It’s snowing on Giving Tuesday in New England. Warm some hearts by making some meaningful donations to organizations that need your help. I hope at least one of them will deal with NET cancer.
Things to think about on Giving Tuesday
I’ve spent a lot of time thinking about what to do with Giving Tuesday. Part of me just wanted to suggest people give to the big NET cancer foundations like NET Research Foundation and Carcinoid Cancer Foundation. I’m sending checks to both of them today.
…do what you can where you can.
But I made a promise to a patient before she died that I would find a way to raise awareness and educate primary care doctors and nurse practitioners about NET cancer, while also trying to raise awareness among the general public. It’s taken me some time to work out how to do that. The #30NETfactsin30days campaign was the first salvo in that effort. It–and the next step–are relatively inexpensive to do, but not likely to have a huge impact.
Raising PCP and NP awareness
Things that could have a bigger impact are expensive. We need a Continuing Medical Education video/course that will teach doctors the basics of NET cancer–and we need to market that program in the places medical folks will see it. We need PSAs for television and radio for the general public. Just the production costs for any of those projects are expensive, never mind what it costs to actually get something on the air.
I made a promise…
I’d really like folks to make donations today to the NET cancer cause. Make a decision to do what you can where you can. No matter what you do, I’ll be grateful. If you want to donate to Walking with Jane’s Laura Hoke Fund for Physician Education and Awareness you can find a link on our Facebook page. All donations made there go to that fund. Or, if you want to donate by check, you can send checks to Walking with Jane, P. O. Box 9721, Fall River, MA 02720. Put Hoke Fund in the memo line.
This day in November I remember the moment I decided to keep Jane alive when I could just as easily let her die. Doing so would have saved her 18 days of suffering. I know that decision made a positive difference in the lives of others. But i still wonder if I made the right call for her.
A moment
“Breathe with me,” I chanted as I held her hand in both of mine..
Her chest rose and fell, rose and fell, rose and fell.
And then it stopped.
I could have done nothing–perhaps should have done nothing. I could have just held her hand and let her slip away. The nurse was in the hall listening to rounds on the patient in the next room. The doctors were all focussed on that.
A decision
We’d talked about this moment–the moment she stopped breathing. But I wasn’t sure. I wasn’t ready.
I opened my mouth and called for the doctors and nurses. “Something’s not right here,” I said in a voice I knew would carry but not convey panic.
They came into the room. They put an oxygen mask on her face and put me behind a screen while they bought a few minutes to figure out what was going on and if her life was over.
Another moment
The hospitalist came onto my side of the curtain. “We think we know what is going on. We think we know how to fix this–how to give her a fighting chance. But we’ll have to intubate her. What do you want to do?”
And there it was–the thing we hadn’t planned for, hadn’t known to talk about–the vast area of gray where we had expected black and white–a simple decision that was no longer simple. But it was a decision I had to make quickly–one way or the other.
And so I chose to let her fight–to put off letting her die for another time–and hopefully another place somewhere in the distant future.
Consequences
Nine years later, I still don’t know if what I did was the right thing or the wrong thing in that moment. I only know I made the choice.
I know the good that came out of that choice. I know there are people who have had that same heart surgery who lived unexpected years because I made that choice. I know we became a beacon that had nothing to do with cancer for some other lives. I know Walking with Jane does not exist without that decision.
I suspect there’s a lot of money beyond what I’ve contributed directly that found its way into NET cancer research that might not have otherwise. I suspect some of the bright lights in the NET cancer community aren’t doing what they are doing if I make a different decision in that moment. I suspect there are dozens of positive outcomes for others because of what the following 18 days created.
Things are different
But I also know those 18 days are 18 days of suffering that Jane did not have to endure. I know how Orpheus felt at the top of those stairs one step away from joy–how it feels to have that joy thrown into endless night by a single turn–by a single moment.
I don’t know how one balances the one against the other–the human costs against the human outcomes. I can’t see how my life is different if I make a different call. I only know it is. I can’t say how the lives of others are different–for better or for worse–if I make a different call. I only know there are lives that are different.
A moment…
“Breathe with me,” I chanted as I held her hand in both of mine.
Her chest rose and fell, rose and fell, rose and fell.
And then it stopped…
And what followed changed things. That’s all I know
Jane went into her first coma on this date in 2010. The coma was caused by what doctors call a carcinoid crisis, which is when the NET cancer tumors suddenly begin producing hormones in even larger quantities than normal. In Jane’s case, the excess serotonin they produced crashed her respiration and blood pressure. Her doctors addressed this with fairly massive doses of octreotide. She would remain in a coma for about 35 hours. She would endure two more such attacks and comas–the last one resulting in her death on December 10.
People complain that Franklin and Jobs didn’t do enough to educate people while they were alive. If what they were going through looked anything like what Jane went through, I understand their reluctance completely.
Editor’s Note: If you are a NET cancer patient, you should not read what follows. It is filled with triggers that will not be good for you. If you are a lay-caregiver of a NET cancer patient, you should not read this for the same reason. If you are a widow or widower who has experienced the death of your other half as a result of NET cancer, you need not read this. You have already had the experience the piece describes and it will upset you, at best. It will have triggers in it you don’t need to deal with. I’m writing this piece for doctors and the general public, not for patients, lay-caregivers, or the bereaved.
