A case study, calciphylaxis: an exercise in human caring.
Prior to spring 2007, Abby was an independent 36-year-old leading an ordinary life. She had no reason to think she would not be going home when she entered the emergency room in March with painful wounds to her legs. Not a stranger to the health care system, Abby already had a significant medical history that included end-stage renal disease (ESRD), systemic lupus, portal vein thrombosis, and thrombotic thrombocytopenia purpura. At the time of hospital admission, she was taking various medications routinely, including prednisone, erythropoietin (Epogen [R]), lactulose, acetaminophen (Tylenol [R]), calcium acetate (PhosLo[R]), sevelamer hydrochloride (Renagel[R]), and warfarin (Coumadin[R]). In addition to the complaint of painful wounds that would not heal, she also described increasing weakness that, in combination with the pain to her legs, was decreasing her ability to ambulate and care for herself.
After many consultations, Abby first was diagnosed with warfarin-induced necrosis. She had been taking warfarin for 10 years due to her portal vein thrombosis, but the medication had been discontinued for a short time and then restarted. According to Abby, the lesions began to appear shortly after she resumed the medication. Based upon this initial assessment, the warfarin was discontinued and Abby was prescribed a low-molecular-weight heparin. Additionally, a biopsy of her wounds was performed to provide a definitive diagnosis. Results indicated Abby did not have warfarin-induced necrosis as previously thought, but she did have calciphylaxis.
Pathophysiology of Calciphylaxis
Calciphylaxis, also known as uremic gangrene syndrome or calcific uremic arteriolopathy (Rogers, Teubner, & Coates, 2007), is a tissue calcification process in which calcium is deposited in skin, soft tissues, and internal organs (Beitz, 2004; Ferreres et al., 2006). It occurs most often in patients with ESRD (Galimberti et al., 2005). Essentially, the decreased renal clearance causes an increase in phosphorus and then calcium. Elevation of these electrolytes causes the parathyroid gland to secrete additional parathyroid hormone to compensate, but this leads to increased mobilization of calcium and phosphorus (Beitz, 2004; Guldbakke & Khachemoune, 2007). The result is deposition of the mobilized calcium in tissues. As this happens, circulation is compromised or blocked by the calcification within the microvasculature. Distal tissue becomes oxygen-deprived and quickly dies.
Identifying at-risk persons is complicated. Calciphylaxis occurs in 38% of patients with ESRD who have received a kidney transplant (Guldbakke & Khachemoune, 2007), and no correlation seems to exist between the time of kidney transplantation and onset of symptoms. The time ranges from a few months to 20 years. The risk for women is three times higher than the risk for men (Ferreres et al., 2006; Guldbakke & Khachemoune, 2007). Age also does not seem to be a factor; the range is 6 months to 83 years old, with a mean age of 48 years (Guldbakke & Khachemoune, 2007). In addition to the patient with ESRD, calciphylaxis has been found in patients with any disease state that alters calcium and phosphorus metabolism. This includes persons with Crohn's disease, cirrhosis, hyperparathyroidism, lupus, and cancers that cause hypercalcemia (Beitz, 2004). Trying to foretell who eventually will develop calciphylaxis is not easy.
Progression of calciphylaxis is fairly predictable. Patients initially report a small area of redness and tenderness that progresses to intense erythema and severe pain. The pain is thought to be due to the ischemia and necrosis of the affected tissue and muscle (Guldbakke & Khachemoune, 2007). Diagnosis is made through biopsy (Beitz, 2004; Guldbakke & Khachemoune, 2007). Indurated plaques and nodules appear next, leading to necrosis with eschar. If the necrotic tissue becomes infected, it quickly progresses to sepsis and ultimately death. Any diagnosis of calciphylaxis has a poor prognosis. By the time clinical symptoms present, it frequently is too late to reverse, and mortality doubles when ulcers occur (Guldbakke & Khachemoune, 2007). Sepsis is the most common cause of death (Guldbakke & Khachemoune, 2007; Rogers et al., 2007). Calciphylaxis has up to an 80% mortality rate regardless of treatment (Rogers et al., 2007).
[FIGURE 1 OMITTED]
Some sort of vascular injury or hypercoagulable state is believed to be involved in the evolution of calciphylaxis (Galimberti et al., 2005). Possible lesion triggers include administration of warfarin, corticosteroids, or immunosuppressive drugs; transfusion of blood products; or local trauma (Galimberti et al., 2005). Abby, who had taken both prednisone and warfarin, had multiple possible triggers.
The Initial Goals of Care
Many necrotic lesions were already present on Abby's lower extremities; staff already were behind in providing appropriate care. Initially the wounds on her legs were debrided extensively and vacuum-assisted wound closure was applied in the operating room. At this point, the goals of treatment for Abby were to manage her pain, change VAC dressings three times per week, and continue antibiotics. The initial plan included improving or reversing Abby's disease process while maintaining her functionality.
During her stay on the surgical unit, Abby experienced a sudden drop in both blood pressure and hemoglobin and hematocrit (El & H). She was transferred to the medical-surgical intensive care unit (MSICU), where six units of packed red blood cells were transfused and albumin was given for her hypotension. It was thought that the drop in H & H was related to subcutaneous bleeding following the debridement. Abby soon developed a fever; septic syndrome treatment was initiated, including administration of levofloxacin (Levaquin[R]) and piperacillin (Zosyn[R]). Over the span of 11 days, Abby's white blood cell count went from 11,700 to 1,500 cells per microliter as she developed piperacillin-induced leukopenia. The piperacillin was discontinued, and two additional antibiotics were started. After Abby stabilized, she was transferred from the MSICU to a medical telemetry unit.
