Dear Readers,

I’ve decided to change the pace at ML, so that I might focus more intensively on some other writing projects. My plan is to post weekly.

Thank you for your continued readership, comments and shares. With your support and interest, this blog will continue to grow.

–ES

scene from “50/50″

The other evening I watched 50/50, a film about a 27 year old man with a rare kind of cancer, a malignant schwannoma. The tumor is growing and pushing into the protagonist’s lower spine. The movie, based in part on the true story of scriptwriter Will Reiser, surprised me by its candor.

Actor Joseph Gordon-Levitt smoothly portrays Adam Lerner, who soon finds out he has cancer. The opening scene captures him jogging in an early morning. He’s seems a nice, cautious and perceptive young man in a relationship. His rowdier buddy, played hardily by Reiser’s real-life friend Seth Rogan, proves loyal during Adam’s chemotherapy and, later, big spine surgery.

By its cast, I expected this might be a guys’ flick. Yes, there are jokes about sex and cancer. But the film reveals the young man wincing during sex because he’s in pain and can’t hide it. The young women are pretty much all attractive, but they’re not interchangeable props; the relationships are complicated and plausible.

An unexpected perk in the movie is the realistic family dynamic. Lerner’s mother, a worrying sort, wants to be there for her son and doesn’t trust that his girlfriend will sufficiently help him. Anjelica Huston effectively fills the mother’s role. Lerner rarely answers her calls, while she’s biding her time with a husband who, due to Alzheimer’s, doesn’t comprehend what’s going on. She respects her son’s privacy, but feels, understandably, isolated and scared.

The doctors are flawed, of course. The oncologist at the start doesn’t directly tell Lerner of his diagnosis but, instead, speaks into a dictaphone about the malignancy. He refers Lerner to an analyst of some sort, a young woman with little experience, for talk therapy.

When Lerner goes for surgery, the pre-op scene is frighteningly realistic to anyone who’s ever had a young family member go through this kind of surgery. The family and friends are worried. The patient, calmest of all among the group, can’t determine what will be his fate.

The term schwannoma derives from Schwann cells. These elongate cells normally envelop long nerves and rarely become malignant. Most schwannomas, or neurofibromas, are benign; these can cause pain and other symptoms by pressing on nerves, but don’t usually don’t spread or grow quickly. The names can be confusing, as there are several similar-sounding terms for these growths. Some people inherit a disposition to these non-malignant tumors. Rarely, as seems to have been the case in this story, a schwannoma takes an aggressive, invasive and sometimes lethal course. Another name for the cancerous form is malignant peripheral neural sheath tumor, or MPNST.

50/50 refers to the odds of Lerner’s survival, about which he read somewhere on the Internet shortly after his diagnosis. I’d give the movie a high score, 90+, mainly for its lucid, accessible approach to a cancer patient’s experience and concerns.

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Even the most empowered patient – one who’s read up on his drug regimen, and engaged with his physician about what and how much he wants to receive, and visited several doctors for second opinions and went on-line to discuss treatment options with other patients and possibly some experts – can’t know, for sure, exactly what’s in the bag attached to his IV pole.

Counterfeit Avastin (images from FDA)

Scary because patients are so vulnerable –

The problem is this. If you’re sick and really need care, at some point you have to trust that what you’re getting, whether it’s a dose of an antibiotic, or a hit of radiation to a bone met, or a drug thinner, is what it’s supposed to be. If vials are mislabeled, or machines wrongly calibrated, the error might be impossible to detect until side effects appear. If you’re getting a hoax of a cancer drug in combination with other chemo, and it might or might not work in your case, and its side effects – typically affecting just a small percent of recipients — are in a black box, it could be really hard to know you’re not getting the right stuff.

What this means for providers is that your patients are counting on you to dot the i’s. Be careful. Know your sources. Triple-check everything.

The bigger picture — and this falls into a pattern of a profit motive interfering with good care — is that pharmacists and doctors and nurses need time to do their work carefully. They need to get rest, so that they’re not working robotically, and so that they don’t assume that someone else has already checked what they haven’t. And whoever is buying medications or supplies for a medical center, let’s hope they’re not cutting shady deals.

This issue may be broader than is known, now. The ongoing chemo shortage might make a practice “hungry” for drugs. And with so many uninsured, some patients may seek treatments from less-than-reputable infusion givers. The black market, presumably, includes drugs besides Avastin.

