Thursday, April 15, 2010

A Call to Jerusalem

I'm not at all sure what is going on and that's not a very comfortable spot for a doctor to be in. The call from the ER doctor tells me that he's admitting a very sick Boeing engineer to the intensive care unit. Abdul Yasu (not his real name) has been in normal health, until he had sudden onset of stabbing left chest pain and violent coughing this morning. This was followed by sweats, a fever to 104, and trouble breathing. His blood pressure is thready and he is wheezing.

The strange thing is that I know Abdul. He's a neighbor living just a few houses away and our kids have gone to school together. A brilliant aeronautical engineer with an equally brilliant wife mathematician Maryam, they immigrated from northern Galilee about 30 years ago. They were Christian Palestinians looking for a better life for their children. Boeing and Seattle have been their secular savior. Abdul has explained to me that his name in Arabic means "servant of Jesus" - a name he is proud of. He last visited his native land three years ago.

Abdul gives me a weak smile when he sees me walk into his ICU room. The cardiac and oxygen monitors are already in place and he's receiving an aerosol treatment for his wheezing: "Praise God that you're here to see me Dr. deMaine. I'm very sick."

That is not news to me! Maryam is at the bedside, very worried and their son who works at nearby Microsoft is on the way over.

Trying to be methodical and not miss anything obvious, I try to get more history. Any recent colds? Any unusual exposure to fumes, sprays or toxins? Any allergy history? Any history of heart or lung disease or smoking? Any history of HIV exposure (this important question is always hard to ask in front of family)? Tuberculosis history? Any friends or family ill? Any new medications or change in medications. Any new health food or alternative medications?

The answers were all to the negative. His list of chronic medications is very short. A diuretic for blood pressure, a multivitamin, and a baby aspirin. That is it. He's not taking any over-the-counter or herbal medications.

About this time the chest X-Ray arrives and I'm very concerned. There's a huge 10 cm cavity in the middle of the left lung which has very strange characteristics. Usually a cancer will be thick walled and irregular. Usually TB is also thick walled with a dense area of infection around it in the upper part of the lung. Abdul's large cavity (basically a hole) in the left lung is very thin walled and has some fluid gathered in the bottom - much like a cup holding water.

Looking further there is a possible second spot in the other lung, about 2 cm in diameter - something we would call a "coin lesion" in medical descriptive talk. This adds to my worry. An old X-Ray is located from two years ago that's entirely normal.

The exam doesn't add much to my knowledge base. There are no enlarged lymph nodes or skin lesions. The throat is clear. The lungs reveal diffuse raspy wheezing. He's not coughing up anything for testing, specifically no blood or off colored sputum. The heart is regular but fast at 120. The abdomen is benign. Orders are written for cytology (cancer check), and for multiple cultures - routine, TB, and fungus.

The blood work begins to return. The main remarkable feature is in the white blood count. This is very elevated with lots of eosinophils. These are the kinds of cells containing red-stained granules that we commonly associate with an allergic reaction. His count is 24% when it would normally be 0-2%. Thankfully the kidney function, liver function, and blood sugar are all normal.

All this was very puzzling. What could cause an sudden allergic-type reaction and a new lung cavity? I begin to think I might have an answer but I need a quick trip upstairs to the medical library. There are a number of syndromes involving eosinophils and the lungs, but in Abdul's case the acute dramatic onset of symptoms suggest a rare diagnosis - a pulmonary hydatid cyst which has acutely ruptured and produced his critical status.

I haven't seen a patient like this for 30 years. My current knowledge of what's called tropical medicine is very rusty. Fortunately in the 1960's I had a great teacher named Harold Brown. He was a Professor of Tropical Medicine at Columbia's School of Medicine and the author of a definitive medical textbook about parasites. The good doctor taught me about intestinal parasites at the CDC in my preparation to care for Peace Corps Volunteers in Afghanistan. Affectionately known as "Stoolie Brown", he made the parasitic intestinal diseases come alive.

Hydatid disase starts as a worm in a domestic or wild dog's intestine. The eggs are shed into the dog feces which often litter the pastures where sheep graze. The unwitting sheep (or an occasion human) ingests contaminated vegetation. The eggs hatch in the new host and penetrate into the liver (or lungs) where they form cysts. The cycle is completed when other uninfected dogs feed on the liver or lung cysts and develop intestinal worms. Humans in sheep raising countries are "innocent bystanders" becoming infected when the salads they eat are contaminated.

