Think –and dress–zebra today

Today is Rare Disease Day. Wear zebra if you have it. If you don’t, then find some.

Do more than wear zebra.

Why zebra? Because doctors are trained to look for horses not zebras when they hear hoofbeats. By that they mean that symptoms are more likely to indicate a common disease rather than a relatively rare one. Most of the time, a bloody nose is just a bloody nose and a cough is more likely from a cold.

Why Rare Disease Day matters

But a cough can be cystic fibrosis. A bloody nose can be a brain tumor. And persistent belly pain and diarrhea can be NET cancer. As the Carcinoid Cancer Foundation puts it, “You can’t detect what you don’t suspect.”

Their efforts often seem puny…

Today, we want everyone to remember that sometimes hoofbeats don’t mean horses–they don’t mean round up the usual suspects. They mean, “I’m a zebra–and I am here to kill you.”

The problem with being a zebra

I watched my wife be kicked to death by one such zebra. By the time we figured it out she had just four months left to live. But she had been fighting that zebra for 30 years while her doctors kept looking for horses. Unfortunately, her zebra was one most had never heard of. We are working to change that.

Fox’s kind of celebrity activism is relatively rare.

But even when there is great awareness of a particular rare disease there is often too little doctors can do to help the patient beyond putting a true name on what they have. Research into rare diseases is rarely well-funded. Drug companies are not much interested in funding diseases they can’t make a profit on. Governments are pressured into funding the big name diseases by their constituencies. Let’s face it: there are more votes in breast cancer, lung cancer, and prostate cancer than there are in NET cancer, neurofibromatosis, and Morsier Syndrome.

Rare diseases and cost-benefit analysis

We hear terms like cost-benefit analysis all the time. They are used to explain why we fund research for this disease but not for that one. But when you have a loved one who is a victim of that line of thinking the idea does not provide any measure of solace.

We hear terms like cost-benefit analysis all the time.

Despite the fact I would not wish any of these rare diseases on anyone, I sometimes secretly wish someone famous would find themselves or their families facing a particular disease too many other families are facing. I look at what Michael J. Fox has done for Parkinson’s Disease sometimes with a sick kind of envy.

Rare disease activism

But Fox’s kind of celebrity activism is relatively rare. Wendy’s founder Dave Thomas and Apple founder Steve Jobs both died from versions of NET cancer. Neither of their fights with the disease has made much difference in the fight against NET cancer. As someone who lost his wife to that particular rare disease, I can understand why they and their families did not get more involved in raising awareness about NET cancer.

I watched my wife be kicked to death by one such zebra.

When you have NET cancer every ounce of your energy–and the energy of your family–goes into fighting what you have. The death can be so horrible at the end the family cannot think about the disease without being drawn back into that whirlpool. I wonder sometimes if my own grief process would not have been better off if I had just walked away from NET cancer after Jane’s death. Working on cancer in general instead would have kept me from daily interactions with the disease and might have let me bury those final memories. Instead, they remain fresh in my mind.

The rare disease bake sales

But that is a road not taken at this point. It is a road many families who lose loved ones to rare diseases choose not to take. They organize bake sales and bike rides and entertainments to raise the money to support research into the rare diseases they are either afflicted with or have a loved one afflicted with. Their efforts often seem puny compared to the massive breast cancer walks that seem to pop up everywhere these days.

I’m a zebra–and I am here to kill you.

But the efforts of those folks as they struggle to raise what amounts to a rounding error on what we spend on the more well-known diseases are anything but meaningless to the researchers struggling to find treatments and cures for these orphans in the storm.

Every dollar counts

In December, I met with Jennifer Chan, who was Jane’s oncologist at the Dana-Farber Cancer Institute. I was there to deliver our final check for last year–a check that took Walking with Jane’s donations to the Program in Neuroendocrine and Carcinoid Cancer to over $49,000 for 2012. I know exactly what it took to generate that seemingly tiny figure.

