Quality of life in children with chronic pancreatitis: An overlooked challenge

Abstract

Chronic pancreatitis (CP) is no longer considered a disease of adults alone; it is increasingly being recognized in children as well. It is a chronic inflammatory condition of the pancreas that can lead to exocrine and endocrine insufficiency over time. The clinical aspects and medical management of pediatric CP are often discussed; however, its impact on the quality of life (QOL) remains largely unaddressed in routine clinical care. The review focus on physical, emotional, educational, and social challenges experienced by children with CP and their families. Pain-related disability, frequent hospitalizations, and the risk of developing exocrine and endocrine insufficiency significantly affect daily functioning and mental well-being. Therefore, improving QOL in children with CP requires a multidisciplinary care strategy, including effective pain management, nutritional optimization, and psychological support.

Key Words: Chronic pancreatitis; Quality of life; Children; Pain; Challenges

Core Tip: With advancements in the field, our knowledge has expanded in understanding and managing pediatric chronic pancreatitis (CP). However, its impact on quality of life (QOL) is often overlooked. Our aim is to shift our attention from the clinical aspects to real-life experiences faced by children. We highlight how the CP affects different domains of QOL. By exploring the available evidence, the review calls for the integration of QOL assessment into routine clinical care and advocates for a multidisciplinary approach. Recognizing and addressing these hidden burdens is essential to truly improving care for pediatric CP beyond symptom control.

INTRODUCTION

Chronic pancreatitis (CP) is a progressive inflammatory disease that leads to irreversible structural and functional damage to the pancreas, resulting in chronic or recurrent pain, exocrine insufficiency, endocrine insufficiency, and a lifetime risk of pancreatic cancer (adenocarcinoma)[1,2]. Although uncommon, its incidence has been reported to increase over the past two decades[3]. Quality of life (QOL) is an important outcome measure in chronic diseases such as CP.

QOL has been well studied and documented in adults with CP[4-10]. However, the pediatric literature remains sparse. Age and development-specific challenges faced by children differ from those of adults. These not only include chronic abdominal pain, but also growth failure, school absenteeism, emotional disturbances, impaired social interactions, and poor academic performance.

This knowledge gap underscores the urgency of our work. Children with CP often experience chronic, debilitating pain requiring frequent hospitalization for medical and/or endoscopic management. This is important because chronic pain significantly impacts physical, emotional, social, and educational domains of life. Addressing QOL in this context is not only required for comprehensive clinical care, but also for integrating QOL assessment into routine care and development of psychosocial support systems.

This review aims to fill this gap by providing a comprehensive overview of the available literature on QOL in children with CP. We will discuss the domains of life most affected, review the currently available tools used for QOL assessment, summarize findings from existing studies, and outline directions for future research.

CHRONIC PANCREATITIS IN CHILDREN

Smoking and alcohol consumption are the most common etiologies of CP in adults; however, in children, the underlying etiologies differ significantly. An underlying genetic predisposition is identified in the vast majority of idiopathic cases in both Asian and Western cohorts. Mutations in the PRSS1 and CFTR genes are commonly reported in Europe and North America, whereas SPINK1 mutations are more frequently seen in Asia[1,11-14]. Abdominal pain is the most frequently reported symptom in children, with episodic pain being more common than the continuous pain[1,15]. The long-term consequences of CP in children include diabetes mellitus, steatorrhea, and malnutrition. The reported prevalence of endocrine and exocrine insufficiency in children ranges from 6%–9% and 10%–17%, respectively[1,15,16]. Malnutrition is usually secondary to factors such as dietary restrictions, exocrine insufficiency, endocrine insufficiency, and complications like pseudocyst formation.

Challenges faced by children in developing countries

In developing countries like India, children with CP, particularly those from rural areas, often face significant barriers to timely diagnosis and intervention. Agrawal et al[15] reported better QOL scores among urban children than those from rural areas, although the difference was not statistically significant. These disparities may result from limited disease awareness, inadequate family support, restricted access to specialized healthcare services, and financial constraints. Consequently, access to essential treatments such as endoscopic procedures, medical therapy, and nutritional support may be delayed or unavailable, directly impacting the QOL of affected children.

