Sickle Cell Information Center Guidelines

Edited by James Eckman, M.D. and Allan Platt, PA-C

Diarrheal illnesses in patients with sickle syndromes require special attention in terms of diagnostic and therapeutic considerations. Dehydration that can occur, especially in children can precipitate pain episodes and other complications. Salmonella infections must be excluded because of the tendency of these to disseminate causing sepsis, septic arthritis, or osteomyelitis.

Subjective Data

Present Illness. Note onset of diarrhea, recurrent, stools per day, formed, watery, blood, cramping, mucous, nausea, vomiting, fever/chills, appetite, weight loss. Determine exposure to symptomatic contacts, foods including undercooked chicken or hamburger, Fish and shellfish poisoning are due to toxins present in the tissues of fish, other toxins, foreign travel, laxatives, antibiotics (Antibiotic use currently or in the preceding 4-6 weeks can be associated with Clostridium difficile), and homosexual activities.

Past Medical History. Hospitalizations and surgery (bowel surgery), diabetes, pancreatitis, and alcohol use. Present medications and drug allergies.

- General. Weight loss, distress, mental status, skin turgor, rashes, dry mucosa. Assess severity of illness and dehydration. Altered mental status and dehydration with orthostatic changes are seen in severe illness. Irritability, lethargy, and moderate dehydration are seen with moderately severe illness.

- Vital Signs. Temperature, Supine and upright blood pressure/pulse.

- Neck. Thyroid size, bruit.

- Abdomen. Inspect for scars, peristaltic waves, masses. Bowel sounds increased, absent, high pitched bruits. Hepato-splenomegaly, masses, tenderness, rebound.

- Rectal. Masses, blood, guaiac.

- Minimal Evaluation. CBC with differential and reticulocyte count. Electrolytes, BUN, and creatinine.

- Additional Tests. Fecal leukocytes are often present in diarrhea caused by Salmonella and Shigella species. or invasive E. coli, whereas they are usually absent in toxicogenic bacterial or viral disease, stool for ova and parasites, stool culture (All patients with fecal neutrophils or unclear etiology), blood cultures, proctoscopic exam, duodenal aspirate, supine and upright abdominal x-rays. Consider contrast studies and endoscopy for chronic or recurrent diarrhea.

Differential Diagnosis

- Viral. Epidemic so family and friends may be affected. Nausea, vomiting, mild cramping, myalgias, fever, and malaise are common. Diarrhea is usually watery with few neutrophils.

- Bacterial Infectious. Crampy abdominal pain, fever, and tenesmus are common. Diarrhea in liquid often bloody and positive for neutrophils. Salmonella, Shigella, Campylobacter, or Yersinia may be cultured.

- Bacterial Toxic. Nausea, vomiting, cramping are common. Diarrhea may be explosive. History positive for travel or foods causing illness in others. Staphylococcus, - Protozoa. Travel, untreated water, weight loss, tenesmus, or bleeding may be present. EIEC = entero- invasive E. coli; EHEC = enterohemorrhagic E. coli; ETEC = enterotoxigenic E. coli; EPEC = enteropathogenic E. coli; E. coli 0157:H7.

Giardia is relatively common and associated with foul liquid diarrhea, gas, abdominal distension and pain. Entamoeba causes bloody mucous diarrhea with abdominal pain and tenesmus.

- Drugs. Laxatives, antacids, antibiotics can cause acute and chronic diarrhea. Pseudo-membranous colitis secondary to Clostridium difficile must be considered.

- Functional/Irritable colon. Occurs with emotional stress, fatigue, nervousness, and during daytime only. May follow gastroenteritis. Diarrhea is watery, frequent, contains mucus and no fat.

- Impaction. Overflow diarrhea from impaction is seen in patients on narcotics. Diagnosis is established by palpation, rectal, and abdominal films.

- Malabsorption. Lactose intolerance with lactase or sucrase deficiency is a common cause of diarrhea in this population. Celiac disease, pancreatic insufficiency, immunodeficiency need to be considered with chronic diarrhea.