If I were receiving an infusion today, like chemo or anesthesia or an infusion of an antibody for Crohn’s disease, I’d worry a little bit extra. I mean, who will check every single vial and label and box? Think of the average hospital patient, and how much stuff they receive in an ordinary day — including IV fluids that might be contaminated with bacteria.

It’s scary because of the loss of control. This circumstance might be inherent to being a patient — in being a true patient and not a “consumer.”

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Dr. John Mandrola is a cardiologist who practices in Louisville, Kentucky. He blogs about electrophysiology — heart rhythms – and other aspects of his work, like general heart disease and internal medicine, at Dr John M. He’s into fitness and cycling, besides.

Part of the fun of blogging is you get to know people. For instance, this morning I learned that John grew up in Connecticut. We must have been near neighbors at some point. Well, sort-of.

Please do check out the Valentine’s Day edition of Grand Rounds, and Dr. John M.

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At the time of her husband Fred’s diagnosis, both she and her husband held several jobs but he lacked health insurance. In a video, Regina describes how his diagnosis and care were delayed.

“73 Cents” refers to the price, per page, Regina needed to pay to get a copy of her husband’s chart when he entered a new medical facility. According to the film, she was told she’d have to wait 21 days to get his records, even though he was acutely ill and dying.

Regina, now a widow with two young sons, pushes for patients’ rights to access to their health  records and, more generally, for a patient-centered approach to medical care. The film-makers’ point: The unreasonable price of the medical records, combined with the delay in receiving them, exemplifies unnecessary harms patients encounter in an outdated, disjointed health care system.

Regina has several ongoing projects, including the Walking Gallery. In that, she represents health care stories on the backs of people’s jackets. The idea is to take the message of the mural – which is one patient’s story, and necessarily static – and take it further.

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structure of MTX (PubChem; NCBI)

I was astonished to learn that methotrexate supplies are running short. This chemotherapy may soon be unavailable to patients who need it. And it’s not just kids with leukemia, as the Times story highlights effectively.

Methotrexate is an old, bread-and-butter cancer kind of drug, a basic ingredient in standard regimens for many tumor types. I’ve personally administered this old drug to patients with breast cancer, lymphoma, leukemia, head and neck tumors, ovarian cancer, colon cancer and people whose tumor cells spread to the brain. Doctors prescribe this drug, also, in a few non-malignant conditions, like rheumatoid arthritis.

Methotrexate has been used in cancer wards for over 50 years. And like other beyond-patent meds, it’s become less profitable to manufacture MTX compared to much costlier new agents. Hard to perceive this shortage as anything but a tragedy — that the business of health care renders valuable, inexpensive drugs out of reach.

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1. In 2010, 32.4% of adults who’d seen a health care professional were advised to begin or continue with exercise or other physical activity. That fraction’s up significantly from 2000, when a slim 22.6% of patients recalled their doctors telling them to get a move on.

2. Among folks over 85 years, nearly 29% say they were told to exercise. That number’s nearly doubled since 2000, when only 15.3% of elderly patients reportedly received this kind of advice.

3. Adults with diabetes were told to increase their activity more often than those with high blood pressure, cardiovascular disease and cancer. Compared with healthy weight adults, obese people were twice as likely to have been told to exercise by a physician or other health professional.

An underlying message is that doctors should be prodding their patients to exercise. From the report:

Research points to the benefits of physical activity for reducing the risk of chronic health conditions (1–4). Engaging in regular physical activity can reduce medication dependence, help maintain functional independence, and improve the quality of life for older adults (5,6). Physicians and other health professionals can be influential sources of health information, and exercise counseling by primary care physicians has been shown to increase patients’ participation in physical activity (6–9).

There was discussion about this yesterday on Twitter, stemming in part from a USA Today article on the report. And here’s the essence of the short-form debate:

Some suggested that doctors don’t tell patients to exercise because they, themselves, are overweight. Or it’s because they don’t feel comfortable recommending for others what they don’t do themselves. While this might explain some physicians’ behavior or discomfort with the topic, it can’t explain that of the majority.

So why don’t more doctors prescribe exercise for their patients?

Reasons I wonder about include a lack of time for “non-essential” communication, especially in clinics. In specialists’ offices, the omission of exercise could have to do with the visit’s purpose. A gastroenterologist or internist who evaluates a patient for a problem like diarrhea, say, might not think to ask about exercise. For some doctors it might be, problematically, an attitude issue — that they just don’t care that much, or think it would be a waste of time to discuss the matter of exercise.

