures Key aspect of care both pre and post transplant

36 Best practices in caregiver education: lessons from the literature Education of patients, family caregivers: strategies for decreasing CR BSI Intensive education Patients in group receiving more detailed written instructions,, at least 6 vs. 2 training sessions, monthly clinic follow up with significantly lower rates of CR BSI (Santarpia et al, 2002) Clinical trial of interactive, videotaped interventions to reduce infection Focus on infection control, self monitoring, i partnership problem solving Significant decrease in CR BSI at 6 and 18 month interval (Smith CE, 2003)

37 Best practices for parent /caregiver education Location of training hospital vs. home ESPEN HAN working group survey: lower complication rates in patients primarily trained at home Higher rate of RN f/u (50%) in home trained group vs. hospital trained group (3.4%) Bozzetti,2002

38 Tell Your Patients About The Oley Foundation FREE information and support for patients on home tube or IV feedings (800) 776 OLEY

39 Parent/caregiver education Use the right tools to get the job done Appropriate reading level Appropriate methods Simulation Learning curve for technical skills steepest at beginning Allow learner to experience mistakes in simulation vs. real life Teach back Ask learner to explain/demonstrate info

40 Parent/caregiver education Ensure quality of teaching: Consistency Procedures How we do things Expectations Who will do things Avoid taking over for the parent Knowledge Ensure teachers have appropriate info to provide needed instruction Culturally sensitive Appropriate use of translators for learner with limited/no English proficiency

41 Parent/caregiver education Be patient Be respectful of the process Recognize the ongoing nature of educational needs Be prepared to reassess/ re educate as changes /problems rise

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44 Home Infusion Therapy Post Intestinal Transplant Enteral Nutrition Formula choice may vary center to center Elemental first 3 to 6 months Peptide based Standard formula Dli Delivered via gastric or jejunal j route Time to wean off enteral feeds varies, dependent on: Prior oral feeding experience Degree of oral aversion Graft function

45 Home Infusion Therapy Post Intestinal Transplant Intravenous hydration May be required initially if depending on volume of ostomy outputs May contain supplemental electrolytes: Magnesium Replacement commonly needed ddwith tacrolimus Enteral form may not be well tolerated initially Bicarbonate Supplement may be required to offset GI tract losses Enteral form may not be well tolerated initially

46 Home Infusion Therapy Post Intestinal Transplant Anti viral therapy Epstein Barr virus (EBV) linked to development of Post Transplant Lymphoproliferative Disease (PTLD) in post transplant patients Intravenous Ganciclovir: May be used for EBV prophylaxis in immediate post transplant period May be used for treatment with active infection

47 Home Infusion Therapy Post Intestinal Transplant Other potential therapy needs: Antibiotics Colony stimulating factors Intravenous iron Anticoagulant therapy IVIG Cytogam Parenteral nutrition

48 Key Aspects of Care Post Intestinal Transplant REJECTION INFECTION

49 Key Aspects of Care Post Liver/Intestinal Transplant Signs of rejection Fever Increased ostomy/ stool output (IT) Changes in character of ostomy /stool output (IT) Abdominal pain Vomiting Jaundice (OLT) Increased liver function tests (OLT) Lethargy Signs of infection can overlap in the IT pt Endoscopic surveillance biopsy gold standard for definitive diagnosis

50 Key Aspects of Care Post Liver/Intestinal Transplant Medication management: Immunosuppressive therapy Tacrolimus Steroids Mycophenolate mofetil (IT) Sirolimus (IT) Infection Prophylaxis Antiviral Ganciclovir, Acyclovir PCP Trimethoprim and Sulfamethoxazole Fungal Nystatin

51 Key Aspects of Care Post Liver/Intestinal Transplant Medication Management Continued! Electrolyte Supplements Magnesium Bicarbonate Vitamin /Mineral Supplements (IT) Zinc Iron Vitamin D

52 Key Aspects of Care Post Liver/Intestinal Transplant More medication management. Acid suppression H2 Antagonist or proton pump inhibitor Antidiarrheal agents (IT) and many other players to be named later

53 Key Aspects of Care Post Liver/Intestinal Transplant Monitor for short and long term complications: Growth Renal disease Malignancy Post transplant diabetes mellitus Cardiovascular complications HTN Adherence Psychosocial Sh School performance HRQOL Reproductive health

54 Challenges for the Home Infusion Professional Very fragile, complex patient population Both pre and post transplant Often multiple l teams, centers involved in care Communication and coordination of care between centers, providers can be complex Service delivery post transplant may be impacted by: Location of transplant center Location of patient Home = out of hospital, local to transplant center Home,home really at home!

