CALCIPHYLAXIS, A CASE SERIES – PAIN & SYMPTOM MANAGEMENT RECOMMENDATIONS AND ROLE OF RENAL SUPPORTIVE CARE

W YEUNG 1, R PAIS 2

1Department of Nephrology, Wollongong Hospital, Wollongong, Australia, 2Department of Palliative Medicine, Royal Prince Alfred Hospital, Sydney, Australia

Background: Calciphylaxis is a rare condition caused by calcification of microvessels in the dermis and subcutaneous tissue, leading to development of severe painful skin lesions which progress to areas of necrosis and ulceration over time.  Pain management is often difficult and refractory to usual opioids.  Mortality is high, reported at up to 80% within the first year of diagnosis, and is most commonly due to sepsis related to wound infection.  To date, there is little evidence regarding treatment of calciphylaxis and no specific pain management guidelines for this condition.

Case Report: We present a case series of three patients with end stage kidney disease (ESKD) who were diagnosed with calciphylaxis.  Pain was a common distressing symptom and was refractory to usual analgesia.  These patients had severe mixed nociceptive and neuropathic pain, requiring a combination of various analgesia modalities for management including an opioid and gabapentinoid.  One patient required the use of methadone and a subcutaneous lignocaine infusion near the end of her life.  Pain contributed to significant morbidity, reduced poor quality of life and poor functional status. These patients received input from a palliative care physician, dietician, nurse consultant and social worker in addition to their usual nephrologist as part of renal supportive care.  This service encompassed shared decision-making and aligning treatment plans with patient goals through advanced care planning.  This is particularly relevant in calciphylaxis, a condition with such high mortality.

Results: The most prevalent and severe symptoms reported by 250 CKM patients at baseline were lethargy (85%), poor mobility (76%), drowsiness (71%), pruritus (60%) and pain (56%). 59% of CKM patients had significant improvement in total symptom score by their 3rd visit to the RSC clinic (mean IPOS-Renal score 13.8, 95%CI 12.9-14.7 vs. 11.6, 95%CI 10.6-12.6; p<0.001). CKM patients reported a mean of 8 (SD±3.5) symptoms at baseline, 7 of which were described as severe by >50% of patients. CKM patients had an average of 8 admissions per year with median length of stay (LOS) of 8 days, compared with 19 unplanned admissions in the dialysis group with a median LOS of 35 days. The age-standardised hospitalisation rate was 8-fold higher among dialysis patients compared with CKM patients.

Conclusion:Pain management can be challenging in patients with ESKD, who are at higher risk of opioid toxicity due to differences in drug metabolism and excretion.  These patients would benefit from renal supportive care, not only for symptom management but also holistic care.


Biography:

Dr Gigi Yeung is a nephrology staff specialist at Wollongong Hospital, specialising in renal supportive care. She has experience working in renal departments of multiple tertiary hospitals across New South Wales. She completed the Clinical Diploma of Palliative Medicine whilst working as the renal supportive care fellow at Royal Prince Alfred Hospital and Concord Hospital.

 

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