Whipple disease

In 1997, after an illustrious 45-year career in the ministry, Arthur DeKruyter of Oak Brook, Illinois, looked forward to a peaceful yet active retirement with his wife of 50-plus years, Gladys. A year later, however, the normally robust pastor felt a drain on his once-tireless energy reserves.

"In September 1998, I began to feel tired in the afternoons," says DeKruyter. "It would get to 3:00, and I just couldn't keep going anymore. I found myself lying down and sleeping for two or three hours. Then little by little, I lost my appetite for dinner. Breakfast was fine, lunch was fine, but dinner was not good. I just didn't care about food, which was totally out of character."

Unknown to anyone else, DeKruyter also observed that his urine appeared darker and cloudier than usual. Blessed with good health throughout life, he dismissed the symptoms as temporary--possibly symptoms of the flu.

Concerned about her husband's health, Gladys mentioned the persistent fatigue during a visit to her cardiologist. A family friend, the cardiologist immediately phoned the popular pastor, asking him to come in for a checkup.

"He poked under my ribs on the right-hand side and hit my liver. I about jumped up to the ceiling," he remembers. "He said, `Oh, my. You've got some problems. Let me call our friend who is a gastroenterologist over.'"

The gastroenterologist ran blood tests and suspected, according to DeKruyter, "a major problem." He immediately referred the former pastor to Mayo Clinic for confirmatory tests.

Six weeks after the vague symptoms began to surface, cancer was found in the head of his pancreas. Fortunately, the cancer had not spread, or metastasized, to surrounding organs, making DeKruyter eligible for what many refer to as the "whopper of all surgeries," the Whipple. While at Mayo, he underwent the complex procedure.

Three-and-one-half years later, DeKruyter is alive and doing well with no signs of further cancer. He was one of the lucky ones. By the time most patients are diagnosed with pancreatic cancer, the disease is already in an advanced stage and has spread to other vital organs. Only a small percentage of pancreatic cancers are detected soon enough to qualify a patient for the Whipple, which to date offers the best chance of cure.

To find out more about the Whipple and early detection, the Post spoke with one of the country's leading experts on the procedure, Dr. John Cameron. Dr. Cameron is chief of surgery at The Johns Hopkins Hospital and Alfred Blalock Professor and chairman of the department of surgery at The Johns Hopkins University School of Medicine.

Q: You are cited as performing more Whipple operations than anyone in the world. How many have you performed?

A: I've done maybe seven or eight hundred Whipple operations. Presently, I operate four days a week. It is interesting that the surgeon Allan Whipple, for whom the procedure is named, only did 32. Until recently, no one did more than a handful of these procedures. At Johns Hopkins Hospital, we do over 200 Whipples a year. The next busiest hospital in the world does maybe 100 annually. The flow of patients to Johns Hopkins from throughout the country and the world has increased substantially over the past 15 years or so.

Q: Have you witnessed an increasing incidence in pancreatic cancer?

A: There was a substantial increase in pancreatic cancer in the country from 1940 through 1990. In the last 10 years, it has leveled off and maybe even decreased a little bit. It is now the fifth leading cause of cancer deaths in the United States, so it is definitely a major health problem.

Q: Is choosing a hospital and surgeon with experience a key factor in the success of the operation and in the patient's outcome?

A: The first paper on the subject of volumes and outcomes relating to the Whipple procedure came from this hospital in 1995. Actually, we held a press conference after the study clearly demonstrated for the first time that the results of an interabdominal gastrointestinal procedure performed in a high-volume hospital were better than in a low-volume institution. The study compared the procedure outcomes at Johns Hopkins with other hospitals in Maryland. Roughly, the mortality at Hopkins was about two percent, while the outcome at other institutions was about 20 percent. Hopkins' interest in the Whipple operation resulted in regionalization. In other words, physicians with patients who needed a Whipple in the state sent their patients to us. Presently, we do 85 percent of all the Whipples in the state. That regionalization resulted in a clear drop in hospital mortality and the saving of lives.