I have seen Death in my life
I have seen death and suffering in my life. I spent part of my life as a police and fire reporter. I have arrived at fatal accident scenes moments behind–and once minutes ahead–of police and EMTs. I’ve walked into a burned out trailer with a fire marshal just after a fire was put out to find a charred body feet from the door. Those events were the stuff of my nightmares for years.
…much greater than anyone can imagine.
Then I sat the deathwatch with my wife and her sister as we watched my mother-in-law drown in the final throes of pulmonary fibrosis for three days and nights. That death was so ghastly her priest called it a martyr’s death. It drove the nightmares of my days as a journalist out and replaced them with new ones.
Nightmare diarrhea
Those nightmares lasted four years before being replaced with the images of my wife’s last days. Those images will never leave me. She did not drown, as her mother did. But what happened to her–and to me–was, somehow, far worse.
I have seen death and suffering in my life.
Aretha Franklin and Steve Jobs died from pancreatic NET. In some respects, it is a very different form of NET than the GI NET Jane dealt with. Their tumors were likely producing pancreatic hormones. Jane’s GI tumors were producing serotonin. The thing large quantities of some of them causes is food to be moved through the intestines very quickly, resulting in ongoing and massive diarrhea. I can’t be sure what happened in the case of Franklin and Jobs. I know what happened to Jane in too much detail.
What diarrhea does to dignity
Not having complete control over your bowels is worse than embarrassing in most human cultures. It reduces one, at times, to an infantile state. When that lack of control results not simply in a stinking stool in your underwear, but in diarrhea streaming down your legs and onto the floor, you feel worse than anyone can imagine. People can tell you it’s all right–that it’s your disease doing it–but that is the kind of thing that destroys your image of yourself.
…ongoing and massive diarrhea.
Many of us in the NET cancer community wish that Steve Jobs or Aretha Franklin had said something while they were alive about their disease. They could have done much to raise public awareness of the disease and how awful it is. They might have inspired more money for research that might have moved us closer to a cure. But they were proud people, living in a culture in a time and a place where we can only joke about what happens in a bathroom. It’s difficult to discuss, even with a doctor, being constipated. And diarrhea makes constipation look easy to talk about.
Simple human dignity
Jane was a proud woman. The night she went into the hospital for heart surgery to replace the valves her cancer had destroyed, they told her she had to go to the seventh floor of the building across the street. “I’m sorry,” she said to me, nearly in tears. “I can’t go that far. You’ll have to push me in a wheelchair.” She was visibly upset by that seemingly simple thing.
…they were proud people…
Jane hated the very idea of a wheelchair. We’d argued, sometimes loudly, about using one to get from one point to another in the hospital on earlier visits. It was an argument I’d decided I could not win. But I’d had to help her off the toilet at home that morning. I’d had to help her off the toilet in the Women’s Room at a rest stop on the way to the hospital. Her self-image and sense of dignity was in trouble long before the operation the next day set the stage for its absolute annihilation.
Diapers and dignity
Over the next four weeks, my wife became an infant. It destroyed us both. I replay those scenes over and over again in my mind. I look for things I might have done differently that might have made it better–might have left me with some small piece of a soul. There’s nothing there.
Jane hated the very idea of a wheelchair.
I diapered my first baby when I was six. I was the oldest of what would turn out to be six children. I put diapers on three of them–and helped by handing my mother diapers and powder on a fourth. I likely diapered the last one more often than my father did. Cleaning up an infant is often a stinky, messy job.
Changing Jane
Jane was my first adult. I knew she was having trouble. I came back from dinner one night to be told I should stay in the waiting area–that she’d had a bowel movement and her bedding and bedclothes needed to be changed. It took half-an-hour before they came to get me.
…my wife became an infant.
I don’t recall the exact details of what happened leading up to me helping change her. I just knew it was a two-person job and I wasn’t letting her lie in that mess one second longer than necessary. I told the nurse if he told me what to do, I’d help. He took me at my word.
A clinical description
I put on the gloves. I rolled her onto her side and held her there. The nurse did what he needed to do and I watched closely so I’d be able to do it myself if I needed to. I don’t remember the exact order of the process. He used the same kind of disposable cloth they use on babies to clean the diarrhea off her buttocks and legs. He rolled the sheets up close to her back. Then I lowered her back onto the bed. I walked to the other side of the bed, rolled her onto her other side. The sheets came off and he finished cleaning her up. Eventually, I lowered her back down on clean sheets in clean nightclothes.
Jane was my first adult.
I don’t know how many times I helped change her. She told me she was more comfortable when I was there to help. The nurses told me I got good at it–that they’d hire me. I like to think they weren’t just being nice. Even when they had someone immediately available, they let me stay in the room while they did it. It made Jane happy.
Breaking minds, destroying dignity
But it also broke her in ways that are hard to describe. She became emotionally more fragile and increasingly more child than wife. She became, for a time, completely paranoid–convinced the doctors and nurses were plotting something. She asked me to eat in the room rather than in the cafeteria so I could keep an eye on them. Periodically, I would drive back to Fall River to pick up clean clothes, cash a check, and pay the bills. She would be angry at me when I came back, even if I’d arranged for visits from friends and family for her while I was gone. Even if she were asleep much of the time. I’d left her alone for too long.
