Just after eight o'clock one morning in November 1996, I was paged and asked to call Marianna Montero at our emergency room. I had not heard from Marianna since her father, Eduardom had died of lymphoma seven months earlier. After Eduardo's death, Marianna and her mother, Isabella, had left Boston for Hyannis to live with extended family. Isabella, had gotten a job cleaning in a bank at night. Marianna was a sophmore at the local high school.
"ER, John Healy speaking," a young male voice said crisply.
"This is Jerry Groopman, I'm returning a call from Marianna Montero."
"She's in the visitors area, Dr. Groopman. I'll get her."
Some vanilla Muzak played on the line
"Dr. Groopman, thank God, thank God," Marianna exclaimed. "Mama couldn't breath again. I took her twice to the clinic at Cape Cod Care in Hyannis. They said it was just a routine case of asthma. Last night she couldn't see from one eye, and her arm was weak. I had to bring her here. I'm scared she going to die. And I didn't..." Her words dissolved in a sea of sobbing
"Marianna," I said firmly, "whatever it is, we will help. I'll be right over."
I put aside the data I had to review for my morning lab meeting and headed for the ER. It was a chilly autumn days, a north wind swirling fallen brown leaves from the sidewalk. Traffic was already bumper-to-bumper on Brookline Avenue, the main route to the Harvard Medical ahrea. I was relieved I had come in early and not been paged while stuck in my car.
Isabella was sitting bolt upright in bed. Her rich black hair was wet and matted, and droplets of perspiration circled her brow. There was a bluish tinge to her lips, and I noted how, with each breath, the sinewy strap muscles of her neck retracted in forceful upward arcs. Marianna was seated at her side, clutching the metal rail of the hospital bed.
"Hola, Isabella," I said loudly above the din of the beeping cardiac monitor and the gurgling oxygen flowing from a tank next to her bed. I gripped her moist left hand and gave it a squeeze. Isabella answered with a wan smile under the oxygen mask covering her nose and mouth.
Marianna stood up from her chair. She had grown since I last saw her in the spring. Tall and lean, she had her mother's jet black hair and sculpted cheeks. Her dark eyes were wide with fear.
We embraced, and I told her I was glad she had brought Isabella to Boston. In a calm, deliberate tone, I explained I needed to review the ER notes and talk with the medical staff and would then return to discuss what had been found and shall be done.
Isabella's triage form was on a clipboard at the nursing station. I focused on the salient details.
It certainly wasn't a routine case of asthma. First, it was unusual to develop the disease at Isabella's age. Most cases begin in childhood. Moreover, the ventolin inhaler and the aminophylline, standard treatments that relieve it by opening airways, and erythromycin, an antibiotic to treat a complicating infectiong, were ineffective.
Isabella was on the brink of respiratory failure. Her respiratory rate was nearly twice normal. The level of oxygen in her arterial blood, normally around 98, was 53, precariously low. If this was not corrected by the oxygen mask, her organs would not function for long. Equally worrisome was the level of carbon dioxide. Carbon dioxide is the toxic gas generated by our metabolism and exhaled during respiration. Normal arterial carbon dioxide is around 40. The elevated level of 47 signaled a serious problem with her ventilation. Further buildup of carbon dioxide would acidify Isabella's blood, dull her brain, and become potentially fatal.
The chest X ray revealed diffuse intersitial thickening. The interstitium is the weblike lattice of tissue that supports the lung. On one side of this lattice are the air sacs, or alveoli; on the other side are the small blood vessels, or capillaries. Some process had thickened the intersitium throughout Isabella's lungs, thereby blocking her vital exchange of oxygen and carbon dioxide. Asthma per se did not cause these changes in the lung tissue.
I then read the findings from Isabella's physical examination. She had lost vision in her right eye. Her retina, viewed through an ophthalmoscope, showed large tracks of fresh blood marching along its dilated veins. On the roof of her mouth was a fan of blood-tinged spots, called petechiae. Fine crackling sounds were heard when she inhaled and exhaled deeply. Neurological testing confirmed weakness of her right hand and arm. I couldn't immediately think of a single diagnosis to explain all that was happening.
"It was just...the last...few months I had trouble breathing," Isabella affirmed between gasps. I instructed her to answer slowly, as best she could, so as not to worsen her air hunger.
The shortness of breath started at work. Her job involved vacuuming the offices in the main bank branch of Hyannis. To reach the corners, she had to move large furniture. She also cleaned the heavily used restrooms, scrubbing soiled toilets and mopping scuffed tiled floors.
"I went with her each time to the clinic at Cape Cod Care," Marianna interjected. "I told the nurses that for Mama to complain something had to be really wrong."
Dr. Matthew Sperry was Isabella's doctor at the HMO. He performed the employment physical when she started at the bank. She expected to see him at the clinic when she had trouble breathing, but he had been too busy. Instead, a friendly but harried nurse attended to her problem.
The nurse heard wheezing when she listened to Isabella's lungs. Maybe the dust in the bank, or the cleaning fluids, had triggered an allergy, the nurse said.
Dr. Sperry was informed of the findings, and the ventolin inhaler was perscribed. Isabella used it as instructed when she felt her chest tighten and her breathing strained, but it offered little benefit. Isabella told the nurse she was hardly able to do her chores at the bank. Each sunrise she returned to her apartment and collapsed in exhaustion.
"I had to call many times to get Mama seen again," Marianna sharply recounted. "Finally, they gave us another appointment."
On that second visit, marianna insisted her mother be examined by a doctor. They had to wait until the last appointment of the day was over.
Dr. Sperry listened to Isabella's chest and confirmed there were wheezes. He said she had asthma and wrote a prescription for aminophylline, to be taken orally three times a day, along with the antibiotic erythromycin. He said Isabella might have developed bronchitis, which worsened her asthma. Dr. Sperry cautioned both drugs could make Isabella nauseated but was sure the would "do the trick."
Isabella faithfully took the medications, although they didn make her queasy. But her symptoms still did not improve. I asked if a sputum examination, blood tests, or chest X ray were performed at either visit. Marianna said she was certain none of these had been done.
Isabella began to cough in harsh spasms. Her lips turned a deeper blue and the cardiac monitor showed that her heart rate had jumped to 135. As the spasms gradually subsided, Marianna stared at me with mute terror.
Before I had a chance to examine Isabella, Rob Salerno, director of the ER, stepped into the doorway of the room. He beckoned me to the corridor.
"She won't be able to keep this up," he stated bluntly. "She's lucky she made it in from Hyannis. I just got her second set of blood gases back. The face mask isn't holding her. She needs to be intubated and placed on a respirator."
Rob Salerno is an excellent ER doctor. He is expert in the technichal details of critical care medicine: the nuances of simultaneously administering multiple potent drugs, each with potential side effects, to resuscitate a patient in shock; the titration of intravenous fluids to fill a failing heart with needed volume without overloading the lungs or taxing the kidneys. And, equally important, he has a seasoned sense of who is going to precipitously deteriorate and how to intervene before the situation gets out of control.
Rob Salerno left to make the arrangements for the intubation as I returned to the Monteros.
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