Thoughts on Jane’s 65th birthday

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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.

Putting the symptoms together

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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.
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.

The pain of NET Cancer Day

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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.

#30NETfactsin30Days

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November 30: #30NETfactsin30days

First purpose

30 Net Facts in 30 Days has taken five moths to pull together. It grew out of discussions with a number of people about one of the central problems facing NET cancer patients: Too few primary care practices even know the name NET cancer, let alone its symptoms. 

The day my wife and I heard her diagnosis, the doctor’s opening words were he had “never heard of this form of cancer before.” That was in August of 2010. Most newly diagnosed patients would likely hear similar words today, if their doctor were being fully honest with them. I meet PCPs and NPs with great frequency. I’ve had two dermatologists tell me they’d heard of NET cancer because of a rash that appears in the late phases of the disease. The rest plead ignorance.

Second purpose

But I wanted the campaign to do more than educate medical practitioners. The general public is equally unaware of the disease despite it having more patients–and frequently more diagnoses per year–than other diseases that have become nearly household words. As a result, wherever possible, we’ve tried to stick to everyday language.

Twitter posts have to be short, so most days I’ll post commentary as a separate post on this site to take things a bit further, if you’re interested.

How you can help

A new fact will be added to this post each day for the month of November. Those facts will also be posted on Walking with Jane’s Facebook page and on our Twitter page. Feel free to copy and post each fact as it becomes available on your own social media page.

And please share this information with the medical professionals in your life.

Daily Posts

​November 1: The number of new diagnoses of NET cancer in the US is rising faster than other cancers: an over 6-fold increase from 1973 (1.09 per 100 000) to 2012 (6.98 per 100 000). #30NETfactsin30days  Commentary

November 2: NET cancers begin in neuroendocrine cells, a type of cell found throughout the body with traits similar to nerve cells and hormone-producing (endocrine) cells. #30NETfactsin30days What are neuroendocrine cells?

November 3: NET cancers commonly start in the gastrointestinal tract (GI) and are the second most prevalent form of GI cancer in the US. While neuroendocrine cancers frequently start in the gastrointestinal tract, they can form almost anywhere, including—but not limited to–the lungs, thymus, testicles, and ovaries. What are incidence and prevalence?

November 4: Neuroendocrine cancers are very diverse. Many low-grade, well-differentiated neuroendocrine tumors are slow growing, but high-grade, poorly-differentiated neuroendocrine carcinomas can be aggressive. #30NETfactsin30days

November 5: NET cancers can produce hormones in life-altering quantities. Some, but not all, neuroendocrine patients have symptoms related to hormone production.    Commentary

November 6: Treatment options for neuroendocrine cancers depend on features, including pathology (differentiation status, grade), where and how far it has spread, whether there are symptoms related to hormone secretion. #30NETfactsin30days #NETcancerday #LetstalkaboutNETs

November 7: There may be many undiagnosed cases of NET cancer. Autopsy studies found NET cancer in nearly one percent of the population. That’s potentially 3 million undiagnosed cases in the US alone. #30NETfactsin30days #NETcancerawareness #Letstalkabout NETS #cureNETcancernow  (This is the one that keeps me up at night.)

Week Two

November 8: NET cancer patients are often given multiple incorrect diagnoses before being diagnosed. It often takes 5-7 years from first symptoms to diagnosis.  Recognizing these symptoms to make an early diagnosis is critical. #30NETfactsin30days  #NETcancerawareness #Letstalkabout NETS #cureNETcancernow  Commentary

November 9: Many NET cancers are diagnosed at an advanced stage after they have already spread. NET cancers discovered earlier are often discovered incidentally on testing done for something else.

November 10: Symptoms of NET cancer can include flushing, diarrhea, intestinal bloating, sudden mood shifts, high or low blood pressure, heart disease, insomnia… If it’s a symptom of too much of particular hormone, it could be NET cancer. #30NETfactsin30days #NETcancerday #LetstalkaboutNETs #cureNETcancernow Commentary

November 11: Patients with carcinoid syndrome can experience symptoms of flushing and diarrhea related to serotonin production by their tumors. Elevated serotonin can be detected using blood and urine tests (5-HIAA). #30NETfactsin30days #NETcancerday #LetstalkaboutNETs #cureNETcancernow

November 12: Constant diarrhea or 4+ loose stools per day is not normal. It can be a symptom of NET cancer and carcinoid syndrome. #30NETfactsin30days #NETcancerday #LetstalkaboutNETs #cureNETcancernow

November 13: If a woman is having hot flashes, it could be a sign of NET cancer, not menopause.