But does Abdul really have Hydatid Cysts in his lungs? We're taught as students to first look for uncommon presentations of common diseases, so I still need to rule out cancer, tuberculosis, etc. Abdul agrees to a bronchoscopy, CT scan of the chest and abdomen, and more blood work - specifically a serology for echinococcus (the medical names for hydatid cysts). The CT scan shows no cysts in the liver and demonstrates the large air and fluid filled cavity within the left lung. This is a 2.4 cm small low density (water density) rounded lesion at the base of the right lung.

He is so sick, I did the bronchoscopy at the bedside. With mild sedation and lidocaine aerosols, I slide the bronchoscope through the left nostril and follow its course on the video screen. The vocal cords are healthy and move well, The trachea and bronchial passages look normal, much like one is gazing up a hollow tree trunk. I do a saline lavage of 100cc's in order to get fluid from the affected area of the left lung. Then I do a biopsy of the mucous membranes nearby. I decide not to try to get into the cavity of the lesion which might induce more havoc.

By the next day, he's feeling much better. The biopsy and lavage are all negative except for the presence of eosinophils. I walk over the the radiology department to review the CT scan with my favorite radiologist. She notices that both on the chest x-ray and CT scan that there isn't just fluid at the bottom of the cyst - there's what she calls the "Water Lilly sign" - crumpled cyst wall at the bottom. She pulls out the CT text books which confirm the suspicions.

All this is beginning to finally make sense. The thin walled cyst acutely ruptured on the day of admission, spilling it's liquid product into the patients lung airways. He had an anaphylactic-type reaction, which can be fatal to some persons - so Abdul is fortunate to survive.

The following day the blood serology confirms a very high level of antibodies to echinococcus, the causative organism. False positives can occur but given the clinical presentation I'm sure I finally have a diagnosis.

But what should be done? I have no experience here, despite doing an Infectious Disease Fellowship years ago. So I pick up the phone and make several calls; to the CDC, and several medical schools with expertise in tropical medicine. The consensus was to begin treatment with a drug called Albendazole, but the big downside was that it provided a cure in only 30% of the patients. Surgery was an added option.

I discussed this with Abdul and Maryam. Their immediate response was, "You must call Maryam's brother in Jerusalem." He is a thoracic surgeon at Hadassah Hospital and must see a lot of this disease.

Dr. Nassir is just back from additional training at the University of Pennsylvania and was most helpful. After hearing the story, he explains that Hydatid Cysts are endemic in northern Galilee and a relative common reason for lung surgery there. He is definite, "It must come out."

Fortunately, my thoracic surgery consultant has worked abroad and is fearless in tackling a case like this. Abdul and the family feel comfortable going ahead and removing the cyst, which means about a third of the left lung. The operation itself doesn't take place for about two months, waiting for the Albendazole drug treatment to kick in and hopefully sterilize the infection so surgery wouldn't spread it to new sites.

The surgery goes well and Abdul makes an uneventful recovery. The Albendazole is stopped after about 18 months. Over the next five years, we follow his chest x-rays and with an occasional CT scan. The remaining right lung spot doesn't disappear, so it is removed with thoracoscopic surgery. It indeed is a small hydatid cyst but sterile and would not have caused problems.

His "near death" experience no doubt affects him and perhaps makes him even closer to his religious faith than before. Beyond that, he's very proud to be an American and considers himself most fortunate - and I think we'd agree.

Comment: A case such as this never leaves me. I feel fortunate to have spent two years in Afghanistan and a year in Saudi Arabia practicing medicine. With modern travel, we can see diseases such as malaria, worms, etc arriving back here where we are very unfamiliar with the diagnosis and treatment. With travel medicine clinics and referral areas of expertise like the CDC we can get the backup we need - if we ask the right questions and make the right calls. This one to Jerusalem certainly helped my patient - and me.

I did present this case at a local chest conference as an unknown - and of course didn't mention the patient's name. The Pulmonary Fellow really struggled with the presentation and couldn't come up with a diagnosis: the reason being that he forgot to ask about "Travel History". Once it was clear that the patient had lived and traveled in the middle east, the diagnosis wasn't so hard. It reminds me that our lives and medical care are really accidents of zip codes - a lottery over which we have limited control. But Abdul managed to win the lottery. His father had told him the streets of America are paved with gold. Abdul tells me this and smiles, "And you know they really are!"

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