Pain Management in a Patient with Intestinal Failure in the Palliative Care Setting: Case Report

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Case Presentation: This case of gastrointestinal hypoganglionosis demonstrates the necessity of alternative analgesic routes—such as subcutaneous ketamine—when conventional opioids fail due to intestinal malabsorption and dysmotility. Effective care required interdisciplinary collaboration, yet persistent discordance between the patient’s curative expectations and her life-limiting prognosis hindered timely advance care planning and community support transitions. Conclusions: Prognostic uncertainty, compounded by the condition’s rarity, highlights the need for early palliative care integration in non-malignant conditions, to ensure that care goals align with patient and family priorities. The case advocates for adaptable care models that bridge inpatient and community services, even amid prognostic ambiguity, to prevent fragmented care during critical transitions. gastrointestinal hypoganglionosis intestinal failure pain palliative care Figures Figure 1 Background Palliative care for patients with non-malignant, chronic conditions presents unique challenges that differ from traditional cancer-focused palliative care (1,2). In particular, the management of pain in patients with intestinal failure, encompasses multifaceted clinical, psychosocial, and systemic considerations that test the boundaries of conventional palliative approaches (3). Adult hypoganglionosis is an acquired, rare and complex condition that affects the enteric nervous system, resulting in gastrointestinal dysfunction and associated pain (4). The disease is characterised by a reduction in the number of ganglion cells in the myenteric and submucosal plexuses of the intestinal wall, leading to impaired intestinal motility and function (4-6). It is considered a subset of intestinal innervation disorders, accounting for approximately 3% to 5% of all such classified conditions (4-6). Clinical features include chronic constipation, recurrent abdominal pain, distension, nausea, vomiting and intestinal obstruction (5,7). Diagnoses itself can be challenging due to the rarity and non-specific nature of its symptoms, which include endoscopic evaluation and full-thickness intestinal biopsies for histological examination (4,7). The pain associated with hypoganglionosis results from several factors: a) chronic intestinal distension due to impaired motility, b) intermittent intestinal obstruction, c) intestinal wall inflammation, and d) visceral hypersensitivity (4,6,7). This case illustrates the interplay between complex pain management and prognostic dilemmas in a patient with acquired gastrointestinal hypoganglionosis and intestinal failure, followed by a narrative review of the current evidence on this topic. Case Presentation Key Aspects of History A patient in their late 20s, who since 2020, had multiple hospital admissions for recurrent small bowel obstructions [SBO] presenting as undifferentiated abdominal pain. She had no significant past medical or surgical history. In June 2022, after her eight hospital admission in under two years, she underwent a right hemicolectomy due to a caecal volvulus. Subsequent histopathology led to the diagnosis of gastrointestinal hypoganglionosis in the terminal ileum and caecum. During this admission, given she was unable to tolerate enteral feeding post-operatively, she was commenced on total parenteral nutrition [TPN] of three litres daily via a tunnelled small bore central venous catheter [CVC]. She was subsequently discharged home, with close outpatient follow up by her gastroenterologist and her multidisciplinary Intestinal Failure [IF] team. In January 2023, she was diagnosed with stage four endometriosis. During her admission between March 2023 till September 2023, she underwent multiple peripherally inserted central catheter [PICC] insertions in her upper and lower limbs for prolonged courses of intravenous hydration, antibiotics, parenteral analgesia and anti-emetics. Her analgesia was managed by the Acute Pain Service [APS] team during this and subsequent admissions. She was trialled on the following medications: sublingual buprenorphine 200 to 400 micrograms pro re nata [PRN] four-hourly, oral tramadol 50mg to 100mg PRN four-hourly, baseline analgesia of topical weekly buprenorphine patches 5 microgram/hour and oral tapentadol sustained release 50mg twice a day. The maximum doses of these analgesic regimens were not reached due to inadequate abdominal pain relief, prompting frequent changes to her analgesia plan. Additionally, she did not perceive any benefit from topical or oral analgesia formulations, which often led to medication noncompliance. Eventually, short courses of intravenous ketamine infusions were identified as the only regimen effective in alleviating her abdominal pain. She was frequently discharged home after infusion without any additional background or PRN analgesia. The pain team scheduled outpatient appointments to monitor her analgesia control, but she was unable to attend the three appointments that were provided. During an extended admission from November 2023 to February 2024, she underwent a significant surgical procedure involving exploratory laparotomy, adhesiolysis, ileo-colic resection, and end-ileostomy, which was complicated by a superior mesenteric vein and a PICC-line associated thrombosis. Due to her significant medical comorbidities, she experienced a rapid functional decline, rendering her wheelchair bound. Her mobility limited was limited to 15 metres due to fatigue, and her baseline body weight was recorded at 32 kilograms as of August 2024. Palliative Care Involvement The palliative care [PC] team was first consulted during an admission in August 2024 for a partial small bowel obstruction [SBO] (Figure 1). Her main presenting complaint was colicky, abdominal pain with spasms, which differed from the cyclical pain previously associated with her endometriosis. Physical examination showed a distended abdomen with diffuse tenderness and reduced bowel sounds. Based on the imaging and clinical findings, a nasogastric tube and foley catheter into her ileostomy were inserted for gastric decompression, and