At a glance
What is current policy and why change it?
The National Liver Review Board (NLRB) reviews requests from transplant programs for candidates whose model for end-stage liver disease (MELD) score or pediatric end-stage liver disease (PELD) score does not accurately reflect their medical urgency for transplant. The NLRB can approve or deny the request. If the NLRB denies the request, the transplant program can appeal the case.
The NLRB uses policy, guidance documents, and operational guidelines to inform their decision to approve or deny a request. Based on feedback and lessons learned, the committee wants to update those documents so the process works better for programs and candidates.
Further Enhancements to the National Liver Review Board
Dr. James Trotter, Chair of the OPTN Liver and Intestinal Organ Transplantation Committee, reviews the Further Enhancements to the National Liver Review Board policy proposal.
Terms you need to know
- Candidate: An individual on the organ transplant waiting list
- Transplant program: An organ specific facility within a transplant hospital
- Operational Guidelines: Standard operating procedures for how the representatives on the NLRB are appointed and how and when reviews for exception requests are approved, denied or appealed
- Guidance Documents: Documents that provide specific clinical information to transplant programs and NLRB members to use when evaluating common exceptions
- 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.
- Appeals Review Team (ART): Nine randomly selected members from the NLRB who meet by conference call to review appeals for exception cases denied by the NLRB
- Portopulmonary hypertension (POPH): Pulmonary arterial hypertension complicated by portal hypertension
- Hepatocellular Carcinoma (HCC): A cancer arising from liver cells (hepatocytes)
What’s the proposal?
The proposal includes changes to policy, operational guidelines, and guidance documents.
What’s the anticipated impact of this change?
- What it’s expected to do
- Provide updated clinical requirements in policy for transplant programs submitting an exception request for a candidate with portopulmonary hypertension (POPH)
- Provide a more effective process for reviewing Post-Transplant Explant Pathology forms for candidates listed with hepatocellular carcinoma (HCC)
- Provide more guidance for National Liver Review Board (NLRB) members when reviewing exception score requests for candidates with polycystic liver disease (PLD)
- Create a pediatric specific Appeals Review Team (ART) to review appeals for pediatric candidate exception requests
- Add a member of the Liver and Intestinal Organ Transplantation Committee as the ART leader
- What it won’t do
- This proposal will not impact how liver candidates are prioritized on the match run to receive a potential transplant
Themes to consider
- Additional National Liver Review Board (NLRB) improvements
Status: Public Comment
Sponsoring Committee: Liver and Intestinal Organ Transplantation Committee
Strategic Goal: Promote the efficient management of the OPTN
Anonymous | 08/06/2020
The proposal should be adjusted to include adding exception points for patients with PSC and PBC. The MELD score does not accurately reflect level of sickness and quality of life of these patients, especially in the common scenario where kidney function remains high despite extremely deteriorated liver function. Please consider adjusting for this. Thank you.
Kelly Porter | 08/06/2020
Part of making these changes is to make it easier for exceptions to be reviewed. There would be less exceptions to review of patients with PSC and PBC eere automatically granted exception points due to the severe impact these diseases have on the physical, emotional, and psychological wellbeing of these patients. 3 points, 1 for each of these impacted areas, would be appropriate.
Deven Daehn | 08/06/2020
PSC patients are unfairly forgotten when livers are available due to lower MELD scores. Doctors should be allowed to give at least 3 discretionary points for these patients as they suffer greatly but it's not reflected in the MELD score. My husband is a PSC patient and I was fortunate to be a match for living liver donation. Because his kidneys worked perfectly, he more than likely would not have made it to the top of the list for a deceased donor before developing cholangiocarcinoma. Many PSC patients don't have a living donor option and suffer from hepatic encephalopathy, ascites, jaundice, severe fatigue, insomnia and so much more for years. They often become so sick that they can't be transplanted. The MELD score should account for PSC patients and the suffering they endure despite the lower MELD. Please help PSC patients!!
John Lowery | 08/06/2020
The proposed modifications do not go far enough. Patients with PSC or PBC typically have MELD scores that do not adequately represent the actual severity of their condition. Exception points (3 or more) should be awarded to PSC & PBC patients to more reasonably reflect their health status.
Anonymous | 08/06/2020
These enhancements to the National Liver Review Board are a good start, but further enhancements are needed. Patients with rare diseases like PSC and PBC consistently fall victim to a system that does not accurately depict the severity of their disease. My wife is now subject to surgeries every 6-8 weeks and has been forced to leave her job because of the debilitating fatigue and pain associated with her PSC. Despite this, we have been told her MELD score is too low to even be listed. How long should patients have to suffer before they are even given an opportunity to improve their condition and quality of life? There needs to be a way to provide exception points to account for the severity of liver diseases that do not present the "right" symptoms to produce a high MELD score.
Beth Sicignano-Merritt | 08/06/2020
My husband has been suffering with PSC for over 10 years and is on the transplant list at MUSC in SC. His MELD is 22 and has been in that range for nearly one year. He is very ill and yet because MELD is the primary factor that UNOS considers he waits and suffers in pain. I strongly believe that UNOS should consider giving PSC patients exception points. We are a small community but our voices need to be heard. Our loved ones are suffering for years unlike others on transplant lists who often receive a transplant in a matter of months. Please give this serious consideration! The long term suffering of PSC patients is a vital consideration and should warrant immediate discussion. It's likely one of the most urgent concerns that this board should consider this year. Please hear our voices!