…doctors are trained to look for horses not zebras…

But I also know just how important that sum is to work on this one rare disease–and how important all the small sums that are raised by small groups of dedicated individuals are to all the researchers who work on the non-glamour diseases that don’t get much press or much attention.

Today is a day to act

Today is Rare Disease Day. Do more than wear zebra. Pick a rare disease–one that doesn’t get much attention–and do what you can to help.

Mourning my mother

My mother died of Alzheimer’s about ten months before my wife Jane died of NET cancer in 2010. When my mother died Jane had already begun the steep decline that would lead to her death and there was little time for me to mourn her passing.

I am in a horrible and lonely race…

And Jane’s death has so overwhelmed me that I have allowed it to obscure my mother’s death and the grief I should feel about it. The truth is, I try not to think about my mother’s death because Alzheimer’s terrifies me–and I saw just enough of how it destroyed my mother to be far more frightened of it than of death itself.

Alzheimer’s beginnings

My mother loved to read, she was a powerful writer, and a woman who enjoyed creating art, whether on stage or on canvas. She kept the checkbook balanced and enjoyed playing with numbers in all their forms. She was a fan of cooking shows because they gave her new recipes to try to new foods to experiment with. There was nothing passive about her—any more than there was anything passive about Jane.

I can imagine no greater torture…

She began her battle with Alzheimer’s in her early 60s—only a year or two older than I am now. Jane noticed something was off about her during my parent’s first visit to our new house, but my father managed to keep us all in the dark until my youngest brother got him drunk enough one night to confess what was going on with mom. Even then, my father worked to keep her at home—until she pulled a knife on him.

Alzheimer’s power

The last time I saw my mother she still knew who I was. My parents had come out to visit my aunt and uncle in New Castle, PA and Jane and I drove down there to visit. But much of the woman I knew had already disappeared. She’d lost her understanding of numbers by then—and with it a substantial chunk of her independence. Cooking requires numbers—as does handling any kind of money. Her patience with anything was long gone.

I understand genetics well enough…

The written word was also beginning to slip away. She no longer wrote and reading was becoming increasingly difficult. Eventually, it all became squiggles on a page—and finally, not even that.

The long knowledge

When he finally was convinced he could no longer take care of her, my father searched out the best Alzheimer’s facility he could find. He visited her virtually every day—except for the periods he was hospitalized because of his kidneys and heart by-pass surgery. People comment sometimes on how much I loved Jane and how well I took care of her. Compared to my father’s treatment of my mother in her last days, I am a miserable failure.

…she knew precisely what was happening to her.

My mother’s battle with the disease was so long and so drawn out that she knew precisely what was happening to her. I can’t imagine not being able to understand numbers—to watch my ability to add, subtract, multiply and divide slowly slip away—and knowing those vital skills were vanishing before my knowing eyes.

The written word

And I expect that it was far worse to lose the power to write and the power to read—and to know those things were going away every bit as inexorably as the numbers had.

Eventually, it all became squiggles on a page…

I don’t want to deal with my mother’s death because it is more than likely my own. I understand genetics well enough to know there is a good chance I carry the same genes that sparked her Alzheimer’s. I know she did crosswords and jigsaw puzzles and number puzzles—all the things that are supposed to hold that nastiness at bay. But it destroyed her, just as it did one of her sisters and one—or more—of my great aunts.

My Alzheimer’s terror

There are a handful of things I am good at: I can analyze, I can think logically, I can learn, I can write, I can speak, I can teach. Those are the tools I have built my life with. Take them away and I can do nothing. Take them away and I am nothing. And the cruelty of Alzheimer’s is that you have to knowingly watch them disappearing—fully aware for years what is happening and completely unable to stop it from happening. I can imagine no greater torture for someone like my mother—for someone like me.

There was nothing passive about her…

I remember visiting my grandparents back in the mid-1970s. They had a next-door neighbor who had lost his wife some years before. He had recently developed full-blown Alzheimer’s but continued to live alone in the house he and his wife had shared. I remember his plaintive howls in the night that no one could comfort. And I worry those will be my howls.

