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Complications From Gastroschisis- The Untold Story

November 25, 2004

Jaydin was a 7-month-old female who was transferred to our facility from an outside hospital by ambulance after her father brought her into the emergency department (ED) for fever of 105, irritability, and epistaxis. At the outlying hospital blood cultures and a complete blood count (CBC) were obtained. She was ashen and having some difficulty breathing and the decision was made to admit her to the pediatrie intensive care unit (PlCCJ) for observation. Her initial labs were significant with a platelet count of 5,000; WBC 5.5; HgB 7.4; HCT 22.3; 30 segmerited neutrophils; 9 bands; 52 lymphocytes; and PT 14.3 with INR 1.6. Her D-dimer was 0.5 but less than 1; PTT 37; fibrinogen 293. Her LFTs were all elevated.

Significant Past History

Jaydin was born with gastroschisis. This was complicated by necrosis of the bowel and left her with short gut syndrome. She has spent most of her short life in the hospital, starting in the neonatal intensive care unit (NICU), and has only been home twice for a total of 1 month. Since birth she had been maintained on total parenteral nutrition (TPN) with minimal gastrostomy tube (QT) feeds. Her course had been complicated by cirrhosis of the liver, and she was currently listed for a liver-small bowel transplant.

Assessment Findings

General appearance: A well-developed baby, crying and alert, but demonstrating guarding with touch to her left side. Conjunctiva icteric bilaterally, bloody drainage from nares, moist, pink mucous membranes.

Vital signs: HR-144; Temp-100.1 ax; RR-56; BP-70/40; SpO; 100% on RA.

Neuromuscular: Normocephalic, anterior fontanelle soft. Neck is supple. Moves all extremities equally. Alert and appropriate for age.

Respiratory: Lungs clear to auscultation bilaterally, minimal grunting.

Cardiovascular: Normal sinus rhythm, 2+ pulses, and brisk capillary refill. Broviac catheter intact in left upper chest.

Gastrointestinal: Abdomen firm, slightly distended, GT intact with small amount serosanguinous drainage, + bowel sounds, guarding with touch to left abdomen, liver about 1 cm below costal margin, no splenomegaly.

Genitourinary: Dark yellow urine. No other abnormalities noted.

Integument: Skin was jaundiced and buttocks excoriated. Broviac dressing with green, moist drainage.

Initial Management Plan

The initial focus for this patient was to treat her signs of shock and find a cause for her febrile illness. Her differential diagnoses were GT infection, line infection, advanced liver disease, and cholesthasis. A repeat CBC was ordered to confirm an initial platelet count of 5000. A comprehensive chemistry panel, D-dimer, and fibrinogen were also ordered. Cultures were redrawn from her Broviac and peripherally. The Broviac site was cultured since there was drainage noted at the site. Boluses of normal saline were pushed at 10 cc/kg until her blood pressure responded by rising to 116/65. Then D5/NS + 20 meq KCI at 30 cc/hr was initiated until her home TPN arrived. She was also started on Vancomycin (147 mg IV q8 hours, 60 mg/kg/day) and Ceftazadime (350 mg IV q8 hours, 150 mg/kg/day). An infectious disease consult was obtained and the initial CXR was reread.

Her father had arrived by now, but without her current TPN so a finger stick blood glucose level was obtained and found to be 85. Jaydin was TPN dependent and did not tolerate being off TPN for long periods of time. Her blood glucose would need to be closely monitored. What do you think is going on?

Continuing Management Plan

Jaydin’s blood cultures came back positive for gram-positive cocci resembling staphylococcus. This was her second line sepsis in a month. Her last infection had grown Klebsiella, enterococcus, and Candida. That line was pulled and a new one placed. Vancomycin was not the most effective antibiotic for her infection, so her antibiotics were changed to Linezolid 30mg/kg/day divided q8 hours IV. She continued to spike fevers and was placed on a cooling blanket. Surprisingly, her cultures from the exudate from the Broviac site were repeatedly negative. She was also transfused with platelets and PRBCs .

Once Jaydin was stabilized with fluid, transfusions, and initial antibiotics, she was moved to the general pdiatrie inpatient unit. She had been a frequent inpatient and was well known to the staff there. She continued to be followed by both gastroenterology and infectious dis ease specialists. She also was supported nutritionally through TPN and was kept NPO. Platelet transfusions were required every other day and were supplemented by intermittent PRBC infusions. We also evaluated her home nursing to make sure they were using identical techniques for dressing changes and preparing the TPN.

Pathophysiology

Short gut syndrome is a possible complication of surgical intervention for necrotizing enterocolitis (NEC) if extensive sections of dead bowel must be removed. In a small proportion of patients, chronic TPN dependence will develop. For those patients, life-threatening complications such as central line infections and liver chirrosis may develop (Vennarecci et al., 2000). Central venous catheters are a growing concern since they allow for organisms to bypass the body’s natural line of defense. Microorganisms that are responsible for central line infections come primarily from the hub, the patient’s skin, or contaminated infusate (Hadaway, 2003). Currently, in the literature, the incidence of Broviac or Hickman catheter infections is 0.5-6.8/1000 catheter days (Flynn & Barrett, 2004).

