Paul L. Weiden, MD, FACP
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For the past decade, I have traveled monthly to Juneau, Alaska, to see patients who have oncology or hematology problems and are referred to me by their primary care provider. Although Juneau is Alaska’s state capital, it is located in southeast Alaska and is surrounded by mountains, glaciers, and the sea, so it is accessible only by boat or a 2-hour flight from either Anchorage or Seattle. The medical community of primary care providers in Juneau is strong, but the population of just 31,000 precludes the full-time presence of many medical specialties, including oncology and hematology. In collaboration with the local medical community and hospital, we can treat patients with many hematologic and oncologic diagnoses in Juneau, but conditions that require intensive supportive care or complex multispecialty care necessitate transfer to Anchorage or to the lower 48 states, most frequently to Seattle.
The following story will sound familiar to most of my medical colleagues, but I thought it nevertheless remarkable and indicative of some of the issues we face daily in serving our patients. Recently, on the first of my three heavily booked clinic days in Juneau, I received a call from a local family physician about a patient who had been found to be pancytopenic during a preoperative evaluation before spinal stenosis surgery. Despite an already full schedule, I asked the office staff to add him on at the end of the day.
Logistic Challenges for Bone Marrow Biopsy
The patient appeared to be the picture of health, save for documented slow progression of pancytopenia over the preceding 3 months. The diagnosis was not obvious, so a marrow examination was needed. The office staff worked diligently and uncomplainingly behind the scenes to rearrange my schedule for the second day in Juneau so I would be able to perform the marrow biopsy that afternoon. The only time the procedure could be shoehorned into the calendar would be just a little too late to meet the deadline for the daily courier who takes specimens from Juneau to Seattle for special studies, so the samples would not go out until the following day. That would have to be adequate.
The next day, additional laboratory data were returned. The only other abnormalities were a markedly elevated D-dimer and a borderline low fibrinogen level, which suggested disseminated intravascular coagulation. But why? Then I recalled that the patient had a digital papillary adenocarcinoma of the finger resected 5 years previously,1 and I wondered if he might now have metastatic disease in his marrow. The bone marrow biopsy went well and was completed quickly, and the technologist rushed up to the laboratory in hopes of still catching the courier.
Diagnosis of Acute Promyelocytic Leukemia
The day after the bone marrow biopsy, I met the patient and his wife in the office (somehow that visit was also worked into my schedule). I informed them that, unfortunately, the initial marrow stain was not adequate to make a diagnosis, but I promised I would keep track of the patient’s situation, although I was returning to Seattle that evening. I also told them that he might need care outside of Juneau. I learned that he had been pleased with previous care he had received at the University of Washington in Seattle, but he had family in southern California and might prefer to go there if a longer stay was anticipated.
Juneau, Alaska. Photo: GETTY IMAGES
The next morning, back in Seattle, I received a call from the Juneau hospital pathologist. A better stain of the marrow aspirate was suggestive of acute promyelocytic leukemia (APL), and that diagnosis had already been confirmed by the Seattle reference laboratory on the basis of immunohistochemical stains. (Yes, somehow the specimen did get to the courier in time to be flown out on the afternoon of the marrow examination!) As most readers of Journal of Clinical Oncology know, patients with APL have an 80% to 90% chance of cure if they start modern therapy in a timely fashion, but they also have a 10% to 30% chance of early death before or in the initial days of therapy, usually as a result of devastating thrombohemorrhagic complications.2,3
Prompt Treatment Plan With Timing Complications
It seemed that the next steps would be relatively simple. APL is a medical emergency, so we needed to start the patient promptly on the medication that must be started quickly (all-trans-retinoic acid [ATRA]) to prevent the thrombohemorrhagic complications, and we needed to get him to Seattle to start definitive chemotherapy.
First, however, I needed to explain this to the patient, but there was no answer on his cell phone. Fortunately, his wife did answer her phone, and she accepted my quite brief explanation of the situation. She assured me that she
I reflected on all of those steps that we as physicians take for our patients, which are often behind the scenes and usually not recognized or compensated, but which are critical nonetheless.— Paul L. Weiden, MD, FACP
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would find her husband, get him to stop by the hospital to pick up the first dose of ATRA, and get him on the 1:00 PM flight to Seattle. I then paged the hospital pharmacist (three calls were required) only to learn that there is no ATRA in Juneau. So, then I called the patient’s wife back for an update. I learned that she had informed her husband and his boss, but she had also discovered there was no space on the 1:00 PM flight to Seattle. In addition, she told me that her husband had developed a large ecchymosis from the previous day’s blood draw, a sign that he was at increasing risk of clinically significant hemorrhage. Therefore, preventing a 7-hour delay until the 8:00 PM flight to Seattle could be critical.
An emergent airlift flight to Seattle would cost about $70,000, but if that would avoid a fatal cerebral hemorrhage, these health-care dollars would be well spent. But I decided first to call the only commercial airline that flies from Juneau to Seattle and use my so-called premium frequent flyer status to see what I could do.