Whatever the reasons are that most doctors don’t bring up the issue, one might ask this: Why do adults need doctors to tell them about the health benefits of regular exercise? After all, it’s common knowledge – the kind of thing taught in elementary school, like nutrition should be — that regular exercise is good for most people. As we age, being out of condition makes every task in life, like walking a few blocks, harder.

In an ideal world, we’d have most adults exercising regularly, and doctors who’d occasionally intervene and counsel patients about what they shouldn’t do because of a particular medical condition, like arthritis or heart limitation. I guess we’re not there yet -

All for this week,

ES

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Cystic fibrosis is an inherited disease that affects the lungs, sweat glands and, in males, the vas deferens — the tube that carries sperm from the testes. Patients are prone to infection (especially pneumonia), overheating, infertility and other problems. Most symptoms arise from defects in transport of ions, like chloride, through membranes. Cystic fibrosis is often disabling and, typically, life-shortening. In 1989, Francis Collins and Lap-Chee Tsu reported in Science they’d discovered the gene responsible.

Location on CFTR gene on human chromosome 7

Now, there’s a wealth of information about the Cystic Fibrosis Transmembrane Regulator (CFTR) gene. It’s located on human chromosome 7 at q (long arm) 31.2, and encodes an ABC (ATP-binding cassette) type ion transporter protein. According to an analysis in Nature this week, scientists have identified over 1500 CFTR mutations.

Kalydeco goes by the generic ‘ivacaftor’ and has been tested published reports as ‘VX08-770–102.’ This compound targets the defect caused by one mutation, termed G551D. Only 4% of cystic fibrosis patients have this genetic variant. Over 90% have a different mutation, F508del. These distinct mutations alter the protein distinctly. From Nature News:

… In patients with the G551D mutation, the channel fails to open properly, so ions are un­able to pass through. About 90% of people with cystic fibrosis have a different mutation, called F508del, which results in proteins that do not fold into their proper shape and so get targeted for degradation, reducing the number of channels…

Drugs in the pipeline will target the more common F508del mutation. Meanwhile, it’s easy to envision more than a few eager parents, patients and optimistic pediatricians wanting to prescribe the new drug for their cystic fibrosis patients who don’t have the G551D mutation. The number of people living with cystic fibrosis in the U.S. is 30,000, according to the Cystic Fibrosis Foundation, so the FDA’s approval applies to approximately 1,200 individuals — the 4% — who carry the G551D mutation.

This seems a perfect example of a new, expensive drug that may really help just a few people, and for which there’s a clear genetic marker. Now, if we could only assume the assay for the mutation will be done correctly in each case…

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The site offers a menu of “basics” about clinical trials, addressing topics like:

1. What are clinical trials and why do people participate?
2. What is clinical research?
3. Who participates in clinical trials?
4. What do I need to know if I am thinking about participating?
5. What questions should I ask if offered a clinical trial?
6. How am I protected?
7. What happens after a clinical trial is completed?
8. How does the outcome of clinical research make a difference?

There’s an imperfect glossary of terms, a selection of patients’ and investigators’ stories, information for kids in research, and other resources including a link to the clinical trials database:  ClinicalTrials.gov.

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Rachel Cheetham Moro (1970 – 2012) was a critical thinker who vigorously supported BCAction and the NBCC’s 2020 deadline. She was a generous and thoughtful on-line friend to many women in the metastatic and more general BC community, where she used the handle @ccchronicles. Her blog provided a running, witty commentary on breast cancer news and trends. Interspersed, she detailed occasional and lately, more frequent visits to the hospital, a Florida vacation, and reflections on her earlier years. In a recent post, she included this wonderful high school photo.

high school photo, from the Cancer Culture Chronicles

Dr. Susan Niebur was a mother in her late 30s, an astrophysicist and blogger who generously shared her experiences at her Toddler Planet blog and elsewhere, including on Twitter as @whymommy. She dealt with inflammatory breast cancer starting in April, 2007. In recent months she wrote less frequently, but  positively somehow, while taking radiation treatments for painful bone mets, going in and out of the hospital and, most recently, receiving hospice care at home.

Susan Niebur in 2011, Toddler Planet

Each of these women inspired many people I know. They were brave and open, and helped others to understand what it’s like to face progressive, metastatic disease. Their words didn’t only affect people with breast cancer, but influenced also their loved ones, and individuals who face all sorts of limiting illness.

Thank you, both, for what you’ve taught me about life.

ES