I put on the gloves.
And it broke me in ways that are hard to describe. You can’t help clean the shit from your other half’s body without it changing you. It deepens the relationship in unexpected ways at the same time it frays your soul. It felt like we were both creating these cool exteriors to hide the raw emotions that were coursing through us both. Even now, as I look at the description of changing her soiled sheets and gown, I see it so clinically–say it so clinically–knowing that to do otherwise would be to start crying and never be able to stop.
I live with it
It’s not that I don’t cry. I do–great hacking sobs that leave me drained emotionally and physically. In the midst of those episodes, I really do feel like the tears will never stop. And then they do–and I feel better for a time. Sometimes that time lasts for months–to the point I start to think maybe, at last, things will stay better.
She would be angry at me…
Then I come around a corner, catch something in my peripheral vision, wake up from a dream–sometimes a dream that has nothing to do with Jane or hospitals or mortuaries or cemeteries–sometimes for no reason at all–and it all rushes back in and I’m standing or sitting by that bed or rolling her on her side or looking out that hospital window…
Why we don’t talk about it
I understand why neither Aretha nor Steve talked about their NET cancer while they were alive. I watched what it did to Jane, year-by-year, month-by-month, week-by-week, day-by-day, hour-by-hour, minute-by-minute, second-by-second. They were all proud people who spent their lives in control–and who saw that control vanishing in the most embarrassing ways imaginable.
It’s not that I don’t cry.
And I understand why their families don’t want to talk about it now they are gone. To talk, even in vague terms, about what happened to the person they loved–and about what happened to them as they watched that person sliding into the abyss of diapers and a second childhood–comes at a heavy cost. It’s taken me nine years to put even this much down on paper and release it for public view.
Real courage, real dignity
Each of you reading this would do anything to protect those you love. Each of you would want to protect their image, even in death. We want the world to believe they died a brave, clean death filled with honor and strength and courage–and a kind of calm beauty.
I watched what it did to Jane…
Somehow, a bed soaked in diarrhea doesn’t create that image, for all that that reality makes what they endured so much greater than anyone can imagine. There may be little dignity in such a death, but it rehires far more courage more courage, far more patience.. And it is why NET patients and caregivers need more than sympathy or dignity–they need a cure.
When things get dark, I go for a walk in the woods. It clears my head, but rarely delivers answers.
The frustrations of teaching
I went for a walk in the woods two weeks ago with a good friend. We’d both had a tough week and needed some time in nature to try to put our heads back on straight. She teaches high school kids. A good part of what I do now is teach adults–or try to.
I still don’t know how to do what needs doing.
I find teaching adults far more frustrating than I ever found teaching high school students. Listening to my friend, it became clear that job has become far more difficult–largely because adults with no idea of what goes on in the classroom have increasingly forced their way into day-to-day practice. The paperwork now required would make it impossible for either Jane or I to do what we did.
What we think we know
Adolescents think they know everything. Adults know they know everything. Both groups are wrong. Sometimes teenagers can be reasoned with. Their brains are more fluid–more open to persuasion. They have limited experience and often have discovered how wrong they can be.
I went for a walk in the woods…
Adults… Adults believe their experiences have taught them everything already. They can’t perceive how the very different experiences of others have led them to very different conclusions. Worse, new facts or new evidence don’t seem to change their perspective very much. They believe what they’ve always believed because that’s the way they learned it over the course of their lives.
The problem with adults
Ultimately, both our problems stem from adults and what they’ve done or refused to do–or what they are doing and what they refuse to do. I admit, kids are crazy. But more often than not, adults made them that way. But kids can change fairly quickly and relatively easily with the right help at the right time–except when the adults refuse to let them.
Adults know they know everything.
My problem is I deal almost exclusively with adults. Their beliefs about everything were forged sometimes 40 years ago. Sometimes their beliefs are based on knowledge of that same age–knowledge that no longer is valid. We’ve discovered a lot of things over the course of my lifetime–things that have stood the world on its head.
What we believed vs. what was true
For example, about the time I was born, a computer scientist supposedly said he could not imagine why we would ever need more than about four computers on the entire planet. Others said flying to the moon was impossible–and even if it were, it would not happen in my lifetime. And cancer? We had no answers and never would.
We’ve discovered a lot of things…
Of course all those statements and ideas proved entirely untrue. I have a computer in my phone that is far more powerful than the best computer we had in 1952–and seemingly everyone has one. Cars all have computers in them. Every writer I know uses one nearly every day. Every teacher, too. No scientist could get through the day without one.
Moonshots
We’ve been to the moon and sent two probes beyond the edge of our solar system into interstellar space. We’ve discovered worlds spinning around distant suns and begun to perceive the structure of the universe.
…four computers on the entire planet.
And then there’s cancer. I hear several times a month from people that cancer research is the biggest boondoggle in history. “We’ve spent all this money and we still have no cure? The drug companies and the researchers are covering up the cure so they can make more money.”
