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The Wound That Nearly Got Away: A Case Presentation.

Juvenile dermatomyositis (JDMS) is a chronic multisystem disease characterized by vasculitis of small arteries and capillaries of muscle, skin, subcutaneous tissues, and the gastrointestinal tract (Malleson, 1990). Early in its course, the disease is marked by the presence of proximal muscle weakness and a distinctive rash; calcium deposits in the tissues develop later. The incidence of JDMS has been reported at 3.2 per million population, with the onset usually occurring between 5 and 14 years of age. After the first decade of life there is a female predominance (Halbert, 1996). The etiology is unknown.

The onset of symptoms may be acute (50%) or insidious (Halbert, 1996). In 50% to 90% of patients, the presenting feature is the characteristic erythematous rash. Muscle weakness is usually gradual in onset and characteristically affects the major proximal muscles symmetrically. The incidence of calcinosis ranges from 30% to 70%. The lesions are frequently located at sites exposed to pressure (e.g., elbows, buttocks, knees) and seem to reflect severity and duration of the disease. Gastrointestinal involvement as a result of impaired muscle function and underlying vasculopathy may result in difficulty in managing secretions, masseter atrophy ("chipmunk" appearance), and both bowel ulceration and perforation. Ophthalmologic symptoms can include prominent or dilated blood vessels at the eyelid margin (Pachman, 1995). Renal and cardiac systems may also have changes due to vasculitis.

Medical management involves prompt, aggressive corticosteroid therapy in the early stages of the disease. Physiotherapy is essential to prevent contractures. Nursing management is focused on relieving anxiety related to the disease for the patient and family, attending to nutritional requirements, providing skin care to pressure areas to prevent ulceration, and coordination of the health care team (Bale & Jones, 1997).

Mortality from JDMS has been reduced from 37% in the 1960s to 8% more recently, with the prompt use of steroid therapy. One-third of patients will have a full recovery within 2 years. The remainder will have a more chronic course and may have some degree of functional disability from residual calcinosis, muscle wasting, and flexion contractures (Halbert, 1996).

Case History

Sally (a pseudonym used to protect patient confidentiality) is a 9-year-old girl who was diagnosed with JDMS in December 1996 by her primary care provider and begun on a subtherapeutic steroid dose of 20 mg daily. She was admitted to a tertiary hospital in March 1997, where her diagnosis was confirmed with muscle biopsy and laboratory tests indicating elevated muscle enzymes. Her treatment included an increase in the steroid dose to 60 mg a day; bed rest for 5 weeks; and management of chronic constipation, a pre-existing condition exacerbated by JDMS.

One month later, Sally was transferred to the pediatric surgical ward in our hospital because of increasing pain and abdominal distension. An abdominal X-ray confirmed a bowel perforation, a laparotomy was performed to repair the bowel and a protective loop colostomy created. Two wound drains were inserted and a central venous catheter (CVC) was inserted through which total parental nutrition (TPN) and patient controlled anesthesia (PCA) infusions were commenced.

Sally's stay in our ward for the 5 months proved to be a challenge not only for Sally and the multidisciplinary team, but especially for her mother. Sally's mother was in the process of a stressful marital split that provided an additional burden for her. With the family's agreement, psychiatric liaison support was coordinated for Sally, her mother, and the immediate family. Sally's mother roomed in with her for the entire 5-month stay in our ward, while her 11-year-old sister stayed with her grandparents at the nearby Ronald McDonald House.

Sally's condition was frequently unstable due to sepsis and respiratory compromise, resulting in four pediatric intensive care unit (PICU) admissions. The wound from her laparotomy dehisced, and she developed an enterocutaneous fistula. Two additional laparotomies were required for bowel perforations, the second resulting in a total colectomy and ileostomy formation. She had both chronic and acute pain for most of her 5-month stay on our ward, and in consultation with the pediatric pain service, this was managed with either oral or intravenous morphine. From the time of Sally's initial laparotomy until discharge from our ward, she would remain primarily lying flat, due both to discomfort in her joints and to the extent of her abdominal wounds.

Medical management of Sally's JDMS was several-pronged and included the following: (a) corticosteroid therapy in varying doses; (b) methotrexate and cyclosporin as immunosuppressants in an attempt to arrest the progress of the disease; (c) management of insulin resistance that had resulted from prolonged steroid use; and (d) management of obesity. Growth hormone was given in an attempt to increase muscle bulk and to assist wound healing, albumin infusions were administered to increase serum albumin levels, and an anabolic steroid was attempted in an effort to reduce the effects of prolonged steroid use. TPN was required for varying periods to address Sally's malnutrition, and frequent antimicrobial therapy regimes were used for septic episodes.

