From the Guidelines
To help a young adult cope with a diagnosis of Primary Sclerosing Cholangitis (PSC), it is crucial to prioritize their emotional well-being and quality of life by providing access to a multidisciplinary team of healthcare professionals, as recommended by the most recent guidelines 1. When dealing with PSC, a rare and chronic liver disease, it's essential to focus on the patient's overall well-being, including their physical and mental health.
- Connecting them with a hepatologist or gastroenterologist who specializes in PSC is vital, as this disease requires specialized care 1.
- Encouraging them to learn about their condition through reputable resources, such as patient support groups, can help them better understand their disease and feel more in control.
- Providing information on existing patient support groups, as suggested by the guidelines 1, can offer valuable emotional support and practical advice. Some key aspects to consider when managing PSC include:
- Medication management, which may involve ursodeoxycholic acid (UDCA) to help manage symptoms, although its effect on disease progression remains debated.
- Symptom management, such as treating itching and fatigue, which can significantly impact the patient's quality of life.
- Regular monitoring with blood tests and periodic imaging to track disease progression and catch complications early.
- Encouraging healthy lifestyle choices, including avoiding alcohol, maintaining a balanced diet, and staying physically active as tolerated. Clinicians should explore and assess quality of life issues in people with PSC as part of routine standard of care, as emphasized by the guidelines 1. By prioritizing the patient's emotional well-being, providing access to specialized care, and encouraging healthy lifestyle choices, healthcare professionals can help young adults with PSC cope with their diagnosis and improve their overall quality of life.
From the Research
Coping with Primary Sclerosing Cholangitis (PSC) Diagnosis
To help a young adult cope with the diagnosis of Primary Sclerosing Cholangitis (PSC), consider the following:
- Management of symptoms and addressing complications is the main focus of PSC treatment, as no effective medical therapy for halting disease progression has been identified 2
- Ursodeoxycholic acid (UDCA) may be prescribed to improve biochemical parameters of cholestasis, although its effectiveness in improving clinically relevant endpoints is still unclear 3, 4, 5
- Liver transplantation is currently the only life-extending therapeutic approach for eligible patients with end-stage PSC, with an excellent outcome compared to other liver transplantation indications 2, 5
Treatment Options
Some treatment options for PSC include:
- Ursodeoxycholic acid (UDCA) at doses of 17-23 mg/kg/day for up to a year to determine if a reduction in serum alkaline phosphatase is observed 5
- Endoscopic interventions of the bile ducts, limited to clinically relevant strictures for balloon dilatation, biopsy, and brush cytology 4
- Novel therapeutic strategies, including apical sodium-dependent bile acid transporter and ileal bile acid transporter inhibitors, integrin inhibitors, peroxisome proliferator-activated receptor agonists, and others, which are in various stages of development 4
Quality of Life
To improve quality of life for young adults with PSC:
- Monitor and manage symptoms, such as abdominal pain, pruritus, jaundice, and fatigue 2
- Address complications, such as bacterial cholangitis, dominant strictures, gallbladder polyps, and cholangiocarcinoma 2
- Consider the potential impact of UDCA withdrawal on liver biochemistry and quality of life, as some patients may experience deterioration in pruritus and other symptoms 6