Before there were cures
In 1952, childhood cancer was nearly an absolute death sentence. Childhood leukemia absolutely was. Breast cancer? Lung cancer? Get your affairs in order. And many people still think this is the case.
And then there’s cancer.
Sidney Farber wasn’t an oncologist. He was a pathologist at Children’s Hospital in Boston. He had the corpses of all these children arriving on his slab on a seemingly daily basis. It drove him to act. He’d go to parents of terminally ill children and get them to let him try chemicals that killed cancer cells in a test tube. Most did no good. But he kept trying.
The changes we don’t see
Today, 90 percent of children diagnosed with leukemia live, many into adulthood. We have a treatment in trials that seems to work for many childhood glioblastoma cases. I know a large group of women, including my younger sister, who are 20-year or more breast cancer survivors. We have cures for lots of forms of cancer–just not all of them yet. Those cures don’t work on every kind of cancer–don’t work on every cancer they are designed to take down.
Sydney Farber wasn’t an oncologist.
Once or twice a year I get to spend time with physicians and researchers working at the cutting edge of cancer. Sometimes, the news is almost too good to be believed–what’s happening with some cancers and immunotherapy leaves me speechless. Sometimes, the news is worse than I can begin to imagine. Every study, every experiment, every trial, adds a new piece to the puzzle, brings a new piece of evidence to the case. Sometimes those things open new lines of investigation. Sometimes they end up being dead ends.
Learning from failure
But we don’t know where something will lead until we try it. Early rockets blew up on take-off more often than people think who were not there to see it. But every failure led somewhere–taught us something we needed to know. People forget that some of our early Mars missions ended in complete failure. Today, we have rovers traveling that planet finding all kinds of interesting things.
Those cures don’t work on every kind of cancer…
Cancer research works the same way. Farber didn’t give up when his first patients died. He didn’t give up when the second group of patients died–or the third or the fourth… Success grows from failure more often than from immediate success.
What we now know
I keep telling myself that. It’s not helping very much this month. Nine years ago, my wife was dying from a cancer her doctor had never heard of, that few oncologists ever expected to see in their entire careers.
…every failure led somewhere…
Today, we know NET cancer stands second only to colon cancer in terms of patients in treatment for GI cancers. We know it stands as the most common of the rare cancers that make up about 30 percent of all human cancers. We know it stands on the verge of crossing out of the legal definition of a rare disease.
Nothing changed when much changed
But medical schools still don’t talk about it much. It appears on no national medical exam. Virtually no primary care physician or nurse practitioner knows it exists if they haven’t encountered a patient with it–a patient who likely heard the same words Jane did when she was diagnosed: “I’ve never heard of this kind of cancer before.”
I keep telling myself that.
I’ve worked at changing that for close to nine years now. Some things have certainly changed during that time. We have online support groups for patients; twoorganizations have put out books on the subject to help patients become more knowledgeable about the disease and better advocates for themselves; nationally, we’re raising–and spending– more than 10 times as much money for research as we were in 2010. I don’t pretend I’ve had a lot to do with any of that–much of it I’ve had absolutely no involvement in.
The other awareness
But awareness outside the NET cancer community has not changed at all. Many of us have shouted from the rooftops. Primary care physicians have not heard us. Nurse practitioners have not heard us. Medical curriculum groups have not heard us. The general public has not heard us.
But medical schools still don’t talk about it much…
Until that changes people will continue to be diagnosed with advanced disease we have no way of curing. We’ll only be able to ease their suffering some of the time when the drugs and treatments we have work. We may buy them some time with a better quality of life than they might otherwise have.
Changing ignorance
And there will continue to be patients who are never diagnosed; who will die without even the treatments we have that might help them with their symptoms, might get them to a graduation or a wedding or the birth of a grandchild.
Many of us have shouted from the rooftops.
We need to get those medical schools and nursing schools and medical curriculum designers to listen. They need to know the universe of neuroendocrine cancer exists–that what they think they know about the size of the problem is badly out-of-date–and dangerously wrong.
The walk in the woods calmed my mind but brought me no answers. I still don’t know how to do what needs doing. But I’ll keep trying. I hope you will, too.
By the time of Jane’s heart surgery, her cancer had consumed much of her liver. The next step in her treatment would have been to address that issue. Today, doctors might go after the primary tumor instead.
The birthday that might have been
Jane would have turned 65 today. We would have done something special. I’d have written her a poem, given her presents and flowers and a couple of cards. We’d have gone to dinner someplace we both liked. Given the milestone 65 represents, perhaps we’d have spent the weekend on the lake or flown somewhere exotic.
…the moment Jane stopped breathing.
Instead, I took flowers to her grave. The weather is raw and blustery. I didn’t stay long. I could hear her in my mind telling me to get somewhere warm. I woke up in the dark this morning. I immediately missed her. That happens most mornings–but this morning was harder than usual. I lay there with tears in my eyes.
Jane’s last birthday
Her last living birthday we spent in the hospital together. She was two days out of surgery and improving rapidly. They wanted to move her to the step-down unit, but there was no bed available. Instead, they began the process where she was. They took her off oxygen and removed the remote monitors. They began weening her off the other drugs she was on.