CONCEPT OF QOL

According to the World Health Organization (WHO), QOL refers to individuals’ perception of their position in life, as influenced by their cultural context, value systems, personal goals, expectations, and concerns[17]. QOL is a multidimensional concept. The concept of health-related QOL (HRQOL) has evolved to focus on those components of overall QOL that directly impact either physical or mental health[18]. Measuring HRQOL has gained importance in recent decades as the healthcare paradigm has shifted from a purely biomedical model to a more integrated biopsychosocial approach. The impact of chronic illness on overall QOL can be quantified using HRQOL instruments. HRQOL instruments available for children and adolescents are multidimensional and asses physical, emotional, school, psychosocial, and social health subdomains[19].

AVAILABLE QUESTIONNAIRES

Several validated tools available to measure HRQOL in children and adolescents[20]. Commonly used pediatric questionnaires include the Pediatric Quality of Life Inventory (PedsQL 4.0) core generic scale and the Child Health Questionnaire-Parent Form (CHQ-PF50)[21,22]. Other generic tools, more commonly used in older adolescents and adults, include the WHO QOL scale and the Short Form Questionnaire (SF-12/SF-36). However, these are less commonly used in younger children[23-25].

There are also disease-specific instruments, well-validated in adults, to assess the QOL in pancreatic disease. These include European organization for research and treatment of cancer QOL questionnaire-pancreatic modification, pancreatitis QOL instrument, and pancreatic exocrine insufficiency questionnaire[7,26]. However, their use in children remains limited due to a lack of validation in the pediatric population.

A detailed discussion of all available disease-specific questionnaires is beyond the scope of this review. We will briefly discuss the two commonly used pediatric generic tools below.

PedsQL 4.0

The PedsQL 4.0 is the most widely accepted and commonly used tool for children and adolescents. It includes a generic core scale and disease-specific modules for conditions such as asthma, rheumatological disorders, diabetes, cancer, and cardiac disease. It is a multidimensional scale encompassing four different domains consisting of 23 questions (physical-8, emotional-5, school-5, and social-5). The average time required to complete the questionnaire is approximately four minutes. It includes both child self- report and parent proxy-report across age groups ranging from 2 to 18 years. It is a validated tool available both in Hindi and English[27,28]. A five-point Likert response scale is used to record responses. Items are reverse-scored and linearly transformed to a 0-100 scale (0 = 100, 1 = 75, 2 = 50, 3 = 25, 4 = 0). Low HRQOL is defined as a score below 2 standard deviation from the mean of normative sample[21]. Scores are derived by summing item responses and dividing by the number of items answered. The physical health summary score (8 items) corresponds to the physical functioning subscale. The psychosocial health summary score (15 items) is the average of the emotional, social, and school functioning subscales. This scale has been used in two peer-reviewed studies assesing QOL in children with CP[15,29].

CHQ-PF50

The Child Health Questionnaire is another widely used generic core scale designed to assess QOL in children aged 5-18 years old. It encompasses 11 multidimensional scales along with 2 summary scores. A parent proxy version is available in two formats (50 or 28 items), while an adolescent self-report version consisting of 87 items is available for children aged 10-17 years old. Completing the questionnaire typically takes approximately 20 minutes. It assesses the QOL in the past 4 weeks. Scores on the items were calculated to provide physical health and psychosocial health scores. Each scale is scored from 0 to 100 with a mean ± SD (50 ± 10); higher scores signify better QOL[22].

How does chronic pancreatitis impact the QOL in children?

The impact of chronic pancreatitis is multidimensional, affecting all the four core domains of QOL-physical, emotional, school, and social (Figure 1).

Figure 1  Conceptual framework illustrating the impact of chronic pancreatitis on different domains of quality of life in children.

How is the QOL in children with CP across the regions/countries?