Lisa Longwell | 08/06/2020
I am a Liver transplant recipient as of April 2015, and a currently listed Liver candidate since April 2018 due to dysfunctional graph. I am waiting now 2+ years due to a low MELD score 13 or 14. I suffer from Portal Hypertension and mild HE. With acute Acites needing Parenthesis every 5 day removing 13.3 lbs or 6+ liters of fluid each time since October 2018. A total of 143 Parentheses preformed to date with a total fluid removal of close to 1 TON. I was listed for a short time in 2018 for a Kidney also because of declining function and because I had kidney failure after the first transplant needing dialysis for about 7 months. Within a year time frame I have since been removed from that list due to some kidney improvement. Each day is more difficult to function due to fatigue, difficulty sleeping, fuzzy thinking, edema. I was submitted for review for exception points this year with no success. I believe there should be some priority other than MELD score for a 2nd Transplant Requirement due to a failing 1st transplant. Please consider some type of criteria to gain exception points for a 2nd transplant recipient, waiting time limit, low meld score, acute acites to have a chance at re-transplant sooner than this. Perhaps for every month after 1 year of waiting the candidate could gain a point up to a certain limit so that the chance of transplant increases while the life of the person decreases. This would give the person some sense of feeling like they have a chance. With my deepest sincerity and hopeful consideration
Anonymous | 08/06/2020
Exception points should be allocated as many PBC/PSC patients encounter life-threatening issues that have no bearing on meld score.
Michelle Werson | 08/06/2020
I would like to share my story with you today in an effort to provide just one example of how important it is that patients with Primary Sclerosing Cholangitis receive exception points to their MELD score. I was diagnosed with PSC in the fall of 2016. In hindsight, I can see now that it is likely I was showing signs of this disease as much as 20 years prior to my diagnosis. Within 6 months of my diagnosis, I underwent the transplant evaluation process at the Mayo Clinic in Jacksonville, Florida, and was listed for transplant. From the time of my diagnosis until transplant, I was hospitalized several times with cholangitis attacks as my disease rapidly progressed. I had several complications from the disease, but the greatest of which was having esophageal varices. During a standard ERCP, my doctors discovered this complication and performed a procedure commonly known as "banding" in an effort to prevent the rupture of the varices. Unfortunately, the procedure did not prevent rupture, and I had a massive hemorrhage on February 17, 2018. Several doctors at 2 different hospitals fought for hours to stop the bleeding to save my life. I was sedated during most of this time, but not before I experienced the trauma of bleeding out. It's the kind of pain I will never forget. After the doctors at Mayo Clinic stopped my bleeding, I was intubated for 2 days and received 9 units of blood. When I was weaned off sedation and extubated, I was discharged straight from the ICU to go home in the hopes that I would survive long enough to make it to transplant. Afterall, there wasn't anything else they could do to help me. My MELD score at this time was 17. I am so incredibly grateful for a medical team who fought for me and petitioned for exemption points on my behalf. By March, I had received 5 exemption points, bringing up my MELD score to 22. As you well know, that is still quite a low number to hope to get a transplant with. However, on April 2, 2018 I received the phone call that would save my life a second time in less than 2 months. A liver had become available to me, and on April 3rd I received the gift of life. This was possible not just because I had received exception points, but because I was willing to accept a high-risk liver. Specifically, a liver that was Hepatitis C positive. This was a risk I was happy to take in order to spend more time with my children, who were 5 and 8 at the time. Without those exception points, I'm afraid I might have become a statistic of those who die while waiting for a transplant. That is a fate that no one deserves. I will continue to appeal to the public to become an organ donor, but today I appeal to you to consider providing special exemption points to those specifically diagnosed with Primary Sclerosing Cholangitis. The MELD score system DOES NOT accurately reflect how ill someone one with PSC really is. The method of calculation does not take into account the specific nature of this disease, and is therefore biased against those with PSC. I am grateful that I can raise my voice on behalf of those who have gone before me and did not survive to transplant, and on behalf of those who come behind me, facing the uncertainty of their future. Everyone deserves a fair chance for effective treatment while we wait for a cure.
Julie Steele | 08/07/2020
Please consider giving exception points to those with PBC and PSC. The quality of life can be so out of sync with their actual MELD score. As a former liver cancer patient I can say having those exception points really made a difference for me.
Johanna Dodge | 08/07/2020
Meld guidelines for PSC should be updated to give us a fighting chance at life. We are a group of fighters who are small, but are slowly being heard. This fight will go on as our voices continue to grow. Do not wait any longer to hear our voices. Please. Update the qualifiers so our meld scores are taken into account as well. Not just the majority. Isn’t this what America and medicine is about? Improving as we increase our knowledge?