Instead, I took flowers to her grave
We had talked about what I would do once she moved out of the ICU. I’d check out of my hotel room the next day and head home. I’d visit on the weekend and maybe once during the week. Jane didn’t want me driving back and forth to Boston every day after work. She needed to focus on healing–not worrying about me driving home. Her doctors agreed: I’d just be in the way. Jane joked she’d gotten a new heart for her birthday.
Harbingers of death
Everything shattered a bit after 7 p.m. The night nurse came on and started running vital signs. That’s standard procedure in the ICU–or anywhere else in a hospital. What she found, she didn’t like. And things went rapidly downhill from there.
She needed to focus on healing.
Jane’s pulse-oxygen level had dropped into the upper 80s. It’s supposed to be well into the 90s–anything below 90 marks a problem somewhere. We were up all night. They did scans and blood tests and could find nothing to explain what was going on.
Carcinoid birthday greetings
What was going on was the first of four carcinoid crises likely triggered by the physical therapy and the weening her off the octreotide drip that helped fight off carcinoid crises in operative post-operative NET cancer patients. No one realized it at the time. It was one of the things Jane taught her doctors. Unfortunately, they didn’t figure that out until she was in her final coma.
Everything shattered a bit after 4 p.m.
Jane never lost consciousness that night. I suspect that was another piece of the carcinoid crisis as insomnia is a symptom of high levels of serotonin. But I chalked up her wakefulness to what was going on around us.
When your best isn’t enough
I wonder now how much brain damage she sustained that night. She was still recovering from the anesthetics from the surgery and was a little loopy to begin with. I never saw her pulse-ox levels dip below 80. But she was never quite right after that night. Still, Jane was always hyper-sensitive to anesthesia, so I just don’t know.
Jane never lost consciousness that night.
I just don’t know too many things from that time period. I second-guess everything I did and said during that final month of her life. I console myself with the knowledge I did the best I could with what I knew and believed at the time. But there was too much suffering there for the final outcome.
The cost of knowledge
I was at a funeral this week. One of the readings was the story of Jesus raising Lazarus from the dead. I know that act made his family and friends happy. But we never hear from Lazarus. I wonder where he was and what that miracle took him away from.
I second-guess everything
And I wonder what additional suffering I inflicted on Jane by calling her back from the edge of death on two later occasions before we let her go home. Those things haunt me. I know the good that came of those decisions. I know what the doctors learned, know how the nurses felt about what they saw. I know how those extra days have shaped my life since–for both good and bad.
Happy birthday or sad birthday?
But I just don’t know if I did the right thing–don’t know how to balance any of it. I doubt I ever will. I’m struggling with a lot of things–trying to figure out what I’m doing, whether I’m making enough of a difference for the energy I’m putting into things, whether it’s time to do something else.
Those things haunt me.
Today is hard. It is always hard. I remember how happy we were for so much of the day–and how rapidly things fell apart as day turned to night. I feel enormous bitterness and enormous sadness. And then I tell myself how little of the good that has happened since exists without that day and the ones that followed.
That thought does not console me. People tell me it should. But they have no idea what I lost–and no idea what the world lost–the moment Jane stopped breathing.
NET cancer was once considered a zebra among zebras. That meant it was well down the list of diseases doctors thought about when presented with a set of symptoms that today screams neuroendocrine cancer at me. Increasingly, I wonder just how much of a zebra this cancer really is. Sometimes, I think of it as a horse disguised in stripes.
Symptoms don’t always help
The focus of this week’s #30NETfactsin30days posts is largely on the symptoms of the disease. There are problems with that focus. The first is that many patients never exhibit any symptoms at all until the day they are faced with an intestinal blockage or some other life-threatening situation. Not every neuroendocrine cancer appears to secrete a hormone, so finding one based on looking for hormone issues does not always work.
We need your help, too.
And even those that do secrete hormones can go for years without producing more than vague, irritating symptoms that people learn to cope with. That vagueness also complicates things for doctors trying to decipher what is going on. Lots of things can cause intermittent bouts of diarrhea, lots of things can cause flushing, lots of things can cause wheezing…
The specialist problem
To further confuse things, patients may see one specialist for one set of symptoms and remain mum about those same symptoms with another specialist in another field. My wife Jane, for example, talked with a gastroenterologist about her stomach issues and her gynecologist about her hot flashes. The one told her she had irritable bowel syndrome, likely brought on by the stress of teaching high school students, since it seemed to subside over vacations. The other blamed that flushing on early menopause.
…many patients never exhibit any symptoms at all…
That Jane also suffered from regular bouts of insomnia she largely kept to herself–blaming her love of coffee and tea for it–compounded the problem. All those things taken together scream NET cancer to me today. Hindsight is a wonderful thing. But none of her doctors had the ability to put all those symptoms together because they were only seeing parts of the story.
Zebra symptoms
Even had they seen all of her symptoms, though, they still were unlikely to come to the conclusion she had neuroendocrine cancer. As Jane’s heart surgeon said to me the night before Jane died, the first thing we teach young doctors is, when you hear hoofbeats think horses not zebras. That means look at the most common diagnosis for a set of symptoms and treat that first. Only if that treatment does not work should you think about other causes–and the next disease on the list is unlikely to be a zebra.
… they were only seeing parts of the story.
