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2017 Research Grant Award Spotlight: Lazaridis and Hemming Karlsen, “Dissecting the Genomic Variants of PSC Outcomes”

 

PSC Partners Seeking a Cure is pleased to present Living With PSC, a podcast moderated by Niall McKay. Each month, this podcast explores the latest research and knowledge about primary sclerosing cholangitis (PSC), a rare liver disease. From patient stories, to the latest research updates from PSC experts, to collaborations that are necessary to find better treatments and a cure, this podcast has it all!

In the 18th episode, Host Niall McKay talks with Dr. Nathan Baggett, medical resident and PSCer who has undergone multiple liver transplants.
“I never wanted PSC to put limits on how I live my life,” says Dr. Baggett.

Check out this episode!


 

PSC Partners Seeking a Cure is pleased to present Living With PSC, a podcast moderated by Niall McKay. Each month, this podcast explores the latest research and knowledge about primary sclerosing cholangitis (PSC), a rare liver disease. From patient stories, to the latest research updates from PSC experts, to collaborations that are necessary to find better treatments and a cure, this podcast has it all!

In the 17th episode, Host Niall McKay talks with Dr. John Eaton, MD, transplant hepatologist and gastroenterologist at Mayo Clinic, about the future with regards to discovering new treatments and, eventually, a cure for PSC.
“I am more optimistic than I have ever been about the future prospects of caring for people with PSC,” states Dr. Eaton.

Check out this episode!



PSC Partners Seeking a Cure is pleased to present Living With PSC, a podcast moderated by Niall McKay. Each month, this podcast will explore the latest research and knowledge about PSC. From patient stories, to the latest research updates from PSC experts, to collaborations that are necessary to find better treatments and a cure, this podcast has it all!

In the 16th episode of Living with PSC, Niall McKay talks with Dr. Jerome Schofferman, MD, about his dual role as PSC patient and physician and what it has taught him about living with a chronic illness, treating patients, and including caregivers in the process.

Dr. Schofferman, MD., is a retired Internist and Pain Medicine Specialist from Sausalito, CA.

Check out this episode!

PSC Partners Seeking a Cure is pleased to present Living With PSC, a podcast moderated by Niall McKay. Each month, this podcast will explore the latest research and knowledge about PSC. From patient stories, to the latest research updates from PSC experts, to collaborations that are necessary to find better treatments and a cure, this podcast has it all!

In the 15th episode of Living with PSC, Media Producer Niall McKay talks with Co-Chair of the PSC Partners Medical Advisory Committee Dr. Christopher Bowlus, Division Chief of Gastroenterology & Hepatology and Professor at UC Davis School of Medicine, about drug and clinical development in the search for treatments and a cure for primary sclerosing cholangitis (PSC).


Check out this episode!


PSC Partners Seeking a Cure is pleased to present Living With PSC, a podcast moderated by Niall McKay. Each month, this podcast will explore the latest research and knowledge about PSC. From patient stories, to the latest research updates from PSC experts, to collaborations that are necessary to find better treatments and a cure, this podcast has it all!

In the 14th episode of Living with PSC, Media Producer Niall McKay has a conversation with Dr. James Tabibian, a gastroenterologist and director of endoscopy and resident research director at Olive View-UCLA Medical Center, about the possible use of Vancomycin as a treatment for primary sclerosing cholangitis (PSC).

Check out this episode!

PRURITUS

Advice From Mom/Caregiver/Pharmacist

Pauline Tostenrud

PSCers Often Have An Itch to Scratch

As many PSCers and their caregivers know, itching, known as pruritus, is a big issue for those affected by this disease. The bile acid buildup from PSC is believed to cause this itching, and suggestions about ways to deal with this problem are sometimes confusing. As a pharmacist and the mother of a double-transplanted PSCer, I am well-aware of the issue. In this blog, I hope to share ideas PSCers and their caregivers can discuss with their doctors to help manage the intensity of itching and minimize skin damage from scratching.

Pruritus

The treatment of itching related to PSC is not the same as itching from mosquito bites or allergic responses and should be managed by hepatologists and dermatologists, especially when it becomes so severe that scratching causes skin damage. The damage can lead to infection, which requires antibiotics and can affect transplant status. Keeping skin moisturized and healthy with vigilant self-care may help patients cope with itching. Doctors should be involved in development of treatment plans to help optimize the strategy and minimize discomfort.

Self-Care

My daughter’s journey with itching is a balancing act of moisturizing and massaging guided by consultation with her doctors. We have found that the best treatment for her is twice a day lengthy massaging with ceramide-containing cream and once daily massaging of an ointment, preferable after a short, cool shower. She takes an antihistamine at bedtime to both minimize daytime episodes of encephalopathy and encourage the nighttime sleep cycle.