My personal race

There is increasing terror in that memory just as there is increasing terror in the thought of my mother’s death. I am in a horrible and lonely race: will my mind stay powerful enough long enough to help find the answer to the NET cancer that killed my wife—or at least to put together something capable of doing that after I am gone?

My mother died of Alzheimer’s…

I have no answer to that—only the certainty that I have to keep running the race for as long as my body and mind will let me.

NET cancer financial appeal

Most of the time I try to use this space to educate people about NET cancer, Jane’s struggle with it, and what we are trying to do to help find a cure. But twice a year I make a direct, formal appeal here for donations to help us find answers to the riddle that is NET cancer.

I’ll be thankful for your help.

It was cold when I got up this morning. It was cold when I went out to drop off some letters. It will be hot chocolate weather tonight and I will have some while I watch an hour or two of television before retreating to bed.

NET cancer creates widows and widowers

But I will do all of that alone. Since Jane died of NET cancer in December of 2010, I do almost everything alone. I shovel snow, I buy groceries, I clean the house—all things we once did together without thinking about how we would miss even those seemingly inconsequential moments if one of us died.

I hate asking people for money.

I remember August 16, 2010 vividly. It was the day they told us about Jane’s NET cancer—a form of cancer her doctor had never heard of—and her first oncologist had never seen. There was nothing average about her cancer. There was nothing average about the days leading to her death. And there has been nothing average about the days since.

Only one way to kill late stage NET cancer

No one had ever beaten late stage NET cancer, but Jane was determined to be the first. In the end, she beat it the only way anyone ever has—by dying and taking it with her. We would have tried anything, but the truth is there was nothing to try except a series of operations with very long odds of success.

Cancers viewed as zebras get little funding respect…

We tried anyway. And her doctors told me when it was over that they had learned a lot from her case—but even more from her indomitable spirit.

The NET cancer zebra

We know a lot more about NET cancer than we did when Jane was diagnosed. But we still have no cure. We still have no reliable way to diagnose the disease. And the average primary care physician knows as little about it as Jane’s did. They call NET cancer a zebra—a disease more likely to show up on TV’s House than in an actual practice.

…we still have no cure.

But there are 120,000 people in the US who know they have the disease—and no one knows how many more have it and will never know, even as it kills them. It looks like so many other diseases that the average NET cancer patient is incorrectly diagnosed three times before someone gets it right.

The finances of NET cancer

Jane was misdiagnosed seven times and was seeing the doctor about what looked like a fluid retention problem when they finally got it right. She could just as easily have been told she had heart disease and have that on her death certificate instead of the real cause.

No one had ever beaten late stage NET cancer…

Cancers viewed as zebras get little funding respect either. Last year, about 12,000 people were diagnosed with NET cancer in the US. Total spending on NET cancer research amounted to less than $5 million. About 12,000 cases of cervical cancer were diagnosed last year. The American Cancer Society alone budgeted over $16 million for research on that. They spent about $1 million on NET cancer.

But while about 12,000 people died in 2012 of NET cancer, only 4,220 died of cervical cancer.

My tin cup is out there

I hate asking people for money. I’d like to resolve all our research needs with the stroke of a pen. But I am not financially rich and–barring I hit the lottery—I’m not going to be.

I remember August 16, 2010 vividly.

So I’m here with my tin cup to ask for your help. I’ll promise you this: All the money Walking with Jane raises will go to finding a cure for NET cancer and educating doctors about it or to the American Cancer Society to research all cancers and help support cancer patients and their families as they fight the long lonely battle against cancer. You can choose where what you donate goes.

The NET Cancer Walker

And whatever walk I am on–whether the Marathon Walk or the Relay for Life–I’ll be thankful for your help.

It was cold when I got up this morning.

To quote Helen Keller: Alone we can do so little; together we can do so much.

Pax et lux,

Harry Proudfoot

Chairman, Walking with Jane, Inc.

Walking with Jane, Inc. is a 501(c)(3) non-profit charitable corporation

organized under the laws of the Commonwealth of Massachusetts.