“Unfortunately, a zebra killed your wife,” he finished.
A zebra among zebras
And neuroendocrine cancer was a zebra even among zebras for Jane’s doctors. It was considered thought so rare that Congress eliminated funding for research into it in 1968. They didn’t come back to it until 2008, when they funded a conference of doctors with an interest in the subject to talk about what they would do if there was federal money to fund something.
…the first thing we teach young doctors…
Congress funded actual research for the first time since 1968 in 2010.
If you don’t suspect it…
It was thought so rare that apparently medical schools didn’t it mention it much, if at all. Even today, I regularly hear from both doctors and nurse practitioners the same kind of thing Jane’s doctor told us the day she was diagnosed: “I’ve never heard of this form of cancer.”
…a zebra even among zebras…
The Carcinoid Cancer Foundation has a wonderful aphorism about NET cancer that applies to almost any relatively rare disease: “If you don’t suspect it, you can’t detect it.” It’s the absolute truth. No medical practice orders tests for a disease unless they have strong reason to suspect that disease is present. But it is impossible to suspect a disease you’ve never heard of.
Raising awareness of symptoms
Part of what the #30NETfactsin30days campaign is about is raising consciousness among the doctors and nurse practitioners who are most likely to encounter neuroendocrine cancer at an early stage when swift intervention could make a real long-term impact on the lives of patients.
“I’ve never heard of this form of cancer.”
But part of it is also about reaching out to potential patients. Jane had symptoms for 30 years before diagnosis. She was in superb physical shape. She played singles tennis every morning of every summer up until the summer of 2009. She often played another couple of hours of doubles with men in the afternoon. We walked 2-5 miles every day from spring to late autumn. We frequently went on 20-30 mile bike rides on ancient steel-framed bikes. She had the same body fat as an elite female athlete.
Looking too good to be sick
Despite all that, she had regular bouts of diarrhea, painful stomach bloating if she ate later than 5 p.m., frequent insomnia, blood pressure low enough that she sometimes passed out getting up from the toilet, hot flashes from her late 30s to the end of her life that never went away, a persistent rash on her face she covered with make-up and never gave a second thought to…
Jane had symptoms for 30 years…
She looked too healthy to be sick, felt too healthy to be sick. She didn’t look or feel like cancer was eating her alive. But it was. And it wasn’t all in her head.
The price of ignorance
We need better ways for doctors to communicate what they are seeing to other doctors; better ways to coordinate diagnoses; better ways to bring the supposed zebras to the attention of the frontline doctors and nurses who see patients earliest and most regularly.
She looked too healthy to be sick…
The consequences of our ignorance are enormous for both patients and their loved ones. Jane died less than four months after being diagnosed. By the end, she’d been stripped of every ounce of human dignity she possessed. The person I was died with her that night. The future we dreamed of died that night.
We need to do all we can to prevent that from happening to anyone else. I work at it as hard as a broken soul can. I’m not enough. We need your help, too.
People tell me my wife was beautiful. I certainly thought so. But the beauty of her mind and soul dwarfed her physical beauty. NET cancer could not touch those.
The Tenth of November
I never look forward to World NET Cancer Awareness Day. It reminds me of too many things. It is the day of the month on which Jane died, though she died in December and not November. But the tenth of every month tends to be difficult.
We need to kill NET cancer.
Still, November 10 is worse than all but December 10. It was the night before what would prove our last trip to Dana-Farber together–the last night that had any sanity in it at all. Not that there had been much sanity in our lives for more than a year.
The price of ignorance
The H1N1 virus had hit Jane particularly hard in October the year before. She’d missed a month of work between it and the pneumonia that followed. In truth, she never fully recovered from that.
…the last night that had any sanity…
I wonder how many years that flu cost us. Sometimes I think it opened the door that let the NET cancer she’d unknowingly been fighting for 30 years gain the upper hand. Other times, I think the NET likely weakened her enough to let the H1N1 really kneecap her. Probably both statements are true. Both things killed her, though the cancer was always going to be the root cause.
The face of NET
She was in tough shape on November 10. We both knew things were coming to a head. The thrice-daily octreotide injections were doing little to thwart the diarrhea, the bloating, or the swelling that had started in her legs that spring and had spread to her abdomen. She’d stopped trying to go up and down the stairs to the cellar. She’d stopped helping with the cleaning, though she did still manage to cook dinner much of the time.
…she never fully recovered…
She was a better cook than I was, so I cleaned up most nights. When I cooked, she cleaned. Now, it didn’t matter who cooked, I did the dishes. We’d always shared the yard work and the housework. She didn’t like that those tasks now all fell on me. She told me she felt guilty. I told her she’d do the same for me if I were the one fighting cancer.
Talk in the face of NET
We talked that night. She listed the things we needed to make sure we asked the next day. I added a couple of things. My official job was to make sure we came away with answers to everything, as wells taking the notes. My bigger job was staying supportive and positive no matter what we heard.
She told me she felt guilty.
We talked, too, that night about other things. We always did. We talked about our days, what we’d read, what our students were doing. The long drive to and from Boston sat on the table in front of us. It stayed quiet, as did the cancer once we’d finished with it. We both knew they were there.