Jeff Donovan | 08/07/2020
I am writing to ask that you give exception points to patients with advanced Primary Sclerosing Cholangitis and Primary Biliary Cholangitis. I have been diagnosed with PSC for 11 years and I am currently feeling okay. However, I have watched so many in our PSC community struggle to get liver transplants because the MELD score often does not reflect how sick these patients are. Currently, someone that has had an addiction to alcohol and has been sober for 6 months will have a higher MELD score than someone that has been living with this debilitating disease for many years. Please consider allowing exception points for these autoimmune patients that the extreme sickness is not reflected in their MELD score. Thank you. Jeff Donovan
Lezlee Peterzell-Bellanich | 08/07/2020
Dear UNOS National Liver Review Board Members, First of all, thank you for your commitment to saving lives through organ donation. Your dedication and hard work is so appreciated. One of the goals is to create the most fair system for people waiting for life saving organ transplants. The current UNOS policy on exception points for people suffering for years with Primary Sclerosing Cholangits (PSC) or Primary Biliary Cholangitis (PBC) is way too restrictive forcing patients to remain in “Meld Score Purgatory”, usually unable to rise to a competitive MELD score which would qualify them for a liver transplant. One important reason is that half of the MELD score measures kidney function and often PSC and PBC patients have livers that barely operate but kidneys that are working fine. Because the MELD score is traditionally low for this small percentage of liver diseased patients, they often look for living donors which risks two lives, is difficult to find, and does not solve the initial bile duct problem to begin with! Furthermore, unless a patient has health insurance which allows them to travel outside of their state, they are potentially locked into a region with higher median MELD scores increasing their chances of death before transplant. My husband has been suffering psychologically and physically from PSC for 18 years. We temporarily moved from New York to Florida to “chase the MELD score” problem yet he is still low on the list. He has jaundice, severe itching, dramatic muscle wasting, extreme fatigue and yet his MELD score actually went DOWN after being listed at Mayo Clinic for transplant for the past 10 months. We recently pleaded with his doctor to ask for exception points, but they were denied by UNOS because of these narrow conditions for points so he is stuck in a holding pattern unless something dramatic happens. We did have two dry runs but only for high risk livers. At this point, PSC/PBCers feel desperate and worried it will never happen so they put themselves at further risk simply because of this score that works against them. My husband’s own hepotoligists in New York and Florida agree that the MELD score is stacked against PSC and PBC patients. One of the Mayo Clinic surgeons told us that in his experience, PSC patients do much better with deceased donors because they need the whole cadaver liver with all the original plumbing reducing their chances of vessels shredding or PSC recurrance. But how can they get a quality liver if their MELD score doesn’t change? The new changes make it even more difficult for doctors to get exception points for their PSC/PBC patients unless they are hospitalized with an infection or have developed cancer. The psycholigical component of living sometimes for decades with this disease is currently not taken into account. This is unacceptable. The MELD score does not tell the whole story of a human being with these rare diseases. They need to be treated differently and given more weight as soon as patients are listed for transplant. We are advocating that PSC and PBC patients get exception points immediately at transplant without having prolonged suffering and risk of death. It is radical but warranted. Since average MELD scores are higher in certain regions, it is only fair to adjust the exception points to that region. You might be aware but a petition was started three days ago explaining to the public what is happening. In three days, we have over 1000 signatures in support of change. UNOS Board Members– as a recent UNOS Ambassador, I understand that you are key to changing this policy on behalf of PSC/PBCers. Please. Listen to us. Please don’t take years to make a decision. How much research do you need? Listen to the people with PSC and PBC who are living with this and being negatively affected by the MELD score. We need this changed as soon as possible. Thank you. PS: YES. I will just say it. PSC/PBC Lives Matter!
Peter Mellom | 08/07/2020
Guidance documents should reflect the candidate’s subjective condition in terms of pain and suffering, therein adding to the candidate’s MELD score. The NLRB should defer to requests from the transplant programs to increase MELD scores because of the greater knowledge gained by physician/patient contact. There is no reason why the objective criteria stated in the adult MELD exception review policy be given greater weight over the physician’s focus on the candidate’s immediate suffering and need.
Kimball Peed | 08/07/2020
PSC ravaged my well-being for 30 years before transplant. Living with PSC and "liver itch" (pruritus) was horrible. At the time of transplant, I was very near death. My old liver looked like it had been burned. Because of the way the MELD score is calculated (my liver-related numbers were atrocious, but my kidney function numbers were too good), I was forced onto the edge of mortality before my MELD score gave me a chance to receive the gift of life through transplant. And, my pre-transplant decline meant that my recovery was prolonged. Moreover, I had to be admitted to the hospital multiple times via the ER before transplant, where the good doctors and nurses made me better but reduced my MELD score, taking away that chance to be transplanted. PSC does not happen because of anything a person does or doesn't do. But PSC victims get penalized by the MELD scoring methodology relative to other liver patients. Please fix this. Thank you.
Anonymous | 08/08/2020
Using only a MELD score to determine a PBC and PCS patient's candidacy for liver transplant is unreasonable. Often times the MELD score does not accurately represents the patient's true condition. Each should be evaluated as an individual and not as a number represented on a chart that was most likely created by someone who has never suffered from one of these debilitating diseases. Waiting until someone is on their deathbed but still healthy enough to survive a transplant is cruel and inhumane. Many of us candidates are forced with making a life and death decision based on a number. For example; I intend to keep working until my employer actually has to fire me for not being able to perform or until I die (possibly on the job). Why? Because I need the health insurance. I often think what it would be like if people in my position could receive an exception and receive the life saving transplant before I have lost everything. At that point, I will die because I will no longer be able to afford the treatment and medications. Imagine if cancer patients had to wait until they met a specific score before they could receive treatment; "I know you have cancer but here are these pills you can take that may or may not help you but when you reach a score of X, we will put you on a list to receive chemotherapy". The proposal is a good start but it does not go nearly far enough to make sure that patients who are truly suffering receive a second chance at life. I encourage you to strongly consider allowing exception points to PBC and PSC patients. We are just as worthy of the life saving transplant as anyone else and we shouldn't have to beg to be seen as such.
Britta Moellenbeck | 08/08/2020
The proposal should be adjusted to include adding exception points for patients with PSC and PBC. The MELD score does not accurately reflect level of sickness and quality of life of these patients, especially in the common scenario where kidney function remains high despite extremely deteriorated liver function. Please consider adjusting for this. Thank you.