APS were referred for pain management. Due to concerns about gastric absorption for oral medications given her partial SBO and previous intolerances to sublingual buprenorphine, analgesia was strictly limited to parenteral. Initial attempts with intravenous tramadol, clonidine, and fentanyl provided limited relief to her abdominal pain. Eventually, a ketamine infusion of 2 mg/hour was effective, with adjuvant intravenous clonidine of 25 micrograms three-times a day. Her infusion was gradually titrated up to 8 mg/hour over two days without causing haemodynamic instability or hallucinations. On the fifth day of the ketamine infusion, her PICC line was blocked. Due to the previous PICC associated thromboses she had obtained in her contralateral limbs, no other long-term venous options were available, as her tunnelled CVC was reserved for TPN. The APS subsequently consulted with the in-patients’ PC team to discuss alternative analgesia options. Analgesia was subsequently transitioned to a continuous subcutaneous infusion [CSCI] of 200mg ketamine over 24 hours. Additionally, the intravenous clonidine was adjusted to a subcutaneous 25 micrograms administered three-times a day. As her SBO resolved, she was transitioned from the CSCI to oral ketamine lozenges 25 to 50mg PRN six hourly, and second-line hydromorphone 0.2 to 0.4mg PRN hourly, without any other background analgesia. Despite the initial referral to palliative care indicating her prognosis of a few months, the patient and her family did not accept this prognosis. Her mother sought second opinions from private gastroenterologists for pursual of active treatment. Due to such discordance, gastroenterology and palliative care endeavoured to address prognosis and advance care planning, including limitations on high-level interventions, such as surgery and intensive care. However, the patient and her family consistently maintained that her condition was not a life-limiting illness. As her condition stabilised, the PC team encountered challenges in her discharge planning and had to address the constraints of community palliative care services for a patient who was unwilling to acknowledge that her disease was life-limiting. The community team, considering these premises and her pursuit of active treatment, initially could not accept the patient for providing community medical and nursing support for sublingual ketamine and parenteral hydromorphone. Efforts were made to re-engage APS and Chronic Pain Service [CPS] to establish a collaborative care model within the community. However, they were hesitant to re-engage due to her previous non-compliance with CPS outpatient clinics. Despite the lack of engagement from the APS and CPS teams, and notwithstanding the patient’s denial concerning her prognosis, the inpatient palliative care team were able to arrange for the community PC service to provide clinical support upon discharge. She was discharged home after 50 days as an inpatient, with intravenous cyclizine 50 mg three times a day and daily TPN administered via her tunnelled CVC, without the need for background nor intermittent analgesia. Despite these efforts, the patient was found in septic shock from an infected tunnelled CVC line by the community PC team and was readmitted to the gastroenterology inpatient unit within 24 hours of discharge. A medically driven goals of care was conducted by her gastroenterologist and the intensivist to exclude intensive care admissions and rapid response calls. The consensus was that such interventions would be futile. The medical ceiling of care was for ward-based treatment for reversible causes, which including the removal of her tunnelled CVC line, which she agreed to have removed. While waiting for her procedure, the patient suffered a sudden cardiac arrest on the ward and died shortly after, with her family by her side, within 36 hours of her discharge from the same hospital. Discussion Challenges in Pain Management for Intestinal Failure Patients Intestinal failure [IF], characterised by the inability to maintain adequate nutrition or fluid-electrolyte balance without parenteral support, often results in chronic visceral pain due to structural and functional gut abnormalities (8,9). Inflammatory changes, bacterial overgrowth and dysmotility contribute to nociceptive and neuropathic pain mechanisms (10,11). The reliance of parenteral nutrition introduces additional risks, including intravenous catheter infections, and intestinal failure associated liver disease, which exacerbate discomfort (9). Opioids remain a cornerstone of analgesia management in IF but are fraught with challenges. Prolonged use is linked to opioid-induced hyperalgesia, dependency, and its potential to exacerbate gut dysmotility (10,12,13). Oral opioid analgesics are often ineffective due to intestinal absorption challenges, necessitating intravenous, sublingual or transdermal routes (8,10). Addition of adjuvants such as gabapentin can address neuropathic components whilst minimising dependency, but circles back to lack of gut absorption through the oral route (14,15). Notably for our patient, gabapentin or pregabalin were not trialled due to concerns of gastrointestinal malabsorption; however parenteral clonidine was proven to be an effective analgesia adjunct. Transdermal options such as buprenorphine and fentanyl patches, are widely used for chronic visceral pain in IF. Buprenorphine’s partial μ-opioid agonism and κ-antagonism reduce constipation risk compared to full opioid agonists (16). Fentanyl patches provide steady analgesia but require intact dermal perfusion, which may be compromised in malnourished and cachectic patients (17). Sublingual buprenorphine and fentanyl avoid intestinal absorption issues in IF patients, and avoid inconsistent absorption from jejunostomies or ileostomies, providing rapid relief during acute pain flares (16,17). However, limitations include worsening of ileus and motility disorders (especially in patients with short gut syndrome), bacterial overgrowth, opioid dependency and regulatory restrictions on opioid naïve patients, particularly with fentanyl (10,16,17). Furthermore, 30% of patients develop erythema and pruritis at the application sites for transdermal formulations, which complicates