Miracles needed
I can’t say what she felt that night. I know I was terrified. She’d lost too much weight, too much strength. She was dying and there was nothing I could do but hold her hand, massage her feet, and hope the long-odds approaches both we and her doctors had in mind would work. I put on a confident face. She did the same.
We both knew they were there.
We didn’t need a miracle–we needed several. It turned out we needed miracles we didn’t even know we needed–that the doctors didn’t even know we needed. It was November 2010–and what we knew about NET cancer with certainty could be reduced to a 3×5 index card. And some of that was wrong.
Long day’s journey into NET
The next day, the traffic was awful. But we came away hopeful. We met with a dietician who gave us some thoughts on things Jane might eat that would help. Jane’s doctor wanted to set up a procedure for the following week to draw out some of the fluid from Jane’s abdomen that would alleviate some discomfort and help get a handle on what was going on.
She was dying…
But when we got home–after three hours in traffic–I had to carry Jane up the stairs. It was the first time that happened. We chalked it up to each other as the end result of a long day and too much time sitting in traffic.
NET consequences
The next day, Jane’s heart surgeon called. A month before we’d talked about surgery “after the holidays.” Now, after seeing the results from the day before and talking with Jane’s other doctors, he wanted to do it Monday or Thursday. We went with Monday. That meant driving to the hospital late Sunday afternoon
I had to carry Jane up the stairs.
I’m not sure Jane would have made it until Thursday now. Given the month that followed, I’m not sure she would not have been better off. She would have died at home in her own bed on her own terms. But we both wanted her to live–to have a fighting chance–to reach the goal she’d set the day she was diagnosed.
What it was worth
She had the surgery. Her heart was worse than they expected. With a lesser surgeon, she would have died on the table. The doctors learned a lot in the days that followed. The people around us learned a lot in those 25 days. I learned a lot in those 25 days.
…to have a fighting chance…
But it cost her more than I can say. And I have never been the same. The tenth of the month has come around 107 times since I caught her last breath on my lips.
The deepest wounds
I did not cry that night. I was too numb–too numb for a long time. And I have work to do. But the tenth of every month breaks open every wound and leaves me with tears and memories–memories of the end.
…it cost her more than I can say.
We need to kill NET cancer. It tears apart the soul of the patient who has it–tears apart the souls of those they love.
My landscaping nightmare helps me deal with the nightmares that await me in the dark of my bedroom. The project outside reminds me that every nightmare has a solution–but the solution often requires lots of though and lots of work. Equally, it requires thinking about the worst-case scale of the problem.
What keeps me awake
Three things keep me up at night. The first derives from the empty space beside me where Jane is supposed to be. I’ve tried for years to describe what that is like–how many nights it makes me reluctant to go to bed and deal with that reality.
Jane’s story becomes the norm.
I won’t go into that again here. I’ve written too much about grief, here and elsewhere, over the years. Not much has changed over the eight years, ten months, and 29 days since Jane died. I’ve just gotten better at coping with the pain.
The diagnostic nightmare
The second thing that keeps me up is the reality that too many patients go through too many incorrect diagnoses before learning the cause of their suffering is neuroendocrine cancer. Despite the improvements in awareness and scanning technologies, patients still go through years of shuffling between doctors and multiple diagnoses that tell them they have something else.
…the empty space…
I wonder how many patients die of NET cancer believing they have something else. Jane could have dropped dead in front of her students with a piece of chalk in her hand–and I would have been told she died of heart failure or a stroke. Her students told me later of episodes in her classroom her last year teaching that sound like a stroke–sudden changes in her voice, in her handwriting, moments of confusion…
Why diagnostics matter
The valves in the right side of her heart were dying. The serotonin from the tumors in her liver was not being metabolized. Instead, it was frying her valves into a brittle, fibrotic mass that leaked fluid into her legs and abdomen. We wouldn’t learn about that until September.
…too many incorrect diagnoses…
Her liver was toast–90 percent of it eaten by neuroendocrine tumors. It was a marvel she could walk, let alone climb stairs or teach. And then, she couldn’t.
How many don’t know?
By the time Jane was accurately diagnosed, it was really too late to do much of anything. We tried anyway. Doctors tried to treat the symptoms, tried to decrease the tumor load, tried to repair the damage to her heart, made plans to try to address the liver problem. And we made plans for the years ahead we thought she might find a way to enjoy.
Her liver was toast…
I wonder how many patients die without knowing what they had? How many die without the treatments that might ease their suffering? How many loved ones think they know what killed their wife, husband, mother, father, child–and are wrong? Not, mind, that it makes much difference in most respects: loss is still loss.
Autopsy studies
The number may be bigger than we think because the number of patients is potentially much bigger than we want to think. In February, 2010, eleven months before Jane died, researchers published a paper on the results of an autopsy study.
We tried anyway.
They hadn’t gone in looking for anything in particular. They just wanted to see what was there. They found neuroendocrine tumors in close to one percent of the bodies. I encountered that study only after Jane had died.
Backing up that study
Three years ago, I had lunch at a NET cancer conference with a doctor from a major medical center. He told me one of his colleagues was engaged in a similar study–and finding similar results.
…researchers published a paper...
