I had the opportunity over the past 10 years to care for a number of patients with PF at Children's Hospital. The data on those patients is shown below.
- 12 patients, 7 females - mean age 14 months
- 10 survived, 8 were intact neurologically
- 8 required amputation, some of multiple extremities
- infections - Neissera in 8, varicella in 2 and 1 each with Strep and E.coli
- surgeries done were colostomies, gastrostomies, tracheostomy and the amputations
The treatment protocol includes, as mentioned, 2 antibiotics begun in the ER, commonly vancomycin and ceftriaxone. ICU admission is required with placement of central venous and arterial lines. Resuscitation from shock and respiratory and ventilatory support is essential. In an animal model of sepsis, a double volume exchange transfusion (XT) using reconstituted PRBCs/FFP has been shown to be a useful adjunct in at least slowing or reversing this process. One advantage of a XT is it provides additional clotting factors. For pressor support we use dopamine, dobutamine, vasopressin and epinephrine. My preference is vasopressin over norepinephrine and milrinone as an afterload reducing agent. Steroids have been selectively used, more often in patients with adrenal hemorrhage, the so-called Waterhouse-Friedricksen syndrome.
The wound management is very much like that of a complex burn. We use silvadene mixed with nystatin applied daily by the nursing staff. Wound biopsies are done frequently for culture and excisions are performed in an intermediate time frame after a period of vascular stability. We would like to be assured of the patient's potential for survival and be convinced that there won't be a bad neurological outcome before we invest time resecting the tissue. What you want to do is excise all the necrotic tissue down to intact well-vascularized fascia, then use some form of wound closure.