Vol. 5 •Issue 18 • Page 31
Pediatric Nephrotic Syndrome
A case study reveals treatment and follow-up plans for a rare pediatric disorder
By Mary Jem Baylon, BSN, RN, Wendy Butler, RN, Chhaya Patel, RN, JodiKingley, MS, RD, CNSD, Mirta Torres, SW, Elvira E. Castro, MSN, RN, & Deborah A. Jasovsky, PhD, RN
In any urban community hospital, a variety of common pediatric illnesses are treated. However, we were all challenged when 15-year-old Rose* was diagnosed with a rare pediatric disorder, nephrotic syndrome. The incidence among children is between 2 and 7 cases per 100,000/year.1
Boys are affected more often than girls, and the onset is usually in the preschool years. Rarely do pediatricians, or pediatric nurses, encounter this type of patient in their practice.2
What Is Nephrotic Syndrome?
The kidney’s function is to filter large amounts of fluids, remove waste products and exchange electrolytes across the cell membrane daily. Any alteration of the renal function that causes an increased glomerular membrane permeability would allow larger plasma proteins such as albumin to pass through the membrane and cause hypoalbuminemia.3 This occurs because of an increased rate of urinary excretion of albumin and an increased catabolism of the albumin pool. A low level of albumin in the blood and a corresponding high level of protein in the urine are expected with this diagnosis.4
Therefore, nephrotic syndrome is an alteration of renal function due to an increase in the glomerular basement membrane permeability to plasma protein such as albumin. Signs and symptoms include generalized edema (anasarca), weakness, marked or persistent proteinuria and hypo.albuminemia. It is critical to detect and treat this syndrome early as it can lead to thromboembolisms, infections, hyperlipidemia and edema.4
Three types of nephrotic syndrome have been identified: primary minimal change nephrotic syndrome (MCNS), secondary, and congenital (CNS).4
MCNS is the most common type. It accounts for 80 percent of the cases and occurs at any age, and it’s more commonly found in males. The pathogenesis is not yet fully understood; it may be a metabolic, biochemical, or an immune disturbance that causes an increased permeability to proteins.
The secondary type is frequently associated with secondary renal in.volvement or with systemic diseases. A rare autosomal recessive gene localized on the long arm of chromosome 19 is the cause of CNS.
Patient History & Physical
Rose was born full-term in Puerto Rico via a normal vaginal delivery and weighed 6 lbs., 3 oz. Immunizations were up to date, and she had a childhood history of bruising easily. Family history included kidney stones.
Her first admission at age 13 left her symptom-free for 2 more years. Recently, she had two more admissions and three visits to the ED. Her last visit resulted in a 10-day admission for fever, left side flank pain, left side chest pain and intermittent vomiting for 3 days.
Admitting diagnoses included acute pyelonephritis, nephrotic syndrome and streptococcal pneumonia. Rose was awake, alert and oriented on admission with periorbital and pedal edema, abdominal tenderness and distention. Vital signs were 101.9º F, 132, 20, 112/55 with a pain scale of 6 and weight of 135 lbs. (61.4 kg).
Rose had many of the expected clinical manifestations listed in Table 1.5,6
Significant Diagnostic/Lab Tests
Rose had a series of tests to determine her diagnosis. The right abdominal and pelvic ultrasound with contrast revealed mild anasarca, ascites, a trace of a pericardial effusion and abnormal bowel loops. The kidney biopsy showed membranoproliferative glomerulonephritis.
The WBC was 41,000 mm3 on admission; it climbed to 76,000 mm3 before decreasing to 65,000 mm3 after the treatment began. The blood cultures showed streptococcus pneuo.mococcus and a bacteremia with gram-positive dyplococci consistent with pneumonia. The source was most likely to be secondary to a spontaneous peritonitis. She also had oral candidiasis.
Her urinalysis showed a hazy yellow specimen with a specific gravity of >1.03; proteins >300; trace ketones, large blood and a small amount of bilirubin. After 5 days of treatment, the urine was negative for blood and ketones, and the specific gravity was 1.015.
Family and Age-related Issues
As a typical teenager, Rose was concerned about her appearance, which was altered with edema and could impact her self-esteem. Her new low-sodium diet was a challenge with her cultural preference for Hispanic food. Her family kept bringing inappropriate food from home, which necessitated a lot of diet counseling.
Her diet changes would also impact her life.style. Teenagers are usually active with friends after school, but her doctor visits and a decrease in activity level could alter her involvement in after-school activities. For Rose, a teenager, privacy was another important concern, and the disease also impacted her parents’ desire to protect her.
As nurses, we recognized Rose’s need to understand and live with her new disease. We involved her in the plan of care and gave her as many choices as were possible. We stressed the need for compliance to avoid future hospitalizations and the long-term consequences of this disease such as serious infection, blood clots, malnutrition, acute kidney failure, CHF and end-stage renal disease.
Keeping the lines of communication open with the patient, family, physician, dietitian and social worker were challenging as the patient had limited English-speaking skills and her mother had none. Translators were needed for all communication.
