3002 Seminar. Problem-Based Learning: Evaluating and Managing the Patient with Recurrent Infections DO NOTE TURN THE PAGES UNTIL INSTRUCTED TO DO SO!

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1 3002 Seminar Problem-Based Learning: Evaluating and Managing the Patient with Recurrent Infections DO NOTE TURN THE PAGES UNTIL INSTRUCTED TO DO SO! Discussion leaders: Kenneth Paris, MD Richard L. Wasserman, MD, PhD History NP is a seven year-old boy whose mother made an appointment with you because of her concern that her child was getting sick too often. Her pediatrician has reassured her that there are no significant problems and that he is just getting normal kid stuff. She explains that he gets antibiotics sixeight times a year, usually for purulent rhinorrhea and had an episode of x- ray documented lobar pneumonia several months ago. The episodes of infection are particularly troubling because they trigger asthma exacerbations and result in many days of school absence. 1

2 Review of Systems/Past Medical History: HEENT: Frequent otitis media beginning at four months-of-age lead to the placement of myringotomy tubes at 12 months and repeat tube placement with adenoidectomy at 18 months-of-age. There was frequent otorrhea after tube placement. Myringotomy tubes have been out for four years and otitis has occurred only once or twice a year for the past three years. At about 18 months-of-age, purulent rhinorrhea became a recurrent problem. The pediatrician typically treats after about seven days of rhinitic symptoms, uses amoxicillin and treats for 10 days. Nasal stuffiness and rhinorrhea often do not clear on antibiotics but the nasal discharge changes from purulent appearing to clear. Commonly, purulent discharge reappears three to five days after discontinuing antibiotics. He uses cetirizine on an as needed basis. Respiratory: Reactive airway disease was diagnosed prior to one year-ofage. At this time, any amount of exercise triggers cough. There are symptoms of cough or wheeze more than three days a week. Night awakening only occurs with infection triggered exacerbations. He takes monteleukast daily. Exacerbations are treated with albuterol aerosols and he gets a steroid burst four times a year. Hospitalizations: None. Intravenous antibiotics: None Environmental History: Lives in a single family home with his parents, three siblings and two dogs. He shares a room with a nine year-old brother. The bedroom is carpeted and the ceiling fan operates every night. The house has not suffered water damage. Social History: Noncontributory Family History: His mother has allergic rhinitis and had asthma as a child. Neither parent, nor the siblings, nor aunts or uncles nor first cousins have any history of infection problems. 2

3 Previous testing: Allergy testing: The pediatrician did a panel of in vitro allergy tests at age three. There were significant reactions to dog and dust mites. Spirometry: Not done. Immunologic testing: Not done. Physical Examination: Except for pink, moderately swollen nasal turbinates the physical examination is normal. Height and weight are at the 75 th percentiles. 3

4 PLEASE LIST YOUR RECOMMENDATIONS Diagnostic studies: Initial management changes:

5 Results Allergy testing: Skin Prick: Histamine Control 15 X 15 mm Wheal 0 Wheal Dog 10X10 Wheal Cat No reaction DM Mix 10 X 10 mm Wheal Alternaria No reaction Bermuda Grass 10X10 mm Wheal Oak Tree No reaction Spirometry: FVC 94% Predicted FEV1 88% Predicted FEV1/FVC 94% (No Bronchodilator Given) 5

6 Immunologic testing: Test Range Units Result IGG MG/DL 410 L IGA MG/DL 44.1 IGM MG/DL 71.6 IGE IU/ML 11.6 IGG Subclasses Range Units Result IGG MG/DL 260 L IGG MG/DL 78 L IGG MG/DL 21 IGG MG/DL 17 Initial Specific Antibody Titers: Description Range Units Result SEROTYPE 1 >1.29 MCG/ML <.3 L SEROTYPE 3 >1.29 MCG/ML <.3 L SEROTYPE 4 >1.29 MCG/ML <.3 L SEROTYPE 6B >1.29 MCG/ML 1.0 L SEROTYPE 7F >1.29 MCG/ML <.3 L SEROTYPE 9V >1.29 MCG/ML.6 L SEROTYPE 11A >1.29 MCG/ML.20 L SEROTYPE 12F >1.29 MCG/ML.8 L SEROTYPE 14 >1.29 MCG/ML.4 L SEROTYPE 15B >1.29 MCG/ML 1.3 SEROTYPE 18 >1.29 MCG/ML.73 L SEROTYPE 19F >1.29 MCG/ML <.3 L SEROTYPE 23F >1.29 MCG/ML.9 L SEROTYPE 33F >1.29 MCG/ML 2.1 Acceptable titer to 1/14 serotypes tested 6

7 Specific Antibody Titers Post-Pneumovax Description Range Units Result SEROTYPE 1 >1.29 MCG/ML.50 L SEROTYPE 3 >1.29 MCG/ML 1.87 SEROTYPE 4 >1.29 MCG/ML 1.14 L SEROTYPE 6B >1.29 MCG/ML 1.22 L SEROTYPE 7F >1.29 MCG/ML.46 L SEROTYPE 9V >1.29 MCG/ML.39 L SEROTYPE 11A >1.29 MCG/ML.20 L SEROTYPE 12F >1.29 MCG/ML 3.15 SEROTYPE 14 >1.29 MCG/ML 2.06 SEROTYPE 15B >1.29 MCG/ML 1.89 SEROTYPE 18 >1.29 MCG/ML.73 L SEROTYPE 19F >1.29 MCG/ML 1.05 L SEROTYPE 23F >1.29 MCG/ML 1.10 L SEROTYPE 33F >1.29 MCG/ML 3.00 Acceptable titer to 5/14 serotypes. Four fold rise in titer to 1/14 serotypes. Comprehensive metabolic panel normal. CRP MG/DL <0.3 C MG/DL C MG/DL

8 SIX MONTHS AFTER THE INITIAL VISIT Interval History Asthma and rhinitis management has been optimized. There are no asthma impairment symptoms and there are intervals when there are no rhinitic symptoms. The patient contacts you for each illness. There have been four courses of antibiotics to treat purulent rhinorrhea. In each instance, symptoms clear within five days of starting treatment. The patient has remained well for at least two weeks after each antibiotic course. What are the best next steps?

9 GAMMA GLOBULIN THERAPY Intravenous One infusion every 3-4 weeks Requires medical staff for IV Schedule with infusion center or nurse Systemic side effects are relatively common (up to 25% of infusions) Infusion site reactions are rare Widely differing peak and trough levels Common starting dose mg/kg/mo Subcutaneous Several infusions weekly Self infused Schedule independently at the family s convenience Systemic reactions are typically less frequent and less severe than with IGIV Infusion site reactions are common but usually well tolerated Minimal week to week variation in IgG levels Recommended starting dose mg/kg/mo up to mg/kg/mo 9

10 Objectives: 1. Recognize typical symptoms and infections associated with antibody deficiency symptoms. 2. Become familiar with laboratory evaluation for patients with recurrent sinopulmonary infections. 3. Become familiar with the interpretation of specific antibody titers and the use of immunization in the diagnosis of immunodeficiency syndromes. 4. Become familiar with treatment options (antibiotics, immunization, IgG replacement) for patients with antibody deficiency syndromes. 5. Understand differences in IgG replacement methods and the selection of an infusion method for patients with antibody deficiency. 10