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Ricky Safer: Reaching a Milestone in Our International PSC Registry

PSC Partners Seeking a Cure

 

RESPONDING TO REQUESTS FOR HELP

TIPS FROM A PSCer and

HER CAREGIVER HUSBAND

“Just let me know what I can do to help.” – a phrase we’ve all heard or even said to someone going through a trying time. Oftentimes, that statement adds extra stress to the person who needs the help. They might not know how to respond or feel compelled to say anything to make the friend or family member feel helpful. The end goal isn’t to add an extra burden – but rather to provide meaningful help. There are a few different scenarios that you might encounter as a patient or caregiver, and we’ve provided tips on how to handle each situation below.

You could use some help. Don’t be afraid to take people up on their offers to help. They are asking because they really do want to do anything they can for you, and don’t feel like you owe them anything. A good start is to sit down and create a list of things that could be “outsourced”. There are many things that could be done by a family member or friend. Keep a running list of errands or tasks that could be done by someone else. It will make the friend/family member feel helpful, and checks things off your ever-growing to-do list! Examples of tasks that could be outsourced are things like:

  • Grocery shopping
  • Running to the hardware store to pick up something (lightbulbs, etc.)
  • Dropping off and picking up laundry
  • Meal preparation / meal train
  • Playdates for the kids
  • Checking on pets
  • Driving to and from appointments
  • Being an advocate for you in appointments or with paperwork (this requires a close relationship as personal information would need to be shared)

Every time someone asks what they can do to help, send them something from the list. It may take them 10 minutes to do, and they will feel helpful doing it. They know that you would willingly do the same for them if the roles were reversed. If the list becomes too much to manage on your own, ask your caregiver to manage the list for you and send out tasks as they feel appropriate.

You have enough help, but people keep asking. Some people are blessed to have many friends and family members close by to help with everything they need. Things may be under control, but people always want to help. Perhaps the neighbor next door might ask what they can do to help. A quick explanation of the family members/friends that are around to help you will diffuse the immediate ask. Saying something like, “Thanks so much for the offer, but I’ve been so lucky to have my parents/friends around to help with everything I need at the moment. I’ll be sure to reach out should anything come up.” Or, send them something from the task list.

You don’t actually need help. Although dealing with a chronic illness is draining, sometimes getting out and doing things for yourself keeps you going physically and mentally. If someone asks what they can do to help, but you don’t actually feel like you need help, you could say something like, “Thanks so much for the offer. I’ve had a hard time staying hopeful during the harder days, so I would love your prayers,” or ask for a donation to PSC Partners by saying something like, “Thanks so much for your offer, but I’m getting by just fine, currently. However, some others in the same situation as me aren’t doing as well. If you’d like to help us all, donating to PSC Partners would be very much appreciated.”

Blog written by Tyler and Nicola Tessier

Nicola and Tyler Tessier reside just outside of Boston with their two young (and active!) boys. Nicola received a life-saving living donor liver transplant from her brother nine years ago and is doing well. When not advocating for organ donation and a cure for PSC, you can find them both working in the marketing industry.

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!

 

Q & A with Winning Athlete & PSC Patient Yvette Matthews

For this month’s blog, we would like to introduce you to Yvette Matthews. Yvette was diagnosed with PSC in 2001, and received a liver transplant in 2013. Prior to her diagnosis, Yvette was a college athlete and a semi-pro beach volleyball player. So, understandably, her PSC diagnosis came as a complete shock, like it does for many in our community. Yvette’s health eventually began to decline, and, after many years of living with the complications of PSC, she received a transplant. Within a year after her surgery, Yvette was slowly able to regain the strength and spirit to train once more for competition. She successfully competed in her first Transplant Games in 2014. She has been to several Transplant Games since, and is now competing in the National Senior Games as well.  Yvette has been an active volunteer for PSC Partners and has attended several of our conferences over the years, always bringing her vivacious spirit and energy to the event. Yvette also has been a key advocate for organ donation.

We were excited to chat with her on the phone last week and talk to her about her PSC diagnosis, competition, liver transplants, and more.

Q:  Describe your life before your diagnosis with PSC?

A:  I was really healthy and working out. I played little league softball and enjoyed riding my bike. I was a college athlete at UMBC where I competed in basketball, hockey, and lacrosse. I was a semi-pro beach volleyball player.

Q: Tell us about your diagnosis and experience with PSC.

A: I was diagnosed in 2001 at a routine physical. My liver enzymes came back elevated. I felt fine, and I had no symptoms, so I literally ignored it. I was 100% sure the doctors were wrong. 

After several years, I started to feel like I was slowing down. The fatigue continued to increase, and I started to develop fluid in my abdomen, which needed to be drained every week. I was just super tired. I didn’t realize until after my transplant how tired I was.

Q:  After your transplant, did you ever think you would compete again?

A:  No. I thought I would get my transplant, be alive, and that would be about it. I assumed that was the way it was. A few months after my transplant, I attended my niece’s wedding in Baltimore. That’s when I started believing that I could do stuff. That event pulled me out of it, and I started working out, again. I don’t remember how I found out about the National Transplant Games, but I started going to meetings and to train for it. In the summer of 2014, I competed in my first National Transplant Games and won nine medals. I was so happy, excited, and motivated. Since then, I have competed in three more National Transplant Games and one World Transplant Games.