long term use (16,17). Recent advancements in pain relief for IF patients include teduglutide, a glucagon-like peptide-2 [GLP-2] analogue that enhances intestinal adaptation by increasing villus height and crypt depth, and reduces abdominal pain by mitigating mucosal inflammation (18); eluxadoline, a peripherally acting μ-opioid agonist/δ-antagonist that reduces visceral hypersensitivity in diarrhea-predominant conditions (15); and low dose transdermal clonidine which reduces colonic hypermotility and visceral pain via α 2 -adrenergic modulation (19). However, high costs, accessibility barriers and risk of biliary complications prevent from mainstream use. Intervention strategies such as coeliac plexus neurolysis and splanchnic nerve blocks have been considered for an appropriate subsect of patients. Coeliac plexus neurolysis targets sympathetic nerves innervating the upper abdominal viscera. In IF patients with chronic pancreatitis or radiation enteritis as the predominant feature of their abdominal pain, coeliac plexus blocks reduced pain scores by 50 – 70% for three to six months (20). Similarly, by targeting splanchnic nerves near the T11 – T12 vertebrae, this block is preferred for patients with retroperitoneal fibrosis or extensive abdominal adhesions (21). Although not extensively studied, epidural or intrathecal blocks are generally considered unsuitable for managing pain related to IF. This is due to the higher risk of autonomic instability, potential motor blockade which could exacerbate muscle atrophy in malnourished patients and, and higher rates of catheter-related infections due to their immunocompromised status (22). Non-pharmacological interventions, such as cognitive-behavioural therapy and acupuncture are underutilised despite evidence supporting their role in reducing opioid reliance (23,24). Interdisciplinary Care Coordination and Service Delivery Models Given our patient’s complex needs, multidisciplinary coordination amongst specialties, including gastroenterology, surgery, APS, CPS and palliative care was required. Multiple studies demonstrate that multidisciplinary teams significantly improve pain control and quality of life in cancer patients. A prospective study of 92 cancer inpatients found that individualised interventions by an interprofessional team consisting of surgeons, pain physicians, psychologists and nurses led to a reduced pain burden and pain scores, for patients receiving concurrent chemotherapy (25). Notably, structured follow-up via phone calls reducing annual hospitalisations by 44% in chronic pain programs, underscoring the importance of continuity (26). Consequently, collaboration between palliative care and pain medicine specialists is most effective when embedded within joint clinical frameworks. A survey of palliative care physicians found that institutions with dedicated collaboration systems and regular case conferences reported 2.5 times higher referral rates to pain medicine specialists, and co-managed 37% of cancer pain cases compared to 6% in less collaborative environments (27). Procedural interventions such as coeliac plexus neurolysis were utilised 7.8 times annually in collaborative teams versus 5.5 times in more confined models (27). Despite physicians acknowledging the importance of their respective role to patients’ care, key barriers to referrals in non-collaborative systems including role ambiguity, lack of funding for interdisciplinary time (which in turn disincentivises collaboration) and insufficient time for intricate discussions (28-30). Successful collaborative palliative service models include a combination of hospital-based consult teams and community-based palliative care. Inpatient palliative care teams reduce intensive care unit admissions by 25% through early symptom management and discharge planning, whereas community palliative care programs reduce hospitalisation rates by almost 40% (31,32). A more collaborative interdisciplinary care coordination with the in-patient and community service models might have helped avoid our patient’s unsuccessful discharge to the community and the limited time spent there before her final admission, which ultimately led to her demise. Notably she was not linked to any rare disease communities such as “Rare Voices”, which, through their capacity building and authentic engagement, recognise shared healthcare experiences of rare diseases and offer practical examples to address patient and family needs (33). The Role of Palliative Care in Non-Malignant Conditions Non-malignant diseases such as chronic obstructive pulmonary disease [COPD], end-stage congestive cardiac failure and dementia account for 60% of palliative care needs, but only 14% of specialist palliative care referrals (34,35). Prognostic uncertainty, compounded by variable disease trajectories, causes delays in referrals, as clinicians hesitate to label patients as “palliative” (35,36). This is exemplified in this case, where the patient and her family were in-denial of her life-limiting disease, given the rarity of gastrointestinal hypoganglionosis, and its limited data on long-term outcomes (5). Tools like the surprise question ("Would I be surprised if this patient died in the next year?") and the Supportive and Palliative Care Indicators Tool (SPICT) improve identification but lack sensitivity in early-stage illness (32,36). Early palliative care integration, concurrent with disease-modifying treatments, enhances quality of life. For example, COPD patients receiving earlier palliative care report 30% fewer hospitalisations and improved dyspnoea management, via prioritising the relief of burdensome symptoms such as dyspnoea, fatigue and pain over prognosis (36,37). Strategies involving early goals of care discussions and facilitating early advance care planning reduces intensive care unit admissions by 50% in geriatric populations with advanced heart failure (38). Embracing uncertainty and adopting early integration of palliative care principles at the onset of diagnosis may have yielded a different and more comfortable outcome for the patient and her family. Conclusion This case study of a young woman with intestinal failure due to hypoganglionosis provides several important insights into palliative care: challenges