Anonymous | 08/09/2020
I suffer from PSC and have been sick with this disease for 15 years. I'm currently on the transplant list for over a year. My kidneys work fine, since this is a disease of the bible duct system. However, my legs and abdomen are swollen, the constant pain is rough, my brain is foggy and the worst part is I'm so tired that I can barely stand some days. My quality of life is pretty bad but I'm doing my best to keep positive. This disease would be so much easier to cope with if there was hope but my MELD score has and likely will hold steady at 22 so I linger and waste away praying for the end some days. I know that PSC is rare so I'm sure there's not a lot of us squawking but we NEED exception points. I've seen folks come in with liver diseases like alcoholic cirrhosis and their MELD shoots up pretty quickly. They're spared the long term suffering PSC patients endure. I'm always happy for them but sitting at the sidelines suffering terribly for years makes it difficult to not speak up, for myself and fellow PSC sufferers. I'm asking with great earnestness that exception point be given to PSC patients on the list. Please consider adding them based on the time a PSC patient has been on the list. I promise you that although the MELD does not reflect it the symptoms of this disease progress and worsen. It plays havoc on a person's body and soul. It's painful and it breaks the spirit of even the strongest of us. Action MUST be taken. Long term suffering is a point of consideration for exception points for PSC patients. Thank you for your work and allowing patients to weigh in! We (my family) and I sincerely appreciate the opportunity to be heard! Mike M
Holly Andersen | 08/09/2020
Please award exception points to liver transplant candidates with PSC. Especially those with Cholangiocarcinoma who are eligible. It is urgent that they receive a donor liver. They may not be "sicker than meld" but they are more "urgent than meld". Thank you
Anonymous | 08/10/2020
I am a PSC patient. I am a pre-transplant patient and have been on the liver transplantation list since June 2019. PSC patients suffer from ascites, jaundice, fatigue and many other conditions. We suffer from psychological and physical problems. We need your help. We are asking for 3 Exception points for PSC and PBC patients who are awaiting transplant.
Kara Hartman | 08/10/2020
As the mother of a 23 year old son with PSC, I am begging that you allow exception points for this horrible disease. Just because a patient looks ok on the outside says nothing about how they’re feeling on the inside. My son has had over 15 hospital stays due to Cholangitis attacks. He has missed countless days of school, work and many vacations due to his recurrent PSC related issues. When the day comes that he’ll need a transplant, I worry his MELD score won’t reflect the severity of his illness. There’s not an hour that goes by on any day that I don’t worry about this. He should be enjoying his life, not burdened with the stress of worrying about the future and “what ifs”. Please imagine if your son, daughter or loved one was going through this. I’d move heaven and Earth for my son but I can’t do it without the support, empathy and understanding of people in positions that can truly help. I pray PSC gets the exception points deserved. Kara Hartman
Renee Bacote | 08/11/2020
I am a 50 year old, end stage PBC patient with a MELD score of 12. I sincerely and respectfully beg for these changes to be implemented as soon as possible. Help me live!!!
Daniel Sedgh | 08/16/2020
Please consider giving a small number of exception points added to the MELD scores of people suffering from late stage PSC and PBC for the disease itself; OR take the median regional MELD score and reduce it slightly for PSC/PBC so they can more quickly receive their life saving liver transplant before going into a more potentially fatal situation which could cause complications during transplant surgery.
Jim Johnsrud | 08/16/2020
I strongly encourage the addition of exception points for PSC patients waiting for a liver transplant. I am a PSC patient and although I look and feel fairly well, I have had life-threatening complications with UC, gall bladder removal leading to acute liver decompensation, bile duct infections leading to sepsis, spontaneous lower GI bleeding,development and worsening of esophagael varices which is extremely worrisome to me. My MELD hovers around 15 and I have actively advocated for a living donor for years with no success, understanding that cadaveric whole liver organ is better suited for successful transplantation in people with PSC.
Neil Eglash | 08/16/2020
Please consider adjusting MELD scores for patients who have PSC. Low MELD scores are the norm for patients like me and we get passed up on the liver list even though we suffer greatly. The itching alone almost caused me to jump off the 18th floor -- not to mention the liver pain and swelling! Please! This would be such a merciful gift to PSC patients. Thank you for listening.
Robert Grant | 08/18/2020
I do think that PSC patients, who often have a long and arduous struggle should have some exception to the standard MELD score
Anonymous | 08/18/2020
UNOS - Please consider giving a small number of exception points added to the MELD scores of people suffering from late stage PSC and PBC for the disease itself OR take the median regional MELD score and reduce it slightly for PSC/PBC so they can more quickly receive their life saving liver transplant before going into a more potentially fatal situation which could cause complications during transplant surgery.
Sharon Swadner | 08/18/2020
I have PBC and everyday is a struggle. It is unbelievable that my disease is considered less important than other liver diseases when it comes to a transplant. Please make the liver transplant process the same for everyone.
Deborah Iacoboni | 08/19/2020
Please consider exception points for PSC and PBC liver disease for transplant. I had PSC for 20 years before my transplant October 2009. I am so thankful and very lucky. PSC is a debilitating disease that just wastes you away. It causes life long problems even after transplant, such as bone loss (osteoporosis) short term memory loss, because of the HE and muscle mass loss. I believe that if you would give exception points and the transplant before your body wastes away, that quality of life after transplant would be much better. 20 years is a very long time to slowly die. That’s what this disease is. A long slow death.