Q: Obviously, Johns Hopkins is one of the leaders in the field. What other centers across the country perform a high volume of these procedures?

A: Other institutions that do really large volumes include Memorial Sloan Kettering in New York, MD Anderson in Houston, Massachusetts General, the Mayo Clinic in Rochester, UCLA, and possibly Barnes Jewish Hospital in St. Louis. All these hospitals do work as well as is done anyplace.

Q: Can you tell us about the Whipple operation?

A: The Whipple operation was popularized by New York surgeon Dr. Allan Whipple in 1932. Actually, it was first performed in 1909 by a German surgeon named Kausch in Berlin. Although Kausch published about the procedure in 1912, it was rarely performed until Whipple in 1932, who did three of the procedures in a fairly short time interval. Two of the patients survived, which helped spread information about the operation, so since then it has become known as the Whipple operation. In the best institutions in this country, the operation carried about a 25 percent hospital mortality rate. In other words, one out of every four patients who had the operation died without ever leaving the hospital. Because it had such a high mortality rate, many physicians and surgeons thought the operation should be abandoned.

Then about 15 to 20 years ago, several institutions, principally Johns Hopkins, began taking a specific interest in the procedure, studying it, and learning how to perform it more successfully. The mortality dropped precipitously from 25 percent down to less than two percent. In one stretch, we did 190 procedures in a row without one mortality. By assembling a small team of surgeons, nurses, radiologists, anesthesiologists, and interventionists interested in caring for patients with pancreatic cancer who undergo the Whipple operation, the mortality dropped to this very low range. In this hospital, the mortality for a Whipple operation--which is generally considered the biggest general surgical operation a surgeon can perform--is no different than, say, a colon operation.

When I became chief of surgery at Hopkins in 1984, I said that the operation should not be performed on anyone over the age of 70. But no one was dying from the operation and we were improving our results, so we decided that we would do it on patients in their 80s, but no one older. Later, we said that over 80 is OK also. Over the last ten years, we have operated on more than 100 patients over age 80, seven over age 90, and one patient from West Virginia who is 103 years old. They were all, of course, very healthy individuals. The mortality in that group is virtually the same as the mortality in the under-age-80 group. In specialized centers, it's become a very safe operation in virtually every age group.

Q: Is the Whipple operation the best option for a patient facing a diagnosis of pancreatic cancer?

A: It's the only thing to do with a chance of long-term survival.

Q: What is the prognosis for patients after the procedure?

A: It all depends. We just reviewed our results in 700 patients on whom we performed the Whipple. As background, pancreatic cancer can involve all of the pancreas, its head, the neck, the body, or the tail, but most of the cancers are found in the head. The Whipple is performed only in patients with cancer in the head of the pancreas.

In those 700 patients, the five-year survival rate is 20 percent. While still very modest, 20 years ago the five-year survival rate was zero, so a lot of progress has been made.

If you take the patients who have negative margins and lymph node involvement--in other words, they have been diagnosed early enough so it hasn't spread to their lymph nodes and there are over 100 or so in this series--the five-year survival rate is about 40 percent.

What is needed is a tumor marker that can help identify high-risk patients, so we can pick up patients at an earlier stage of disease, before the tumor has become inoperable and spread to the lymph nodes.

Q: Are the markers presently used, such as the carcino-embryonic antigen (CEA) or carbohydrate antigen (CA) 19-9, helpful in detecting high-risk patients?

A: They are too nonspecific. A person can have an elevated CEA or CA 19-9 from smoking cigarettes, other tumors, or chronic pancreatitits. We need a molecular marker or protein product that will identify patients at particularly high risk.

I'm optimistic that within the next five years, there is going to be either a molecular marker or a protein product, which can be tested for in blood, that will identify high-risk patients who need to be investigated for early pancreatic cancer. That test will make a huge difference.

