From the Guidelines
The treatment of calciphylaxis requires a multifaceted approach focusing on wound care, pain management, and addressing underlying causes, with sodium thiosulfate being a cornerstone medication. The management of calciphylaxis involves several key components, including:
- Intensive wound care with sterile dressings, debridement of necrotic tissue, and infection prevention with appropriate antibiotics when needed.
- The use of sodium thiosulfate, typically administered intravenously at 25 grams three times weekly during hemodialysis sessions, or daily for non-dialysis patients, as it works by dissolving calcium deposits and improving vascular function 1.
- Pain control, which is essential and often requires opioid analgesics.
- The use of phosphate binders like sevelamer or calcium-free alternatives to normalize phosphate levels, while calcimimetics such as cinacalcet help control parathyroid hormone levels.
- Discontinuation of vitamin D supplements, warfarin, and calcium-based medications is crucial.
- Nutritional support with adequate protein intake promotes wound healing.
- For patients on dialysis, intensifying the regimen with daily or prolonged sessions helps improve calcium-phosphate balance.
- Hyperbaric oxygen therapy may benefit some patients by enhancing tissue oxygenation and wound healing.
- In severe cases with hyperparathyroidism, parathyroidectomy might be necessary. It is also important to consider the dialysate calcium concentration, as a lower concentration (eg, 1.5 to 2.0 mEq/L) might be considered in certain situations, such as when a low PTH level is associated with adynamic bone disease 1. However, the primary focus should be on the comprehensive management of calciphylaxis, rather than solely on adjusting dialysate calcium concentrations.
From the Research
Treatment Options for Calciphylaxis
- Calciphylaxis is a rare and life-threatening disease that mainly occurs in dialysis patients, and traditional treatment options are often unsatisfactory 2, 3, 4, 5, 6.
- A multi-modal treatment approach using IV sodium thiosulfate, cinacalcet, and sevelamer has been shown to improve patient outcomes, with a rapid decrease in pain, improvement of general condition, and wound healing within six months 2.
- Cinacalcet has been used as a treatment for calciphylaxis, either as monotherapy or in combination with other treatments, and has been shown to have a high response rate, with 83.4% of patients experiencing partial or complete response to treatment 3.
- Combination therapy with cinacalcet and other treatments, such as sodium thiosulfate and paricalcitol, has been shown to be effective in treating calciphylaxis with hyperparathyroidism 4.
- A multi-interventional treatment strategy, including trigger-agent cessation, wound management, antibiotic therapy, intensified hemodialysis, intravenous sodium thiosulfate, and attempted oxygen therapy, has been shown to be effective in treating calciphylaxis, with 6 out of 7 patients experiencing complete recovery 5.
- A multidisciplinary and multimodal approach, including wound care, antibiotics, analgesics, daily hemodialysis, and strategies targeting calcification, has been shown to be effective in treating calciphylaxis, with the addition of hyperbaric oxygen sessions helping to put the disease under control 6.
Key Treatment Components
- IV sodium thiosulfate: initial dose 119.4 +/- 84.9 g/m(2)/week, maintenance dose 40.6 +/- 9 g/m(2)/week 2.
- Cinacalcet: maintenance dose 36 +/- 32.9 mg/d 2, with a high response rate when used as monotherapy or in combination with other treatments 3.
- Sevelamer: maintenance dose 3320 +/- 1671 mg/d 2.
- Wound care and management: crucial for preventing infection and promoting healing 5, 6.
- Hyperbaric oxygen therapy: may be beneficial in putting the disease under control 6.