in managing pain in intestinal failure; need for flexibility in life-limiting conditions with uncertain prognosis; and importance of interdisciplinary care coordination when treating complex patients. Abbreviations Small bowel obstruction [SBO], total parenteral nutrition [TPN], central venous catheter [CVC], Intestinal Failure [IF], peripherally inserted central catheter [PICC], Acute Pain Service [APS], pro re nata [PRN], continuous subcutaneous infusion [CSCI], Chronic Pain Service [CPS], chronic obstructive pulmonary disease [COPD], Supportive and Palliative Care Indicators Tool (SPICT) Declarations Ethics approval and consent to participate: All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. Consent for publication: Written informed consent was obtained from the patient’s next of kin for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal. Availability of data and materials: Data sharing is not applicable to this article as no datasets were generated or analysed during the current study. Competing interests: The authors declare no conflicts of interest. Funding: This research received no specific function/grant from any funding agency in the public, commercial, or not-for-profit sectors. Authors' contributions : AD wrote the introduction, case presentation and discussion. SK prepared the figures. SK reviewed & edited the case presentation and discussion. The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. 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Cite Share Download PDF Status: Published Journal Publication published 30 Sep, 2025 Read the published version in BMC Palliative Care → Version 1 posted Editorial decision: Revision requested 17 Jul, 2025 Reviews received at journal 10 Jul, 2025 Reviewers agreed at journal 03 Jul, 2025 Reviewers agreed at journal 20 May, 2025 Reviews received at journal 19 May, 2025 Reviewers agreed at journal 13 May, 2025 Reviewers invited by journal 06 May, 2025 Editor invited by journal 07 Apr, 2025 Editor assigned by journal 07 Apr, 2025 Submission checks completed at journal 03 Apr, 2025 First submitted to journal 31 Mar, 2025 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-6344976","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":452779302,"identity":"50591492-2693-43fb-9dac-7c55cad62b11","order_by":0,"name":"Adarsh Das","email":"data:image/png;base64,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","orcid":"","institution":"Sir Charles Gairdner Hospital","correspondingAuthor":true,"prefix":"","firstName":"Adarsh","middleName":"","lastName":"Das","suffix":""},{"id":452779303,"identity":"7e9dbe1a-74ae-4cf6-a416-c9117319c941","order_by":1,"name":"Jayamangala Sampath Kondasinghe","email":"","orcid":"","institution":"Sir Charles Gairdner Hospital","correspondingAuthor":false,"prefix":"","firstName":"Jayamangala","middleName":"Sampath","lastName":"Kondasinghe","suffix":""}],"badges":[],"createdAt":"2025-03-31 12:53:22","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-6344976/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6344976/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1186/s12904-025-01878-0","type":"published","date":"2025-09-30T15:58:11+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":82358513,"identity":"93cb848e-60cd-4dcf-ab5e-1d01cd4860e5","added_by":"auto","created_at":"2025-05-09 11:24:38","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":367408,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cem\u003eAbdominal Computed Tomography (CT) showing gastrointestinal ganglionosis with superimposed bowel obstruction in transverse (A and B), coronal (C) and sagittal views (D).\u003c/em\u003e\u003c/p\u003e","description":"","filename":"Figure1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-6344976/v1/384b7cdeaa0b71c9692a536d.jpg"},{"id":92884464,"identity":"5b9326d5-1086-46e9-9dcf-d38a226a627a","added_by":"auto","created_at":"2025-10-06 16:13:08","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":755397,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6344976/v1/bc641599-4d95-417d-8087-45dcd876557c.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Pain Management in a Patient with Intestinal Failure in the Palliative Care Setting: Case Report","fulltext":[{"header":"Background","content":"\u003cp\u003ePalliative care for patients with non-malignant, chronic conditions presents unique challenges that differ from traditional cancer-focused palliative care (1,2). \u0026nbsp;In particular, the management of pain in patients with intestinal failure, encompasses multifaceted clinical, psychosocial, and systemic considerations that test the boundaries of conventional palliative approaches (3).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eAdult hypoganglionosis is an acquired, rare and complex condition that affects the enteric nervous system, resulting in gastrointestinal dysfunction and associated pain (4). The disease is characterised by a reduction in the number of ganglion cells in the myenteric and submucosal plexuses of the intestinal wall, leading to impaired intestinal motility and function (4-6). \u0026nbsp;It is considered a subset of intestinal innervation disorders, accounting for approximately 3% to 5% of all such classified conditions (4-6). Clinical features include chronic constipation, recurrent abdominal pain, distension, nausea, vomiting and intestinal obstruction (5,7). Diagnoses itself can be challenging due to the rarity and non-specific nature of its symptoms, which include endoscopic evaluation and full-thickness intestinal biopsies for histological examination (4,7). The pain associated with hypoganglionosis results from several factors: a) chronic intestinal distension due to impaired motility, b) intermittent intestinal obstruction, c) intestinal wall inflammation, and d) visceral hypersensitivity (4,6,7).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThis case illustrates the interplay between complex pain management and prognostic dilemmas in a patient with acquired gastrointestinal hypoganglionosis and intestinal failure, followed by a narrative review of the current evidence on this topic.