A review of the literature reveals that, to date, only four peer-reviewed pediatric studies have evaluated QOL in children with CP. The first study, conducted by Pohl et al[29] in 2012, assessed QOL in children (n = 38) with long standing pancreatitis (acute relapsing and CP) compared to healthy age-matched controls[29]. The study concluded that children with pancreatitis experienced poor QOL across all domains, with fatigue being a prominent concern. In a subsequent study, Bellin et al[30] evaluated 19 children with CP or acute relapsing pancreatitis who had failed medical and/or endoscopic management. These children were assessed before and after total pancreatectomy and islet cell auto transplant. The authors reported poor baseline QOL (physical component and mental component scores), with significant improvement one-year post-surgery[30]. Neither of these studies analyzed the factors affecting QOL. The third study, a large multicentre analysis from the INSPIRE consortium, found poor physical HRQOL but normal psychosocial QOL in 368 children with acute recurrent pancreatitis or CP. Emotional, behavioral problems, as well as abdominal pain were identified as factors associated with low physical QOL[31]. Most recently, a multicentre study from India by Agrawal et al[15] assessed QOL in 120 children with CP and reported poor physical and psychosocial QOL[15]. The study reported abdominal pain and poor socio-economic status as significant contributors to poor physical and psychosocial QOL. A detailed comparison of findings of these studies is presented in Table 1.

Table 1 Comparison of different studies on quality of life in children with chronic pancreatitis.

Ref.	Age group (years)	Number of patients	Questionnaire used	QOL findings	Anxiety and depression	Gender impact	Pain impact	Exocrine insufficiency	Endocrine insufficiency	Advanced disease	Calcific pancreatitis	Socio-economic status	Impact of treatment modality
Pohl et al[29], 2012, United States	2–18	38	PedsQL 4.0	Poor physical and psychosocial QOL	Not studied	Not studied	Not studied	Not studied	Not studied	Not studied	Not studied	Not studied	Not studied
Bellin et al[30], 2011, United States	5–18	19	SF-36	Poor physical and mental component score	Not studied	Not studied	Not studied	Not studied	Not studied	Not studied	Not studied	Not studied	Surgery: Improvement in QOL score 1-year post-surgery
Tham et al[31], 2022, Multicentre (United States, Canada, Israel, Australia)	6–18	368	CHQ-PF50	Physical QOL poor; psychosocial QOL normal	19.3%	No	Yes	No association	No association	Not studied	Not studied	Not studied	Not studied
Agrawal et al[15], 2024, India	8–18	120	PedsQL 4.0	Both physical and psychosocial QOL poor	1.6%	Female had poor psychosocial QOL	Yes	No association	No association	No association	No association	Poor socio-economic status associated with poor QOL	Not studied

QOL: Quality of life; PedsQL 4.0: Pediatric quality of life inventory core generic scale; SF: Short form questionnaire; CHQ-PF50: Child health questionnaire-parent form.

Regional variations in etiology impacting QOL: Among the published studies of QOL in children with CP, three were conducted in Western countries and one in India[15,29-31]. A genetic etiology was identified as a major contributor to pediatric CP in all the studies except the Indian study, where genetic testing of idiopathic cases was not discussed. However, a prior study from India including 146 children with CP have reported underlying genetic mutation in the majority of idiopathic cases[1]. The pattern of mutation varies by the region with PRSS1 and CFTR mutations are more common in Europe and North America, whereas SPINK1 and CTRC variants are more prevalent in Asia[14]. Although QOL is impaired across the regions, genetic factors may influence disease burden and thereby indirectly impact QOL.

Regional variations in QOL: Differences in health care accessibility, disease awareness, and psychosocial support system exist across countries. For example, a multicentre study by Tham et al[31] reported normal psychosocial QOL in contrast to Indian study, potentially reflecting a better psychosocial support systems in Western settings[31]. In addition, cultural differences, health-seeking behavior, social stigma, and gender bias may also influence how families perceive and report QOL. These observations underscore the importance of conducting regionally diverse and cross-cultural studies.

Factors affecting QOL in children with CP

Abdominal pain: It is the most frequently reported symptom in children with CP. Published pediatric studies consistently highlight its profound impact on physical and psychosocial QOL[15,29-31]. Therefore, pain management remains the cornerstone of treatment wheather medical, endoscopic, or surgery. Pain significantly impacts daily activities, school attendance, and emotional well-being. In children, improved QOL post- surgery has been clearly demonstrated by Bellin et al[30]. However, longitudinal data evaluating QOL outcomes following medical or endoscopic management in children remain limited. In contrast, adult studies have shown QOL benefits following endoscopic interventions, such as pancreatic duct stenting/duct clearance, and pharmacological therapy, such as use of antioxidant and pancreatic enzyme replacement therapy[8-10].