Kathleen Wilson | 08/21/2020
I am submitting this comment in support of providing exception meld points for patients of PBC and PSC. I have been diagnosed with PBC for 13 years now. My liver has been biopsied and I am now at stage 4 cirrhosis. My meld points are now being calculated. Due to my condition it has caused my health to decline as to where I am now having to step down from my job due to fatigue and brain fog which is causing cognitive issues which are in addition to the swelling of my abdomen, bouts of extreme itching, nausea, joint pain etc that I have come to accept as a part of my daily life. The institution of exception meld points is important for PBC and PSC patients as I personally watched my grandmother suffer and deteriorate from PBC for many years until her death. She was bed ridden and then in and out of the nursing home for years. She had to have a closed casket due to the overall deterioration of her body. I was young when she passed away, but watching this beautiful human being suffer who did not receive a transplant and the emotional pain it caused my mom watching her suffer is additionally why I am in support of this change. I hope that my comment is taken into consideration as the current scoring system does not accurately portray the overall health condition and suffering PBC patients experience. My hopes are that the exception points will be added to patients afflicted with this incurable disease to maintain some quality of life and the opportunity to alleviate suffering before they are too sick to qualify for a transplant. I would not want to see anyone experience the pain and suffering my grandmother did. If a liver was available to her before her complete health decline our family would have been able to have her in our life longer and she would have not have suffered the excruciating pain she did before she died. Thank you for taking my comment into consideration. Your organization is greatly appreciated and everything they do to make organ transplantation fair and just. Thank you again for taking the time to read.
Maryann Austin | 08/22/2020
I am a 53 year old mother of two sons. One is only 14 years old. In 2018, after several years of extreme symptoms, I was diagnosed with PSC. I regularly traveled almost 3 hours for scans and care. With a MELD of around 12, I was not even on the radar for transplant. And, there is no cure for PSC, so treatment consists of monitoring and attempting mitigation of symptoms. Meantime, I was too affected to continue working or living a normal life, but not sick enough for a new liver per the current policies. In February of 2020, I was on my way to meet with my hepatologist, thinking I would be discussing transplant. Finally! A recent ERCP showed that my left lobe was obliterated. Sad that I had to be happy about such news to hope for a transplant. To my shock, my doctor told me that I have cholangiocarcinoma and was not a candidate for transplant. I was referred to another hospital and have now undergone IV chemo. I am currently undergoing several weeks of radiation therapy. I am not sure if I will live or die. If I respond to treatment, I may receive a new liver. I am undergoing testing. Had the current system been more sensitive to the progression of PSC, I may have been listed for transplant before becoming as ill as I am. It seemed that, overnight, my health jumped from too well for help to beyond hope. My life, and the lives of my loved ones, would be very different right now had the MELD system been geared to assist me. Please consider the many PSC and PBC patients who suffer these diseases through no fault of our own, and who are unfairly discriminated by the current policies.
Priscilla O'Shields | 08/22/2020
I am a PBC patient currently listed in California and Illinois via the living donor transplant process. I was diagnosed just over 4 1/2 yrs ago. However after looking back at different symptoms my Dr.s believe I have been living with PBC for nearly 18 yrs now. I am one of those patients that has a lower meld score that doesn’t truly reflect the progression of my disease. My meld score is currently 14. I am extremely jaundiced and itch incessantly. I have constant abdominal pain but my scans always come back the same. I have been through every medication on the market for treatment including immunal suppressants to no avail. My drs have told me there is nothing more that can be done and my only option is transplant, but of course my meld score is too low to expect and offer. On several occasions they suggested that exception points could improve my chances of receiving a donor liver. However, every time they have presented it to the board the exception points are denied. I have been going through the living donor option but that has also proven difficult. All donors that have been tested have all been disqualified for one reason or another. The most recent just last week due to a fatty liver. This is so emotionally exhausting for not only myself but my husband and children. We have come to accept that with out the exception points my disease will have to progress significantly to put me high enough on the list to receive an offer. This process is unfathomable because everyone believes my quality of life would vastly improve and the potential of a positive recovery is substantially better as PBC is my only ailment. I have been blessed to not have overlapping diagnoses. Exception points could change everything for me. They could improve my chances and help me live a longer life. To spend that precious time with my family that is currently overrun by itching and fatigue. Please consider these changes to improve not only mine but many others possibilities to be given this gift of life!!
Anonymous | 08/25/2020
Please adjust the proposal to include adding exception points for patients with PSC and PBC. The MELD score does not accurately reflect level of sickness and quality of life of these patients. The common scenario in these patients is that kidney function remains high despite extremely deteriorated liver function, which means patients are much, much sicker before they have a high enough score for transplant. Please consider adjusting for this disparity by allowing exception points in patients with these diseases.
Kristyn Jarvis | 08/25/2020
Where are the detailed proposed changes for PSC patients? Where is the section on how additional MELD points(3-5) should be given to PSC patients with low MELD and advanced and terminal liver disease? Why are PSC patients ignored in this proposal? It is devastating to watch a loved one suffer so much with a low MELD. The doctors and two transplant centers we are working with say there is nothing they can do. Why are we all helpless against UNOS? They are intentionally prioritizing other types of liver diseases over PSC and PBC. Why would UNOS refuse to update a system that rewards many negative lifestyle choices(obesity, drinking, and drugs) with quick liver transplants but fail to provide the same service for those who have PSC? If even one member of the National Liver Review Board had but one family member with PSC, none of these comments would exist on this page because the change would have already happened. Since the decision makers are unable to recognize what needs to happen, we must flood the comments with the reality they do not wish to acknowledge. Maybe someone is listening to our pain. Maybe someone is listening to the heartbreaking injustice of the MELD score.