Fighting winter storms of love

The snow wiped out my monthly extra visit to Jane’s grave on the tenth of the month. It tried to do the same to my plans to spend part of Valentine’s Day in the cemetery with her. Twenty-six months and four days after her death from NET cancer and I am still nowhere close to being where we both expected I would be by now emotionally.

Friendships I can have, but romantic and physical relationships remain beyond me…

Last night I got notes from two students who were both, I think, stunned that I had not moved further down the trail of dealing with my grief. One even suggested I should be thinking about getting into a relationship at this point–that Jane would want me to be happy.

The sources of happiness

But happiness is not determined by whether I am single or not. Neither Jane nor I believed being a couple was the key to happiness. Rather, we saw doing meaningful work as the single most important thing where that was concerned. Not that our relationship did  not bring us vast reservoirs of joy–it did. But we had both been single a long time before we met and had clear expectations we were likely to remain that way all our lives. We knew happiness was not exclusively the possession of those who wore rings on their fingers.

But the lack of social spontaneity is equally untenable.

In fact, much of what we saw around us argued the contrary. We had both seen marriages that floundered from one unhappy event to the next before dissolving into pure despair and divorce. Marriage was a daring act for anyone. And for us, coming so late to the party, it was even more difficult. For those in their mid-to-late-30s with fully formed personalities and habits, marriage means an intense period of adjustment fraught with all kinds of peril.

Logical love

Jane and I knew the risks of trying to combine our lives but made a conscious decision to make the level of commitment marriage requires. And we worked very hard to evolve what we had beyond where it began. Every day was an act of conscious love, both for each other and for the world we lived in. We had access to each others souls that I cannot explain because I do not fully understand it myself.

Every day was an act of conscious love…

How we could have so badly miss-read what all of that would mean for the other if one of us died is a thing even now I have difficulty comprehending. Part of it had to do with a deeply shared belief in the gradual perfection of the human soul over multiple lifetimes–that we had shared lives in the past and would do so again in the future–but it does not fully explain that miss-reading of how I would react to her death. Plato described true friendship as one soul in two bodies but I have yet to find an analogy that works for true lovers.

The hunger for physical and spiritual love

The hunger for a renewal of that kind of relationship is sometimes unbearable. I am starved not only for the sensation of physical touching but for the touch of that other soul as well. I crave both in the same way a junkie craves heroin or cocaine–and a part of me would settle for a steady supply of even the most watered down version of either one. I am consciously terrified of an unregulated or unexpected hug because of its seductive power. Even someone taking my hand at an unguarded moment  has enormous destructive potential.

…happiness is not determined by whether I am single or not.

So every social interaction is carefully considered–almost choreographed in my mind before it happens. But the lack of social spontaneity is equally untenable. I am afraid that I too often come across as either overly cold or severely psychologically wounded–and both those things, in themselves, can send the very mixed signals I am desperate to avoid.

The shadow of love

But until–and unless–I can emerge from the shadow cast by the love we shared with each other I have to be very careful how I interact with others. Friendships I can have, but romantic and physical relationships remain beyond me, however much a part of me desires it were otherwise. I have to continue to deal with those feelings and desires the same way I dealt with my earlier craving for alcohol in the days just after Jane’s death. I consciously avoided even a glass of wine until I reached a point the glass of wine was no longer a craving but a thing over which I could exert full conscious control.

Twenty-six months and four days after her death…

Relationships that extend beyond friendship may someday be possible for me again. I think Jane would like that–at least she said so before she went into the hospital. But I will not ask anyone to live in the shadow of what we had–including myself. That would be a profanation of who we were and who I am and what I believe.

Wearing zebra print clothing

NET cancer is considered a rare disease. As a result, I post some things on our Facebook page–and elsewhere–about other rare diseases and about Rare Disease Day, which this year is February 28. Part of the effort to raise awareness of rare diseases is a campaign to get people to wear zebra print articles of clothing on that day, much as the American Heart Association tries to get people to wear red on February 2 to raise awareness about heart disease in women.

…sometimes a zebra is far more deadly than a lion.