Sepsis is defined as a systemic response to infection (Chettle, 2003). Some findings suggest that sepsis is a documented infection with at least two of the systemic inflammatory response syndrome (SIRS) criteria (Chettle, 2003). The SIRS criteria include a documented temperature of > 38.0C or

Although the first sign of sepsis frequently is a temperature > 38.0C, some other findings may be thrombocytopenia, prolonged prothrombin and partial thromboplastin times, elevated D-dimer levels, and decreased fibrinogen levels. Signs of erythema or drainage from the insertion site may also be observed. If not treated early, patients can progress quickly from sepsis to septic shock (hypotension) to multiple system organ failure. Most line infections can be treated with antibiotics; however, if there have been repeated infections, the line may need to be pulled. Once the patient has been documented as being infection free, a new line can be placed.

Family Matters

Paramount to any sick child is the ability of the family to support the many complex needs that may arise in order to maintain that child’s health and well being. This was particularly difficult in this case, as Jaydin’s mother was 17 years old, living with a guardian, going to school, and trying to care for her baby. The baby’s father was not very involved and had limited abilities. Foster care for medical necessity was considered, but the family was unwilling to give up their ability to make decisions for Jaydin. A plan had finally been arranged for Jaydin to live in the paternal aunt’s home. Further support would be provided by home nursing 16 hours a day. The mother and father were responsible for her care for 8 hours in the evening. All were instructed on Jaydin’s 24-hour care requirements prior to leaving the hospital. What wasn’t evident to the staff at this time was the turmoil between the mother and the aunt. This was, at best, a difficult family situation for a very sick child with multiple medical needs.

The Rest of the Story

Recently, Jaydin’s cultures have come back negative, and she is once again at the top of the list for transplant. The transplant facility advised Jaydin’s QI specialists to keep her hospitalized until the transplant. Jaydin’s mother has confided to the social worker that there are tensions in the aunt’s home. She has since received some counseling and support to move toward caring for Jaydin on her own after transplant. She has matured a lot since we first met her and has proven she has the desire and will to overcome adversity to care for her child. This would be a challenging task for anyone to take on, especially a 17year-old with virtually no support system in place.

The Lesson Learned

With the advancement of medical technology there are more children living at home who are dependent on high-risk therapies to maintain their health. Home TPNto maintain adequate nutrition is one of these highrisk therapies. Central line infection is a great concern for children receiving TPN. Monitoring to prevent infection and to recognize the early symptoms of infection is crucial for these children. To prevent infection, nurses must assure that families understand the importance of maintaining sterility of the central line and using aseptic technique when preparing TPN. Even if this is done, patients awaiting liverbowel transplants tend to have at least one central line infection while awaiting transplant (Vennarecci et al., 2000). Therefore, it is imperative that families recognize early signs of infection and seek treatment immediately.

Successful management of home TPN is a challenge under ideal conditions. In a complicated patient like Jaydin, nurses must assure that all caregivers are properly educated about safe home administration of TPN and work with the health care team to obtain needed psychosocial supports for family.

This is a Critical Care Critical Thinking Problem (CCCTP) designed to test your problem solving and analysis abilities.

Instructions: Read the CCCTP below. Then outline how you would assess and expect this patient to be managed. Finally compare your rationale and decision to that provided in the shaded area.

We invite your contributions to this section of the journal. An honorarium of $50 will be paid to the author of a published CCCTP.

Please submit material to: Beth Suddaby; Inova Fairfax Hospital for Children; 3300 Gallows Rd.; Falls Church, VA 22042 or e-mail at elizabeth.suddaby@inova.com for author guidelines.

References

Chettle, C.C. (2003, February 10). The body’s overreaction to infection can prove deadly. Nurse Week. Retrieved from www.Nurseweek.com/ce/ce115a_p rint.html

Flynn, P.M. & Barrett, F.F. (2004). Infection associated with medical devices. In R.E. Behrman, R.M. Kleigman, & H.B. Jenson (Eds.), Nelson textbook of pediatrics, T7th edition (p. 859). Philadelphia: W.B. Saunders Company.

Hadaway, L. (2003) Skin flora and infection. Journal of Infusion Nursing, 26(1), 44-48.

Ross, V.M., (2003). Uncertainty about the clinical detection of sepsis. Journal of Infusion Nursing, 26C]), 23-27.

Vennarecci, G., Kato, T., Misiakos, E.P., Neto, A.B., Verazo, R., Pinna, A. (2000). Intestinal transplantation for short gut syndrome attributable to necrotizing enterocolitis. Pediatrics, 705(2). Retrieved from www.pediatrics.org/ cgi/content/full/105/2/e25

Contributed by:

Kim Josephson, MSN, CPNP

Clinical Nurse Specialist

Pediatric Medical/Surgical unit

Inova Fairfax Hospital for Children

Falls Church, VA.

Copyright Anthony J. Jannetti, Inc. Sep/Oct 2004




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