Fatigue: Fatigue is a frequently underreported yet clinically important symptom in children with CP. It may result from chronic abdominal pain and malnutrition secondary to exocrine insufficiency. In children, Pohl et al[29] reported higher fatigue levels in those with long-standing pancreatitis, which were linked to poor HRQOL. Similarly in adults, CP is associated with fatigue, sleep disturbances, and fear of future health complications[7]. However, the associations between fatigue and nutritional status has not yet been systematically studied in pediatric CP, and future research is needed to explore this interaction.

Weight loss: Weight loss in children with CP is multifactorial, resulting from exocrine insufficiency, endocrine dysfunction, and dietary restrictions. However, the primary contributor to malnutrition is pancreatic exocrine insufficiency. In an adult study, Olesen et al[32] reported a strong correlation between weight loss and impaired QOL[32]. It is therefore important to monitor for symptoms of exocrine insufficiency such as steatorrhea. Notably, adult studies have shown that pancreatic enzyme replacement therapy (PERT) supplementation significantly improves nutritional status and overall QOL, underscoring the importance of early diagnosis and treatment[8]. Currently, no pediatric longitudinal follow-up studies are available to assess the impact of weight loss and PERT on long-term QOL.

Impact of socio-economic status: Socio-economic status includes the factors such as family income, education status, and occupation of parent. In a recent study, Agrawal et al[15] reported that children from lower socioeconomic status had poor QOL. Families with financial constraints may face barriers to healthcare access, difficulty understanding disease complexity, and challenges in adapting to lifestyle modifications required for effective disease management.

Gender differences: Gender differences in QOL have been well documented in both pediatric and adult studies. A recent pediatric study from India reported that females had poorer psychosocial QOL compared to males[15]. Similarly, adult studies from the United States and Italy have documented poorer QOL among female patients[6,33]. This gender disparity has been attributed to differences in disease perception and pain coping mechanisms[34]. The Indian study also raised concerns about gender inequality, suggesting that families may prioritize male health over female, a pattern supported by Saikia et al[35], who found that Indians families spend less on healthcare of females[35]. This gender difference is an important finding in Indian study; however, it may not be generalizable to other settings without further cross-cultural validation. Further studies are warranted to explore gender disparities across the different geographical and cultural contexts.

Psychiatric co-morbidities: CP in children associated with an increased risk of psychiatric co-morbidities such as anxiety and depression. In adults with CP, the prevalence of depression and anxiety has been reported as high as 47% and 37%, respectively[36]. However two pediatric studies have reported depression and anxiety together in 19.3% and 1.6% of cases, respectively[15,31]. These findings highlight the need for routine psychological screening and integrated pain and mental health management to improve overall QOL in children with CP.

STRATEGIES TO IMPROVE THE QOL

Multidisciplinary approach

A multidisciplinary model is now considered the standard of care in pediatric pain management[37]. The literature supports its effectiveness in improving health-related outcomes[38]. In children with CP, multidisciplinary, coordinated care involving physicians, nurses, psychologists, physical therapists, social workers, and school personnel is essential to address all dimensions of the children life. Multimodal pain management includes pharmacological management, endoscopic interventions, psychological support, and physical rehabilitation can improve the QOL, even when complete pain resolution is not achievable[39]. In addition, family education, realistic goal-setting, and anticipatory guidance play a crucial role in empowering families and improving treatment adherence and satisfaction.

Psychological support and counselling

Pain-focussed psychological therapies are a newer approach that helps children cope with chronic pain. Cognitive behavioral therapy (CBT) is among the most established methods[40]. To date, no pediatric study has evaluated CBT in context of CP. However, several studies in children with function abdominal pain have demonstrated its efficacy in both short term as well as long term[41-44]. Although these conditions differ in etiopathogenesis, they share common challenges such as chronic pain, school absenteeism, and psychological distress. In adults with CP, only one randomized controlled trial has demonstrated its efficacy for reducing pain and disability[45]. Given this, CBT may be a promising tool for pediatric CP as well, but its role needs validation through dedicated pediatric trials. Therefore, psychological assessment and counselling should be incorporated into routine clinical care.

