The news about Steve Jobs' decision to take a medical leave from Apple until June has been more than a bit disturbing to me—and not because I am among the legions of iPhone devotees. I, like Jobs, was diagnosed with an islet-cell tumor in my pancreas. The experience taught me a lot about this misunderstood cancer—and it has made reading media reports speculating about Jobs' mysterious medical condition, and what possible ramifications his brush with cancer have for his present health, incredibly frustrating.
The media aren't entirely to blame for the confusion. As Slate's Farhad Manjoo points out, Jobs hasn't made it easy to report on his medical battles. According to Fortune, he was diagnosed with an islet-cell tumor in 2003 but didn't publicly acknowledge it until he underwent surgery to remove it in the summer of 2004. Later, in responding to concerns about his visibly deteriorating health over the last couple of years, Jobs and Apple have been reticent, claiming at various points that he was fine, simply suffering from a "common bug," had "digestive difficulties" following his operation to remove his tumor, and had an easily treatable "hormonal imbalance" before admitting upon announcing his leave, without specifics, that the problem was more serious.
My diagnosis in 2007 was a matter of pure and simple luck. After I experienced nighttime abdominal pain, a gastroenterologist ordered up blood work and a CT scan. Over the next few days, the pain subsided, and I considered skipping the scan because I was feeling somewhat better. I was 37 years old. I ate lots of fruits and vegetables, exercised, and stayed away from trans fats. Still, I went for the scan, which revealed two things: I had appendicitis, which was responsible for my pain, and I had a tumor about the size of a "large tennis ball" in the tail of my pancreas. I had a nonfunctioning islet-cell tumor. I quickly learned that the only truly reliable way to treat islet-cell cancer is to cut the tumor out before it spreads. Fortunately, doctors at Johns Hopkins were able to do so. My surgeon, Dr. John Cameron, removed the tumor, cut out 40 percent of my pancreas (he resected the tail in a procedure called a distal pancreatectomy), removed my spleen, and took out my appendix for good measure.
While my tumor was large and had been growing inside me "for years" (my surgeon's words), it was caught before it had spread, and my prognosis is extremely positive. When a friend told me shortly after my diagnosis that Jobs and I shared a disease, I soaked up as much information about Jobs' condition as I could find. More recently, I've watched with a combination of wonderment and dismay as the news media, in their rush to report on Jobs' present condition, have often engaged in a journalistic shorthand—referring to his 2004 disease as "pancreatic cancer." While this description is technically true, it's also misleading. Islet-cell tumors can certainly kill people, but they're drastically different from adenocarcinoma, what we normally think of as pancreatic cancer, which is much more aggressive and common. Eighty percent to 90 percent of pancreatic tumors are adenocarcinomas. More than 37,000 Americans will probably be diagnosed with adenocarcinoma of the pancreas this year, while approximately 2,500 Americans annually are diagnosed with the much rarer islet-cell cancer. All of these crucial distinctions have often gotten lost amid the unseemly feeding frenzy around Steve Jobs.
Islet-cell cancer, like Jobs and I had, is usually curable when caught early; adenocarcinoma, which is usually detected only after it has spread, has a five-year survival rate of 5 percent. (Patrick Swayze has adenocarcinoma, as did Randy Pausch, whose "Last Lecture," recorded before his death, became a viral video sensation.) Another important point to keep in mind, also overlooked by most in the media, is that islet-cell tumors (also known as "neuroendocrine" tumors) are divided into functioning and nonfunctioning categories. While we don't know what kind of a tumor Jobs had—he has never specified—I can tell you that my tumor was "nonfunctioning" because as far as my doctors could tell, it wasn't producing any hormones, and it caused no symptoms.
In contrast to my own tumor, there are five types of "functional" islet-cell tumors. They "present" in a variety of ways, depending on what kind of hormones they produce: insulinomas, which can cause low blood sugar; gastrinomas, which release large amounts of gastrin, a hormone, into the bloodstream and cause ulcers in the stomach and duodendum; VIPomas, which tend to cause severe diarrhea; glucagonomas, which cause severe skin rashes and weight loss, among other symptoms; and somatostatinomas, extremely rare (fewer than one in 40 million people get them) islet-cell tumors with "nonspecific" clinical symptoms including diabetes and stones in the gallbladder. We have no way of knowing what was causing Jobs' "hormone imbalance," but functioning islet-cell tumors do all produce hormones, so this is one plausible explanation.
After I was diagnosed, I was told that modern medicine doesn't have chemotherapy or radiation to use against islet cells. ("We've got nothing that works" went the refrain.) Islet-cell tumors tend to be slow-growing, so chemotherapy designed to attack rapidly growing cells is ineffective. But there are some drugs, including one called streptozocin, that have "response rates as high as 70%" with islet-cells, according to Hopkins' Web page. In some cases, doctors can also use techniques such as hepatic artery embolization and chemoembolization, which essentially destroy the blood vessels that have been feeding the metastases in an attempt to choke off the tumors' blood supply.
We as a country have shortchanged medical research regarding both adenocarcinoma of the pancreas and islet-cell tumors. For starters, the National Cancer Institute has been cutting grants for adenocarcinoma research in recent years, and the funding stream for scientists is drying up. This is happening at the very moment when doctors at the Sol Goldman Pancreatic Cancer Research Center have mapped the pancreatic cancer genetic blueprint—opening up a promising new field of research and possibly new early detection tests and treatments.
At the same time, as with many rarer diseases, pharmaceutical companies have little financial incentive to support scientific research into islet-cell tumors, while the government also shortchanges research into uncommon diseases. "The greatest emphasis is paid to funding the most common tumors, such as those of the lung, breast and colon. When you consider the pitiful federal funding for pancreatic adenocarcinoma, despite this cancer claiming over 34,000 American lives each year, you can imagine where even less common cancers like islet-cell tumors fit into the grand scheme of things," says Dr. Anirban Maitra, an associate professor of pathology and oncology at Johns Hopkins. "Unless there is a commitment to study rare diseases like islet cell tumors, there is unlikely to be significant progress in this disease."
Furthermore, "[A]dvances made in pancreatic adenocarcinoma—and there have been some significant ones, funded by nonfederal dollars—are highly unlikely to be extrapolated to islet cell tumors, simply because they are essentially completely different tumors joined only by the commonality of occurring in the pancreas. It, too, is a major medical orphan."
One professor of oncology and pathology at Hopkins, Bert Vogelstein, has said that if he can find a donor who will support the project, he and his team will do their best to sequence the islet-cell tumor genome within a year. Perhaps, if Jobs' recent medical woes turn out to be related to his islet-cell tumor, there will be greater attention paid to the disease, the way Michael J. Fox helped increase awareness of Parkinson's. If I've learned nothing else since my diagnosis, it's that medical orphans need attention, too.