Table of Contents
Patients, Families and Friends
Health Care Providers
|Specific Problems: Cough and Reactive Airway|
|Health Care Providers - Problem Oriented Clinical Guidelines|
Pneumonias, acute chest syndrome caused by sickling in pulmonary arteries, and fat embolization, are acute complications seen with increased frequency in patients with sickle syndromes. They present difficult differential diagnostic problems because manifestations of chest pain, cough, fever, pulmonary infiltrates, and severe hypoxia are common to all. Treatment includes hospitalization with careful monitoring of hemoglobin and blood gasses, oxygen for hypoxia, judicious hydration and pain treatment, antibiotics, and exchange transfusion in episodes with severe hypoxia, rapid progression, or diffuse pulmonary involvement. Older patients may develop chronic restrictive lung disease, pulmonary hypertension and cor pulmonale.
by Lewis Hsu, MD, PhD
Edited by James Eckman, M.D. and Allan Platt, PA-C
Present Illness. Note the onset of cough, frequency, precipitating factors (exercise, reclining, exposures). Presence of sputum production (quantity, color, time of day), hemoptysis, fever, chills, chest pain, weight loss, earache, postnasal drip, coryza, sore throat, myalgias, headache. History of environmental/occupational inhalation, allergies, hayfever, smoking.
Past Medical History. Previous episodes of pneumonia, tuberculosis or exposure to tuberculosis, PPD status when last tested, hospitalizations, surgery, prolonged sitting, Family history of allergies, phlebitis, or pulmonary emboli. Present medications, drug allergies, alcohol, or marijuana use.
Review of Symptoms. Exclude orthopnea, paroxysmal nocturnal dyspnea, edema, dyspnea, on exertion, or change in nocturia.
Objective DataPhysical Examination
- General. Signs of weight loss, temporal wasting, distress, accessory muscle usage.
- Vital Signs. Temperature, respiratory rate, blood pressure, pulse, pulse oximitry.
- HEENT. Bullous myringitis, pharyngitis, nasal discharge.
- Neck. Thyroid, lymphadenopathy.
- Chest. Listen to cough. Percussion and palpation for dullness, increased resonance, fremitus. Auscultation for rales, rubs, rhonchi, wheezing, change in breath sounds, E to A.
- Heart. Neck veins for venous pressure/pulse, point of maximum impulse, thrills, S1, S2 (Increased P2), S3, S4, rubs or murmur.
- Abdominal. Hepatosplenomegaly.
- Extremities. Cyanosis, clubbing, edema, calf swelling, tenderness, cords, erythemia.Laboratory
- Minimal Lab. CBC with differential and reticulocyte count. Chest x-ray for all new coughs.
- Additional Lab. Sputum smear and culture if cough productive or chest x-ray positive. ECG for heart failure or chest pain. Consider PPD, cold agglutinins, V/Q lung scan.
- Pneumonia. Findings of high fever, leukocytosis with left shift, infiltrate on x-ray, purulent sputum favor the diagnosis of pneumonia. Many patients present with infiltrates and pneumonia may be very difficult to differentiate from pulmonary infarction. The former is more common in children the latter in adults.
- Bacterial Bronchitis. Low grade fever, purulent sputum, hemoptysis, negative chest x-ray, smoking history all support this diagnosis.
- Viral Syndrome. Low grade fever, unchanged WBC or elevated WBC with reactive lymphocytosis, negative chest x-ray, upper respiratory prodrome and dry cough or clear sputum suggest a viral illness.
- Tuberculosis. Fever, weight loss, exposure, upper lobe pneumonia, resistance to treatment indicate possible tuberculosis. Presence of new conversion of PPD suggests diagnosis, chest x-ray may show classic miliary pattern in children, positive sputum smear or culture is diagnostic.
- Pulmonary Infarction. Low-grade fever, pleuritic chest pain, hemoptysis, rub, lack of neutrophil bands on WBC differential, and phlebitis favor pulmonary infarction. Ventilation/perfusion lung scan may be helpful if chest x-ray is negative and previous study is available. Sickle cell patients may have abnormalities on lung scan from previous infarctions making interpretation difficult without old comparative studies.
- Asthma/Bronchospasm/ Reactive Airway Disease may present with chronic, seasonal, or exercise induced cough. "Allergic cough" occurs without true asthma. History of asthma, chronic bronchitis, atopy, wheezing, retractions with breathing, prolonged expiratory phase will support asthma as the etiology of the cough. Bacterial infection may precipitate asthma attacks.
- Congestive Heart Failure. Pulmonary congestion from left heart failure may present with non-productive cough or a cough productive of clear, pink or foamy fluid. History of dyspnea, dyspnea with exertion, paroxysmal nocturnal dyspnea, orthopnea, or ankle edema is characteristic. Presence of neck vein distension, bibasilar rales, S3, and increased ankle edema firmly support the diagnosis. Laboratory evaluation may show increased anemia and pre-renal azotemia.
- Mass Lesions. Hilar adenopathy, tumor, abscesses may present with chronic cough. Weight loss is common. Fever, hemoptysis, stridor, or other lymphadenopathy may be present. Chest x-ray showing a mass requires diagnostic evaluation with biopsy to establish the etiology. Extramedullary hematopoiesis causes pulmonary masses occasionally but almost never produce a cough.
Pain episodes associated with almost any of the causes of acute cough requires admission to the hospital. In patients without an acute pain episode, new coughs must have clear etiologies amenable to outpatient therapy if the patient is to be sent home. Frequent follow-up is indicated until the patient improves.
