Daisy is a 67 year old IDDM in ESRF who presented to a regional hospital in February 2008 with exacerbation of CCF, recent weight gain of 3+ kg, pitting oedema to below knees, lethargy and anorexia.
On admission there was an incidental finding of a small lower abdominal erythema, this progressed over the weeks to a number of lesions and was ultimately diagnosed on biopsy to be Calciphylaxis.
Calciphylaxis is an incapacitating condition with 80% mortality rate and an estimated prevalence among haemodialysis patients of 1 – 4%.
Calciphylaxis
• Abnormal calcium deposits in the blood vessels causing subcutaneous nodules of infarction and skin necrosis
• Commonly in areas of thickened subcutaneous tissue eg breast, abdomen & thighs
• Extremely painful
• Poor healing and high associated infection rate
• Increased risk in female, obese and diabetic patients
• Death usually results from sepsis and organ failure
Surgery and antibiotic therapy did not contain the spread of the lesions. In July Daisy commenced hyperbaric oxygen therapy at a metropolitan hospital. Unfortunately a severe life threatening reaction to the therapy occurred so she was transfer back to her regional unit.
Final resort – expensive Sodium Thiosulphate therapy
Sodium Thiosulphate
• Calcium chelator – increases solubility and mobilization of vascular Ca deposits thereby promoting removal via haemodialysis
• Assists restoration of endothelial cell dysfunction promoting vasodilation
We will follow Daisy’s Calciphylaxis progress through 2008 and ultimately ask............................ treatment but at what cost?