The first airline agent informed me that he was sorry, but nothing could be done. I asked to speak to his supervisor. She told me that, yes, there was one last seat available because of my frequent flyer status. But when I asked her to hold it for 5 minutes until the patient or his wife could call with the required personal information, I was told that would not be possible. So, desperate not to lose this one remaining seat, I asked my wife for her cell phone, called the patient’s wife on it, and, transmitting the information between the two phones, I gave the airline personnel the information needed to get the patient on that remaining seat on the 1:00 PM commercial flight to Seattle. As an aside, the cost of that commercial flight was $500, saving the health-care system (so to speak) $69,500. I wonder where that fits into the current national health-care debate?
I finally had time to speak to the patient and his wife together for a few minutes to give them a brief introduction to acute leukemia, APL, the risk of hemorrhage, why APL is a medical emergency, and the favorable long-term prognosis if appropriate treatment is started without delay. They seemed to understand and to accept this overwhelming new reality. I asked the patient to get on that 1:00 PM flight and told him I would call his cell phone while he was in the air with additional instructions on how to proceed once he landed in Seattle.
Four Anxious Hours on the Phone and Then Some
The next step was to get the patient admitted to the university hospital, and to do what I could to ensure that his treatment was started without delay, even though this would be a late-evening hospital admission. I called the hospital operator and asked for the pager number of the hematology attending physician on call. The operator told me she could not give me that number. I reminded her that I am a clinical professor at the medical school, but to no avail; she still would not give me the number. She did say, however, that she would page the hematology attending physician and ask her to call me.
After several attempted calls back and forth, the attending physician and I finally did connect. But she informed me that I needed to speak to the leukemia attending physician, not the hematology consult attending.… Back to the hospital operator. After several more back-and-forth calls, I spoke with the leukemia attending physician, who assured me she would check on bed status, arrange the admission, and call me back to confirm that all was set. By this time, the patient was in the air. I left a message on his cell phone and then called his wife to update her as well. Wonderful, I thought—all is now arranged. Finally, after nearly 4 anxious hours on the phone, I could relax.
In some cases, a few telephone calls can make the difference between life and death.— Paul L. Weiden, MD, FACP
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But a couple of hours later, I realized I had not received that confirmation call from the leukemia attending physician or from the hospital. Had the admission really been arranged, or had there been a glitch? So, I tried to reach the leukemia attending physician again, but we could not connect. Also, copies of my office notes and the cytogenetic confirmation of the diagnosis from the Seattle reference laboratory had been faxed to my home office, but I needed to get all of this information to whoever was going to be seeing the patient at the hospital that evening to be sure he received his ATRA without delay.
More phone calls to hospital admitting and to the leukemia floor. The receptionist told me to fax her the information, but when I called her back 30 minutes later to confirm receipt, I was informed: “Oops, it looks like that fax machine is not working, please try another number….” Finally, I did connect with the leukemia attending physician. I learned that a bed was available and that they had received the second fax and had all of the necessary data. I was promised by the attending physician that she would not leave the hospital until the patient was seen and his therapy started.
‘Time to Reflect on Quite a Day’
At last, it was time to reflect on quite a day—and to be glad that the patient needed medical clearance for his elective surgery, that his primary care provider recognized the significance of his abnormal blood cell counts, that all of this happened to coincide with the first of the 3 days per month that I was in Juneau, that the office staff in Juneau worked in the patient on Monday and that I could do the marrow examination on Tuesday in time to catch the courier to Seattle on that day, that the pathologist and the reference laboratory recognized the possibility of APL and reported results rapidly, that the patient and his wife and his boss all accepted the urgency of the situation with only the most superficial of explanations, that I am a frequent flyer and that this allowed the airline supervisor to give the patient the one open seat on the 1:00 PM flight, that the university hospital staff all responded appropriately (accepting the reality of a few extra phone calls).
And, yes, to reflect on all of those steps that we as physicians take for our patients, which are often behind the scenes and usually not recognized or compensated, but which are critical nonetheless. These activities may seem endlessly frustrating or extraneous to clinical practice, yet they are intrinsic to the art of medicine and essential to our roles as patient advocates. In some cases, a few telephone calls can make the difference between life and death. ■
At the time this article was published in the Journal of Clinical Oncology, Dr. Weiden was practicing at the University of Washington School of Medicine, Seattle.
1. Duke WH, Sherrod TT, Lupton GP: Aggressive digital papillary adenocarcinoma (aggressive digital papillary adenoma and adenocarcinoma revisited) Am J Surg Pathol 24:775-784, 2000.
2. Park JH, Qiao B, Panageas KS, et al: Early death rate in acute promyelocytic leukemia remains high despite all-trans retinoic acid. Blood 118:1248-1254, 2011.
3. Sanz MA, Lo-Coco F: Modern approaches to treating acute promyelocytic leukemia. J Clin Oncol 29:495-503, 2011.