A multidisciplinary team approach was used to manage the complexity of Sally's condition and needs. At times, the large number of people involved in Sally's care became overwhelming for Sally and her mother. The multidisciplinary team consisted of five medical teams, physiotherapists, occupational therapists, social workers, ward grandparent, nursing staff, play specialist, stoma therapist, and school teachers. All of these people needed to either examine or treat Sally on a daily basis throughout the week. Sally and her family were left with little quality time together and minimal privacy. To address this concern, the nurse caring for Sally each shift would negotiate with Sally and her mother to schedule treatments and rest periods at times that were agreeable to Sally.

Compromised Wound Healing

Sally's complex medical problems greatly influenced her ability to heal. The key factors impeding healing included JDMS; decreased mobility; persistently low serum magnesium, zinc, and albumin; malnutrition; infection; and prolonged glucocorticosteroid therapy. The prolonged steroid use resulted in Cushings syndrome, decreased muscle bulk, and hyperglycemia, all factors that negatively influence wound healing.

JDMS results in increased occlusion of capillaries and arterioles leading to tissue infarctions that may have inhibited wound healing. Sally's bowel showed evidence of inflammatory occlusion of small vessels, yet there was no confirmed evidence of proximal large vessel vasculitis. Sally's mobility was decreased as a result of muscle weakness and calcinosis. Sally's nearly constant use of the supine position, made drainage of exudate from her abdominal cavity wound difficult.

Nutritional deficiencies are recognized as delaying wound healing by protracting the exudative phase and hindering collagen synthesis (Garvin, 1990). Sally's nutritional status varied with the instability of her condition. A number of factors influenced her nutritional status, including decreased gut motility in the initial phases of the disease, nausea and vomiting, multiple abdominal operations necessitating 1her to avoid eating (NPO), a total colectomy, and a period of rotavirus gastro-enteritis. Nutritional support was intensive and involved three periods of TPN for a total of 77 days. Oral intake was encouraged utilizing nutritional supplements whenever possible, yet there were periods when nasogastric feeding was required. Sally's daily caloric intake ranged from 1000-1400 calories.

Magnesium is an essential mineral for cell growth. Sally's serum magnesium levels fluctuated between 0.6 and 0.94 mmol/l (normal 0.75-1.0 mmol/l). Zinc is an essential cofactor for enzymatic activity, including protein synthesis. Patients who are zinc deficient have reduced epithelialization, decreased wound strength, and reduced collagen synthesis (Bale & Jones, 1997). Serum zinc levels were low throughout Sally's admission, ranging from 8-12 mmol/l. The normal zinc level is 12-20 mmol/l. Both magnesium and zinc supplements were given via TPN.

Albumin prevents wound edema by maintaining intravascular volume (Hartley & Campion-Fuller, 1994) and is a transport medium for amino acids, zinc, and fatty acids that are essential elements in wound healing (Garvin, 1990). Sally's serum albumin level fluctuated between 15-40 g/l for 4 months (normal range 35-47 g/l), most probably due to the high volumes of wound exudate. Two albumin transfusions were given to Sally to address this concern (see Figure 1).

[Figure 1 ILLUSTRATION OMITTED]

Sally had several episodes suggestive of systemic infection, although there were no positive blood cultures. Her abdominal wound was chronically infected, culturing staphylococcus aureus, pseudomonas, and candida albicans. As a result, a variety of antimicrobial and antifungal agents were used in her care (see Figure 2).

[Figure 2 ILLUSTRATION OMITTED]

Steroid therapy produces a decrease in the inflammatory response, depresses the development of granulation tissue and angioneogenesis, and inhibits wound contraction (Garvin, 1990; Hotter, 1990). Sally's prolonged, high-dose steroid use was a significant factor in her wound healing. In early August, Sally's serum glucose levels became elevated due to insulin resistance from her catabolic state, stress, steroids, and obesity. Insulin was required to assist glycemic control and promote anabolism. Growth hormone and oxandralone (anabolic steroid) were tried for their anabolic effects of increasing muscle bulk and improving wound healing, but were discontinued due to a resultant increase in insulin demands. Eventually, Sally was able to discontinue the use of insulin.

