The Role of Palliative Care in Pain Management for a Patient with Klippel-Trénaunay Syndrome: A Case Report

Abstract

For patients with serious illness related to a rare condition, it is critical that symptoms are adequately assessed and treated in both the inpatient and outpatient settings. The involvement of a palliative care team can be beneficial in the management of physical and emotional stressors in these situations. This case report explores the role of a specialist palliative care team in the pain management of a patient hospitalized due to complications from Klippel-Trénaunay syndrome (KTS), a rare congenital vascular malformation syndrome associated with chronic pain. With the use of a comprehensive symptom assessment, the implementation of a patient-controlled analgesia demand-button, and the use of validated tools such as the ESAS (Edmonton Symptom Assessment System) and CAGE-AID (Cutdown, Annoyed, Guilty, Eye-opener, Adapted to Include Drugs) screening, the patient achieved improved pain control, functionality, and overall well-being. This case highlights the importance of integrating palliative care in the management of complex diseases such as KTS.

Introduction

Klippel-Trénaunay syndrome (KTS) is a rare, congenital vascular malformation syndrome characterized by a triad of capillary malformations, venous malformations, and limb hypertrophy. ¹ KTS is estimated to affect 2–5 in 100,000 live births. ² It is typically caused by a sporadic mutation in the PIK3CA gene, a gene important for the creation of an enzyme involved in the regulation of cell growth and division. ² Manifestations of KTS are typically isolated to only one limb due to somatic mosaicism. ³ Patients with KTS commonly experience high levels of pain due to complications such as chronic venous insufficiency, skin and soft tissue infections, and vascular malformations. ¹ The treatment of this syndrome primarily focuses on the management of symptoms such as chronic cellulitis, limb hypertrophy, and venous malformations. As >60% of patients with KTS report pain as one of their primary symptoms, ⁴ the adequate management of pain is critical. Psychiatric comorbidities are also common among patients with KTS, with prevalence rates exceeding 15% for depression and 5% for anxiety. ⁴ This case explores the involvement of palliative care physicians and a multidisciplinary care team to manage the symptoms associated with this syndrome.

Case Presentation

A 31-year-old Caucasian female with a past medical history of KTS, chronic cellulitis, and chronic lymphedema of the right lower extremity presented to the emergency department (ED) with 1 day of worsening swelling, erythema, and pain in her right lower leg and foot. She worked in the film and media industry and was able to adequately perform her duties at work despite the chronic cellulitis, pain, and other complications associated with her diagnosis. She previously underwent amputation of her right toes as a teenager due to complications from KTS. She had previously tried sirolimus as a disease-modifying therapy for her condition but had not taken the medication for many years prior to this admission.

Upon arrival at the ED, the patient became hypotensive, was started on vasopressors, and was admitted to the intensive care unit (ICU) for septic shock due to severe cellulitis of the right lower leg. Broad-spectrum antibiotics were started, and she was taken to the operating room for the surgical exploration of her right lower leg. No evidence of necrotizing fasciitis was found during surgery, and she was transferred to the ICU for postoperative monitoring. While in the ICU, efforts were made to adequately manage her pain. She was initially managed with a hydromorphone patient-controlled analgesia (PCA) demand-button at a dose of 0.2 mg available every 6 minutes during the hospital days 1–3. She was later taken off the demand-button PCA on hospital day 3 and transitioned to scheduled oral oxycodone 5 mg every 6 hours, IV hydromorphone 1 mg every 2 hours as needed via nursing bolus, and given scheduled adjunct medications (acetaminophen 1000 mg every 8 hours, gabapentin 200 mg every 8 hours, and methocarbamol 500 mg every 6 hours).

The patient spent about a week in the ICU. Once her hemodynamics stabilized, she was taken off pressor support and transferred from the ICU to the general medicine floor on hospital day 8. Her IV hydromorphone as-needed dose was spaced out to every 8 hours. While in the general medical unit, she noted increasing pain in her right lower leg and was found to have a fever. Computed tomography (CT) revealed a new deep venous thrombosis in the right lower leg, for which she was started on anticoagulation. She then began to have increased superficial purulent drainage in her medial calf incision on her right lower leg, with wound cultures found to be positive for Escherichia coli. For this, she was given meropenem.

Due to increasing pain, her as-needed hydromorphone frequency was adjusted for more nursing boluses as needed in addition to continuing her nonopioid adjunct medications listed above. She reported worsening pain at night and felt that it was, in part, due to the delayed delivery of the needed medication from the nursing staff. This went on for many days, and the palliative care team was consulted on hospital day 18 to assist with pain management.

The palliative care team met with the patient and performed a comprehensive symptom assessment. The patient reported ongoing uncontrolled pain in her right calf and foot as well as intolerable pain when she put her right foot down during physical therapy sessions. She stated that she needed her hydromorphone as-needed dose about every 2 hours to keep her pain at a tolerable level (she noted a personal pain goal of 5/10 in severity as “tolerable”). Her pain regimen was reviewed, and it was calculated that she was requiring ∼120 mg oral morphine equivalent daily.

The palliative care team also measured the patient-reported symptom outcomes through the Edmonton Symptom Assessment System - Financial and Spiritual (ESAS-FS). The ESAS-FS is a comprehensive assessment used to evaluate a patient’s symptoms, and it includes the symptoms of pain. Patients are asked to provide a numerical rating between 0 (none) and 10 (worst possible), needing the average number for each symptom in the past 24 hours. The patient initially scored a total score of 40 on the assessment, with an average score of 4 for physical pain, 5 for depression, 5 for anxiety, and 5 for drowsiness. A CAGE-AID opioid misuse risk assessment was also performed, with the patient reporting a desire in the past to cut back on drinking alcohol. The palliative care team was also able to explore the areas of joy and strength for the patient, learn her life experiences, and gain insight into the patient’s version of dignity.