Which Lotions are Best?

Ointments are occlusive, meaning they hold moisture in the skin, but they often feel greasy. Creams absorb into the skin and usually do not leave the greasy feel. Antihistamine-containing creams and ointments, such as diphenhydramine or hydrocortisone, can be purchased over the counter and might provide limited relief. However, they do not alter the source of itching, the bile acids. Antihistamines also may cause drowsiness, and, if the PSCer is experiencing any hepatic encephalopathy, they might contribute to this problem. The least sedating antihistamines are cetirizine and loratadine.

There are some prescription-only steroid products for the skin, but, keep in mind, long-term use of topical steroids on the skin can have harmful effects. Therefore, depending on skin condition, doctors need to help determine the risks and benefits of topical steroids. Scratching, which can even occur during sleep, might lead to inflammation and skin breakdown and make some topical steroid use suitable to treat the inflammation. Topical steroid use over long term can be harmful, so, again, these should be used only under a doctor’s care.

Topics for Discussion with Physicians

Medications, including cholestyramine, are bile acid sequestrants that bind bile acids in the gastrointestinal tract to help eliminate them. While they often help, these drugs can also bind and prevent absorption of some medications. So, it is imperative that PSCers seek medical advice about timing and dosage amount relative to other medications.

Some alternate medications that may also help include the antibiotic rifampin, as well as the medicines naltrexone and naloxone. Additionally, there are prescription-only drugs from different classes with side effects beneficial for the discomforts of PSC-related itching, such as doxepin, sertraline, and paroxetine, but these may cause dryness and/or drowsiness. These medications have other limitations, as well. For example, if patients take pain management opioids, the naltrexone and naloxone would be counter-productive and not recommended. The PSCer’s liver disease status, such as bilirubin level or clotting factors, might make rifampin an inappropriate choice. Any intolerance to the side effects of sertraline or paroxetine might make these options contraindicated, as well.

Since PSCers often take many medications, drug interactions may complicate the addition of anything new. Therefore, PSCers and their caregivers must work closely with medical providers and pharmacists to find the best options.

Light Therapy

Dermatologists may recommend a specialized light therapy, which is only available through the physician. This is different than tanning, and involves very short treatments with a filtered light therapy (UVA and UVB) at the doctor’s office. Some medications cause light sensitivity, so be sure to bring a complete list of medications to the dermatology appointment. My daughter felt that, over the months before transplant, the light therapy with dermatology provided some relief.

Every case of PSC-induced pruritis is different. Please be sure to check with your doctor before adding or removing anything from your skin-care or medication routine. If you have other suggestions for managing pruritis, comment below.

PSC Partners Seeking a Cure is pleased to present Living With PSC, a podcast moderated by Niall McKay. Each month, this podcast will explore the latest research and knowledge about PSC. From patient stories, to the latest research updates from PSC experts, to collaborations that are necessary to find better treatments and a cure, this podcast has it all!

In the 12th episode of Living with PSC, Niall McKay discusses living donor liver transplants and PSC with John Crowley, whose brother was his living donor.


 

Check out this episode!

By Stacey Eno

I look at my daughter’s picture, a radiant face, a smile so cheerful that it holds no clue of the horrific battle she is still undergoing with PSC. On her pink cheeks and twinkling eyes there is no sign of the pain, fear, and fight that are gnawing her every day. This is my beautiful hero, in her moment of respite, elevating herself beyond her pain for the cause she believes in.

Our story with PSC is no ordinary story, though no PSC story is ordinary. During her freshman year in high school, my sweet and always positive daughter, Hayley Rose Eno, was diagnosed with PSC and ulcerative colitis. She started her PSC journey with the intense symptoms of final stage PSC. She suffered extreme back pain and vomited blood. Doctors discovered that she had portal hypertension which meant the large veins in her esophagus were under pressure (varices) and were causing the bleeding. She already had cirrhosis! She went on to have numerous “bandings” of the varices over the following eight months, about ten procedures in all, to try to stop the bleeding. Her health continued to deteriorate until she needed to have a full liver transplant in December 2016, a week before Christmas.

Her recovery was dotted with a few episodes of mild rejection. After some time, Hayley finally began to feel better. Unfortunately, this period of wellness did not last long. The intense back pain re-emerged in April and she couldn’t eat without extreme abdominal pain. After a few trips back to the doctor who explained it away as “constipation,” we found ourselves back in the emergency room in the beginning of May. We soon found out she had post-transplant lymphoproliferative disorder, or PTLD, a type of lymphoma, and cancer. She had cancerous lesions on her liver, in her stomach and intestines. She had to undergo six rounds of chemotherapy that began at the end of May and ended in August. Doctors say her scans are now “clean.” They are still closely monitoring her and working to find the right levels of immunosuppression for her.

