Marcia Alvarez: The Case

HPI:

Marcia Alvarez is a 21-year-old female patient who is seen in follow up by her physician for persistent cough and shortness of breath.

Her last visit was six weeks ago, when she sought care for nasal congestion, running nose, and congestion in the back of her throat and cough. At that time, she had been prescribed fluticasone nasal spray and oral loratadine but she continued to cough. Her cough is always worse at night and sometimes she can’t seem to stop coughing and occasionally wheezes. During one of those episodes she had been at her aunt’s house and had borrowed her albuterol inhaler; she thought that it provided some relief.

ROS:

No fever or chills.  She has had intentional weight loss through Weight Watchers. She does not think her symptoms are any different at work then at home or elsewhere. No rashes or joint pain.

PMHx:

• Obesity, BMI currently 31
• Allergic rhinitis usually seasonal and worst in the spring and fall. 

Medications:

• Fluticasone nasal spray
• Loratadine 10 mg po daily

Allergies:

• NKDA

Fhx:

Her mom has a history of nasal allergies. Her sister has a history of asthma as a child.  Maternal uncle and cousin who both had leukemia. Not much is known about their history because that branch of the family lives in Puerto Rico. 

SHx:

Miss Alvarez moved to Kentucky from Puerto Rico with her family when she was 12 years old. She is currently a student at the University of Louisville on a part-time basis in addition to working full-time. Ms. Alvarez works full-time at a preschool and has a pet cat. She lives in an apartment and does not have concerns about exposure to mold, insect or rodent infestation, does not smoke, and does not hang out with people who do. She does not drink alcohol or use any drugs.

Exam:

HEENT: normal nasal and throat mucosa, no JVD, TM’s clear, eyes clear

Chest: diffuse, high-pitched expiratory wheezes throughout all lung fields, increased expiratory time. No murmurs, S1 S2 normal

Abdomen: No organomegaly or tenderness, normal bowel sounds

MSK: no joint swelling or deformities, normal range of motion

Skin: no rashes

The physician sends Ms. Alvarez over to the diagnostics lab for pulmonary function testing with and without albuterol. Her flow volume loop is shown.

An albuterol inhaler with spacer and beclomethasone inhaler were prescribed, she was instructed on the correct way to take these medications, and follow up was scheduled in two weeks. Miss Alvarez is concerned about being prescribed a daily steroid, saying  "I thought that steroids can make you gain weight, I’m trying to lose weight, some worried about that. Also, don’t steroids cause diabetes?” After further discussion, she is reluctantly willing to try taking the medication everyday to see if it would make her feel better.

Follow-up: At her follow visit, she reports some improvement in her cough but is still coughing nightly and feels like she still wheezes with activity and cannot be as active as she would like to be due to shortness of breath. She has been taking her inhaled steroid daily and using her albuterol at least four times a day as well as waking up once at night to use it. Her exam is unchanged. Her inhaled budesonide is discontinued and she is started on a combination fluticasone propionate and salmeterol inhaler. She is to continue to take the albuterol as needed. She is scheduled for another follow-up visit in two weeks.

Follow-up: At her second follow-up visit, she reports that she still has not improved much since her last visit, and that she is now coughing up sputum each morning. PFTs are repeated and have not really changed, with her FEV1 only 70% predicted for her height. A tiotropium inhaler was added, and she is also started on oral zafirlukast.  She is very frustrated and points out that she is now taking seven medications and not getting any better. Her physician refers her to a pulmonary specialist for further evaluation.

Before she can be seen by the pulmonary specialist, her breathing and wheezing becomes worse and she is admitted to the hospital. She also reports that her sputum production has been worse in the last couple of days. 

At presentation to the hospital her exam shows:

General survey:  She seems anxious and uncomfortable and in moderate respiratory distress.

HEENT:  Nasopharynx is normal and there is no sinus tenderness. She has no cervical lymphadenopathy or jugular venous distention.

Chest:  she continues to show a prolonged expiratory phase with wheezing in both lungs. She does not have any crackles, egophony, tactile fremitus or dulness to percussion and there are no changes to the remainder of her exam compared to her initial exam.

Tests of blood clotting were normal, as were liver enzymes and bilirubin

A chest x-ray shows:

A chest CT shows:

Due to her prolonged course and lack of response to treatment, pulmonary specialists are consulted. They recommend a bronchoscopy to evaluate the cause of her symptoms and the abnormalities seen on her imaging studies. Thick plastic-like wads of solid mucus were impacted in several segmental bronchi. Pathologic images of samples of this solid matter obtained at bronchoscopy are shown.

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A serum IgE was > 10,000 IU/mL, and IgE and IgE associated with a specific microbe was elevated. The patient was placed on a long tapering course of oral steroids and oral itraconazole, continued on inhaled steroids and albuterol with discontinuation of her other medications, and improved gradually over several months. When seen in follow-up clinic by the pulmonologist, PFTs were obtained showing a near-normal FEV1 and FEV1/FVC ratio.