Wound Management

To care for Sally's wounds, dressings were performed for a total of 138 days, as described below. On many occasions there were two and three dressing changes a day due to excessive ooze or due to the difficulty of adhering dressing materials to Sally's abdomen. The choice of dressing products available is extensive, and as the wound progressed and regressed through the stages of wound healing, different products were used. Choosing which products to use involved consultation between nursing staff, surgeons, wound product suppliers, and specialists in adult wound management. Table 1 provides a summary of product descriptions and how they were utilized in Sally's wound management.
Table 1. Dressing Products Utilized in Sally's Wound Care(*)

Product                Manufacturer      Product Description

Telfa[TM]              Kendall, USA      Low adherent absorbent pad

Tegaderm[TM]           3M                Semipermeable transparent
                                         film dressing

Aquacel[TM]            ConvaTec          Hydrofiber dressing

Duoderm[TM]            ConvaTec          Hydrocolloid dressing

Steri-strips[TM]       3M                Microporous tape with
                                         polyfilament fibers

Comfeel[TM]            Coloplast         Hydrocolloid dressing

Kaltostat[TM]          ConvaTec          Alginate dressing

Jelonet[TM]            Smith & Nephew    Paraffin impregnated gauze

IntraSite Gel[TM]      Smith & Nephew    Hydrogel wound dressing

Allevyn[TM] Standard   Smith & Nephew    Hydrophilic absorbent
                                         dressing

Allevyn Cavity[TM]     Smith & Nephew    Deep wound dressing,
                                         consisting of a foam chip
                                         core with a nonadherent
                                         perforated film covering

Primapore[TM]          Smith & Nephew    A low adherent absorbent
                                         pad with an adhesive,
                                         nonwoven backing surface

Product                Application in Sally's Wound

Telfa[TM]              1. postoperative wound dressing
                       2. protection of healing tissue

Tegaderm[TM]           3. secondary dressing

Aquace[TM]             4. absorption of wound exudate
                       without drying out the wound bed

Duoderm[TM]            5. picture framing of the wound to
                       reduce trauma to surrounding skin
                       from repeated removal of tape

Steri-strips[TM]       6. support wound edges and assist
                       with approximation of wound
                       edges

Comfeel[TM]            7. support the wound
                       8. secondary dressing

Kaltostat[TM]          9. absorption of exudate from the
                       wound

Jelonet[TM]            10. To assist with securing of a
                       primary dressing
                       11. to prevent wound edges from
                       drying

IntraSite Gel[TM]      12. to stimulate granulation by
                       provision of a moist wound
                       environment
                       13. wound debridement and
                       desloughing

Allevyn[TM] Standard   14. to arrest hypergranulation

Allevyn Cavity[TM]     15. absorption of exudate from full
                       thickness wound

                       16. protection of the epithelializing
Primapore[TM]          wound from external damage


(*) Products are listed in order that they appear in the text

Care of initial surgical wound. Sally's initial laparotomy wound (April 1997) revealed two colonic perforations. A loop colostomy was created and two wound drains inserted. The wound was closed with clips and covered with Telfa[TM] (Kendall) and Tegaderm[TM] (3M). The two wound drains were covered with ostomy bags due to the high volumes of exudate; the colostomy was bagged with a one piece pouch system. Seven days after surgery, the wound had increased serosanguinous exudate and was irrigated with saline via syringe and redressed with Aquacel[TM] (ConvaTec) and Tegaderm in an attempt to control drainage. The following day, there was evidence of dehiscence, the staple closures were removed, the wound picture framed with Duoderm[TM] (ConvaTec) to reduce the damage to local skin from repeated tape removal. Steri-strips[TM] (3M) and strips of Comfeel[TM] (Coloplast) were applied across the suture line in an attempt to support the wound. The entire area was then covered with Duoderm. A small sloughing area at the distal end of the wound was cleaned and dressed with Aquacel and Tegaderm.

The left drain continued to have high volumes of exudate, requiring daily or more frequent emptying. The right drain was removed and was dressed with Aquacel and Duoderm. The exudate from the distal laparotomy wound appeared fecal in origin and an enterocutaneous fistula was suspected.

Wound care after second surgery. Increasing abdominal pain coupled with the suspicion of a fistula and pus in the left drain site resulted in surgery in May to re-explore the laparotomy wound. Findings included a sigmoid enterocutaneous fistula; a wound drain was inserted into the fistula and then dressed with Kaltostat[TM] strips (ConvaTec) to aid in the absorption of exudate. The laparotomy wound was left open in two areas to facilitate cleaning of the areas and to allow healing by secondary intention (see Figure 3).