Hayley dreamed of holding a fundraiser to help in part with our expenses and to help others affected with PSC. I had discovered PSC Partners Seeking a Cure through Facebook shortly after her PSC diagnosis. I had found everyone in this community extremely helpful and understanding of the huge problems we faced with PSC. That is what motivated and inspired Hayley to take action. Over this past summer, the hair salon Hayley went to since she was a little girl, Barbara Ross Hairstylists, offered to hold a fundraiser for her on September 24th. This was precisely the positive outlet she needed. She designed shirts, hats and rubber bracelets with the logo, “Fight Like Hayley.” Barbara, the owner of the salon, donated the day’s proceeds for haircuts, blowouts and hair extensions to Hayley. There were also many baskets that the staff and some friends had put together to raffle off. A make-up artist was waiting for her when she walked into the hair salon that morning. The results are clear in her pictures. She looked so beautiful and happy! At the end of the day, she and her friends at the hair salon had raised $1,390 for PSC Partners Seeking a Cure’s research program! That’s my Hayley: She rose above her pain to help all PSCers.

If you have any thoughts to share with Stacey and Hayley, please feel free to do so in the comment section below.

Each year, PSC Partners asks research grant awardees to share a layman’s summary as part of the process for accepting the grant.  We are excited to share the 2017 PSC-related research grant summaries with you! In order to make this information more digestible and shine a light on each individual study, we will share one summary per week until we’ve shared the details for all of them.  Please feel free to add your comments or thoughts at the bottom of each of these summaries for the researchers!

Title: Exploring the PSC Matrisome: Discovery of Serum Biomarkers for the Stratification and Classification of Patients with Primary Sclerosing Cholangitis

Principal Investigator: Massimo Pinzani, MD, PhD, FRCP, Professor of Medicine and Director of the University College London (UCL), Institute of Liver and Digestive Health (ILDH), Sheila Sherlock Chair of Hepatology, London, UK

Lay Summary

Primary sclerosing cholangitis (PSC) is a disease affecting biliary ducts through which the bile flows from the liver into the intestine to ensure key digestive tasks. PSC is characterized by progressive scarring around the bile duct leading to obstruction and accumulation of bile in the liver. With time, scarring and fibrosis involve more and more portions of the liver causing cirrhosis and liver function insufficiency. Importantly, PSC is a silent disease and it is often discovered when the disease is already advanced. Also, when the disease is diagnosed in an early phase it is extremely difficult to establish the stage of disease evolution without performing a liver biopsy. This causes many difficulties in the management of patients with PSC and greatly hampers the possibility of efficient clinical trials with drugs specifically designed for this liver disease. Our laboratory has proposed a new technology based on the analysis of the scarring tissue obtained from the liver of patients with PSC. We have discovered that some proteins are specific from the scarring tissue of PSC and have an extremely good correlation with the disease stage. The presence and the amount of these proteins could be tested with a simple blood test and therefore become a diagnostic biomarker. The funding provided by PSC Partners will be used to support our efforts to further characterize these biomarkers and perform a clinical validation in our cohort of patients of PSC.

 

Each year, PSC Partners asks research grant awardees to share a lay summary as part of our grant process.  In order to make this information more digestible and shine a light on each individual study, we will share a summary each week (in alphabetical order of the Principal Investigator’s last names).  We welcome your comments or thoughts for the researchers at the bottom of each of these blog posts, and are excited to share this new PSC-related research with you!

Title: Dissecting the Genomic Variants of PSC Outcomes

Principal Investigators:

Konstantinos Lazaridis, MD, Consultant Gastoenterology and Hepatology, Professor of Medicine, Mayo Clinic College of Medicine

Professor Tom Hemming Karlsen, MD, Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital, Rikshospitalet

Brief Lay Summary of Study:

Primary sclerosing cholangitis (PSC) is a heterogeneous disease with variable outcomes. At the present time, we are unable to distinguish which patients will rapidly progress to a poor outcome (i.e., need for liver transplant, development of cholangiocarcinoma etc) from those likely to experience a relatively benign disease course. Our research into PSC over the last 5 years has significantly advanced the field and has begun to characterize the genetic predisposing elements underlying the disease development. However, we lack studies about the importance of genetic factors in predicting long-term clinical outcomes of PSC such as disease progression and its complications. We hypothesize that genetic variation plays a key role in the clinical expression, and outcomes of PSC. The objective of this application is to discover the genomic determinants of PSC outcomes (i.e., orthotopic liver transplantation, cholangiocarcinoma, and colon cancer development). Successful outcome of this study will lead to better prediction of PSC progression and introduction of individualized approaches for risk stratification of disease outcomes and ultimately benefit the patients that suffer from PSC.