- Inflammatory Bowel Disease. Crohn’s disease or Ulcerative colitis presents with crampy diarrhea, weight loss, rectal bleeding, tenesmus, fistulae, extra-abdominal symptoms.

- Endocrine. Diabetes can be complicated by autonomic neuropathy, painless, nocturnal diarrhea. Hyperthyroidism causes with weight loss, heat intolerance, tachycardia, tremor, thyroid enlargement and bruit.

- Tumor. May cause diarrhea by partial obstruction or secretions and present with weight loss, pain, rectal bleeding, masses. Colon cancer, carcinoid, neuroblastoma, pancreatic tumors, and Zollinger-Ellison syndrome may cause chronic diarrhea.

- Immune Deficiency. IgA, combined, and HIV associated immunodeficiency may cause watery diarrhea or steatorrhea. History usually includes recurrent infections, stomatitis and skin rashes.

Treatment

Most patients with diarrhea and pain crisis or dehydration should be admitted for intravenous hydration. Infants and children should receive clear liquids for 12 to 24 hours. Infants will require an oral electrolyte solution and formula can be restarted by giving half strength for 24 hours and then full strength if this is tolerated. Apple sauce (not juice), bananas, rice, carrots, and saline crackers are useful constipating agents. In older children and adults milk products should be avoided. Kaopectate may be of value in older children and adults. Lomotil should be avoided completely and loperamide is usually not indicated.

If intravenous hydration is required, treatment is based on the type and severity of the dehydration. In sickle cell, replacement must consider deficits, urinary output, and insensible losses. For isotonic dehydration (sodium 131 - 149), replace deficits: water = 100 - 150 ml/kg, sodium = 7 - 10 meq/kg, potassium = 7 - 10 meq/kg. For hypertonic dehydration (sodium > 150), replace deficits: water = 120 - 180 ml/kg, sodium = 3 - 5 meq/kg, potassium = 3 - 5 meq/kg. For hypotonic dehydration (sodium < 130), replace deficits: water = 50 - 80 ml/kg, sodium = 10 - 14 meq/kg, potassium = 10 - 14 meq/kg. D5W 1/2 normal saline with potassium should be used for isotonic dehydration after hypotension is corrected with D5W with normal saline. Both hypotonic and hypertonic dehydration should be treated with D5W 2/3 or 3/4 normal saline and potassium. Initial estimates of deficit should be replaced over eight hours and estimates of 24 hour maintenance replaced in the next 16 hours. Management may require measurement of stool and urine volumes and sodium, potassium, chloride concentrations in severe or ongoing diarrhea.

Specific Therapy

- Infectious. Patients with viral diarrhea can be treated with oral hydration, acetaminophen, Kaopectate, and a clear liquid diet. Those with severe dehydration or other infectious types require inpatient evaluation and therapy. Salmonella should always be treated and ampicillin 150 - 200 mg/kg/day in four divided doses, trimethoprim 10 mg/kg - sulfamethoxazole 50 mg/kg/day in two doses, or amoxicillin 50 mg/kg/day in three divided doses should be given for five to ten days. For shigella give trimethoprim 10 mg/kg - sulfamethoxazole 50 mg/kg/day in two doses. Campylobacter is sensitive to erythromycin 30-50 mg/kg/day in four doses for five days. Yersinia enterocolitis is treated with aminoglycosides or tetracyclines.

- Bacterial toxic diarrheas respond to bismuth subsalicylate. Giardia responds to furazolidone or metronidazole . Metronidazole followed by diloxamide is used to treat Entamoeba histolytica.

- Drugs. Diarrhea responds to stopping the drug. Pseudo-membranous colitis is treated with oral vancomycin 40 mg/kg/day in four divided doses if C. difficile is documented.

- Functional/Irritable colon. Treatment is supportive and symptomatic. Patients may benefit from a high fiber diet low in dairy products. Psyllium seed or other bulk laxatives may provide benefit.

