Sickle Cell Information Center Guidelines

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

Nasal Congestion, and epistaxsis are common complaints in sickle cell patients. Sinusitis should be treated with antibiotics to prevent complications.

Present Illness. Duration, time of day, allergies, seasonal variation, occupation, lacrimation, sneezing, frontal or maxillary pain/pressure, headaches, worse with bending forward, purulent discharge, halitosis, fever, chills, changes with emotion, temperature change, activity, History of bleeding, ecchymosis, petechiae. type of heating, humidification, dental problems, tooth ache, present medications, habits.

Past Medical History. History of nasal trauma, dental work, hypertension, nasal polyps, drug allergies, family history of atopy, asthma, nasal polyps, aspirin sensitivity.

Vital Signs. Temperature, blood pressure, pulse.

Head. Presence of facial swelling, frontal or maxillary tenderness.

Ears. Examine for otitis media or serous otitis.

Eyes. Conjunctivitis, injection, hypertensive vascular changes.

Nose. Examine for foreign bodies, source of bleeding, polyps, ulcerations, color of mucosa, mucus color and consistency, septal deviation.

Mouth. Pharyngitis, enlarged tonsils, posterior nasal bleeding, caries, tooth abscess, gum bleeding.

Neck. Nuchal rigidity, lymphadenopathy.

- Minimum Evaluation. CBC with differential and reticulocyte count.

- Additional Studies. PT, PTT, bleeding time, and platelet count if other signs of bleeding tendency or epistaxis is severe and recurrent. Nasal smear for eosinophils to prove allergic. Sinus films to confirm acute or chronic sinusitis if indicated. CT scan for severe headache, altered mental status, or focal findings. Lumbar puncture for severely ill child or adult with high fever, severe headache, altered mental status, focal neurologic findings and a negative CT.

- Allergic Rhinitis. Atopic history, seasonal, eye itching, sneezing, nasal polyps, eosinophilia, or occupational related symptoms are present.

- Acute Bacterial sinusitis. Fever, periorbital edema, facial tenderness and swelling, purulent discharge, malodorous breath, leukocytosis, positive x-rays support the diagnosis. Ethmoiditis usually does not occur before age six months, maxillary before one year, and frontal before age 10 years because of delay in sinus development. Pain is behind the eyes with ethmoid, in teeth or zygomatic bone with maxillary, and above the eye with frontal involvement.

- Vasomotor Rhinitis. Symptoms similar to allergic but more related to weather changes, physical stimuli, emotion. Patient lacks atopic history or eosinophilia.

- Viral Rhinitis. Common, with other "cold" symptoms.

- Rhinitis Medicamentosa. History of frequent use of nasal sprays, decongestants, and antihistaminesis present.

- Drugs. Nasal congestion seen with oral contraceptives, reserpine, aldomet, beta blockers, aspirin and non-steroidal antiinflammatory drugs if patient is sensitive.

- Spontaneous Epistaxis. Most common, history of dry heat, may be seasonal, irritants may precipitate.

- Traumatic Epistaxis. History of recent trauma, nose picking, or nose blowing is present.

- Tumors. Sinus tumors may present with pain, congestion, or bleeding.

- Hypertension. This is an uncommon cause of epistaxis in this group.

- Structural. Septal deviation or nasal polyps may predispose to nasal congestion or epistaxis.

- Acute Bacterial Sinusitis. Common organisms are S. pneumoniae, H. influenza, Moraxella catarrhalis, beta hemolytic streptococcus, S. aureus, and anaerobes which can cause overwhelming disease in children with sickle syndromes. Central nervous system involvement or sepsis must always be considered.