[Figure 3 ILLUSTRATION OMITTED]

Daily dressings of the laparotomy wound involved irrigating with saline, picture framing of the wound with Duoderm, Kaltostat shaped to fit the wound bed, securing in place with Jelonet[TM] (Smith & Nephew), and covering with a secondary dressing of Combine (a secondary dressing material consisting of a layer of absorbent cotton enclosed in a sleeve of cotton gauze). The fistula was bagged and irrigated with saline via the bags as required to facilitate the removal of exudate and fecal matter. Bagging of the fistula was done for several reasons. It enabled accurate measuring of the output, allowed visualization of the surrounding skin, and reduced the skin excoriation that may have occurred with frequent changing of conventional dressings such as Jelonet and gauze. The colostomy and drain site bags were changed as necessary. The dressing change was labor intensive, requiring approximately 1 hour of time and two nurses to perform the procedures. Two nurses were needed due to the discomfort caused by dressing changes, patient anxiety, and the difficulty of accommodating two ostomy bags and a large laparotomy dressing on a child's abdomen. During this period, the deepest portion of the wound was down to the peritoneum, with visible bowel.

This dressing regime continued for 10 days and was revised to stimulate granulation in the laparotomy wound, using IntraSite Gel[TM] (Smith & Nephew) on ribbon gauze, Combine dressing and covered with Tegaderm. The right drain site from the initial laparotomy was dressed with IntraSite Gel and Tegaderm, and the fistula remained bagged. The colostomy bag was changed to a two-piece system in an attempt to increase the adherence of the device and protect the skin from excoriation. Daily dressings continued since an attempt to change to dressing changes every 48 hours resulted in contaminated dressings either from drainage from the fistula or leakage from the colostomy.

Two weeks later, a proximal portion of the laparotomy wound developed a small central area of hypergranulation (see Figure 4). This was managed with Allevyn[TM] standard dressing (Smith & Nephew) and covered with Primapore[TM] (Smith & Nephew). The Allevyn standard dressing occluded the wound, thereby inhibiting cell proliferation and arresting hypergranulation.The remainder of the wound continued to be dressed with IntraSite Gel[TM], Allevyn Cavity[TM] (Smith & Nephew), gauze, and Primapore[TM] (see Figure 5).

[Figures 4-5 ILLUSTRATION OMITTED]

Wound care after third surgery. In early July, Sally returned to surgery as she had increasing fistula drainage, abdominal discomfort, and abdominal distension. The laparotomy revealed two bowel perforations, an abscess associated with the sigmoid fistula, and free fecal peritonitis. An appendectomy and formation of loop colostomy were performed, and two wound drains were inserted.

The hypergranulation had been arrested, but small necrotic areas were detected in the laparotomy wound. These were dressed with IntraSite Gel on ribbon gauze and covered with Combine dressings and Tegaderm. The remaining laparotomy sutures were removed later in July, as there was an increase in discharge from the laparotomy wound and the presence of pus in the wound. The wound was cultured, and pseudomonas was found to be present, so Sally was started on intravenous antibiotics (see Figure 6). Dressings were requiring hours of nursing care at this time. The laparotomy wound, the fistula, and the right drain continued to have purulent discharge.

[Figure 6 ILLUSTRATION OMITTED]

Wound care after fourth surgery. Sally returned to surgery in early August for further wound exploration. Pus was present in both iliac fossa, an abscess associated with the sigmoid fistula, and the colon was necrotic. A total colectomy was performed, the old colostomy site closed, and a new ileostomy formed. Two wound drains were inserted and the wound was closed with tension sutures.

The laparotomy wound was now dressed with Kaltostat gauze and Tegaderm, with Jelonet being applied to the wound edges if they dried out. Both drain sites remained bagged. Two weeks later, the laparotomy wound had granulated and epithelialization was well established, therefore, the alternate sutures were removed and Primapore was applied to protect the wound from external damage (see Figure 7). The drain sites no longer required bagging, and Telfa and Tegaderm were applied to manage negligible ooze. At the end of August the remaining abdominal sutures were removed and dressing stopped (see Figure 8). At this time Sally was transferred from our area to a pediatric medical ward for further nutritional management and rehabilitation.

[Figures 7-8 ILLUSTRATION OMITTED]

After 3 months on the medical ward, Sally was able to go home. She continues to have daily physiotherapy, is able to transfer and mobilize in a wheelchair, and can tolerate a full diet. Due to her depressed immune status, Sally's mother has elected to home school Sally and her sister.

Pain Management

In order to help Sally through the lengthy dressing procedures, a program of activities was coordinated by the ward play specialist. Frequently, Sally preferred to watch videos. In addition to supporting these distraction and coping efforts, the pediatric pain team monitored Sally's pain levels on a daily basis. Her pain was managed with a combination of a PCA infusion and oral morphine. The pediatric psychiatric liaison team also utilized cognitive coping strategies to assist with the management of Sally's pain and stress related to her condition and prolonged hospitalization.

