Continued Review of National Liver Review Board (NLRB) Guidance At a glance Current policy When a liver transplant program believes that a candidate’s calculated model for end-stage liver disease (MELD) or pediatric end-stage liver disease (PELD) score does not accurately reflect their medical urgency for transplant, they can submit a request for a MELD or PELD exception score. The National Liver Review Board (NLRB) reviews requests from transplant programs for these exception scores. The NLRB uses policy and guidance documents to decide whether to approve or deny exception score requests. Since implementation in 2019, the OPTN Liver and Intestinal Organ Transplantation Committee has regularly evaluated the NLRB to identify opportunities for improvement. Supporting media Presentation View presentation Proposed changes Create guidance for pediatric liver transplant candidates with cystic fibrosis Update guidance for adult liver transplant candidates diagnosed with hepatic adenomas and Budd Chiari syndrome Anticipated impact What it's expected to do Provide NLRB members with updated guidance to use when reviewing MELD and PELD exception requests for specific candidates Make it more likely that pediatric patient with liver disease related to cystic fibrosis and adult candidates with hepatic adenomas or Budd Chiari syndrome are able to access liver transplant in a timely and equitable manner. What it won't do Will not impact how liver candidates are prioritized on a match run Terms to know Guidance Documents: Documents that provide more information to transplant programs and NLRB members to use when making decisions on exception requests Model for End-Stage Liver Disease (MELD): The scoring system used in allocation of livers to candidates who are at least 12 years old Pediatric End-Stage Liver Disease (PELD): The scoring system used in allocation of livers to candidates who are under 12 years old National Liver Review Board (NLRB): A review board of members drawn from a nationwide pool of liver transplant physicians and surgeons, who review exception requests from transplant programs for candidates whose automatically calculated model for end-stage liver disease (MELD) score or pediatric end-stage liver disease (PELD) score does not accurately reflect the candidate’s medical urgency for transplant. Hepatic adenomas: Rare benign nodules in the liver Budd Chiari syndrome: Medical condition in which the veins that drain the liver (hepatic veins) are blocked or narrowed by a mass of blood cells (clot), causing the blood to back up into the liver. Cystic fibrosis: A genetic disorder that can lead to chronic damage in several organs including the lungs, pancreas, and liver. Click here to search the OPTN glossary Comments UC San Diego Health Center for Transplantation | 09/29/2022 The UC San Diego Health Center for Transplantation (CASD) appreciates the opportunity to provide public comment on the Continued Review of National Liver Review Board (NLRB) Guidance. The recommended modifications are appropriate. CASD does also urge the Committee to continue to explore opportunities to create automatic exception pathways. Attachment OPTN Transplant Coordinators Committee | 09/29/2022 The OPTN Transplant Coordinators Committee thanks the Liver Transplantation Committee for their work and for the opportunity to comment on this proposal. One member expressed support for the proposed changes to adult exceptions, noting that more succinct exceptions are easier to utilize and apply. The member asked for clarification as to whether these exceptions would be automatic, and the presenter explained that these would be reviewed, but that the guidance will provide improved consistency across cases. A member recommended that the Liver Review Board keep track of the number and type of appeals and re-applications needed for different disease types, so that the Liver Committee could review and determine if the guidance and policy are appropriate or need to be updated. It was clarified that this information is reviewed, particularly for efficiency and appeals. A member asked for clarification on the surgical intervention question and related response options. One member expressed support for standardizing cystic fibrosis pediatric exceptions, sharing that this exception should be more standardized. The member expressed support for consistency in appeals Region 6 | 09/28/2022 Sentiment: 3 strongly support, 16 support, 4 neutral/abstain, 0 oppose, 0 strongly oppose Region 10 | 09/28/2022 Sentiment: 2 strongly support, 9 support, 4 neutral/abstain, 0 oppose, 0 strongly oppose NATCO | 09/28/2022 NATCO appreciates the opportunity that the OPTN Liver and Intestinal Organ Transplantation Committee has provided to review their Continued Review of National Liver Review Board (NLRB) Guidance proposal. We provide the following feedback: 1) For Cystic Fibrosis, it is recommended that additional clarity be provided to identify that/if the guidance pertains to liver-only candidates (those not yet requiring a lung transplant) in addition to those requiring liver-lung transplantation. 2) For Hepatic Adenomas, it is recommended that the updated language include exceptions being granted for those individuals with known malignancy or exceptionally high risk for malignancy. 3) For Budd Chiari, it is recommended, and agreed, that a qualifying criterion of “failed surgical management” be included. For example, in the case of a failed shunt or if there is a contraindication to a shunt. In summary, NATCO supports the proposed changes and agrees that each is necessary for enhanced clarity as well as standardization of exceptions being granted. Society for Pediatric Liver Transplant | 09/28/2022 The Society of Pediatric Liver Transplantation (SPLIT) and the North American Society for Gastroenterology, Hepatology and Nutrition (NASPGHAN) support this additional guidance to include parameters for specific exceptions for children with cystic fibrosis related liver disease (CFLD). As currently calculated, MELD and PELD scores do not accurately reflect the risk of liver-related mortality or morbidity for most children with cystic fibrosis. In the biliary cirrhosis which is a hallmark of CFLD, hepatic indices may never be very elevated, despite advanced disease and fibrosis; portal hypertensive complications are most commonly the cause of liver-related complications. In addition, many children with cystic fibrosis are malnourished and have lower muscle mass, which contributes to lower creatinine and a lower calculated MELD. Current exceptions that exist for lung-liver transplant candidates rarely apply to