\u003c/p\u003e"},{"header":"Case Presentation ","content":"\u003cp\u003e\u003cem\u003eKey Aspects of History\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eA patient in their late 20s, who since 2020, had multiple hospital admissions for recurrent small bowel obstructions [SBO] presenting as undifferentiated abdominal pain. She had no significant past medical or surgical history. In June 2022, after her eight hospital admission in under two years, she underwent a right hemicolectomy due to a caecal volvulus. Subsequent histopathology led to the diagnosis of gastrointestinal hypoganglionosis in the terminal ileum and caecum. During this admission, given she was unable to tolerate enteral feeding post-operatively, she was commenced on total parenteral nutrition [TPN] of three litres daily via a tunnelled small bore central venous catheter [CVC]. She was subsequently discharged home, with close outpatient follow up by her gastroenterologist and her multidisciplinary Intestinal Failure [IF] team.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eIn January 2023, she was diagnosed with stage four endometriosis. During her admission between March 2023 till September 2023, she underwent multiple peripherally inserted central catheter [PICC] insertions in her upper and lower limbs for prolonged courses of intravenous hydration, antibiotics, parenteral analgesia and anti-emetics. Her analgesia was managed by the Acute Pain Service [APS] team during this and subsequent admissions. She was trialled on the following medications: sublingual buprenorphine 200 to 400 micrograms pro re nata [PRN] four-hourly, oral tramadol 50mg to 100mg PRN four-hourly, baseline analgesia of topical weekly buprenorphine patches 5 microgram/hour and oral tapentadol sustained release 50mg twice a day. The maximum doses of these analgesic regimens were not reached due to inadequate abdominal pain relief, prompting frequent changes to her analgesia plan. Additionally, she did not perceive any benefit from topical or oral analgesia formulations, which often led to medication noncompliance. Eventually, short courses of intravenous ketamine infusions were identified as the only regimen effective in alleviating her abdominal pain. She was frequently discharged home after infusion without any additional background or PRN analgesia. The pain team scheduled outpatient appointments to monitor her analgesia control, but she was unable to attend the three appointments that were provided. During an extended admission from November 2023 to February 2024, she underwent a significant surgical procedure involving exploratory laparotomy, adhesiolysis, ileo-colic resection, and end-ileostomy, which was complicated by a superior mesenteric vein and a PICC-line associated thrombosis. Due to her significant medical comorbidities, she experienced a rapid functional decline, rendering her wheelchair bound. Her mobility limited was limited to 15 metres due to fatigue, and her baseline body weight was recorded at 32 kilograms as of August 2024.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cem\u003ePalliative Care Involvement\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eThe palliative care [PC] team was first consulted during an admission in August 2024 for a partial small bowel obstruction [SBO] (Figure 1). Her main presenting complaint was colicky, abdominal pain with spasms, which differed from the cyclical pain previously associated with her endometriosis. Physical examination showed a distended abdomen with diffuse tenderness and reduced bowel sounds. Based on the imaging and clinical findings, a nasogastric tube and foley catheter into her ileostomy were inserted for gastric decompression, and APS were referred for pain management. Due to concerns about gastric absorption for oral medications given her partial SBO and previous intolerances to sublingual buprenorphine, analgesia was strictly limited to parenteral. Initial attempts with intravenous tramadol, clonidine, and fentanyl provided limited relief to her abdominal pain. Eventually, a ketamine infusion of 2 mg/hour was effective, with adjuvant intravenous clonidine of 25 micrograms three-times a day. Her infusion was gradually titrated up to 8 mg/hour over two days without causing haemodynamic instability or hallucinations. On the fifth day of the ketamine infusion, her PICC line was blocked. Due to the previous PICC associated thromboses she had obtained in her contralateral limbs, no other long-term venous options were available, as her tunnelled CVC was reserved for TPN. The APS subsequently consulted with the in-patients\u0026rsquo; PC team to discuss alternative analgesia options. Analgesia was subsequently transitioned to a continuous subcutaneous infusion [CSCI] of 200mg ketamine over 24 hours. Additionally, the intravenous clonidine was adjusted to a subcutaneous 25 micrograms administered three-times a day. As her SBO resolved, she was transitioned from the CSCI to oral ketamine lozenges 25 to 50mg PRN six hourly, and second-line hydromorphone 0.2 to 0.4mg PRN hourly, without any other background analgesia. \u0026nbsp;\u0026nbsp;\u003c/p\u003e\u003cp\u003eDespite the initial referral to palliative care indicating her prognosis of a few months, the patient and her family did not accept this prognosis. Her mother sought second opinions from private gastroenterologists for pursual of active treatment. Due to such discordance, gastroenterology and palliative care endeavoured to address prognosis and advance care planning, including limitations on high-level interventions, such as surgery and intensive care. However, the patient and her family consistently maintained that her condition was not a life-limiting illness. As her condition stabilised, the PC team encountered challenges in her discharge planning and had to address the constraints of community palliative care services for a patient who was unwilling to acknowledge that her disease was life-limiting. The community team, considering these premises and her pursuit of active treatment, initially could not accept the patient for providing community medical and nursing support for sublingual ketamine and parenteral hydromorphone. Efforts were made to re-engage APS and Chronic Pain Service [CPS] to establish a collaborative care model within the community. However, they were hesitant to re-engage due to her previous non-compliance with CPS outpatient clinics.