Steve Jobs and Me
A layman’s guide to islet-cell tumors in the pancreas.
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."
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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.