That sounded like a reasonable idea to me, so I began posting about it a couple of weeks ago. Then I got a note from a friend last night asking how getting people to wear zebra print items was going to help people with rare diseases. It was a good question. The note got me to realize that while there were lots of pieces of PR making the rounds about wearing zebra none of us were really doing a very good job of explaining to people why we wanted them to do that.

Why a zebra?

Doctors refer to “rare diseases” as zebras. It comes out of their training in medical school and as interns: “When you hear hoofbeats, think horses, not zebras.” The mantra is there to remind doctors the most likely cause of a particular symptom is a common illness rather than a rare one: a cough is more likely a chest cold than it is lung cancer, more likely a standard lung cancer than mesothelioma–the type caused by exposure to asbestos–or cystic fibrosis.

…early discovery is important for all rare disease patients.

But just because a disease is rare–in the US that means there are fewer than 200,000 people directly affected by the disease–does not mean no one has ever heard of the disease. Cystic fibrosis and ALS–the disease often referred to as Lou Gehrig’s Disease–are rare diseases by that definition. Yet it would be hard to find an American–let alone a doctor–who is not aware of each of those. A month does not go by that I don’t get a solicitation looking for money to support research into both.

It’s not the size of the zebra

Unfortunately, for every “rare disease” people have a significant awareness of, there are hundreds, perhaps thousands most people–and most doctors–have never heard of. And it has little to do with relative prevalence. Cystic fibrosis, which has a large and effective foundation raising funds for it, for example, was responsible for about 3,700 deaths from 1999-2006. Over seven years it killed about one-third as many people as NET cancer kills in a single year. Cystic fibrosis affects about 30,000 people in the US–and 70,000 worldwide. ALS affects about 30,000 people at any given time in the US–about a quarter as many people as have been diagnosed with NET cancer.

Doctors refer to “rare diseases” as zebras.

Both cystic fibrosis and ALS are terrible diseases. I have seen both up close and would do nothing to cut the funding for either disease. But I have a long list of other rare diseases I have seen up close that affect more people but do not get the attention or the funding they need from either the general public or the federal government.

Rare diseases and doctors

More importantly, awareness among doctors of most other rare diseases is also very low. They may see the disease but not recognize it as easily as they might cystic fibrosis. And like cystic fibrosis, early discovery is important for all rare disease patients. The sooner the disease is spotted, the sooner whatever treatments we have for the disease can start–and that can make a significant difference for the quality of life those patients’ experience.

NET cancer is considered a rare disease.

We need everyone to be much more aware of the many lesser-known “rare diseases” that are out there. Greater public awareness will lead to more funds so that we can do more research. Greater physician awareness will lead to earlier diagnosis, earlier treatment, and a better quality of life for both patients and their families.

Join the zebra herd

So please, find some kind of zebra patterned item you can wear on February 28. Encourage the people you know to do likewise. Educate yourself about a rare disease or two that you have not heard of. Help us raise awareness not only about the NET cancer zebra–but about all the zebras that are out there–because sometimes a zebra is far more deadly than a lion.

Five days of new sadness

I’ve just come back from the funeral of a 30 year-old woman who should have lived another 50 years. She’d earned a masters degree in English and was taking the courses she needed to apply to medical school following a first bout of cancer a few years ago. We had talked last fall at the funeral of her photography teacher who was also a colleague of mine. He died of brain cancer, though he had fought off at least two other cancers previously.

…the greatest failure comes when one fails to try.

My student had just come out of the hospital a couple of weeks before but she looked good and we made plans to meet for coffee a few weeks later. Unfortunately, she got hung up on an errand and we missed each other. She had planned to attend the Relay for Life Kick-off Dinner in late January, but didn’t make it. The weather was tough that night—and maybe she knew then that there was something going wrong.

The winter of my discontent

She died unexpectedly last Friday. I got an email from another former student Saturday morning.

It is about saving them all.

I’d already begun to slide into grief earlier in the week. Winter is not an easy time for me since Jane’s death. The house is too quiet. The neighborhood is too empty. The cold makes walking difficult. And the absence of sunlight seemingly too easily leads to Vitamin D deficiencies that can’t always be made up by supplements.