\u003c/p\u003e\n\u003cp\u003eDespite the lack of engagement from the APS and CPS teams, and notwithstanding the patient\u0026rsquo;s denial concerning her prognosis, the inpatient palliative care team were able to arrange for the community PC service to provide clinical support upon discharge. She was discharged home after 50 days as an inpatient, with intravenous cyclizine 50 mg three times a day and daily TPN administered via her tunnelled CVC, without the need for background nor intermittent analgesia. Despite these efforts, the patient was found in septic shock from an infected tunnelled CVC line by the community PC team and was readmitted to the gastroenterology inpatient unit within 24 hours of discharge. A medically driven goals of care was conducted by her gastroenterologist and the intensivist to exclude intensive care admissions and rapid response calls. The consensus was that such interventions would be futile. The medical ceiling of care was for ward-based treatment for reversible causes, which including the removal of her tunnelled CVC line, which she agreed to have removed. While waiting for her procedure, the patient suffered a sudden cardiac arrest on the ward and died shortly after, with her family by her side, within 36 hours of her discharge from the same hospital.\u0026nbsp;\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003e\u003cem\u003eChallenges in Pain Management for Intestinal Failure Patients\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eIntestinal failure [IF], characterised by the inability to maintain adequate nutrition or fluid-electrolyte balance without parenteral support, often results in chronic visceral pain due to structural and functional gut abnormalities (8,9). Inflammatory changes, bacterial overgrowth and dysmotility contribute to nociceptive and neuropathic pain mechanisms (10,11). The reliance of parenteral nutrition introduces additional risks, including intravenous catheter infections, and intestinal failure associated liver disease, which exacerbate discomfort (9). Opioids remain a cornerstone of analgesia management in IF but are fraught with challenges. Prolonged use is linked to opioid-induced hyperalgesia, dependency, and its potential to exacerbate gut dysmotility (10,12,13). Oral opioid analgesics are often ineffective due to intestinal absorption challenges, necessitating intravenous, sublingual or transdermal routes (8,10). Addition of adjuvants such as gabapentin can address neuropathic components whilst minimising dependency, but circles back to lack of gut absorption through the oral route (14,15). Notably for our patient, gabapentin or pregabalin were not trialled due to concerns of gastrointestinal malabsorption; however parenteral clonidine was proven to be an effective analgesia adjunct.\u003c/p\u003e\n\u003cp\u003eTransdermal options such as buprenorphine and fentanyl patches, are widely used for chronic visceral pain in IF. Buprenorphine\u0026rsquo;s partial \u0026mu;-opioid agonism and \u0026kappa;-antagonism reduce constipation risk compared to full opioid agonists (16). Fentanyl patches provide steady analgesia but require intact dermal perfusion, which may be compromised in malnourished and cachectic patients (17). Sublingual buprenorphine and fentanyl avoid intestinal absorption issues in IF patients, and avoid inconsistent absorption from jejunostomies or ileostomies, providing rapid relief during acute pain flares (16,17). However, limitations include worsening of ileus and motility disorders (especially in patients with short gut syndrome), bacterial overgrowth, opioid dependency and regulatory restrictions on opioid na\u0026iuml;ve patients, particularly with fentanyl (10,16,17). Furthermore, 30% of patients develop erythema and pruritis at the application sites for transdermal formulations, which complicates long term use (16,17). Recent advancements in pain relief for IF patients include teduglutide, a glucagon-like peptide-2 [GLP-2] analogue that enhances intestinal adaptation by increasing villus height and crypt depth, and reduces abdominal pain by mitigating mucosal inflammation (18); eluxadoline, a peripherally acting \u0026mu;-opioid agonist/\u0026delta;-antagonist that reduces visceral hypersensitivity in diarrhea-predominant conditions (15); and low dose transdermal clonidine which reduces colonic hypermotility and visceral pain via \u0026alpha;\u003csub\u003e2\u003c/sub\u003e-adrenergic modulation (19). However, high costs, accessibility barriers and risk of biliary complications prevent from mainstream use.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eIntervention strategies such as coeliac plexus neurolysis and splanchnic nerve blocks have been considered for an appropriate subsect of patients. Coeliac plexus neurolysis targets sympathetic nerves innervating the upper abdominal viscera. In IF patients with chronic pancreatitis or radiation enteritis as the predominant feature of their abdominal pain, coeliac plexus blocks reduced pain scores by 50 \u0026ndash; 70% for three to six months (20). Similarly, by targeting splanchnic nerves near the T11 \u0026ndash; T12 vertebrae, this block is preferred for patients with retroperitoneal fibrosis or extensive abdominal adhesions (21). Although not extensively studied, epidural or intrathecal blocks are generally considered unsuitable for managing pain related to IF. This is due to the higher risk of autonomic instability, potential motor blockade which could exacerbate muscle atrophy in malnourished patients and, and higher rates of catheter-related infections due to their immunocompromised status (22). Non-pharmacological interventions, such as cognitive-behavioural therapy and acupuncture are underutilised despite evidence supporting their role in reducing opioid reliance (23,24).