Family Involvement

Family centered care in pediatrics is based on the development of a partnership between health professionals and the child and their family (Smith, 1995). Throughout Sally's hospitalization, nursing staff worked toward upholding the partnership. While in our ward, we acknowledged her mother's expertise as a parent and attempted to balance this with our clinical expertise as nurses to work toward a partnership in care focused on Sally's best interests.

At the beginning of each shift, the nurse assigned to care for Sally would negotiate a schedule for Sally's care with Sally and her mother and establish the care procedures with which Sally's mother would like to be involved. When Sally's condition was most critical, it was difficult to find ways that Sally's mother could assist directly in nursing care. However, during the time Sally was having large doses of oral morphine, it was agreed that a security safe containing the morphine tablets be placed in Sally's room to enable her mother to dispense the analgesia as required. This enabled Sally's mother to monitor and respond to Sally's analgesia requirements and in doing so, regain some independence from nursing staff.

Sally's mother was initially unable to look at Sally's wound. After a period of 3 weeks, as she became more comfortable with the sight and smell of the wound, Sally's mother assisted the nursing staff by helping to remove the old dressing and by mopping up saline as we irrigated the large cavity wound.

A white board in Sally's room kept note of the shift schedule. A team of primary nurses was assigned to care for Sally, thus ensuring that she was cared for by a consistent group of nursing staff familiar with her needs. Weekly meetings were held with the multidisciplinary team and Sally's mother to discuss Sally's progress and the management of her condition.

A volunteer ward grandparent was assigned to work with Sally to ensure that her mother would have the opportunity to leave the ward if she chose to. The grandparent visited for 2 hours Sally. The hospital school teachers gave Sally school work as her condition would allow, and Sally's sister attended the hospital school while staying at Ronald McDonald House. Sally's mother displayed unfaltering commitment and gave continual emotional support to Sally throughout her stay in the hospital. This was made possible by the immense support she received from her extended family and aided by the partnership in care model practiced in our hospital.

Summary

Sally had multiple bowel perforations and survived despite the odds. Her care needs were complex and challenged both medical and nursing staff. Sally's prolonged hospitalization created stresses for Sally and her family. Chronic and acute pain management greatly influenced Sally's response and, as a result, her mother's response to nursing care. The concurrent marital problems placed an additional burden on Sally's mother that affected the hospital stay.

In addition to placing stress on Sally and her mother, Sally's complex care needs were also stressful for the nursing staff. Sally's care was excessively time consuming, requiring her to have her own nurse for a large part of her stay. Dealing with the stresses faced by Sally and her family, as well as the complexity of providing wound care for this child, posed challenges to the staff. However, the staff rose to the challenge. Nursing staff gained experience in working with a family during a lengthy, difficult hospital stay, thus gaining insight into the management of chronic medical and surgical patients. Nursing staff also increased their knowledge of wound management principles and, as a result of caring for Sally, learned to become flexible and innovative, extending our knowledge and experience to discover new and individualized ways of managing a complex wound.

Acknowledgment: The author wishes to thank Mr. Vipul Upadhyay, Dr. Liz Wilson, and Jenny Hayward, for reviewing this article.

References

Bale, S., & Jones, V. (1997). Wound care nursing. A patient centered approach. Cardiff, Wales: Baillere Tindall.

Garvin, G. (1990). Wound healing in pediatrics. Nursing Clinics of North America, 25(1), 181-191.

Halbert, A.R. (1996). Juvenile dermatomyositis. Australasian Journal of Dermatology, 37, 106-108.

Hartley, B., & Campion-Fuller, C. (1994). Juvenile dermatomyositis: A Roy nursing perspective. Journal of Pediatric Nursing, 9(3), 175-182.

Hotter, A.N. (1990). Wound healing and immunocompromise. Nursing Clinics of North America, 25(1), 193-203.

Malleson, P. (1990). Controversies in juvenile dermatomyositis (Editorial). Journal of Rheumataology, 17, 731-732.

Pachman, L.M. (1995). Juvenile dermatomyositis. Pathophysiology and disease expression. Pediatric Clinics of North America, 42(5), 1071-1093.

Smith, F. (1995). Children's nursing in practice: The Nottingham Model. London: Blackwell Science.

Bridget Kool, BHScN, RGON, was Clinical Nurse Educator, Starship Children's Hospital, Auckland, New Zealand, at the time of this writing. She is currently a Masters of Public Health student.
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Author:Kool, Bridget
Publication:Pediatric Nursing
Article Type:Statistical Data Included
Geographic Code:1USA
Date:Jan 1, 2000
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