Children with pain crisis, severe headache, fever > 102^oF, who appear toxic, have orbital cellulitis or facial swelling, or neurologic findings should be admitted for I.V. therapy with cefotaxime plus clindamycin or a semisynthetic penicillin. Chloramphenicol is sometimes used with ampicillin, however, the marrow suppression may pose significant problems because of the hemolysis. Sinus films, CT, and lumbar puncture may be indicated. Surgical drainage needs to be considered if response to antibiotics is not prompt. Antibiotics in children without severe illness include amoxicillin 50 mg/kg/day P.O. q 12 hours up to 40 kg; T 160 mg - S 800 mg (1 Bactrim DS) q 12 hours > 40 kg, erythromycin/sulfisoxasole E 50 mg/kg/day divided in four doses given q 6 hours. If there is no response in 48 hours admit patient for I.V. antibiotics, central nervous system evaluation, and consideration for surgical drainage.

Adults with pain crisis or fever of >38.5^oC admit for hydration, I.V. antibiotics, and observation. If patient not severely ill, treat with P.O. antibiotics such as Bactrim DS 1 tab P.O. q 12 hours, ampicillin or erythromycin 500 mg P.O. q 6 hours for 10 days. Have patient return if not better in 48 hours. Consider sinus films, fungal culture, admission for I.V. antibiotics, and surgical drainage if not improving. Both adults and children are often given oral and topical decongestants, however, their role is not well defined in acute sinusitis from infection.

- Allergic Sinusitis. Decongestants like pseudoephedrine, antihistamines, or combinations in drops, syrups, or pills in doses appropriate for the age and weight of the patient. Warn about sedation, use with caution in hypertensives or diabetics. Topical sprays can be only used for short periods only (3 to 5 days) to prevent rhinitis medicamentosa. Consider using nasal chromolyn or beclomethasone spray if not improved. Advise patient about pets, hypoallergenic pillows, frequent changing of furnace filters, and cleaning. If problems are severe and recurrent, hyposensitization should be considered.

- Viral Sinusitis. Treat with oral or topical decongestant antihistamines and intervene with antibiotics early for purulent discharge or sputum production.

- Rhinitis Medicamentosa. Prolonged use of topical decongestants can cause tachyphylaxis and persistence of symptoms. Taper off nasal spray by diluting 50/50 with H2O Q.O.D. Start oral antihistamine/ decongestant combination and consider adding nasal chromolyn or beclomethasone spray.

- Spontaneous or Traumatic Epistaxis. Nose bleeds can present special problems in patients with sickle syndromes who are severely anemic because blood loss can be significant. Acute hemorrhage should be controlled by having patient recline with pressure and/or cold packs to bridge of nose. If uncontrolled or severe, obtain CBC with platelet count, coagulation studies, and obtain an otolaryngology consult immediately. For recurrent epistaxis, consider allergic rhinitis, bleeding disorders, vascular malformation, and hypertension. Humidify bedroom air, lubricate nasal mucosa with petroleum jelly, and avoid nose picking and aspirin.

- Tumors. For recurrent or persistent unilateral pain or swelling, obtain sinus films and otolaryngology consultation.

- Hypertension. The acute treatment of epistaxis is the same but control of the blood pressure is the must important long term therapy to prevent recurrence and other complications of hypertension.

- Structural. Structural problems in patients with nasal symptoms or epistaxis should prompt referral to otolaryngology for evaluation. If nasal polyps are present, warn patient about possible sensitivity to aspirin and related medications.

The most important preventive consideration when a child or adult with a sickle syndrome presents with upper respiratory infection is to always consider central nervous system involvement or sepsis. Early use of antibiotics for purulent discharge is indicated to prevent move severe infections in this population.

Recurrent rhinitis, frequent "colds", or epistaxis requires complete evaluation. This includes a careful allergy history, environmental evaluation, and exclusion of other medical or physical causes.

Patients and parents need to both be reassured about rhinitis and epistaxis and educated to seek medical care very early for recurrence or increase in symptoms, especially fever, altered mental status, or lethargy. Instruction about the proper use of medications including side effects of antihistamines including drowsiness which may preclude driving or using machines, problems with over use, and the need to limit duration of use. Parents and patients may need to be instructed to be very observant and assist in identifying foods or exposures that cause problem in those with recurrent symptoms.

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