November 14: NET cancer can cause symptoms including pain, abdominal cramping, or cough that are related to the effects of the tumor on normal organs. Commentary

Week Three

November15: Another common site for NET cancers to start is the pancreas. Pancreatic NETs can also secrete hormones, including insulin, gastrin, glucagon, and others, with life-altering results.

November 16: A persistent cough is not always an allergy. Sometimes it’s a symptom of a NET cancer in the lung.

November 17: Jane’s Birthday–NET cancers may remain small before spreading. The cancers often spread to the liver where they can be numerous and large in size. Commentary

November 18: NET cancers occur nearly equally among men and women. Commentary

November 19: If a NET cancer is discovered early enough, surgery can sometimes offer a cure.

November 20: For many patients with advanced NET cancer, cure may not be possible.  But we have some drugs and procedures that can slow growth of disease and reduce tumor load and symptoms to improve quality of life. 

November 21: There have been improvements in the survival of patients with advanced neuroendocrine tumors in recent years related to approval of new treatments. (Most treatments for neuroendocrine cancers are relatively new.)

Week Four

November 22: Aretha Franklin and Steve Jobs both died from NET cancer. US President Dwight Eisenhower was discovered to have had a form of NET cancer (paraganglioma) after his death. Commentary

November 23: Elevated levels of serotonin in patients with carcinoid syndrome can affect the valves of the heart, usually causing the valves on the right side of the heart to not work normally. Carcinoid Heart

November 24: NET cancers can affect people of all ages. While it was once believed NET cancers were a disease of those over 50, we have seen increasing numbers of younger patients in recent years—some as young as nine.

November 25: While NET cancer was once considered a rare disease, it may have crossed the line for the legal status of that categorization (fewer than 200,000 patients).

November 26: NET cancer is not like other cancers. When you find one, seek out a neuroendocrine cancer specialist. Carcinoid.org maintains a list.

November 27: Multidisciplinary care–evaluation by specialists from multiple disciplines of medicine–is critical to formulating the best treatment plan for NET cancer patients.

November 28: Finding NET cancer early is a key to a long-term positive prognosis. There is currently no universal screening test for neuroendocrine cancer. It is one of the Holy Grails for researchers.

The last two

November 29: In most cases, we do not know why patients develop NET cancer.  In some patients, there is an inherited predisposition to developing neuroendocrine cancers and other endocrine disorders.

November 30: Pheochromocytoma and paraganglioma are rare forms of NET that form in nerve tissue inside (pheochromocytoma) or outside (paraganglioma) the adrenal gland. They can make catecholamine hormones.

My nightmare nights

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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.
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.

Neuroendocrine cancers: problems and dilemmas

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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.
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.

…the only curative therapy…

A large chunk of that kind of research funding is created by the NET Research Foundation (NETRF), though many NET cancer centers do raise and spend significant amounts to do that work–amounts that may, together, equal what NETRF supplies. The Neuroendocrine and Carcinoid Tumors Program at the Dana-Farber Cancer Institute, for example, raises over $1 million each year to fund research.

Barriers to diagnosis

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.

Death, incidence, prevalence–a NET perspective

awareness, Death, NET Cancer Awareness Day/Month

We know so little about NET cancer that we cannot even put an accurate number on how many people die from the disease each year. That level of ignorance scares me to death.

 

We know so little about NET cancer that we cannot even put an accurate number on how many people die from the disease each year. That level of ignorance scares me to death.

Diagnoses and deaths

About 328,030 Americans were diagnosed with gastrointestinal cancers in 2018. There were 165,460 deaths. That’s 27 percent of all the cancer deaths in the US. (These numbers come from a presentation at the Dana-Farber Cancer Institute Visiting Committee.)

…more research than we are doing now.

In contrast, 234,030 people were diagnosed with lung cancer and 154,000 died; 334,200 new breast cancers were diagnosed and 42,260 people died.

Death by the numbers

In terms of both diagnoses and deaths, colorectal cancer is the worst of the GI cancers. Last year, 145,600 Americans were diagnosed with it: 51,020 died. It is also among the worst cancers overall in terms of mortality–only lung cancer killed more Americans last year.

There were 165,460 deaths.

Traditional pancreatic cancer was diagnosed approximately 56,770 times last year; 45,750 died from it. It is the third most common form of cancer death in the US. (These numbers come from a presentation at the Dana-Farber Cancer Institute Visiting Committee.)

Where NET cancer sits

Liver/biliary cancer is the fifth most deadly form of cancer. With 35,740 deaths a year, it trails breast cancer by about 6500 deaths a year. But it is a far less common diagnosis at 54,400. (These numbers come from a presentation at the Dana-Farber Cancer Institute Visiting Committee.)

…only lung cancer killed more Americans last year.