Cultural Barriers
The language barrier was overcome through the many Spanish translators available in the pediatric unit. This Spanish family was close-knit, so mom was usually found at the bedside, unless she was working. Rose’s sibling stayed at home, though cousins and friends visited frequently.
Cultural issues needed to be addressed in the pain management plan. In the Hispanic culture, pain is not verbalized and denial of pain is preferred. The nursing staff had to rely on visual cues and timely administration of acetaminophen to help manage Rose’s pain. Comfort was also accomplished with repositioning, visitor limitation, privacy and the use of her own phone and television.
Safety & Consultations
The nurses needed to balance Rose’s need for autonomy and freedom with her need for assistance. Rose complained of dizziness and needed monitoring and assistance when ambulating to the bathroom or on short walks in the hallway.
Contributing to her “fall” risk was the furose.mide (Lasix®) used to treat her edema and hyper.tension. Her blood pressure and other vital signs were carefully monitored. She also wore an allergy ID band for aspirin.
Consultations with infectious disease specialists for fever and the positive blood culture, and a pediatric nephrologist for a kidney biopsy, were included in her treatment plan. Nutrition and social service requirements, including discharge-planning needs, were also included.
Medications
The most important medications for Rose in.luded: prednisone, furosemide, Vasotec® (enalapril maleate), cefipime (Maxipime®), vancomycin (Vancocin®) and Flagyl® (metronidazole).
Prednisone is a corticosteroid given to reduce the excretion of protein in the urine. The nurse emphasized with Rose and her mother the im.portance of keeping future physicians informed that Rose was immunosuppressed as a result of this treatment and that no vaccinations could be given while taking this medicine.
Furosemide is a diuretic given to prevent reabsorption of water, sodium and potassium by the kidney tubules resulting in the excretion of excess fluid. Oral supplements of potassium were given during this time.
Vasotec is an anti-hypertensive given to reduce high blood pressure. The nurses stressed the need for Rose to continue taking this medication as scheduled even when she is feeling better.
Cefipime, vancomycin and Flagyl are antibiotics used to treat and prevent infection. Strict intake and output monitoring was needed, especially since vancomycin is excreted slowly in the presence of an impaired renal system. Toxicity may occur rapidly so blood pressure is monitored during administration and a sudden drop could indicate red man syndrome.
This syndrome is particularly important to watch for, as those who have a change in the immune system in the face of stress can manifest the red man syndrome with a sudden and profound drop in blood pressure, with or without the eruption of maculopapular rash over the face, neck, upper part of the chest and upper extremities.7,8
Goals of Nutrition Therapy
The goal for any compromised pediatric patient is to provide adequate calories and protein to promote growth and development. In Rose’s case, special attention was needed to address the presence of edema and to reduce the risks associated with disease progression. Her nutrition needs included an assessment of height (63 inches) which placed Rose in the 50 percentile; her weight (135 lbs., or 61 kg); and the desirable body weight of 123 lbs., or 56 kg., for the 50th percentile.
Nutritional side effects to consider for Rose include growth retardation based on protein calorie malnutrition and corticosteroid treatment. Catch-up growth of this 15-year-old was not a concern, as 16 years is the average end of skeletal growth for females.
Physiological issues to address include hypoalbuminemia due to increased rate of urinary excretion of albumin and increased catabolism of albumin pool, and hyperlipidemia (elevated VLDL and LDL), where the long-term risk for coronary artery disease is unknown in children. A Step One diet emphasizes a reduction in dietary cholesterol and saturated fats to reduce lipids. The diet is required for this long-term syndrome,9 and a sample menu is available in Table 2.
The diet modifications addressed the following.
• Energy — at least the recommended dietary allowances (RDAs) for desirable body weight.
• Protein — RDA for age with special attention not to replace urinary protein loss and to keep protein within .7 to 1.0 g/kg dry or ideal body weight.
• Sodium — restrict when edema present and limit to 2,000 mg per day.
• Fluid — restrictions based on the desired effects of diuretics.
• Vitamins — insufficient amounts of calcium and B12 in the modified diet necessitate a daily calcium supplement and MVI with minerals.
• Cultural preferences — as many Spanish foods as possible were kept, with restricted portions identified.
Social Concerns
The biggest problem facing this family was the lack of medical and prescription coverage for Rose. Her mother received referrals to NJ Kid Care Medicaid, Supplemental Security Income and the American Kidney Association. Rose was registered with the Special Child Health Services in New Jersey.
During hospitalization, we needed to address past non-compliance with the medical treatment plan. Issues of transportation and medical coverage impacted the family’s ability to comply. As the family lives in a large Hispanic community, language was not a barrier, but it was addressed as it related to accessing services. The family was able to identify someone who could interpret for them over the phone when necessary.
Plan of Care
The plan of care needed to incorporate the key findings from the assessment, which include pain, edema and self-image.4 Table 3 identifies the most critical elements, although we recognized the need to incorporate culture and patient education, too.