- Impaction. Treat with manual extraction, Fleets enemas, stool softeners (colase 100 - 200 mg P.O. T.I.D).

- Malabsorption. Discontinue milk and dairy products, obtain GI consultation, or admit for persistent symptoms.

- Inflammatory Bowel Disease. Obtain consultation from a gastroenterologist to coordinate evaluation and therapy.

- Endocrine. Treat the endocrine disorder with consultation from an endocrinologist. Consider bulk laxatives, antibiotics, and consultation from diabetologist for diabetes.

- Tumor. Surgical removal is usually indicated.

- Immune Deficiency. Treat underlying immunodeficiency and look for specific pathogens in the stool.

Nursing Considerations:

? Dehydration is a potential problem with diarrhea, patients with sickle cell syndromes are at significant risk for complications associated with dehydration

 Signs and symptoms should be reported immediately ( dry lips, dry mucous membrane, or poor skin turgor)

 Management includes aggressive fluid replacement. Encourage increased fluid intake (water, flat ginger-ale or sprite, electrolyte replacement beverages, or other clear liquids as tolerated)

 Instruct patient or caregiver to avoid caffeine, milk and high-fiber foods that irritate the bowel, worsening the situation

 Be aware that diarrhea is one of the side effects of some antibiotics and some pain medications such as NSAIDS, encourage patients to take the medicine with plenty of water or other fluids

 Teach parents proper handwashing and encourage use of disposable baby wipes instead of washcloths to prevent spread of infections or cross contamination

Prevention

Patients with sickle syndromes are at significant risk for complications from diarrhea induced dehydration. Diarrhea must be managed with aggressive fluid replacement to prevent other sequelae .

Salmonella sepsis, osteomyelitis, and septic arthritis occurs is much more common in individuals with sickle cell anemia. Stool cultures are indicated for most patients with significant diarrhea in this population. Appropriate antibiotics should almost always be given when this organism is isolated to reduce the incidence of these complications.

Parent and Patient Education

Parents of infants and young children must be taught the to administer clear liquids, the ABCs (Apple sauce, bananas, and carrots) of diarrhea treatment, and to detect dehydration. Early presentation for medical evaluation is stressed. Inability to take in oral fluids during an episode of diarrhea is a medical urgency requiring immediate consultation with a health care provider. Patients should be careful to avoid under-cooked meats, eggs, shellfish, and fish at risk for toxins.

References

Hand WL and King NL. Serum opsonization in sickle cell anemia. Amer. J. Med. 64:388-395, 1978.

Guerrant RL , Lohr JA, and Williams EK. Acute infectious diarrhea. 1. Epidemiology, etiology, and pathogenesis. Pediatr. Infect. Dis. J. 5:353, 1986.

Williams EK Lohr JA, and Guerrant RL. Acute infectious diarrhea. 1. Diagnosis, treatment, and prevention. Pediatr. Infect. Dis. J. 5:353, 1986.

Schrier RW. Renal and Electrolyte Disorders, 3rd Edition, 1986.

Powell, D.W. Approach to the Patient with Diarrhea. In T. Yamada (ed.), Textbook of Gastroenterology. New York: Lippincott, 1991. P. 732.

Cheney, C.P., and Wong, R.K.H. Acute infectious diarrhea. Med. Clin. North Am. 77:1169, 1993.

Goldberg, M.B., and Rubin, R.H. The spectrum of Salmonella infection. Infect. Dis. Clin. North Am. 2:571, 1988.

Centers for Disease Control. Update: Multistate outbreak of Escherichia coli 0157:H7 infections from hamburgers--Western United States, 1992-1993. M.M.W.R. 42:258-263, 1993. (See reprint of this in J.A.M.A. 269:2194, 1993.)

Tarr, P.I. Escherichia coli 0157:H7: Clinical, diagnostic and epidemiologic aspects of human infection. Clin. Infect. Dis. 20:1, 1995.

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Copyright © 1997 Sickle Cell Information Center
Last modified: October 08, 2000