Sickle Cell Information Center Protocols

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

Infections of the throat are no more common in sickle cell anemia patients, but prompt treatment may prevent complications such as sepsis, meningitis, pain crisis or aplastic crisis. Since patients have a predisposition to these complications, their occurrence must always be considered especially when evaluating children with upper respiratory symptoms. The incidence of adenotonsillar hypertrophy (ATH) in SCD appears increased and not related to infectious diseases. We suggest that ATH represents a part of the natural course of compensatory lymphoid tissue enlargement in children with SCD. This causes obstructive sleep apnea syndrome with symptoms of snoring. Adenotonsillectomy can correct the of symptoms and improve alveolar hypoventilation. Overnight polysomnography is diagnostic.

Present Illness. Document onset of sore throat, ability to talk, pain location and radiation. Note any associated symptoms such as fever, chills, rash, nausea, vomiting, abdominal pain headache, earache, coryza, cough, sputum production, myalgias, and fatigue. Seek symptoms of airway compromise including stridor, drooling, restlessness, inability to lay on back, or air hunger. Document the ability to swallow liquids and keep fluids down. Inquire about similar illness in family members and friends. A history of snoring may be caused by adenotonsillar hypertrophy.

Past Medical History. Record past history of rheumatic fever, nephritis, general allergies, drug allergies, and present medications.

- Vital signs. Temperature, pulse, blood pressure, respiratory rate.

- General. Respiratory distress, stridor, drooling, "hot potato voice".

- Head. Occipital, pre and post auricular lymph nodes.

- Ears. Otitis media, bullous myringitis.

- Eyes. Lid edema, conjunctivitis, papilledema.

- Nose. Inflammation, mucopurulent discharge, polyps.

- Mouth and Throat. Pharyngeal inflammation and exudate; petechiae on soft palate, enlargement of tonsils, exudate, asymmetry; uvulitis or deviation; oral ulcerations, gum swelling, tooth tenderness.

- Neck. SUPPLE! Presence of anterior or posterior cervical adenopathy, tenderness. Size and tenderness of thyroid gland.

- Chest. Retraction, use of accessory muscles, clear to ascultation and percussion.

- Heart. New heart murmur or change in heart murmur.

- Abdominal. Tenderness, increase in splenomegaly/hepatomegaly.

- Skin. Rash

- Minimum Evaluation. CBC with differential and reticulocyte count, throat culture, and urinalysis.

- Additional Laboratory. Blood cultures for high fever, rigor or toxic appearance. Mono spot for posterior cervical adenopathy (consider CMV, toxoplasmosis titers, RPR, and HIV test if negative). Consider lateral neck x-ray for unilateral swelling and chest x-ray for cough or respiratory distress. For tonsilar hypertrophy and obstructive sleep apnea, order sleep studies.

- Bacterial Pharyngitis. Streptococcus (Group B and G), N. gonorrhoeae, H. Influenza, Mycoplasma and rarely diphtheria can cause pharyngitis. Tonsillar exudate, cervical adenitis, soft palate petechiae, strawberry tongue, sandpaper rash (scarlet fever) support S. pyrogenes infection. Epiglottis is seen with H. influenza infection. Bullous myringitis occurs in mycoplasma infection. Vincent’s angina has superficial ulcers, foul odor, grey exudate, submaxillary gland enlargement. Consider syphilis with painless ulcerations and gonorrhea in any sexually active patient.

- Viral Pharyngitis. Posterior cervical adenopathy, petechiae on soft palate, splenomegaly, and atypical lymphocytosis favor infectious mononucleosis or CMV infections. Acute HIV infection causes a mono-like syndrome. Many other viral infections, including influenza, adenovirus, and enterovirus, can cause pharyngitis.

- Fungal Pharyngitis – Candida or thrush presents with inflammatory ulcers, often covered by cotton like plaques that yield characteristic yeast on Gram’s stain. It is often associated with immune deficiency states.