Slightly less than one percent doesn’t sound like a lot–until you scale it up. The US population is roughly 330 million. One percent of that is 3.3 million. To me, that’s a big number. And it scares me.
The stuff of nightmare
We can’t say with certainty what the number of undiagnosed neuroendocrine cancer cases is. Three million is a worst-case scenario. But it is the stuff of my nightmares.
And it scares me.
Primary care doctor awareness is enormously important. Nurse practitioner awareness is enormously important. ER personnel awareness is enormously important. Catch this cancer early in its progression and surgery can create a cure.
Catch it late, and Jane’s story becomes the norm. It’s a norm that I would wish on no one. And it is too often the case. It, too, is the stuff of my nightmares.
The NETwalkers Alliance raises money through the Boston Marathon Jimmy Fund Walk for neuroendocrine cancer research. Morgan (left) has walked the entire route the last two years with Walking with Jane founder Harry Proudfoot (right) who lost his wife to NET cancer in 2010. Morgan was the last teacher Jane mentored and has been involved with Walking with Jane from the start.
Neuroendocrine cells
All neuroendocrine cancers come from neuroendocrine cells. Neuroendocrine cells are the way the nervous system and the endocrine system communicate and create the hormones and peptides our bodies need to do everything from build muscle to digest food to run away or stand and fight. They are involved in everything our bodies do to survive and thrive.
Lives depend on that attention and awareness.
Neuroendocrine cells are found just about everywhere in the body. As a result, neuroendocrine cancers can appear almost everywhere in the body. On the surface, at least, that makes them different from the ways we think about most cancers. When a cancer occurs in the lungs, we call it lung cancer. When a cancer happens in the pancreas, we call it pancreatic cancer. When we get cancer in the liver, we call it liver cancer.
Matters of body geography
But a neuroendocrine cancer in the lung, pancreas, liver–or anywhere else–is often a very different disease from the “normal” cancers that occur in those organs. They may have very different symptoms. They can’t be discovered using the same tests and scans. They frequently don’t respond to the same chemo and radiation treatments.
…neuroendocrine cancers can appear almost everywhere…
And some, but not all, produce hormones in significant enough quantities to have an impact on body chemistry. Those changes can speed up digestion, slow respiration, speed up heart rate, raise or lower blood pressure, change moods–essentially affect anything a hormone or peptide can affect.
Testing for neuroendocrine
Tests for hormone levels can be difficult to administer, as well as expensive. Until recently, for example, testing for serotonin, a hormone commonly produced by neuroendocrine tumors, involved a patient collecting all of their urine for 24 hours. That urine would then be sent to a lab to be individually analyzed for chemicals related to the breakdown of serotonin. Based on those levels, the lab would then extrapolate how much serotonin was being produced and whether or not that fell into normal ranges.
…an impact on body chemistry.
And no doctor is likely to order tests without better cause than a fishing expedition based on a group of vague symptoms that may or may not be explained better by something else.
More complications
Another complication in diagnosing and treating a neuroendocrine cancer is the very number of possible hormones involved and their impact on an individual patient’s symptoms. High serotonin levels produce very different symptoms from high adrenaline levels. And each may require a different treatment regimen.
Tests…can be difficult to administer…
As if that were not bad enough, neuroendocrine cancers come in at least two very different structural types. NET cancers are low grade, well-differentiated tumors that often grow slowly. That slow growth may be part of the reason traditional radiation and chemo therapies don’t work well on them.
The carcinoma difference
Neuroendocrine carcinomas, on the other hand, can be very aggressive. They are high grade, poorly differentiated structures. Their aggressive nature may make them more open to more traditional-looking therapies. But the success rate is not particularly high.
As if that were not bad enough…
In truth, for both types of neuroendocrine cancer, the only curative therapy we have is surgery. And that only works when the cancers are discovered quite early. Otherwise, our treatments can ease symptoms and/or slow the advance of the disease.
Researching neuroendocrine
Neither neuroendocrine cancer form is well understood. And we simply lack the funds to do enough fundamental research into their biology to create the knowledge that could lead to either early detection or a cure.
Two significant barriers exist in the fight against neuroendocrine cancer. The first is awareness among primary care physicians and nurse practitioners about the disease. The Carcinoid Cancer Foundation uses the phrase, “If you don’t suspect it, you can’t detect it” to underline why physician knowledge and awareness matter. I would go one step further and suggest that doctors can’t suspect what they’ve never heard of. In too many cases, too many doctors and nurse practitioners have never heard of the disease.
…much research funding is created by NETRF
The second barrier is public awareness. Neuroendocrine cancer is the second most prevalent GI cancer in the United States. Only colon cancer has more diagnosed patients. Yet few people outside the neuroendocrine cancer community and their immediate families have ever heard of it.
Raising Awareness
I talk with doctors and nurses in a variety of settings. Even many oncologists have never heard of neuroendocrine cancer. I speak in public settings with some frequency. I am too often greeted by incredulity that such a cancer can have flown so long under the radar.
Two significant barriers exist…
Neuroendocrine cancers need a greater level of awareness and attention from both doctors and the general public. Lives depend on that attention and awareness. The #30NETfactsin30days Campaign is an effort to create that greater awareness.
