Question & Answer Panel Session I - Drs. Hughes, Chopra and Bergasa

(Note that some questions were tabled for afternoon breakout sessions which explicitly address the questions. The responses are not direct quotes but summaries of discussions).

Summaries prepared by Arne Myrabo.

For Dr. Hughes – Why are there yellow areas on the transplant map?
Those are areas in which MELD scores are very low or there are no transplant centers. (See PowerPoint presentation)

For Dr. Hughes – Are there ongoing discussions about the inequities that you show? Are there efforts to restructure the regions based on availability of organs?
Right now there are no discussions to restructure the regions. The question is how can we change the allocation scheme to allow higher MELD patients to be transplanted across regions. Any time that UNOS tries to make a change in organ allocation, it is a multi-year process. First they have to model that allocation algorithm, and then they have to present the data. And finally they have to give time for public comment. That’s why they post these procedural changes to allow the public to have input. The last item that was discussed was called “Share 29,” which would allow a MELD score of 29 to be shared more globally than is currently the case. That actually was turned down (it’s a voting system), as the majority has to agree.

For Dr. Chopra – Why are you doing less liver biopsies these days? I was told biopsy is the gold standard for diagnosing PSC. What are additional diagnostic tools?
PSC involves bile ducts both inside and outside the liver. A limitation of biopsy is that the tiny little piece you get may not pick up the disease process. Secondly, there is sufficient evidence to show that different parts of the liver are affected differently by the disease. If you take a biopsy from two different sides of the liver, you may get two different stages of the liver disease. The “gold standard” is a combination of blood tests and pictures or images of the liver. Today, an MRCP at a reputable facility can usually produce that diagnosis. ERCPs are reserved for situations that require a therapeutic solution such as a new or a dominant stricture. Biopsies are still performed where overlap is suspected or follow-up blood tests suggest autoimmune problems.

For Dr. Bergasa – My son has UC and PSC. He already has cirrhosis. He had an upper endoscopy and colonoscopy two weeks ago. After the procedure he developed persistent itching. He takes vancomycin and Urso. Did the procedure or sedation trigger the itching?
I don’t know exactly, but this is an excellent example of what manipulation of the bile ducts can do. This is one reason why we do more MRCPs and fewer ERCPs. It is possible, but not likely, that the sedation may have partly mediated the itch. The itch that has persisted may be due to the bile duct manipulation.
 

For Dr. Hughes – Your last slide was data from 2011. Do you have more recent data, and is the same differential in MELD scores present in 2013?
The data comes from the Scientific Registry of Transplant Recipients. The 2012 data is still being analyzed and confirmed.

For Dr. Chopra – How many people present with IBD after PSC diagnosis?
In my practice, about one third of new patients do not have IBD. I have one patient that developed IBD 10 years after PSC diagnosis. It is uncommon, but it does happen. I think we will see this more frequently, since liver function tests are often done routinely now.

For Dr. Bergasa – Can you explain why itching gets worse with sweating (as with exercise) or with certain clothing? Is there a basis for itching increasing at night?
Anything that increases stress may increase endogenous opioids, which may increase itching. Scratching behavior tends to peak from about noon to 6. At night, scratching behavior persists. The mind is occupied with many tasks during the day. At four in the afternoon, the sensation starts to peak, as preoccupation with daytime tasks decreases, so the dominant sensation becomes itching.
Dr. Slivka noted that some questions related to exciting research on replacement artificial organs and stem cell research were not directly applicable to this session.

For Dr. Hughes – What do you think about giving children priority? Does a pediatric patient get to transfer points when transitioning to an adult center? Do kids get any preferential treatment?
We didn’t discuss the PELD score, but there is priority given to children for younger donors. A donor under 35 is going to have priority going to a child. These changes have dramatically reduced the number of children waiting for transplant for any organ.

For Dr. Chopra – You say you prefer not to do MRIs on pregnant women, but there’s no radiation involved in an MRI. Is there a concern with the magnet and the fetus?
You can do second trimester MRIs.

For Dr. Chopra – Why is it that PSC overlap with autoimmune hepatitis is considered to do better?
Pediatric PSC may be a different variant. The studies are so small that it is possible that there is no difference. (Dr. Slivka noted that he has had patients that were diagnosed with autoimmune hepatitis as children, PSC-AIH overlap as teenagers and “regular” PSC as adults.)

For Dr. Bergasa – What is the link between allergies and PSC? How can allergies be explained, and is there any interplay with pruritus?
Anything that can trigger a response to an environmental factor can result in itching. The itch does not have to be related to the itch of cholestasis (bile flow impairment). Wool is a common complaint, especially if sweating. Fabrics such as cotton and silk are better.

For Dr. Hughes – How often are the appeals for MELD granted? Does it differ by region, and why? What percentage of requests for upgrades are for PSC patients?
The data is not available to us, so I don’t know the answer. Most of the appeals we submit are for cancer or PSC. From one region to another there is variability, and that depends on how competitive that region is for organs.

For Dr. Chopra – Is it important for an asymptomatic PSC patient to live close to a transplant center? PSC is a rare disease.
You need to have access to a liver doctor or GI doctor with a special interest in PSC.

For Dr. Chopra – Should children of parents with PSC be screened?
At this time we do not recommend routine screening for PSC.

For Dr. Bergasa – 28% of female patients note increased pruritus symptoms during the pre-menstrual timeframe. Have you noticed other fluctuations in symptoms during the rest of the menstrual cycle?
No, pruritis is seen mostly in the pre-menstrual state. The data came from a questionnaire.

For Dr. Hughes – Is there pressure to modify the MELD calculation to better reflect quality of life metric?
Not currently. Many transplant surgeons are not happy with the fact that the MELD score does not seem to be equitable. It doesn’t allow the physician any leeway to help the patient who has severe ascites or encephalopathy. Most of the patients listed today have a MELD score of around 15 – and that is where most of the deaths occur. There needs to be a method to allow judgment to play a role.

For Dr. Chopra – Do you routinely check antibodies such as pANCA, and how frequently?
The tests are done at diagnosis only. There is no indication that the numbers will change with time.

For Dr. Bergasa – Is escalation in itch an indication of deterioration of liver function?
No, just the opposite. The liver makes the pruritogen (the substance that causes itch), so as the liver function decreases, the production of the pruritogen decreases.