The cultural influence and age-appropriate behavior were also incorporated into the care plan. Although the pediatric nurses were experienced in taking care of Spanish-speaking patients, the need for medications and diet restrictions required a lot of time and reinforcement for patient and family education.
Patient Outcomes
During this hospitalization, the desired outcomes for Rose were accomplished by discharge.
• Pain free upon discharge. Physical and verbal expressions of pain were absent.
• Exhibits minimal evidence of fluid accumulation. Edema in all locations was lessened and specific gravity brought within normal range. I&O ratio appropriate and Rose complied with decreased fluid intake and optimal scheduling of sodium.
• Progressive return to activities within disease limitations as disease subsides. Rose and her mother were informed that the teen needs to rest when she is tired and a full return to normal activities could be expected as the treatment plan continues and the disease subsides. Rose acknowledged the need to balance all future activities with rest and the importance of returning to school and resuming social activities within the limitations of her disease.
• Absence of infection. Rose and her mother were instructed to avoid exposure to anyone with infection as a relapse could occur. The nurse stressed the need for handwashing and told them to report any signs and symptoms of infection to the doctor immediately.
• Verbalization of knowledge and demonstration of follow-up care. Both Rose and her mother were able to verbalize signs and symptoms of disease and the importance of immediate medical attention if present. They acknowledged each medicine, its side effects, pledged to take the medicine as prescribed and understood the importance of follow-up care in the clinic and with specialists. They were able to articulate how to monitor the urine for protein and signs of edema by measuring weight or watching for puffiness in the extremities or around the eyes.
To date, Rose has avoided another hospitalization.
References
1. Chesney, R.W. (1999). The idiopathic nephrotic syndrome. Current Opinion in Pediatrics, 11, 158-161.
2. Schwartz, M.W. (Ed.). (1997). The 5-minute pediatric consult. Baltimore: Williams & Wilkins.
3. Wong, D. & Eaton-Hockenberry, M. (2001). Wong’s essential of pediatric nursing (6th ed.). St. Louis: Mosby Inc.
4. Jaffe, M. (1998). Pediatric nursing care plans (2nd ed.). Englewood, CO: Skidmore-Roth Publishing.
5. Belkengren, R. & Sapala, S. (1999). Pediatric management problem of idiopathic nephrotic syndrome. Pediatric Nursing, 25(3), 304-305.
6. Hogg, R., et al. (2000). Recognizing and treating the nephrotic syndrome: Avoid unnecessary delays. Contemporary Pediatrics, 17(11), 84-93.
7. Hui, Y.L., et al. (2002). Red man syndrome following administration of vancomycin in a patient under spinal anesthesia — A case report. ACTA Anaesthesiologica Sinica, 40, 149-151.
8. Wooten, J. (2001). Red man syndrome. RN, 64(11), 45-48.
9. Sedman, A., et al. (1996). Nutritional management of the child with mild to moderate chronic renal failure. Journal of Pediatrics, 129(2), S13-S18.
Resource
NIDDK (2001). Childhood nephrotic syndrome. Retrieved July 9, 2003 from the World Wide Web: http://www.niddk.nih.gov/health/kidney/pubs/cns/cns.htm
*Some details about the patient were changed to protect her identity.
Mary Jem Baylon, Wendy Butler, Chhaya Patel, Elvira E. Castro and Deborah A. Jasovsky are nurses in New Jersey. Jodi Kingley is a dietitian and Mirta Torres is a social worker in New Jersey. Because of HIPAA regulations, the authors did not wish to be identified with their facility.
Table 1: Clinical Manifestations5,6
• Pleural effusion
• Weight gain
• Facial puffiness
• Ascites
• . Labia or scrotal swelling
• Edema
• Hypoalbuminuria
• Lethargy
• Weakness
• Anorexia
• Proteinuria
• Irritability
Table 2: Sample Menu for 15-year-old Female
Breakfast.1/2 cup of cream of wheat 1/2 cup of 1 percent milk 1 cup of orange juice
LUNCH Sandwich: 2 slices of whole wheat bread, 1 slice low sodium turkey breast, and lettuce/tomato 2 tablespoons of mayonnaise 1 fresh apple 1 cup of raw carrots 1 brownie 16 oz fruit drink
AFTERNOON SNACK 4 low sodium Ritz crackers 1 tablespoon regular peanut butter 1 cup of lemonade
DINNER 1 oz* pork chop 1 cup of baked French fries 1/2 cup of steamed broccoli 1 tablespoon of regular margarine 1 cup of grape juice
EVENING SNACK 2 cups of popcorn (oil-popped, no salt) 16 oz regular cola
*1 oz = 1/3 of a deck of cards
Diet analysis: 2,171 calories, 43 g protein, 350 g carbohydrates, 75 g fat. This menu is considered to be a high carbohydrate, low protein and moderate fat diet.
Source: USDA Healthy Eating Index information, http://147.208.9.133/default.asp
