Pediatric cholestatic liver disease: Successful transition of care

Cleveland Clinic Journal of Medicine. 2019 July;86(7):454-464 | 10.3949/ccjm.86a.18140
July 1, 2019|Cleveland Clinic Journal of Medicine
Praveen Kumar Conjeevaram Selvakumar, MD;Vera Hupertz, MD;Naveen Mittal, MD;Kris V. Kowdley, MD;Naim Alkhouri, MD
Author and Disclosure Information

Praveen Kumar Conjeevaram Selvakumar, MD
Department of Pediatric Gastroenterology and Hepatology, Cleveland Clinic

Vera Hupertz, MD
Department of Pediatric Gastroenterology and Hepatology, Cleveland Clinic; Clinical Assistant Professor, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, OH

Naveen Mittal, MD
Texas Liver Institute, University of Texas Health, San Antonio, TX

Kris V. Kowdley, MD
Liver Care Network and Organ Care Research, Swedish Medical Center, Seattle, WA

Naim Alkhouri, MD
Director of the Metabolic Center, Texas Liver Institute, University of Texas Health, San Antonio, TX

Address: Naim Alkhouri, MD, Metabolic Center, Texas Liver Institute, University of Texas Health, 607 Camden Street, San Antonio, TX 78215; alkhouri@txliver.com

Dr. Kowdley has disclosed financial relationships (consulting, independent contracting, serving on advisory committee or review panels, teaching and speaking, or holding intellectual property rights) with Enanta Pharmaceuticals, Gilead Sciences, GlaxoSmithKline, High Tide Health, Intercept Pharmaceuticals, and UpToDate.

Release date: July 1, 2019
Expiration date: June 30, 2020
Estimated time of completion: 1 hour

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ABSTRACT

With recent medical advances, more patients with childhood-onset liver disease and more pediatric liver transplant recipients are surviving into adulthood, generating distinctive challenges to adult primary care providers. Young adults with pediatric liver disease are a unique cohort of patients with different evaluation and monitoring strategies, treatment, complications, and comorbidities. This creates a critical need for successful transition of these patients into adult care, with incorporation of a formal transitional model and multidisciplinary team.

KEY POINTS

  • The causes of cholestasis in children are different from those in adults, with genetic inherited causes more common in childhood.
  • Cholestasis in children can be caused by biliary tract obstruction such as in biliary atresia or defects in forming and excreting bile acids and other components of bile.
  • With the growing number of people with childhood-onset liver disease surviving into adulthood, it is important for internists to be aware of unique problems and challenges in continuing management of this population.
  • In addition to medical comorbidities, these patients may also have impaired psychosocial functioning and quality of life.

TRANSITION TO ADULT CARE

While the number of patients with childhood-onset liver disease and pediatric liver transplant recipients who survive into adulthood is increasing, there are no established guidelines or formal models for transitioning these patients into adult care. Consequently, studies on transitional process have examined various issues such as patient and parent frustration, poor medical knowledge among patients during transition, lack of parental facilitation, and inadequate knowledge on disease process among adult-care hepatologists.52–54

Figure 2. Transitional model in childhood-onset cholestatic diseases.
A position paper on transitional care from Europe has put forth important strategies.55 A multidisciplinary team including a pediatric provider, nurse practitioner, transplant coordinator, psychologist, social worker, and adult provider is vital for a successful transition. Key requirements for an adolescent patient include knowledge about the disease, demonstration of responsibility toward disease management, and self-management of healthcare needs such as scheduling appointments or insurance needs. This demanding task could be streamlined if pediatric liver transplant programs could adopt a formal transitional model (Figure 2). The transition could start as early as age 10 to 12, with gradual transfer of health management responsibilities from parents to adolescents from age 13 to 17.56

A prolonged period of transition up to age 25 is preferred in complicated cases. Distinctive consideration for transition should include those with neurocognitive developmental delay from underlying disease or hepatic encephalopathy before transplant. These patients need additional support and time to achieve independence in health management before transition.57 Validated questionnaires are available to assess readiness to transition into adult care,58 implying that the decision to transition should not be based solely on age. 

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