The nurses who cared for her still were focused on the goals of pain management and healing. Pain persisted with even minimal movement, and Abby was very lethargic due to the large doses of analgesic. Because her appetite had decreased, Abby was given nutrition through a nasogastric tube. During the 4 months of her hospitalization, Abby's albumin level remained between 1.2 and 2.0 gm/dL; a normal value is 3.5 gm/dL or greater. This indicated a protein deficiency that would affect her ability to heal (Delville, 2008). Wounds continued to erupt on her legs and torso (see Figure 1).
The wound-ostomy team followed Abby's progress closely, and guided the staff with her wound care. Dressing changes were scheduled into the nurses' busy day. On average, four people needed 3-4 hours to complete dressing changes. Staff had difficulty coping with their own emotions; there seemed to be no way to change the dressings without causing severe pain to Abby. A trip to the post-anesthesia care unit for sedation during one dressing change was attempted. After sedating her enough to manage her pain, she was nearly obtunded for 8 hours. This clearly was not a safe option for Abby when she needed dressing changes 3-4 times per week.
After Abby had been hospitalized for more than 2 months, new lesions continued to appear and seemed to be spreading aggressively on her arms and back. Old wounds began to smell, and the VACs were discontinued. Abby was bed-bound, not eating, and wanted the feedings via nasogastric tube discontinued. It appeared to the nursing staff that family and friends were encouraging, but failed to accept this as a terminal illness. Both Abby and her family were spiritual, and while it did not seem they were ready to accept the prognosis, the health care team had to face some difficult realities. Abby was not going to heal and go home. The goals for nursing had changed.
New Goals for Abby
The staff focused on keeping Abby comfortable and maintaining her dignity as infection was overwhelming her body. After many conversations between Abby and the health care team, a do-not-attempt-resuscitation order was written by the physician during her final weeks. The nursing staff had grown close to Abby and although her care was much more time-intensive than the other patients on the medical units, nurses requested to have Abby assigned to them. The staff seemed determined to ensure Abby had the best possible experience and care. During this time, many nurses were forced to wrestle with their own mortality. Always the question of doing the right thing was foremost in their minds. On a hot day in July, Abby quietly slipped away.
Inova Fairfax Hospital 0FH) is a large tertiary facility that sees thousands of patients every year. Jean Watson's Theory of Human Caring is the framework for nursing care at IFH. Many caring moments influenced Abby and her family as well as the staff. Watson (2006) indicated a "caring moment between the patient and nurse has a field of its own, which is greater than either one, and transcends both patient and nurse and becomes part of the life history of both" (p. 90). "It is in entering into and participating with the great mysteries of the sacred circle of life and death that we engage in healing" (Watson, 2003, p. 199). Abby did not spend more than 3 or 4 weeks on any individual nursing unit, but she was that one patient a nurse simply cannot forget. She helped every nurse who came in contact with her understand that nursing is a caring art.
Acknowledgment: We would like to thank our nursing colleagues Kathy Mullins, RN, and Nancy Bluefeld, RN, CWOCN, for their participation in the preparation and presentation of this case.
Beitz, J.M. (2004). Calciphylaxis: An uncommon but potentially deadly form of skin necrosis. The American Journal of Nursing, 104(7), 36-37.
Delville, C.L. (2008). Are your patients at nutritional risk? The Nurse Practitioner, 33(2), 36-39.
Ferreres, J.R., Marcoval, J., Bordas, X., Moreno, A., Muniesa, C., Prat, C., et al. (2006). Calciphylaxis associated with alcoholic cirrhosis. Journal of European Academy of Dermatology and Venereology, 20, 599-601.
Galimberti, R.L., Farias, E.D.R., Parra, I.H., Algranati, L., Kowalczuk, A., & Imperiali, N. (2005). Cutaneous necrosis by calcific uremic arteriolopathy. International Journal of Dermatology, 44(2), 101-106.
Guldbakke, K.K., & Khachemoune, A. (2007). Calciphylaxis. International Journal of Dermatology, 46(3), 231-238.
Rogers, N.M., Teubner, D.J., & Coates, P.T. (2007). Calcific uremic arteriolopathy: Advances in pathogenesis and treatment. Seminars in Dialysis, 20(2), 150157.
Watson, J. (2003). Love and caring ethics of face and hand--an invitation to return to the heart and soul of nursing and our deep humanity. Nursing Administration Quarterly, 27(3), 197-202.
Watson, J. (2006). Caring theory as an ethical guide to administrative and clinical practices. JONA'S Healthcare Law, Ethics, and Regulation, 8(3), 87-93.
Cheryl Schmitz, MS, RN-BC, CEN, currently on sabbatical, was Clinical Nurse Specialist, Adult Medical Surgical Services, Inova Fairfax Hospital, Falls Church, VA, at the time this article was written.
Liz Reyes, RN, is a RN II, Medical Telemetry Unit, Inova Fairfax Hospital, Falls Church, VA.
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|Title Annotation:||Clinical Practice|
|Author:||Schmitz, Cheryl; Reyes, Liz|
|Article Type:||Case study|
|Date:||Jul 1, 2009|
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