- Peritonsillar Abscess. Unilateral peritonsillar swelling with exudate and deviation of the uvula occurring in association with high fever, leukocytosis with left shift suggests peritonsillar abscess.

- Retropharyngeal Abscess. Unilateral swelling in posterior pharynx, fever, leukocytosis with left shift, difficulty swallowing, stridor, neck pain suggests retropharyngeal abscess.

- Tonsilar hypertrophy and obstructive sleep apnea. – Chronic tonsilar hypertropy and abnormal sleep studies.

Criteria for Admission.

Patients with sickle syndromes and infectious pharyngitis should be admitted for concurrent pain crisis, stridor, epiglottis, inability to maintain oral hydration, or fever over 38.5oC. Patients with peritonsillar or retropharyngeal abscesses need admission. Patients who just have peritonsillar cellulitis can be treated as outpatients unless pain crisi is present , they progress to abscess formation, develop neck swelling, appear toxic, or fail outpatient management.

- Infectious Pharyngitis. Sickle cell anemia patients are high risk for severe illness with streptococcus infection justifying empiric treatment with antibiotics while culture results are pending. Oral penicillin treatment is: adults, penicillin VK 250 mg P.O. q 6 hours; children, 50 mg/kg/day in four divided doses every 6 hours. Parenteral benzathine penicillin can be given as one dose I.M.; 1.2 million units adults and children over 40 kg, 900,000 units children 27 - 39 kg, 600,000 units children 14 - 26 kg, 300,000 units children 3 - 13 kg. Alternatively, use erythromycin. adults, 250 mg P.O. q 6 hours; children, 20 - 40 mg/kg/day in four divided doses q 6 hours for 10 days with penicillin allergy or to also cover mycoplasma and chlamydia. All regiments will prevent rheumatic fever. Force fluids, rest, acetaminophen for pain in children and acetaminophen or aspirin in adults.

- Fungal Pharyngitis. Treatment is with clotrimazole troches, fluconazol or nystatin.

- Peritonsillar Abscess. Obtain ENT consult to consider aspiration. Incision and drainage is usually indicated. Initial therapy for cellulitis is procaine penicillin by I.M. injection followed by high dose oral penicillin q 6 hours for ten days. Outpatient observation at 24 hour intervals is mandatory to detect abscess formation or extension. Patients who appear toxic, develop extension, or fail to respond to 48 hours of antibiotics should be admitted for intravenous penicillin or clindamycin.

- Retropharyngeal Abscess. This is a surgical emergency requiring immediate admission and ENT consultation. Surgical drainage is indicated. Initial empiric therapy with a semi-synthetic penicillins are given I.V. until smear and culture results exclude staphylococcus aureus infection.

- Tonsilar hypertrophy and obstructive sleep apnea. Refer the patient to an ENT specialist for surgical evaluation.

Nursing Considerations

Monitor parameters such as sore throat which may cause difficulty swallowing (drooling); mild fever, headache, malaise, and joint pain. Patients with mononucleosis may have enlargement of the spleen in addition to local symptoms such as pain related to the infectious process in the throat.

Assess level of pain experienced.

 Administer antieffective agents and analgesics as ordered.

 Assess and document effectiveness.

 Provide warm saline throat irrigation/lozenges if ordered.

 Encourage rest.

Monitor fluid volume deficit related to pain/inability to drink fluids normally.

 Encourage fluid intake.

Observe for signs of dehydration (dry skin, cracked lips and decreased urine output).

 Assist with frequent oral hygiene, patient may be mouth breathing, which adds to discomfort.

Prompt treatment will prevent complications of sickle cell disease such as pain episodes. Proper treatment of streptococcal disease is important to prevent rheumatic fever.

The most important information to impart in the parents and patients is to seek early evaluation and treatment if a URI or sore throat develops to prevent complications from sickle cell disease and late sequela from the potential streptococcal infection. It is also critical for patients and parents to be informed of the importance of completing the course of antibiotics in established streptococcal infections to prevent rheumatic fever.

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