Region 4 | 08/26/2020
Region 4 vote: 8 Strongly Support, 12 Support, 4 Neutral/Abstain, 0 Oppose, 0 Strongly Oppose Region 4 supported this proposal. During the discussion, one attendee commented that the ART "Leader" for the Pediatric NLRB should be an experienced UNOS certified Pediatric physician or Surgeon and not "just a Liver Committee member" which is most likely to be an adult liver physician or surgeon. Another commented that ruling out other causes of POHP can be difficult due to patients who have multiple risk factors.
Anonymous | 08/28/2020
Please allow MELD score exception points for those with PBC. I’ve been listed for over 20 years in three different states and am now putting my death with dignity request in order.
Wendy Babb | 08/28/2020
Exception points should be given to PSC patients. As a PSC patient even my doctors have told me that it is not a fair scoring system. Not only do we get shirked on scoring, but very little research is being done to figure out what this disease is all about. Incredibly frustrating and ultimately life threatening. My husband, children and I thank you so much for taking action.
Region 5 | 08/28/2020
Region 5 vote: 5 Strongly Support, 23 Support, 3 Neutral/Abstain, 0 Oppose, 0 Strongly Oppose. Region 5 supported this proposal and had the following comments: • We strongly support the new pediatric component. • Strongly support the creation of the Pediatric Liver ART. Adult clinicians should participate in the Adult Liver ART and Pediatric clinicians in the Pediatric ART.
Katelyn Turk | 09/02/2020
When we talk about enhancements to policies, it's to positively affect the patient. In this case, we need to add more pressure to get more exception points for those with PSC and PBC. PSC and PBC are both diseases that are rare and when these patients are added to the transplant list, their meld score is too low to receive a deceased liver. As you know, their bile ducts become very narrowed but their blood work may look fine. This is dangerous, as cholangiocarcinoma is the thought of why the bile ducts are failing and we don't have enough resources to completely rule out this cancer. This is what is happening to me. My meld score is currently an 8 and multiple doctors from multiple hospitals are telling me I need to get a transplant within a few months. Problem is my meld score is too low to possibly receive a deceased liver. Instead I need to find someone willing to undergo a transplant to donate part of their liver. If we were to receive these exception points, this would help those with this disease to receive the transplant before possibly testing positive for cholangiocarcinoma. UNOS, please help us. We're a small community of people that need extra points to possibly save our life. Thank you for your time.
Larry Stahl | 09/09/2020
I am a retired primary care physician who was transplanted with a non-living donor liver for PSC. That was 2018 and I was age 67 at the time. I appreciate the opportunity to comment on NRLB policy changes being considered at this time. I was diagnosed in 2007 after insurance labs showed abnormal liver findings. I was referred to a very competent gastroenterologist in my hometown who made the diagnosis after ERCP. I was referred to an in-state transplant center and there received great care for 10 years. Then I developed end-stage cirrhosis with symptomatic hepatic encephalopathy. This required me to go on disability. My excellent hepatologist advised me to strongly consider liver transplant. I completed a transplant evaluation but was not listed for transplant. The reasons for my non-listing were 1) a discovery of coronary artery disease which had to be treated with a coronary stent 2) my age (66) 3) low MELD score. This left me needing to seek referral to another center. My hepatologist referred me to another region where I was evaluated and listed. Four months before receiving my transplant, I had a severe episode of cholangitis with sepsis and septic shock. After receiving in-patient care in my home community, my hepatologist requested exception points. I did indeed receive 3-4 exception points for the septic shock and went on to be transplanted with excellent results. I believe that the exception points awarded for the single episode of septic shock allowed me to be transplanted and saved my life. I understand that the National Liver Review Board and its committee for Liver and Intestinal Transplantation is considering policy changes which may be very beneficial for patients with cholangitic diseases. Two issues are important to me: • The efforts to broaden criteria for portopulmonary hypertension receive my wholehearted support. These efforts will give objective designation to POPH patients which will benefit those of us with low-MELD score diseases. I think the evolution away from subjectivity is a big step in the right direction. I believe these changes will allow end-stage cirrhosis patients who have POPH to be objectively classified and receive consideration near time of transplant. • I also believe that if exception criteria are to be utilized, a person who has had in-patient treated sepsis should receive exception points even if it's only one episode. In my case the one episode resulting in a blood pressure of 50/25 could easily have resulted in my demise. I may well have not survived to experience multiple in-patient episodes of sepsis. Yet multiple in-patient episodes are now required by new UNOS regulations for exception criteria. Thanks for the opportunity to comment on what I consider to be very important problems which I feel will be positively addressed for those of us with PSC, PBC.
Region 7 | 09/10/2020
Region 7 vote: 6 Strongly Support, 8 Support, 3 Neutral/Abstain, 0 Oppose, 0 Strongly Oppose Region 7 supported the proposal and had the following comments: • Attendee commented that hopefully this proposal will address some of the issues members are encountering with allocation.