As I wrote two days ago, we have no up-to-date numbers for NET cancer. The 12,000 cases a year cited in 2012 would put it behind both stomach cancer (27,510) and cancer of the esophagus (17,650) but ahead of all other GI cancers. Even assuming a five percent increase in diagnoses, which would take the number to 16,080, it would still trail those more frequently diagnosed cancers.

But no up-to-date numbers

We don’t have any recent statistics for how many people die from the various NET cancers each year in the US either.

…more frequently diagnosed cancers.

The one thing we do know is, in 2012, about 171,000 diagnosed patients were living with the disease in the US. We had two drugs approved for use in NET cancer patients at the time–and surgery for those whose disease was discovered early enough for that to be helpful.

Making a case for an increase

We’ve since added three FDA approved drugs, liver embolization procedures, and a radiation treatment called PRRT that had been used for some years in Europe. There are additional treatments in the pipeline.

We don’t have any recent statistics…

My guess is that 171,000 patients has increased over the last seven years, perhaps significantly.

Why second?

But even if it hasn’t, NET cancer would still be the second most prevalent form of GI cancer, trailing only colorectal cancer. The others kill too fast to build up that large a number of patients. Simply put, there are very few five-year survivors of pancreatic, liver, stomach or esophageal cancer.

There are additional treatments in the pipeline.

Colorectal cancer is also deadly, but so many patients are diagnosed with it that the net gain in living patients is about 100,000 a year.

Quality of life issues

Yes, NET cancer patients can–and often do–live for many years after diagnosis. I know many patients who have fought NET cancer since before Jane was diagnosed over nine years ago. But quality of life matters–and the quality of life for many NET patients is quite poor: constant diarrhea, insomnia, intestinal pain and bloating are just some of what those patients deal with constantly.

The others kill too fast…

Our treatments, other than surgery, are not curative. They offer symptom relief and slow the progress of the disease. Nor do they work for every patient.

And many suffer for many years before they get a diagnosis. It was 30 years between Jane’s first symptoms and her diagnosis. She was an outlier, but many go 5-7 years before being diagnosed.

Research and awareness matter

The problem is the tumors really can form just about anywhere–and the symptoms can vary based on where the tumor is located and what hormones it is producing. And sometimes, there are either no hormones to detect–or, perhaps, hormones we don’t yet know how to detect.

…many suffer for many years…

All of this underlines–again–the need for greater awareness among both medical professionals and the general public–and much more research than we are doing now.

Searching for the real NET number

awareness, NET Cancer Awareness Day/Month
An accurate number of NET cancer patients could tell us whether we are looking at a zebra or a horse with stripes.
An accurate number of NET cancer patients could tell us whether we are looking at a zebra or a horse with stripes.

#30NETfactsin30days: November 1

The number of new diagnoses of NET cancer in the US is rising faster than other cancers: an over 6-fold increase from 1973 (1.09 per 100 000) to 2012 (6.98 per 100 000). 

What’s the right number?

Few things about this project have been more frustrating than trying to figure out how many new NET cancer patients are diagnosed each year. The last “official” figures come from a 2012 study that found 12,000 new patients and a total of 171,321 diagnosed patients living with the disease.

…we all need to be more aware…

That same study cited an expected growth in diagnoses of five percent a year. For cancer, that’s a fairly high growth rate in diagnoses. Lung cancer cases, for example, are in decline. Most other cancers seem only to grow as fast as the increase in population. The number of breast cancer diagnoses a year, for example doesn’t vary much from year to year.

Complicating the potential number

At five percent growth in diagnoses a year, 2019 should end with about 16,900 new patients. That, of course, also assumes that awareness and diagnostic techniques are no better than they were in 2012. Since that year, we’ve seen two new PET scanning technologies begin to come on line–the Octreoscan and the Gallium-68 scan.

The last “official” figures come from a 2012 study…

We’ve also seen the development of a new 5-HIAA blood test that is lest cumbersome than the 24-hour urine test doctors used in 2012. That test has only begun being used very recently, so it’s likely not had much impact yet–but may well do so in future.

Why numbers matter

The patient population has also likely grown during that period as new treatments have become available–but that’s an issue for another day.

…it’s likely not had much impact yet…

The lack of up-to-date data would be less of a problem with a disease where the diagnoses don’t vary much from year to year. But we’re still trying to define the scope of the problem we face. More patients creates greater urgency for finding treatments–which means a greater need for research funding, as well as a greater need for oncologists with knowledge of how to treat this peculiar disease.

More importantly, it means we need primary care practices to have greater awareness of the disease than many do.

Meanwhile, in Kentucky

Globally, there is evidence that the number of cases is growing outside the US. But there is one piece of evidence that NET cancer diagnoses are growing at a greater than five percent a year rate in the US.

