1. An Introduction to Transplantation Lauren Walker, RN, BSN, CCRN Other Contributors: Lisa Dreyfuss, RN, BSN Hilary Poan, RN, BSN

2. Goals and Objectives: * By the end of the lecture, students will have an understanding of : -The history of pediatric GI transplant -The qualification of being listed for transplant -Common diagnosis indicating a need for a liver or small bowel transplant -Signs and symptoms of liver and small bowel failure -Common preop/postop medications -Signs and symptoms of organ rejection -Lifetime management concerns after transplant

3. History Transplants have been performed for over 50 years in United States:  1950s First Successful Kidney 1954  1960s First Successful Liver 1967 First Successful Heart 1968 First Successful Pancreas 1968 --UNOS http://www.unos.org/whoWeAre/history.asp

4. Transplant History  Then nothing until….  1980s Why? CYCLOSPORIN (early generation Prograf) introduced 1983 First Successful Single Lung 1983 First Successful Double lung 1986 First Successful Intestine 1987 First Living donor liver 1989 --UNOS www.unos.org/whoWeAre/history.asp

5. Organ Allocation: Getting Listed United Network for Organ Sharing (UNOS) maintains the transplant list.  Transplant centers do a thorough evaluation of a candidate  When a person is accepted for transplant by a transplant center, the center contacts UNOS and they are added to the list.  Once listed, the transplant center contacts the candidate to let them know they are listed.

6. Organ Allocation: Allocation When an organ is available, UNOS tracks and allocates the organ Organs are allocated by status. For Georgetown criteria is based on the Pediatric End Stage Liver Disease (PELD) Scoring System  Status 1A – fulminant liver failure (no previous liver failure)  Status 1B – liver failure necessitating the need for a blood transfusion within a 24 hour period for liver candidates  Score from 1-40 based on labs including bilirubin, albumin, INR, age, growth failure. Pt. in need of SB get an automatic 23 points. Priority is as follows:  Local  Regional (DC is in region 2, which also includes - Delaware, Maryland, New Jersey, Pennsylvania, West Virginia)  National

7. Who needs a Transplant? As of 06/6/11 111,502 people are waiting for transplants 16,487 waiting for a liver Mean waiting time kids < 1 yr 223 days Mean waiting time kids 1-5 yrs 262 days 221 waiting for an intestine Mean waiting time kids < 1 yr 358 days Mean waiting time kids 1-5 yrs 425 days National pediatric (up to 17yrs) survival from 1 to 5 years: over 83%

8. Liver Transplant Common indications for liver transplant seen on our unit include:  Biliary Atresia  Alagille’s Syndrome  Hepatitis B  Hepatoblastoma  Hemochromatosis

9. Signs of Liver Failure Increased Liver Function Tests (ALT, AST, Alk phos, bilirubin (direct and indirect) Jaundice Bleeding Ascites Spleno/Hepatomegaly Glucose Intolerance Increased Infection Malnutrition (Vit. A, D, E, K) Dark Urine Puritis Osteoporosis/Fractures

10. Liver Transplant A liver transplant can be done in 3 ways: 1) Cadaver 2) Living-Related Donor (generally left lobe) 3) Cadaver Split Liver

11. Intestinal Failure: Definition The inability of the gastrointestinal system to maintain fluid, electrolyte, and nutritional balance of the body Condition requires supplementation from sources outside of the GI tract

12. History of Intestinal Transplant 1988 1 st successful transplant. Why so late?  Large organ  Lots of lymphoid tissue in intestinal system = immunity  Bacterial flora Outcomes have improved with new medications (Prograf) Currently 23 centers have patients listed for intestinal transplant. Pittsburgh and GUH are the largest. National pediatric (up to 17yrs) survival rate from 1 to 5 yrs: over 71.5% (63.8% for kids under a yr)