\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eInterdisciplinary Care Coordination and Service Delivery Models\u0026nbsp;\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eGiven our patient\u0026rsquo;s complex needs, multidisciplinary coordination amongst specialties, including gastroenterology, surgery, APS, CPS and palliative care was required. Multiple studies demonstrate that multidisciplinary teams significantly improve pain control and quality of life in cancer patients. A prospective study of 92 cancer inpatients found that individualised interventions by an interprofessional team consisting of surgeons, pain physicians, psychologists and nurses led to a reduced pain burden and pain scores, for patients receiving concurrent chemotherapy (25). Notably, structured follow-up via phone calls reducing annual hospitalisations by 44% in chronic pain programs, underscoring the importance of continuity (26).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eConsequently, collaboration between palliative care and pain medicine specialists is most effective when embedded within joint clinical frameworks. A survey of palliative care physicians found that institutions with dedicated collaboration systems and regular case conferences reported 2.5 times higher referral rates to pain medicine specialists, and co-managed 37% of cancer pain cases compared to 6% in less collaborative environments (27). Procedural interventions such as coeliac plexus neurolysis were utilised 7.8 times annually in collaborative teams versus 5.5 times in more confined models (27). Despite physicians acknowledging the importance of their respective role to patients\u0026rsquo; care, key barriers to referrals in non-collaborative systems including role ambiguity, lack of funding for interdisciplinary time (which in turn disincentivises collaboration) and insufficient time for intricate discussions (28-30). Successful collaborative palliative service models include a combination of hospital-based consult teams and community-based palliative care. Inpatient palliative care teams reduce intensive care unit admissions by 25% through early symptom management and discharge planning, whereas community palliative care programs reduce hospitalisation rates by almost 40% (31,32). A more collaborative interdisciplinary care coordination with the in-patient and community service models might have helped avoid our patient\u0026rsquo;s unsuccessful discharge to the community and the limited time spent there before her final admission, which ultimately led to her demise. Notably she was not linked to any rare disease communities such as \u0026ldquo;Rare Voices\u0026rdquo;, which, through their capacity building and authentic engagement, recognise shared healthcare experiences of rare diseases and offer practical examples to address patient and family needs (33).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cem\u003eThe Role of Palliative Care in Non-Malignant Conditions\u003c/em\u003e\u003c/p\u003e\n\u003cp\u003eNon-malignant diseases such as chronic obstructive pulmonary disease [COPD], end-stage congestive cardiac failure and dementia account for 60% of palliative care needs, but only 14% of specialist palliative care referrals (34,35). Prognostic uncertainty, compounded by variable disease trajectories, causes delays in referrals, as clinicians hesitate to label patients as \u0026ldquo;palliative\u0026rdquo; (35,36). This is exemplified in this case, where the patient and her family were in-denial of her life-limiting disease, given the rarity of gastrointestinal hypoganglionosis, and its limited data on long-term outcomes (5). Tools like the surprise question (\u0026quot;Would I be surprised if this patient died in the next year?\u0026quot;) and the Supportive and Palliative Care Indicators Tool (SPICT) improve identification but lack sensitivity in early-stage illness (32,36). Early palliative care integration, concurrent with disease-modifying treatments, enhances quality of life. For example, COPD patients receiving earlier palliative care report 30% fewer hospitalisations and improved dyspnoea management, via prioritising the relief of burdensome symptoms such as dyspnoea, fatigue and pain over prognosis (36,37). Strategies involving early goals of care discussions and facilitating early advance care planning reduces intensive care unit admissions by 50% in geriatric populations with advanced heart failure (38). Embracing uncertainty and adopting early integration of palliative care principles at the onset of diagnosis may have yielded a different and more comfortable outcome for the patient and her family.\u0026nbsp;\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eThis case study of a young woman with intestinal failure due to hypoganglionosis provides several important insights into palliative care: challenges in managing pain in intestinal failure; need for flexibility in life-limiting conditions with uncertain prognosis; and importance of interdisciplinary care coordination when treating complex patients. \u0026nbsp;\u003c/p\u003e\n"},{"header":"Abbreviations","content":"\u003cp\u003eSmall bowel obstruction [SBO], total parenteral nutrition [TPN], central venous catheter [CVC], Intestinal Failure [IF], peripherally inserted central catheter [PICC], Acute Pain Service [APS], pro re nata [PRN], continuous subcutaneous infusion [CSCI], Chronic Pain Service [CPS], chronic obstructive pulmonary disease [COPD], Supportive and Palliative Care Indicators Tool (SPICT)\u003c/p\u003e\n"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate:\u003c/strong\u003e All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication:\u003c/strong\u003e Written informed consent was obtained from the patient\u0026rsquo;s next of kin for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials:\u003c/strong\u003e Data sharing is not applicable to this article as no datasets were generated or analysed during the current study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests:\u0026nbsp;\u003c/strong\u003eThe authors declare no conflicts of interest.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding:\u003c/strong\u003e This research received no specific function/grant from any funding agency in the public, commercial, or not-for-profit sectors.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthors\u0026apos; contributions\u0026nbsp;\u003c/strong\u003e: AD wrote the introduction, case presentation and discussion. SK prepared the figures. SK reviewed \u0026amp; edited the case presentation and discussion. The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All authors have reviewed and approved the submitted manuscript.