After a conversation between the patient and the palliative care team, a shared decision was made to stop the present opioid regimen, re-trial a hydromorphone demand-button PCA, and add a 1-mg IV hydromorphone nursing bolus 30 minutes before the scheduled physical therapy sessions. Her ESAS-FS score the next day totaled 32, a decrease of 8 points from the previous day. She reported improvement in drowsiness, depression, shortness of breath, and anxiety and remained at 4 for physical pain. Over the next few days, the patient continued to report slight improvements in her pain and an increased ability to work with physical therapy during her sessions. While her numerical perception of pain was generally consistent, her overall functionality showed improvements in terms of her spirits, participation with therapies, and engagement with staff. The palliative care team also helped to optimize the patient’s bowel regimen, as she reported that she had not had good bowel movement in 3+ weeks, further adding to improvements in the overall well-being. Additionally, the palliative care team communicated their recommendations with the multidisciplinary team of nurses, physicians, physical therapists, and other staff to ensure that the patient’s needs were heard and addressed.

Over the coming days, the patient was weaned off the hydromorphone demand-button PCA to scheduled oral hydromorphone 4 mg every 4 hours while continuing the IV hydromorphone 1-mg nursing boluses, as needed, for breakthrough pain. She was then discharged to inpatient rehabilitation, where she spent an additional 2 weeks before being discharged home. Because the inpatient rehabilitation was within the same hospital, the same palliative team was able to continue following the patient. She was discharged from inpatient rehabilitation with a pain regimen consisting of gabapentin 300 mg every 8 hours, oral hydromorphone 4 mg every 3 hours as needed, methocarbamol 500 mg every 8 hours as needed, and acetaminophen 1000 mg every 8 hours as needed. The patient was initially receptive to establishing care in our outpatient palliative/supportive care clinic for pain and symptom management, and this was coordinated by our team. After further discussions with her family, though, she ultimately decided to follow up with her established providers closer to home, who were familiar with her condition and symptom needs.

Discussion

The appropriate management of pain in KTS is complex. In the inpatient setting, uncontrolled pain can lead to the inability to perform physical therapy sessions, delay of discharge, and an increase in the risk of hospital-acquired complications through a potentially prolonged stay. As evidenced by the comprehensive palliative assessment, this patient was experiencing pain and symptoms in a variety of psychosocial domains. Through the comprehensive palliative assessment, we were able to recognize suffering through a physical, emotional, and existential lens and respond holistically.

There is a wide body of evidence that opioid demand-button PCA pumps can be helpful for postoperative pain, and this was the case in our patient above. ⁵ As nursing availability may limit the patient receiving pain medication, implementing the demand-button PCA for this patient allowed her to receive pain medication in a timely manner. In addition, the demand-button PCA allowed for more rapid, real-time titration based on the level of pain control and potential opioid-induced adverse events. ⁶ To identify the risk factors for nonmedical opioid use or opioid misuse, the CAGE-AID risk mitigation screening tool was performed in the initial palliative assessment, helping identify that this patient might have an increased risk of nonmedical opioid use and opioid overdose-related complications. Documentation of a patient’s daily opioid needs in the electronic medical record was important to trend stabilization and transition to an oral regimen that could be provided on discharge. ⁷

In addition to the efforts above, continuity of care with the palliative care team after this patient was discharged from the inpatient medical unit to inpatient rehabilitation at the same hospital was greatly beneficial in adequately managing her suffering. If this was not an option, though, sufficient communication between provider care teams and medical record documentation would be imperative. Previous research has indicated that frequent communication between providers and thorough documentation without the use of complex medical jargon are beneficial in easing the transition between providers in palliative care. ⁸ In this case, the ability of the same palliative care team to follow the patient in both settings and thoroughly document her treatment plan in the system-wide electronic medical record allowed for transparency in communication.

Because resources may be limited in the community setting, physicians in the community may find complex symptom management challenging. This, in turn, can make it difficult for patients with these conditions to receive adequate pain management in the community. Review studies on general practitioners’ attitudes toward prescribing opioids for noncancer-related chronic pain indicate that many feel under-resourced when it comes to pain management. ⁹ They report lacking adequate guidelines for prescribing opioids and not receiving sufficient support from specialists who are more experienced in these areas. ⁹ Further efforts to streamline communication between palliative care physicians and primary care physicians in the community are essential to address these gaps and help those with serious illness. Pain management for patients with uncommon syndromes such as KTS presents unique challenges, and it is in the patient’s best interest for both palliative care and primary care physicians to work together in the short and long term.

Conclusion

Pain is a common symptom in KTS, and a comprehensive symptom management plan is essential. In this case, the use of the demand-button PCA and recording opioid use daily to identify trends facilitated the rapid titration of opioids and pain relief. More than physical pain, though, a holistic assessment and plan including the use of validated tools (such as ESAS-FS and CAGE-AID) to balance pain management with risk mitigation, analgesia that is responsive to pain needs, continuity of care between care settings, and communication between medical providers in the inpatient and outpatient settings are necessary. Because conditions such as KTS are rare and complex, clinical providers may be hesitant to engage in symptom management and coordination of longitudinal care. Finally, the palliative team can advocate for the patient’s quality of life, add re-assurance that the patient’s needs are heard, and provide a spectrum of support should the patient’s disease trajectory worsen. KTS is a unique condition, and it is one that benefits from person-centered palliative approaches to care.

Authors’ Contributions

M.E.A. contributed to the conceptualization, original draft preparation, reviewing, and editing. J.S.M. contributed to conceptualization, reviewing, and editing.