More patients creates greater urgency…

In an article published in Oncotarget in April, 2018, researchers looked at more recent data on NET cancer diagnoses for the state of Kentucky through 2015. They found an increase from 3.6 per 100,000 in 1996 to 10.3 per 100,000 in 2015.

A word of warning

Now Kentucky has a higher cancer incidence rate than most of the rest of the country across the board. And its NET cancer incidence in 2012 was 8.4 as compared to 6.98 per 100,000 for the US as a whole. But that’s a greater growth than five percent a year in that locality.

…10.3 per 100,000 in 2015

How big an issue NET cancer will become in the years ahead is an open question. Right now, we all need to be more aware of it–and of its potential.

Vote for Greta for Patient Hero

awareness, Personal statement, Uncategorized

My friend Greta is dying.

 She’s dying of NET cancer.

I can’t stop that. No one can. 

I watched the same disease kill my wife. I know with ghastly precision what Greta is going through—what her family and friends are going through.

Before this final turn, Greta was nominated for the Hero of Hope Patient Award. She’d like to win it. I’d like her to win it.

Her biography is at the link, if you need more than my endorsement to vote for her. 
So follow the link and cast your vote for her.

Today is the last day of voting.

Please.

https://conquer-magazine.com/2019-hero-of-hope-patient-award?fbclid=IwAR1jWAQdxJ8yIMHeDqZ2dd7LCcXIEo2KRhjhw8K99yDcdY09Tx1Qg-LO1zE 

My last arrow? I hate this dragon

awareness, Goals, Marketing NETs, NET Cancer Awareness Day/Month, Personal statement
I lost Jane nearly nine years ago. I'm still fighting her cancer--but I'm nearly out of arrows.
I lost Jane nearly nine years ago. I’m still fighting her cancer–but I’m down to my last arrow.

The arrow I have

I have to admit, I’m worried. In two days we launch a social media campaign on Facebook and Twitter aimed at PCPs, NPs, medical schools and the general public about NET cancer. It’s the last arrow I have in my quiver and I’m far from sure it’s enough to make this dragon even wince. But it’s the arrow I have, so…

I made a promise.

This campaign has to go viral in ways nothing I’ve ever posted–or written anywhere on anything–has done. It has to reach communities I’ve never figured out how to reach effectively. We’ve worked on it for months–and I’m still tinkering with it today–will likely still be doing so tomorrow.

Hoping for an avalanche

Earlier this year, I described what I’ve spent the last nearly nine years doing as standing on top of a mountain throwing pebbles, hoping to start an avalanche that would wake people up to the reality of neuroendocrine cancers.

It’s the last arrow…

I’ve watched in horror as the numbers of diagnoses and the number of patients has climbed year after year. We stand at the edge of NET cancer losing its rare disease status, yet it remains a disease neither medical professionals nor the public has any real awareness of.

Time’s arrow

It’s been a decade since Jane’s H1N1 flu battle opened the gate to her NET cancer’s closing act. It’s been nine years, two months, and 14 days since we knew what she had. We’re barely seven weeks from the ninth anniversary of her death.

…standing on top of a mountain throwing pebbles…

In that time, I’ve raised some money, inspired some people–thrown lots of pebbles–but NET cancer remains largely unknown and largely underfunded. And I’m down to that last arrow–that last pebble. And I’m afraid it isn’t going to be enough.

Death and failure

I’ve lost track of how many patients I’ve known have died this year. There have been too many. I know more will die before this year ends. Each one tears at my soul–each one reminds me we’ve failed–that I’ve failed to honor the promises I made myself when Jane died: to raise awareness so no one hears what we heard the day her doctor told us what she had–“I’ve never heard of this cancer before;” to create the money that would help to find a cure; to find the money that would make an early diagnosis common rather than an exception.

…down to that last arrow…

I thought all those things would be easy. How could anyone who heard Jane’s story not be moved to help? All I needed to do was write and speak and advocate. These were things I knew how to do–have done all my life.

Competing voices

And I failed. There are hundreds–maybe thousands of diseases our there with the same trouble. No one knows about them except the people who have them. No one cares about them except for those affected by them. A thousand things clamor for attention every day. No one has time for the things that don’t have a direct impact on their lives or the lives of their families.

There have been too many.

Starting Friday, we will try again. Over the course of 30 days, we’ll post our 30 facts and hope they get reposted and read and will do some good. I have a plan for a follow-up project in December and January if this even sort-of works.

And then?

And if it doesn’t…

If it doesn’t…I guess I’ll try to make a new arrow or look around for some loose pebbles somewhere.

…we will try again.

I made a promise.

–Harry Proudfoot,

President, Walking with Jane