13. Diagnosis leading to a SB Transplant Structural: NEC, Gastroschisis, malformation/volvulus, trauma, atresia, tumor Functional: Pseudo-obstruction, Megacystis, Microcolon, Intestinal Hypoperistalsis, Hirschsrpung’s disease

14. Indications in Children for Small Bowel Transplant

15. Management of Intestinal Failure Gut Rehabilitation  STEP procedure  Intestinal stretching  Time (as patient grows, gut grows and absorbs more) Lifetime TPN – Will lead to liver failure Intestinal Transplant

16. Diarrhea Constipation Emesis Fluid Imbalance and signs and symptoms of fluid imbalance Electrolyte Imbalance and signs and symptoms of electrolyte imbalance Malnutrition and signs and symptoms of malnutrition Failure to Thrive (FTT) Skin breakdown r/t diarrhea Liver failure and its signs and symptoms if TPN cholestatis occurs Signs of Intestinal Failure

17. Criteria for transplantation Can only be listed for Intestinal transplant with:  Loss of access  Irretractable dehydration  Multiple septic infections  Liver failure r/t TPN

18. Types of Intestinal Transplant Isolated Intestine Liver/Bowel Multivisceral  Liver, intestine, pancreas, stomach

19. The transplanted organ Must be at least 70% size of recipient Minimal downtime/ischemic time (intestine 10 hours or less, liver 24 hours) minimal pressor support before harvest ABO compatibility Negative crossmatch (PRA)

20. Pre Transplant Care Issues TPN Dependent Infection Dehydration Malnutrition GI bleed r/t portal hypertension Waiting Time Socialization

21. Pre-transplant Medications Vitamins (ADEK) Calcitriol Nystatin Iron

22. Post-Transplant Medications Immune Suppression: Prograf, Prednisolone, Rapamune, Cellcept, Baxiliximab Other Common Meds:Prevacid, Imodium, Lomotil, Reglan, Norvasc, Propranolol

23. Post Transplant Issues Immunosuppression Rejection Infection Education Adherence Support

24. Rejection The immune system protects the body from anything that is not self. Because a transplant is foreign to the body, without intervention, the immune system will attempt to destroy it. Goal of immunosuppressants is to inhibit immunological response and therefore prevent rejection.

25. Early signs and Symptoms of rejection General  Fever greater than 38°C  Tachycardia  High or low immunosuppressant levels  Lethargy/irritability  Abdominal pain or distention

26. Liver Rejection Liver  Increased liver function tests  Nausea and/or vomiting  Dark urine  Jaundice  Itchy skin

27. Intestine Rejection Intestine  Increased stools and/or ostomy output  Dehydration  Increasing WBC  Falling hemoglobin, albumin, or iron saturation  Weight loss  Bloody stools/ostomy output  Pale, black, or bleeding stoma  Output with clots or chunks of tissue  Sepsis

28. Rejection Monitoring LFTs for Liver Output and stoma for SB, appearance during scopes ONLY SURE WAY TO KNOW is through a biopsy Rejection is treated with high dose Steroids and Thymoglobulin

29. Major Complication: Infection Most common complication because of immunosuppression HAND WASHING Avoid sick contacts No raw foods, no live vaccines, no cleaning up after pets Prophylactic Meds Surveillance labs for EBV, CMV, Adenovirus

30. Life after Transplant Scope twice a week for the first month Once a week for the next two months Annual scope Blood draws twice a week for the first 3 months Labs once a week until labs are stable Labs at least once every three months Lifetime of immunosuppressants Rejection can happen at any time

31. Lifetime Management Issues Quality of Life Lifetime medication regime Lifetime laboratory surveillance of immunosuppression levels Lifetime surveillance for rejection Annual visits to transplant center

32. Resources Unos: http://unos.org/http://unos.org/ Georgetown University Hospital Transplant Center for Children http://www.georgetownuniversityhospit al.org/body.cfm?id=555650 http://www.georgetownuniversityhospit al.org/body.cfm?id=555650