Psychological Toll, Poor Support Found for Primary Sclerosing Cholangitis Patients

The uncommon diagnosis of primary sclerosing cholangitis (PSC) often came as a shock to both those diagnosed and their family members, according to a British virtual focus group study. And unlike those with more prevalent chronic diseases such as cancer, PSC patients appeared to get short psychological shrift from the healthcare system.

Veronica Ranieri, PhD, MSc, of Tavistock & Portman NHS Foundation Trust, University College London, in the U.K., and colleagues reported that based on extensive patient and family discussions, the PSC Wellbeing Study found that diagnoses of this disease – which has an estimated prevalence in northern countries of 3.85 to 16.2 cases per 100,000 person-years – were often hastily delivered by healthcare professionals.

In addition, as shown in the study in PLoS One, caregivers rarely offered comprehensive explanations of the disease’s consequences and provided little opportunity for patients to ask questions or receive adequate referral for support. The results exposed a particular need for long-term psychological support and greater inter-connectivity in patient services as a means to improve wellbeing, especially since many PSC patients have comorbidities such as inflammatory bowel disease, the researchers said.

“Such cohesion should also extend to ensuring continuity of care, whereby participants could be seen by the same specialist who was familiar with their case at each appointment,” the team wrote. “Secondly, a need for signposting to reliable and accurate information and sources of support around the time of diagnosis, especially for such a rare and ‘invisible’ illness, would help families familiarize themselves with the illness and feel less isolated.”

The disease involves scarring of the hepatic bile ducts, has no definitive therapy as yet, and the prevalence is rising.

“This study took a phenomenological approach as it was interested in understanding the subjective experiences of individual participants and the meaning that participants attributed to these experiences,” the researchers wrote. Analysis revealed substantial psychological distress, including depression and anxiety, in patients and caregivers, including persistent fears about longevity and death, worsening PSC symptoms, or associated illnesses such as cancer.

“In light of these difficulties, we urge physicians to take into account the likely psychological impact the illness will have on the person diagnosed with PSC and their family and, where possible, be open to speaking about it,” Ranieri told MedPage Today. “We also ask that physicians have information ready for those affected to take home with them or have sources of information and support they can easily signpost them to.”

In the study, patients as well as family members described difficulties in adjusting to life post-diagnosis, with problems due not only to decreased levels of functioning but also to a sense of isolation.

On the positive front, some of those surveyed had turned to various coping mechanisms, such as volunteering and participating in distracting activities that provided intellectual stimulation. Other responses in answer to questions about coping included denial, acceptance, attempts to be positive, and slowing the pace of life were reported by individuals who, like many of those living with PSC, had multiple chronic conditions or were living with incurable cancer.

Study Details

The study, conducted during September to November 2018, recruited 30 geographically diverse U.K. residents age 18 or older who self-identified either as PSC patients or their partners, family members, or caregivers.

Participants were selected for different characteristics in terms of age, duration of diagnosis, and history of transplantation. Of the 30 participants, 22 were patients and eight were supporting family members. Participants were allocated to one of four focus groups, each containing six to nine participants.

Participation occurred via an “Asynchronous Virtual Focus Group” (AVFG) in which participants wrote answers to a different question each week for 4 weeks.

Three of the four AVFGs included only patients — 10 men and 12 women ranging in age from 28 to 74 and with disease durations of 6 months to 20 years. Nineteen had not had a transplant.

Family members supporting those living with PSC were all female: five mothers and three partners of patients, whose ages ranged from 31 to 60, and who supported patients with diagnoses varying from 6 months to 10 years.

After analyzing the narratives from the focus groups, the researchers had the following advice for healthcare providers:

• Show sensitivity towards patients and families at the time of diagnosis, many of whom will be in shock at the news. Some patients will be asymptomatic at the time of diagnosis and many will be distressed by the rarity and gravity of the illness

• Get informed if you aren’t familiar with PSC; many healthcare professionals will likely be unfamiliar with it, but patients indicated that informed professionals were a notable source of support

• Don’t assume that someone with high liver function test values is misusing alcohol

• Point patients and their families to trusted sources of information and support, including having a leaflet ready for them to take home with them; direct them to appropriate websites focusing on liver health

• Discuss the psychological impact of PSC; although symptoms of depression and anxiety were often noted by participants, such problems were rarely discussed or treated adequately by healthcare providers, the researchers found

Response to the study has been positive, Ranieri said. “We shared the findings with health professionals at the UK-PSC conference [held in Oct. 2019] and they found it helpful to incorporate speaking about psychological impact in their PSC consults. One thought so far is for liver teams to have at least one individual on the team that patients can turn to to speak about the psychological impact of the illness.”

Study limitations, the team said, included that since participants wrote and edited their own narratives, spontaneity of speech was reduced. Furthermore, participation in the study was literacy- and technology-dependent, which may have excluded some of the target population. In addition, since potential participants responded to advertisements, the sample was subject to self-selection bias. The authors also acknowledged that saturation with regard to caregivers’ accounts and narratives on transplantation was probably not achieved since participants were not specifically asked about transplantation, and fewer caregivers contacted the research team to participate.