The Disease Detective

My story

I am a 49-year-old male who used to suffer from interstitial lung disease. I first began to have symptoms about two and a half years ago, and my lung functioning slowly deteriorated. I received excellent care from my pulmonologists, Vincent Valentine and Alexander Duarte, at the University of Texas Medical Branch (UTMB) in Galveston. They prescribed an extensive regimen of tests; I underwent a lung biopsy bronchoscopy, a video-assisted thoracoscopic surgery (VATS) lung biopsy, and several X-rays and CT scans. At first, they thought that I had hypersensitivity pneumonitis – but after various treatments failed to halt the disease progression, additional testing established a new diagnosis: idiopathic pulmonary fibrosis (IPF). My doctors referred me to the lung transplant center at Baylor St. Luke’s Hospital in Houston, where I was evaluated and put on the list for a lung transplant.

I received my double lung transplant on March 23, 2017. The procedure went fabulously and I made a remarkable recovery. I owe my life to God, my family, and the exceptional medical providers who saw and continue to treat me. Thanks to them, I feel great!

Getting to know pathology

Even before I became a patient, I was aware of pathology. I’m an attorney, so I have previously worked with forensic pathologists on cases to evaluate causes of death. But I now know that pathology is much more varied than I had previously seen. I think of a pathologist as a physician who focuses on the causal study of disease and is involved in the microscopic study of cells and cell abnormalities. I primarily think of pathologists as people who analyze lab specimens under a microscope.

My first encounter with pathology as a patient was after my interstitial lung disease diagnosis. I have a family history of IPF but, for my doctors, it was difficult to distinguish between that and hypersensitivity pneumonitis. The two diseases have somewhat similar clinical symptoms and radiology findings. To help shed light on my diagnosis, I underwent a bronchoscopy and lung specimens were sent to a pathologist. After the pathology report came back, though, there were still uncertainties as to my diagnosis, so I underwent a VATS lung biopsy. Those results clarified my diagnosis, but did not absolutely rule out the possibility of any other disease.

After my transplant, my former lungs were biopsied. The pathology report confirmed that I had suffered from IPF, rather than hypersensitivity pneumonitis. Knowing that was extremely important to me, because if I had had hypersensitivity pneumonitis, I would have been at continued risk of recurring lung damage in my transplanted lungs if I had not removed myself from the antigen causing the pneumonitis. Because of the difficulties we had experienced in pinpointing the offending antigen – in retrospect, an obvious challenge as none existed – I was pleased to have a confirmed diagnosis of IPF.

All of my care providers made sure I understood the nature of my disease from a pathology perspective. My pulmonologist shared and explained my pathology reports with me after my biopsies. Later, I also met my pathologist, Tim Allen, during an online conference call with my pulmonologist and radiologist. During this conference, my three doctors briefly reviewed my case and went over the pertinent radiology and pathology findings. I had questions about my pathology reports and my diagnosis, and Allen explained the reports and the reasons behind the differential and my diagnosis in terms I understood.

After my conference with the multidisciplinary team, I felt I had a better understanding of my disease and greater peace of mind. It was – and still is – important for me as a patient to know as much as I can about my disease, its causes, treatment, and prognosis. My pathologist answered several perplexing questions I had about the findings from the two biopsies I underwent. Without this contact, I would still have lingering doubts as to my diagnosis and the differences in the biopsy reports.

The rights and the wrongs

I really appreciated being included in the multidisciplinary conference between my pulmonologist, radiologist and pathologist. It was the first and only time I was able to speak with a pathologist. That time was very valuable to me, because my pathologist provided me with insight into my disease by using my own biopsy slides to point out to me some of the basis for his diagnosis. I was able to visualize what we were discussing, and to directly see the impact of the disease on my lungs. For those suffering from interstitial lung disease, pathology plays a big role in diagnosis, so I feel that all patients should be able to meet with a pathologist to review their biopsy reports and findings. Meeting with a pathologist should be part of the patient management protocol for these types of diseases.

One thing I would have appreciated was easier access to my pathology reports. UTMB uses an electric medical record system that allows patients access to test results and some reports. My pathology reports were unfortunately not accessible, though I was eventually able to obtain them by other means. As a patient, I want easy access to my medical records – including pathology reports. That’s something I would like pathologists and hospital administrators to know, so that perhaps in the future patients will have access to their complete records.

The patients’-eye view

I think patients often feel out of touch with pathologists’ work because there is little, if any, interaction between patient and pathologist. I would appreciate having an office visit or phone call with a pathologist after undergoing procedures to review and explain the findings in the reports. I want to be fully informed of my disease and the foundation behind its diagnosis.

I would also encourage patients to educate themselves as best they can about their disease and the various methods used in diagnosing it. That way, they can have a basic understanding of what’s wrong, how the doctors go about learning more, and what can be done to address the issue. All patients deserve a basic understanding of what the tests and procedures can and can’t reveal about their disease. I think patients should consider obtaining copies of their pathology reports, and seeking out a visit with their pathologist to discuss the reports if they have questions.

From a layman’s perspective, one often thinks that medicine is black and white – that there is an absolute certainty or concrete answer to every medical condition. I thought that the results from my VATS lung biopsy would provide me with an absolutely certain diagnosis – but, of course, that wasn’t true. The report left open the possibility of a differential diagnosis; it was still possible that I had a different form of interstitial lung disease. I was somewhat disappointed, but I came to the realization that medicine can be an inexact science and that patients should be aware that diagnoses are rarely made with 100 percent conviction.

I realize that medicine, and in particular histopathology, is complicated. It can be difficult to explain pathology results to patients in terms they understand. Being able to translate medical terminology into laymen’s terms is a talent that I suspect is often overlooked and undervalued in the medical profession. For patients who do want that explanation, I feel it is critical to the doctor/patient relationship to be able to meet with each doctor providing specialty care. I feel more involved and informed in my care when I have an understanding of my disease and the reasoning behind its diagnosis. Even if it is not part of the standard protocol, I feel that a meeting with the pathologist should be an option offered to any patient who wants to discuss reports. Getting that expert viewpoint first-hand can be a valuable part of a patient’s journey through care.