55 Case Study J is an 10 month old girl referred for liver/ small bowel transplant evaluation Prenatal diagnosis of gastroschisis Has had 4 GI surgeries Remaining length of small bowel 12 cm Evidence of Intestinal failure associated liver disease Total bili 8.0, AST 151, ALT 60 Albumin 3.1 Platelets 55, INR 1.26

56 Case study PN dependent 18 hours/day D20 2% Trophamine 850 ml 20% Intralipid 45 ml Tolerating limited enteral feeds Elemental pediatric formula 240 ml over 24 hours Has had 4 central venous catheters 2 major CVC related infections 1 loss due to mechanical malfunction

57 Case study Weight : 10 kg (75 90%tile) Length:73 cm (75 90%tile) Physical exam: Jaundiced Abdomen mildly distended Spleen down 4 cm and full Liver down 5 6cm and firm Prominent vascular markings on chest and abdomen

58 Case Study Meds: Ursodiol Loperamide Erythromycin Probiotic Ferrous sulfate Famotidine in TPN

59 Case study After evaluation recommended for dual path: Listing for liver/bowel transplant Intestinal rehabilitation with Omegaven trial 6 weeks post eval, initiation on Omegaven : 2 Hospitalizations: Bacteremia Ecoli E.coli, Klebsiella, Strep Ileus Liver function continues to deteriorate Spironolactone and lasix started PN infusion extended to 24 hr/day due to hypoglycemia

60 Case study Clinic visit 8 weeks post eval: Weight = 12.5 kg increase of 500 gm in 1 week Abdomen significantly distended Labs: Bili total 21mg/dl AST 289, ALT, 289 Albumin 2.4 INR 2.0 Platelets 88 Na 126, K 4.5, Cl 94, CO2 26 Cr 0.1, BUN 10

61 Case Study Fluid and electrolyte management Restrict total fluids to 70% maintenance Change morning Lasix dose to IV Give Albumin 1 gram/kg followed by IV Lasix Recheck lutes in 48 hours Coagulopathy Increase supplemental Vitamin K in TPN

62 Case Study Repeat lytes Na 133, k 3.9, cl 102, c02 28 Cr 0.2, BUN 13 INR 2.0 Wt 12.2 kg J was maintained at home for 6 weeks with weekly albumin infusions, weekly clinic visits and twice weekly lab checks. She was readmitted at 14 months of age due to INR of 2.7,bleeding from GT site and increased irritability.

63 Case study J received a multivisceral transplant 38 days later. J was discharged home 6 weeks later on: Pediatric Vivonex 24 hr/day Tacrolimus, mycophenolate mofetil, prednisone Gancyclovir (IV), Trimethoprim/Sulfamethoxazole, nystatin Loperamide, famotidine Magnesium sulfate Aspirin

64 Case study 5 months post transplant: J has had no re hospitalizations. Stable graft function Steady weight gain Starting to try some food by mouth Current regimen: Peptamen Jr 16 hr/day Gancyclovir switched to oral valganciclovir Sodium citrate/citric acid with a probiotic supplement added

65 Questions?

66 Rf References Beerman LE et al. Clinical outcomes of intestinal transplant patients receiving home infusion services Transplant Proc Sep;32(6): Fishbein TM. Intestinal Transplantation. NEJM 2009;361: Kaufman SS et al., American Society of Transplantation. Indications for pediatric intestinal transplantation: a position paper of the American Society of Transplantation. Pediatr Transplant Apr;5(2):80 7 Larosa, C,et al. Outcomes in pediatric solid organ transplantation. Pediatric transplantation. 2011). 15(2), National Coverage Determination for Intestinal and Multivisceral Transplantation. cms.gov/medicare coverage coverage database McDiarmid SV et al. Selection of pediatric candidates under the PELD system. Liver Transpl Oct;10(10 Suppl 2):S McDiarmid SV et al.transplantation. Prevention and preemptive therapy of postransplant lymphoproliferative disease in pediatric liver recipients Dec 27;66(12): Sundaram SS et al. Study of Pediatric Liver Transplantation Research Group. Outcomes after liver tranplantation in young infants. J Pediatr Gastroenterol Nutr Oct;47(4): Ziring D, et al. Infectious enteritis after intestinal transplantation: incidence, timing and outcome. Transplantation Mar 27;79(6):702 9.