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAcknowledgements\u003c/strong\u003e: Many thanks to the palliative care consultants and nursing staff for their invaluable insight, constructive feedback and suggestions. \u0026nbsp;\u003c/p\u003e\n"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eGadoud A, Kane E, Oliver SE, et al. Palliative care for non-cancer conditions in primary care: a time trend analysis in the UK (2009-2014). BMJ Support Palliat Care. 2020 Jan 13 Epub 20200113. https://doi:10.1136/bmjspcare-2019-001833. Cited in: Pubmed; PMID 31932476\u003c/li\u003e\n\u003cli\u003eMoens K, Higginson IJ, Harding R. Are there differences in the prevalence of palliative care-related problems in people living with advanced cancer and eight non-cancer conditions? A systematic review. J Pain Symptom Manage. 2014 Oct;48(4):660-77. https://doi:10.1016/j.jpainsymman.2013.11.009. Cited in: Pubmed; PMID 24801658.\u003c/li\u003e\n\u003cli\u003eBroadbent AM, Heaney A, Weyman K. A review of short bowel syndrome and palliation: a case report and medication guideline. J Palliat Med. 2006 Dec;9(6):1481-91. https://doi:10.1089/jpm.2006.9.1481. Cited in: Pubmed; PMID 17187557.\u003c/li\u003e\n\u003cli\u003eLee A, Suhardja TS, Simpson I, Lim JT. Rare case of adult intestinal hypoganglionosis and review of the literature. Clin J Gastroenterol. 2021 Apr;14(2):599-607. https://doi:10.1007/s12328-021-01342-5. Cited in: Pubmed; PMID 33502729.\u003c/li\u003e\n\u003cli\u003eAldossary MY, Privitera A, Elzamzami O, Alturki N, Sabr K. A Rare Case of Adult-Onset Rectosigmoid Hypoganglionosis. 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BMJ Open. 2021 Dec 1;11(12):e048417. eng. Epub 20211201. https://doi:10.1136/bmjopen-2020-048417. Cited in: Pubmed; PMID 34853100.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"bmc-palliative-care","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pcar","sideBox":"Learn more about [BMC Palliative Care](http://bmcpalliatcare.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/pcar/default.aspx","title":"BMC Palliative Care","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true},"keywords":"gastrointestinal, hypoganglionosis, intestinal failure, pain, palliative care","lastPublishedDoi":"10.21203/rs.3.rs-6344976/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6344976/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground: \u003c/strong\u003eManaging pain in palliative patients with intestinal failure requires a multidisciplinary approach that addresses complex pharmacological and systemic challenges while navigating prognostic uncertainties.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCase Presentation: \u003c/strong\u003eThis case of gastrointestinal hypoganglionosis demonstrates the necessity of alternative analgesic routes—such as subcutaneous ketamine—when conventional opioids fail due to intestinal malabsorption and dysmotility. Effective care required interdisciplinary collaboration, yet persistent discordance between the patient’s curative expectations and her life-limiting prognosis hindered timely advance care planning and community support transitions.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusions:\u003c/strong\u003e Prognostic uncertainty, compounded by the condition’s rarity, highlights the need for early palliative care integration in non-malignant conditions, to ensure that care goals align with patient and family priorities. The case advocates for adaptable care models that bridge inpatient and community services, even amid prognostic ambiguity, to prevent fragmented care during critical transitions.\u003c/p\u003e","manuscriptTitle":"Pain Management in a Patient with Intestinal Failure in the Palliative Care Setting: Case Report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-05-09 11:24:33","doi":"10.21203/rs.3.rs-6344976/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2025-07-17T08:30:35+00:00","index":"","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-07-10T23:24:04+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"84256573433152360595419575304836682641","date":"2025-07-03T19:15:21+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"138041443448242013244733095089438600034","date":"2025-05-20T23:49:20+00:00","index":"hide","fulltext":""},{"type":"editorInvitedReview","content":"","date":"2025-05-20T02:08:17+00:00","index":"hide","fulltext":""},{"type":"reviewerAgreed","content":"208319963438883331371711804130151125391","date":"2025-05-13T22:11:33+00:00","index":"hide","fulltext":""},{"type":"reviewersInvited","content":"","date":"2025-05-06T04:13:28+00:00","index":"","fulltext":""},{"type":"editorInvited","content":"","date":"2025-04-07T11:43:41+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-04-07T11:35:26+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-04-03T12:40:14+00:00","index":"","fulltext":""},{"type":"submitted","content":"BMC Palliative Care","date":"2025-03-31T12:48:58+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"bmc-palliative-care","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"pcar","sideBox":"Learn more about [BMC Palliative Care](http://bmcpalliatcare.biomedcentral.com/)","snPcode":"","submissionUrl":"https://www.editorialmanager.com/pcar/default.aspx","title":"BMC Palliative Care","twitterHandle":"BMC_series","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"em","reportingPortfolio":"BMC Series","inReviewEnabled":true,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"dfc414da-240c-4966-8991-ab4e654f6797","owner":[],"postedDate":"May 9th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-10-06T16:10:23+00:00","versionOfRecord":{"articleIdentity":"rs-6344976","link":"https://doi.org/10.1186/s12904-025-01878-0","journal":{"identity":"bmc-palliative-care","isVorOnly":false,"title":"BMC Palliative Care"},"publishedOn":"2025-09-30 15:58:11","publishedOnDateReadable":"September 30th, 2025"},"versionCreatedAt":"2025-05-09 11:24:33","video":"","vorDoi":"10.1186/s12904-025-01878-0","vorDoiUrl":"https://doi.org/10.1186/s12904-025-01878-0","workflowStages":[]},"version":"v1","identity":"rs-6344976","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6344976","identity":"rs-6344976","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}