Q: What does competing again mean for you?

A: Personally, getting back into competition makes me feel alive. It is something that has always been a part of me, and it was missing for a while. Being at these games and getting to meet everyone, from the competitors to the family members of donors, is so uplifting. It’s almost like going to the (PSC Partners) conferences.

Q: How are you feeling today?

A: I feel pretty good. I get tired sometimes. I don’t have the stamina that I used to, and sometimes I have to force myself to lie down, but, overall, I’m really feeling good.

Q: What is ahead for you?

A: I have qualified for the long jump in the National Senior Games this June in New Mexico, and I am competing in the World Transplant Games in Newcastle, England this fall.

Thanks for sharing your remarkable journey with us, Yvette. We wish you the best of luck in your upcoming competitions!

Dear PSC Community,

We are excited to announce the launch of our Annual Giving Campaign! PSC Partners’ annual giving campaign is a vital part of keeping the important work we do moving forward. Your support is critical for continuing the momentum that has been building the last couple of years. 

As I look back on our first fourteen years as an organization, I am thrilled to see the huge increase of interest in solving the mystery of PSC and the number of valuable collaborations that are being created. We knew from the start that to make our voices heard in the medical world and among PSC patients and their families, we would need to connect with the major centers treating PSC patients. Our vision was to create both a strong medical network and an engaged patient community and to encourage collaboration between the two groups.

This year has been particularly exciting for our community.  It is wonderful to see researchers sharing data internationally, which will speed up the pace of research. One of the outcomes of the 2016 FDA/AASLD workshop on Trial Design and Endpoints for Clinical Trials, where I met Dr. Veronica Miller, Executive Director of the Forum for Collaborative Research, was the launch of the PSC Forum at this year’s EASL meeting in Amsterdam. Additionally, there are a number of ongoing early phase clinical trials and registries to help motivate research, and we are beginning to have a better understanding of the pathophysiology of PSC. 

There is no question that interest in solving the “black box” of PSC is gaining momentum across stakeholder lines. The creation of the PSC Forum and our integral involvement with this group is giving us much to be optimistic about. However, there is much work to be done to meet our community’s unmet needs. The good news is that we now have the attention of all the right stakeholders including: researchers,  clinicians,  pharmaceuticals and regulators.  Now, more than ever, we need your continued support to maintain our momentum and find a cure!

Over the next month, we will be sharing information and asking for your support to ensure we can continue to provide education and support for patients and caregivers, grow our patient registry and fund the research that is so critical to finding better treatments and a cure.  To kick off the season, we’ve created an annual giving page where you can learn more about our accomplishments this year, where we’re headed and how you can support our continued success.  

Our success lies in our community’s ability to not only donate to keep our momentum going, but also to spread the word. 

We are optimistic about a future with better treatments for primary sclerosing cholangitis and thank you in advance for your support!

Sincerely,

Ricky Safer

Founder and CEO

Primary sclerosing cholangitis (PSC) is a rare liver disease affecting the bile ducts inside and outside the liver. As with most rare diseases, it is difficult to conduct large clinical trials that support the development of effective treatments for PSC.

In 2014, PSC Partners Seeking a Cure collaborated with the NIH Office of Rare Disease Research to create the international PSC Partners Patient Registry. This registry brings information on PSC and those affected by the disease into a single place, and enables academic medical centers and pharmaceutical companies to easily locate patients who opt in to be part of the patient-driven registry. 

The PSC Partners Patient Registry collects data including information on diagnosis, family history, quality of life, medications, surgeries, overlapping diseases, and patient demographics. Our registry is designed to be a resource for researchers, assists in finding appropriate patients for clinical trials, facilitates drug safety monitoring for new drugs, and allows for patient participation at each level of research.

This spring, we hit an important milestone: the PSC Partners Patient Registry reached more than 1,000 patients. We hope that bringing together more PSC patients will speed up research by enabling easier recruitment of participants for clinical trials. This is critically important, as the disease currently has no effective treatments to slow down disease progression.

An unexpected result of enrolling more than 1,000 patients in the registry is that some of those who tried to enroll learned — after answering a series of questions about their diagnosis and symptoms — that they do not actually have PSC. Instead, they have primary biliary cholangitis (PBC), a different disease that also affects the bile ducts. This misdiagnosis is troubling, given that PBC patients could be benefitting from treatments that are effective in slowing the progression of PBC – and they may be missing out on these treatments due to misdiagnosis.

To help clear up any misconceptions, we’ve been working with PSC Support, a nonprofit patient organization in the UK, on an educational campaign to promote a better understanding and more accurate diagnosis of these two rare liver diseases. Given the similarity of their names, PSC and PBC have been confused in medical publications, among regulatory bodies, and in medical practice — as well as in individual patient diagnoses, as seen in our patient registry.

Without an accurate diagnosis, patients with PBC may miss out on important treatments, and PSC patients, who, unlike PBC patients, are at an increased risk for colon and bile duct cancers, may not get important cancer screening.

A clear understanding of the two diseases will likely decrease the chances for misdiagnosis and our well-populated registry of over 1,000 PSC patients can serve to bring us a step closer to finding much-needed therapies for PSC. 

To learn more about the PSC Partners Patient Registry, or to enroll, visit http://pscpartners.org/patient-registry/.