Q: What is the gold standard, in your opinion, for a diagnosis?

A: The CT scan is the gold standard for diagnosing and staging pancreatic cancer. Many patients also have an ERCP--because they present with yellow jaundice--to image where the blockage is and to help in putting a stent in to relieve the jaundice. But diagnosis and staging is done with a CT scan.

Q: How did you become interested in pancreatic cancer?

A: I became interested in part because the operation carries such a high risk of mortality. When I finished my training in the 1970s, I was a young surgeon looking to enter a field where I could make some contribution. The Whipple operation is a formidable, huge general surgical procedure, so I became interested in it and tried to perform it better, improve the results, and lower the mortality. There are many other diseases besides pancreatic cancer for which you perform the Whipple--ulcerative tumors and pancreatitis, for example, but I would say that about 60 percent of Whipples that we perform are for pancreatic cancer.

As I became more and more interested in the operation, we started getting more referrals. Pancreatic cancer was obviously the most challenging of all the operations, for which you do the Whipple. I decided to get others interested in the disease--pathologists, oncologists, radiologists, etc. Of course, it was easy to get other people interested in the disease when we began accruing an increasing amount of surgical material to study and research. Today, Johns Hopkins has more basic research going on in pancreatic cancer than any other institution in the world, because there is so much clinical material.

Q: So at this point, where the cancer begins to develop is completely unknown to us?

A: We know that it starts in the pancreatic ductal epithelium--the lining of the pancreatic duct. But we are learning more. In colon cancer, Dr. Bert Vogelstein at Johns Hopkins is a molecular geneticist who, over the last 15 years, has worked out the molecular events, or genetic mutations, that drive the changes in the epithelium, or lining, of the colon that lead to colon cancer--the activation of the oncogenes or the deactivation of the suppressor genes. The same work is being done now with pancreatic cancer.

Q: Do you see referral patients from all over the United States?

A: Yes. Many will come in after their doctors inform them that they have pancreatic cancer. They go home and tell the family. And a member of the family, such as the granddaughter, will get on the computer and discover our Web site, then tell their grandfather or grandmother that they have to go to Baltimore to Johns Hopkins. They arrive in Baltimore after going over the information on our Web site and, after reading the information, know as much about pancreatic cancer as we do. The Web site provides a marvelous education background so that when patients arrive, you don't have to start at ground zero in explaining what is going on. They already know about the Whipple operation and what the pancreas looks like. It has taught us the value of Web sites.

Q: What is the average length of stay when a patient comes from out of state--California, for example? How long are they at Johns Hopkins convalescing after the operation?

A: Those are good questions. We have what we refer to as a critical pathway for all patients that outlines what will occur on day one, day two, day three, etc. If everything goes perfectly, 50 percent of patients are out of the hospital on day seven or eight. For the other 50 percent, the average stay is probably 10 or 11 days. If they are from the Baltimore area, they obviously go straight home. But if they are from California, for example, we want them to stay in the Baltimore area in a hotel for a week, so we know that they are OK prior to leaving the area. They see us from this time on as an outpatient. But if everything looks fine, they head home.

The Whipple Procedure

The pancreas is located behind the lower part of the stomach. The head of the pancreas is surrounded by the duodenum, the first segment of the small intestine.

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During the standard Whipple, surgeons remove the gallbladder, common bile duct, part of the duodenum, the head of the pancreas, and sometimes part of the stomach.

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The remaining portions of the duodenum, pancreas, and bile duct are rejoined to restore continuity of the gastrointestinal tract.

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The Whipple Diet--Do You Have a Recipe?

The Saturday Evening Post would like to hear from post-Whipple patients about their diets. Are certain foods more agreeable than others? We would like to know. If you are a post-Whipple patient or a caretaker with dietary recommendations or recipes tailored for the post-Whipple palate, please write to us at: The Whipple Diet/SatEvePost, P.O. 567, Indianapolis, IN 46206.

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