Sharon Nanz | 09/13/2020
I support the proposed enhancements and have the following additional concerns and suggestions regarding future updates to NLRB positions. My comments address a need that I urge the NLRB to take under consideration during the next round of enhancement proposal discussions – the need for a close examination of how MELD scores and exceptions scores are assigned to primary sclerosing cholangitis (PSC) patients with end-stage liver disease (ESLD). Because the MELD score calculation includes indicators of kidney health, and many PSC patients with ESLD do not have kidney dysfunction, MELD scores for these patients frequently remain too low for them to get a liver transplant in a timely manner. Yet while they scores are low, their suffering is high. Why I care about this issue: Twice my daughter found herself in ESLD due to PSC, at ages 15 and 21. The first time, while on the waiting list with a MELD of about 14-15, she suffered a major variceal hemorrhage. She vomited blood, required air transport to center that could treat her, and in the interim slipped into a coma for two days due to severe, acute hepatic encephalopathy. Her doctors were concerned enough to test her brain function. Fortunately, she recovered from that episode, but her Tx team told us that she would not live to see the top of the transplant list and that we must line up a living donor. It took five months to test several donors and find a suitable match for her first transplant. Two years after transplant, her PSC returned, as it does in a minority of patients. She developed intractable itching (a living hell of indescribable proportions) and endured 20-25 hospitalizations for cholangitis attacks, 10 PICC lines leading eventually to a port placement (a procedure that resulted in a punctured lung and several days with a chest tube), and crushing fatigue, weakness, and eventually accelerating weight loss and severe ductopenia. Her MELD was 12! Thank God she was listed during a time when she could be awarded exception points without having sepsis, which she might not have survived. TWO different regional review boards recognized her dire plight and raised her MELD to 30. Even at that, it took many months to get her deceased donor transplant at age 21. Suggestions to consider for PSC patients: 1 – Find a way to weight the MELD score differently for PSC patients. A PSCers’s liver can be very decompensated and yet their kidneys are doing OK. Waiting for their kidney to also malfunction could mean that they become too ill to transplant. Employ a scale or system to measure weakness, muscle loss, and involuntary weight loss, as these are good indicators of decline. Find some way to measure the toll the itching takes on affected patients - it some cases, it can cause severe depression and anxiety, putting patients at risk of death by suicide. That is a mortality risk, too. Use the findings to augment the MELD. 2 – Analyze the data for the new acuity circles in such a way that you can find out if these circles are helping PSC patients in any way. If centers’ average MELD scores at transplant are above 20-22 or so, as I expect that they still will be, most PSCers won’t qualify for a transplant until they are on a fast slide towards death. 3 – I know the ethics are tricky on this, but some other liver diseases have treatments and lifestyle changes that could, if successfully employed, avert the need for a transplant. This is not the case with PSC. There are NO approved treatments for PSC, other than a few symptom management medications, which only work for some patients. 4 – In spite of the fact that a percentage of develop recurrent PSC, most PSCers do very well after transplant. Since their disease was not caused by their lifestyle habits or addictions, their new, transplanted liver has an excellent chance of long-term graft survival. Until there are more donors, surely the overall graft survival likelihood should be a consideration. Finally, I commend you on your commitment to wrestling with these difficult issues. They are not easy, and a change in favor of one patient creates a disadvantage to another. Ultimately, the broader answer is a greater number of organ donors. Even broader than that are new therapies that decrease the need for transplants at all. In the meantime, please don’t forget about PSC patients – they are suffering horribly. Mortality risk as defined by the current MELD calculation should not be the only criteria that counts. Thank you.
Region 3 | 09/15/2020
Region 3 vote: 1 Strongly Support; 19 Support; 5 Neutral/Abstain; 1 Oppose; 0 Strongly Oppose: Comments: A patient family representative in attendance shared her concern on behalf of patients with a rare autoimmune disorder PSC and PBC because these patients are very sick but have historically low MELDs and therefore long wait times. Instead of going into a transplant stronger with better outcomes, they are needing to either be hospitalized with sepsis or develop cancer to get exception points and are asking for exception points when being listed. A member asked if the pediatric ART leader would be a pediatric hepatologist and the response is that the Liver Committee is requesting feedback on ART composition including for the pediatric ART. Additional comments submitted online during meeting: • It is quite obvious from a lot of the comments during today’s meeting and online that the proposal should be adjusted to include adding exception points for patients with PSC and PBC • Agree with all proposals
OPTN Membership and Professional Standards Committee | 09/18/2020
The MPSC thanks the LIC for presenting a focused presentation on proposed changes to Policy 9.5.I.i Initial Assessment and Requirements for HCC Exception Requests in its proposal “Further Enhancements to the National Liver Review Board.” The MPSC offers the following questions and comments in regards to the proposed changes: • Looking retrospectively at the pathology is unfair to the member because they didn’t have the pathology when they listed the patient. Why isn’t this asking for the information at the time of transplant with radiology instead of the pathology upon explant? • If the LIC intends to refer members to the MPSC, then it should be stated in the policy. • The LIC may need to consider what their metrics for referring members to the MPSC are and what they would like to achieve by doing so. The MPSC took a sentiment vote of 7 strongly support, 22 support, 1 neutral/abstain, 1 oppose.
Susan Klein | 09/19/2020
Our daughter has been battling Primary Sclerosing Cholangitis and Primary Billary Cholangtis for 9 years. Please consider exception points for people with PSC and PBC, the MELD score does not reflect how sick these patients really are. Thank You.