- Pneumonia. All patients with sickle syndrome and pneumonia should be admitted for I.V. antibiotics and monitoring. Blood cultures should always be obtained and sputum smear and culture may guide initial, empiric therapy. Children should be covered for S. pneumoniae and H. influenza. Third generation cephalosporins (ceftaxime or ceftriaxone) are good choices especially in children on penicillin prophylaxis. Ampicillin or ampicillin plus chloramphenicol are alternatives in severely ill children depending on local patterns of H. influenzae resistance. Chloramphenicol is usually avoided because of the suppression of erythropoiesis. In older children and adults, similar antibiotics are good initial therapy. Mycoplasma pneumoniae, Staphylococcus aureus, Klebsiella species, and other gram negative organisms must be considered based on clinical presentation and sputum smears. Cold agglutinin titers of 1 to64, or greater support mycoplasma infection. In our experience, addition of erythromycin often benefits patients who are culture negative and fail to respond to initial empiric therapy.
- Bacterial Bronchitis. General support includes forcing fluids, bed rest, expectorants with cough suppressants, and stopping smoking. Treat all patients with a new productive coughs with antibiotics.
Amoxicillin. Children < 20 Kg, 20-40 mg/kg/day; Children > 20 kg and Adults, 750 - 1500 mg/day P.O. in three divided doses given q 8 hours for 7 days.
Erythromycin. Children, 30 - 50 mg/kg/day; Adults, 2 gm/day P.O. in four divided doses given q 6 hours for 7 days.
Trimethoprin-sulfamethoxazole (Bactrim/Septra). Children, < 40 kg, trimethoprin 4 mg/kg with sulfamethoxazole 20 mg/kg ; Children > 40 kg and Adults, trimethoprin 160 mg with sulfamethoxazole 800 mg (1 DS) –
- Viral Syndrome. Symptomatic treatment, force fluids, bed rest, Lucoqualatic (Rbitussin) with codeine for non-productive cough. Do not suppress a productive cough. Educate to monitor temperature return for pain crisis, fever >100oF, chest pain, dyspnea, or purulent sputum.
- Tuberculosis. If active tuberculosis is suspected, admit the patient for evaluation and treatment. Patient should be in isolation if cough is productive and chest x-ray shows cavitation or upper lobe pneumonia. Treatment is based on extent of the extent of disease, response to therapy, and antibiotic sensitivity of the organism.
- Pulmonary Infarction. If a pulmonary infarction, acute chest syndrome, is suspected (Chest Syndrome), admit for observation and treatment. Consider antibiotics if there is any question of infection.
- Asthma/Bronchospasm. The cough of asthma may respond to bronchodilators or other standard asthmatic treatments. Care must be used because the cardiac stimulation and diuresis caused by these agents can precipitate complications in sickle cell patients. Vigorous hydration is indicated.
- Congestive Heart Failure. Most patients with hemoglobinopathy develop high output heart failure from increasing anemia, however, myocardial infarction and other primary cardiac diseases are possible. Almost all patients will require admission for careful monitoring during slow transfusion with concurrent diuresis. Immediate treatment includes administration of oxygen at 4 L/min and obtaining emergency ECG, CBC, blood gasses, electrolytes, BUN and creatinine. Myocardial infarction should be excluded in older patients.. (see CHF)
- Masses. Masses on chest x-ray, with or without symptoms, require extensive evaluations which are usually facilitated by admission to the hospital. Treatment is based on the outcome of the diagnostic evaluation.
Use of prophylactic penicillin and proper immunization including Prevnar and H. influenzae vaccine is the single most important preventive measure to prevent pneumonias. Proper early treatment of signs of bronchial or lung infection is important in all patients even if they have been vaccinated or are on penicillin because failures occur. Chest syndrome may be prevented by appropriate hydration, use of incentive spirometers, and avoiding severe physical exertion. Sickle cell patients should never smoke and not be exposed to passive smoking.Patients with Reactive Airway Disease should follow the ATS guidelines for reduction of exposure to allergens and irritants.
Tuberculosis is still a common problem in the sickle population. Annual skin testing is recommended. Children and adults who are exposed to tuberculosis or have conversions of the PPD should receive prophylactic treatment based on the guidelines of the American Thoracic Society and the Centers for Disease Control. Pyridoxine should probably always be administered with isoniazid in these patients.
Parent and Patient Education
The most important educational activities relate to prevention by giving penicillin, keeping immunizations current, and presenting for prompt evaluation when a cough occurs. Smoking education is important for parents and patients.
Barrett-Connor E. Acute pulmonary disease and sickle cell anemia Amer. Rev. Respir. Dis. 104:159, 1971.
Barrett-Connor E Pneumonia and pulmonary infarction in sickle cell anemia. JAMA 224:997, 1973.
Charache S et al. "Acute chest syndrome" in adults with sickle cell anemia. microbiology, treatment, and prevention. Arch Intern. Med. 139:67, 1979.
Solanki DL Berdoff RL. Severe mycoplasma pneumonia with pleural effusions in a patient with sickle cell-hemoglobin C (SC) Disease. Amer. J. Med. 66:707, 1979.
Poncz M et al. Acute chest syndrome in sickle cell disease. Etiology and clinical correlates. J. Pediatr. 107:861, 1985.
Starke JR. Modern approach to diagnosis of tuberculosis in children. Pediatr. Clin. North Amer. 35:441, 1988.
|Last Updated on Monday, 14 June 2010 14:17|