Pain management strategies

Pain management in CP should follow a stepwise analgesic ladder approach, starting with non-opioids (paracetamol, non-steroidal anti-inflammatory drugs) and escalating to opioids under supervision. In patients with persistent pain despite medical therapy and imaging evidence of structural abnormalities (ductal stones or stricture), endoscopic interventions such as dilatation of stricture, clearance of ductal stones with or without extracorporeal shock wave lithotripsy, and pancreatic duct stenting may be warranted. When both medical and endoscopic therapy fail and patient continued to have persistent pain, surgical options such as lateral pancreatico jejunostomy may be required[46-49]. A detailed discussion of pain management strategies is beyond the scope of this review.

Nutritional interventions

Children with CP are at risk of developing malnutrition, primarily due to progressive exocrine insufficiency and endocrine insufficiency, which evolve over time. In adults, exocrine insufficiency typically develops 5-10 years from the time of diagnosis[50]. Pediatric data show that 18% of children with a prior acute pancreatitis episode develop exocrine insufficiency within six years[51]. Exocrine insufficiency, wheather clinical or subclinical, can lead to fat malabsorption, poor growth, and fat-soluble vitamin deficiencies, all of which further contributes to poor QOL. These parameters have been shown to improve following PERT supplementation, as reported in an adult study by Czakó et al[52]. Therefore, nutritional rehabilitation is of paramount importance in the management of pediatric CP and should include PERT when indicated, dietary counselling, regular growth monitoring, pubertal development, and surveillance for fat-soluble vitamin deficiency. Such measures are essential not only for maintaining nutritional status but also to optimize QOL in children with CP.

Role of social support system: In addition to medical and psychological interventions, developing an effective social support system requires a coordinated efforts from health care provider, school, family and community organizations. The key component include educating school staff about the disease, creating flexible academic plans, facilitating peer group support, and actively involving social workers to support the child’s overall well-being.

Mechanistic link between influencing factors and interventions: Chronic pain abdomen in children with CP act as a central driver of QOL impairment, contributes to fatigue, frequent hospital visit, school absence, academic decline, and emotional disturbances. Although none of the published pediatric studies have directly assessed social dynamics such as peer relationship, isolation, school absence and academic decline, these remain clinically relevant concerns arising from chronic pain and frequent medical appointments. Therefore, interventions such as effective pain control (medical, endoscopic, or surgical), PERT, and psychological support (e.g., CBT) are cornerstone of management to interrupt these causal pathways and improve overall QOL.

Future direction and research gaps

There is a pressing need to develop disease specific, age appropriate, and culturally adaptable QOL assessment tools using mixed method research to better evaluate and address the unique challenges faced by children with CP. Additionally, cultural factors, such disease perception, acceptance of disease, health care accessibility, and family support, vary across regions and must be considered when designing specific QOL tools. Such tools should undergo validation in diverse setting to ensure that they reflect the experiences of children across different socioeconomic and healthcare environments. Futhermore, multicentre prospective cohort are required to evaluate the impact of various intervention (medical, endoscopic, or surgical interventions) during follow-up. There is also a need for randomized controlled trials to evaluate the effectiveness of cognitive behavioural therapy in children with CP. Finally, region-specific studies are needed to explore the role of sociocultural and health system factors in shaping QOL outcomes, especially in low- and middle-income countries.

CONCLUSION

CP is a life-long disease that affects more than just the pancreas. It impacts the physical, emotional, social, and school QOL. QOL is an important outcome measure in chronic disease but remains underrecognized in clinical practice despite significant advances in diagnosis and management. Factors such as chronic or recurrent pain, fatigue, poor socio-economic status, and female gender have been reported to adversely affect QOL in children with CP. As QOL is closely linked to both disease burden and treatment response, its routine assessment is imperative. Routine assessment using validated tools like the PedsQL 4.0 core generic scale and the CHQ-PF50 can help clinicians to identify daily challenges experienced by children and their families. However, there is a need for more tailored instruments that reflect the unique experiences of this patient population. In addition to multidisciplinary care, the integration of QOL assessment into pediatric care program should be supported by institutional and national health policies. This includes the routine use of validated QOL tools during outpatient visits, health care provider training in QOL assessment, and integration into electronic health record, and advocacy by healthcare insurance systems to recognize QOL assessment as a reimbursable service. These steps are essential to translate recommendations from theory into practice.