Donna Hart | 09/22/2020
Please consider exception points for patients with PSC and PBC. These diseases are dreadful and they need special consideration in the MELD scoring system. Please read through this story. My son, now 31, has PSC and is 6 months post living donor transplant. He was diagnosed with PSC in 2011 and was placed on the transplant list in 2012 with a MELD of 15. Over the years since, his health has deteriorated, and his quality of life was diminished. He could no longer work or engage in social activities with friends. He became somewhat reclusive due to the prominent yellow color caused by jaundice. The severe jaundice caused constant itching that could not be helped with any medication. (His constant itching caused him to lose all hair on my arms and legs.) The severe fatigue was overwhelming and he spent much of the decade of his 20’s sleeping. His MELD score did not, in ANY way, reflect his illness. Over the past two years has been in the ICU three times with pneumonia and septic shock, gastrointestinal bleeds due to portal hypertension (almost bled to death), and severe malnutrition. When the septic shock episodes happened, yes, his MELD would go up into the 30’s, but because he was septic, a transplant could not be performed. During the first septic shock episode he was given a 5% chance to survive. He’s a fighter and a PSC Warrior and he surprised them all and survived, and then developed a colon bleed that could not be controlled. During the time he was bleeding his hemoglobin was 4 and he was receiving constant blood transfusions (44 units in total…forty-four) until it was determined a TIPS (Transjugular Intrahepatic Portosystemic Shunt) needed to be inserted into his liver to relieve the portal hypertension to stop the bleeding. During this two-year period, he had been transported once by ambulance and once by helicopter to his transplant center due to the severe nature of his illness. At one point my son was granted MELD exception points, and was on target to receive more points, putting him higher on the list and closer to a transplant, but OPTN changed the MELD exception point rules and my son’s MELD was again knocked down. The third time he was in the ICU at his transplant center, out of frustration we decided to have him transported to another transplant center one mile away. Once at the new transplant center, the bacterial infections were controlled, his MELD went down to 19, and he was told he should find a living donor because his MELD did NOT reflect his illness. His liver had gone from fibrosis to cirrhosis and he did not have much time left. PSC had not yet affected his kidneys keeping his MELD score lower. This disease also caused osteoporosis, kyphosis, and scoliosis as he waited on the list for 7 (seven) years. This disease is dreadful. His bilirubin was 30! THIRTY. He was severely malnourished due to malabsorption of nutrients. The months leading up to a living donor transplant he was getting monthly ERCP’s and stents placed in his bile duct to try to keep the bilirubin down a bit. My son was fortunate enough to find a living donor. No one in our family was a match and we had to reach out to the community to find a donor. Fortunately, there were many people who came forward and a match was found. Then due to some suspect CCA cells found in brushings done during my son’s ERCPs, the transplant had to be postponed until a solution was found. If it were not for the innovative thinking of the transplant team at his transplant center, my son might have had to wait even longer for a transplant, and perhaps he would not live long enough to receive it. Finally, in March of this year, my son received his living donor transplant, and was the last living donor transplant to be performed at his transplant center when COVID-19 shut down all “voluntary” surgeries. We are forever grateful. I will also add that my son’s paternal grandmother has PBC and was transplanted in 1992, and still living well. At that time MELD scoring was not used. Her symptoms were much like my son’s, and she also had the severe jaundice. My son’s story is only one story. How many others with PSC and PBC suffer, wait and linger for their MELD to raise, and then become so ill that they are too sick to transplant? The scoring system needs to be altered so these PSC and PBC patients have a better chance for life. Please reconsider how the MELD is calculated for them. Thank you for your time and consideration.
Region 8 | 09/22/2020
Region 8 vote: 4 strongly support, 13 support, 3 neutral/abstain, 1 oppose, 0 strongly oppose Comments: One member stated they are strongly in favor of the pediatric component to the review board.
SPLIT | 09/23/2020
I am so thankful for my SPLIT community for advocating for children on the waitlist. In response to the pediatric specialty ART: this NLRB enhancement is sorely needed. Our transplant team has seen so many comments regarding denials of our pediatric non-automated exception score submissions that seemed to indicate that there was a lack the core pediatric knowledge needed for the review. Our team welcomes feedback from NLRB, but it must be constructive and from peers that know pediatrics. This change will make the scoring on a more even playing field for kids. Currently we are relying on those who are not trained in the field of pediatrics to make decisions for pediatric listed patients, and it is resulting in very inconsistent voting among reviewers that we can tell from the comments are adult specialists with limited to possibly no pediatric experience.
American Society of Transplantation | 09/24/2020
The American Society of Transplantation is supportive of the proposal as written. We agree that pediatric NLRB reviewers should be UNOS certified pediatric physicians or surgeons, as children have very different risk factors than adults.
Region 1 | 09/24/2020
Region 1 vote: 5 Strongly Support, 6 Support, 1 Neutral/Abstain, 0 Oppose, 0 Strongly Oppose Comments: Region 1 supports this proposal. There was a question raised regarding updating the POPH criteria. There was a request made to clarify how to measure pulmonary pressure and whether it requires a catheterization; policy language does require documentation of heart catheterization.
Region 2 | 09/25/2020
Region 2 vote: 13 Strongly Support, 17 Support, 3 Neutral/Abstain, 0 Oppose, 0 Strongly Oppose Comments: • An attendee noted their support of creating a separate pediatric ART, but the Liver Committee member assigned to the ART should have pediatric expertise. It may be beneficial to also include a member from the Pediatric Committee on the ART. • Another attendee recommends that the exception score criteria for polycystic liver disease should include more granular criteria with respect to nutritional status. • Another attendee stated that it would be optimal to include nutritional parameters into exception score consideration for adults, as is already done on an ad hoc basis in pediatrics. It is not enough for the patient to be malnourished; however, there should be ongoing efforts to improve nutrition, including high calorie diets and supplemental overnight nasogastric feedings. Having malnutrition can also be a predictor of poor outcomes so it should not, by itself, give a heightened score.
Hospital of the University of Pennsylvania | 09/25/2020
The exception score criteria for polycystic liver disease could benefit from more granular criteria with respect to nutritional status.
Anonymous | 09/26/2020
Please grant exception points for those with PSC. The MELD score does not accurately reflect how sick they are!