pediatric patients and may become less frequently utilized with the inception of CFTR modifying medications. Specific guidance to include exceptions for children with portal hypertension who have failed or are not a candidate for other interventions, who are experiencing growth failure as a result of their CFLD, or who have a decline in their FEV1 as denoted in the proposed new guidance would more clearly describe the disease of some of our most vulnerable patients. Region 8 | 09/27/2022 Sentiment: 1 strongly support, 14 support, 4 neutral/abstain, 0 oppose, 0 strongly oppose Region 7 | 09/27/2022 Sentiment: 3 strongly support, 9 support, 2 neutral/abstain, 0 oppose, 0 strongly oppose | This was not discussed during the meeting, but OPTN representatives were able to submit comments with their sentiment. One member noted support for this initiative for exception scoring. The guidance addresses two of several diseases that do not fit well into the normal MELD PELD scoring process. There should be exception requests and board reviews for these and other diseases (like PSC). Diseases that can lead to cancers and other issues sometimes do not fit well into the score system; however, patient mortality can be on different paths depending upon what disease they have. With some of the rare diseases, the patient can deteriorate quickly and lose the feasibility of transplantation. The committee should review other diseases for exception ruling. American Society of Transplantation | 09/27/2022 The American Society of Transplantation generally supports the changes outlined in the proposal, “Continued Review of National Liver Review Board (NLRB) Guidance.” We offer the following comments for consideration: Changes to the hepatic adenoma policy are long overdue, and there is broad support for the changes, some of which allow granting of exception points before the development of malignancy. Agree with Budd-Chiari changes, but programs should provide greater granularity and detail to the severity of the hepatic decompensation. Attachment Region 11 | 09/26/2022 Sentiment: 3 strongly support, 14 support, 3 neutral/abstain, 0 oppose, 0 strongly oppose American Society of Transplant Surgeons | 09/26/2022 The American Society of Transplant Surgeons appreciates the opportunity to comment on the Continued Review of NLRB Policy and Guidance. The proposed creation of guidance for pediatric candidates with CF, specifically the proposed FEV1 thresholds set at less than 70% or greater than or equal to a 5% annual decline. ASTS largely supports this proposal as written. However, we are concerned that malnutrition in children with cystic fibrosis may be viewed subjectively and may be related to the pancreatic disease they have—not their liver disease. We support the modification regarding failure of treatments for complications and modified guidelines for pulmonary testing. The proposed changes to guidance for candidates with hepatic adenomas. ASTS supports the proposal as written. The proposed changes to guidance for candidates with Budd Chiari. ASTS supports the proposal as written. Attachment Region 1 | 09/21/2022 Sentiment: 1 strongly support, 5 support, 2 neutral/abstain, 0 oppose, 0 strongly oppose Region 3 | 09/20/2022 Sentiment: 2 strongly support, 8 support, 4 neutral/abstain, 0 oppose, 0 strongly oppose Region 2 | 09/13/2022 Sentiment: 7 strongly support, 12 support, 6 neutral/abstain, 0 oppose, 0 strongly oppose Region 9 | 09/12/2022 Sentiment: 0 strongly support, 8 support, 1 neutral/abstain, 0 oppose, 0 strongly oppose Anonymous | 09/11/2022 About 5 years ago I was notified by letter that at 65 I was too old to be considered for a transplant. Since then I have been told I am too healthy for even a living donor transplant. Supposedly I am compensated but I am Stage 4 with portal hypertension and most other side effects. Recently I found out that my MELD score was not reliable because the input tests were not done on same day-months apart. Next up was bilirubin-that got thrown out because I am too healthy looking for that high-2.8. I also have a note in mychart that I ask too many questions and I can’t be really sick because I don’t go to ER. Hidden age discrimination, ineptitude, or are my expectations too high? Do we need a patient driven review board driven by request for 2nd look? Region 5 | 09/08/2022 Sentiment: 8 strongly support, 18 support, 5 neutral/abstain, 0 oppose, 0 strongly oppose | Comments: A member supported the current proposal and suggested that NLRB guidance to include additional liver related diseases. A member commented that she appreciates the Committee considering pediatric-specific criteria for this guidance and believes it will help to standardize pediatric exception requests and approvals. Pediatric Transplantation Committee | 09/08/2022 The Pediatric Committee thanks the OPTN Liver and Intestinal Organ Transplantation Committee for the opportunity to review their Continued Review of National Liver Review Board (NLRB) Guidance proposal. The Committee provides the following feedback: The Committee agreed with the proposed guidance, specific to pediatric candidates with CFLD. We recognize that the current standard exception was written in reference to lung-liver candidates, not liver-only candidates. Offering the liver alone may improve lung function, and once lung function declines past a certain point, the candidate will may require a liver-lung transplant. However, the Committee suggested clarifying the wording of this guidance since there was confusion as to whether it applied to lung-liver or liver candidates. In addition, we would support future revision of the standard exception guidance so that it more accurately applies to CF liver-only candidates. Region 4 | 08/26/2022 Sentiment: 2 strongly support, 16 support, 3 neutral/abstain, 0 oppose, 0 strongly oppose Anonymous | 08/17/2022 I support the changes for the Pediatric liver transplant candidates: to Create guidance for pediatric liver transplant candidates with cystic fibrosis Anonymous | 08/06/2022 We are requesting a modification of the MELD score process. Genetic abnormalities ie alpha 1 antitrypsin protein deficiency has caused my husbands HCC. His cancer is limited to his liver, he has gone through various cancer treatments and his cancer is under control and Alpha one only affected his liver. He is in good shape good BMI and works out daily. His meld is 10. He will not be able to regenerate his liver due to Alpha 1. His cancer will come back its only a matter of time due to his cirrhosis due to the Alpha 1 Why can't he get points on the MELD SCORE for the genetic disease Alpha 1.