The reality of grief

Grief is not like what they describe in the scientific journals. There is no magic wand that relieves it after the first year, nor apparently after the second. Gradually one becomes better at coping with the emptiness, the guilt, and the anger—but they do not seem to vanish.

He wants to save them all.

I know our friend did not die of NET cancer. She died of another of the seemingly endless varieties of the disease. That her cancer is better funded than NET cancer made no difference. In the end, it killed her just as dead as Jane’s NET cancer killed her.

Every cancer has deadly potential

The truth is that even the cancers we know the most about are still deadly too often.  I have another student whose daughter is fighting one of the childhood forms of leukemia. Thirty years ago she would probably be dead. Today, 90 percent of children diagnosed with her form of leukemia are cured to the point hey can look forward to a normal lifespan.

I have always hated that story.

But for 10 percent of those little boys and girls nothing we have works.

Cancer and cost-benefit analysis

In the world of cost-benefit analysis the bean counters would consider a 90 percent cure rate, “good enough.” But it is not good enough for the doctors, the nurses, the technicians, or the researchers. It is especially not good enough for the children and their families who are in that 10 percent.

The beach is covered in starfish…

Some years ago there was a story making the rounds of teacher conferences about a little boy on a beach. The beach is covered in starfish and the little boy is picking them up and throwing them, one at a time, back into the ocean.

The starfish story

A man comes by and derides the boy for his efforts. “You’re wasting your time,” he says. “You can’t save them all.”

 …for 10 percent of those little boys and girls nothing we have works.

The boy picks up another starfish and throws it into the sea: “But I saved that one,” he shouts at the man.

I have always hated that story.

Saving every cancer patient

I have always hated that story because the boy’s reply is born out of frustration. The truth is he isn’t satisfied with saving a single starfish, or half the starfish, or 90 percent of the starfish. He wants to save them all.

Grief is not like what they describe in the scientific journals.

But he has to shut the bean counter up or he’ll begin to believe the bean counter. And then the starfish will die. And the lives of the starfish all matter to the boy.

Teaching and cancer research

Teaching is not about being satisfied with saving one child from ignorance. It is about educating them all. Medical research is not about saving one person from disease. It is about saving them all.

Winter is not an easy time for me…

Neither teachers nor researchers can afford to be bean counters. They cannot be concerned with cost-benefit analysis. They, like John Donne, are concerned with mankind—and know that every death, whether mental or physical—diminishes them.

The reality of failure

Both know they will fail sometimes, but both know they cannot consider the possibility of failure if they are to do their best work. No matter how much it hurts to fail—nor how great the odds of failure are—the greatest failure comes when one fails to try.

…maybe she knew then that there was something going wrong.

In the starfish story the man leaves the boy to his starfish. He has learned nothing. Perhaps I would have liked the story better if the man, inspired by the boy’s words, began helping to throw the starfish back into the sea.

The battle against cancer ends when we have cures for every cancer for every patient. Help us save some starfish.

Begging in the street–and elsewhere

There are few things I enjoy less than asking people for money. Unfortunately, for the last seven days that is exactly the activity I have been engaged in. Were it not for Jane’s death from NET cancer twenty-five months and 22 days ago I would likely have spent that time reading, walking, and working on one of the books Jane and I had planned to write after we retired.

I hate what NET cancer did to her.

And if NET cancer research were well-funded and supported by large foundations on the scale of breast cancer, I might be contemplating a run for the state House of Representatives or the US senate seat vacated today by John Kerry–or at least be nearing the end of putting together the journalism book I have always wanted to write. No doubt I would still be working on the Greater Fall River Relay For Life, but I would not feel the need–or the pressure–to focus additional energy on raising money and awareness for this little-known form of cancer.

NET cancer and my despair

Nor would I feel an obligation to immerse myself in the kind of research that brings me into daily contact with just how slender our resources are and how that affects the progress toward a cure. That knowledge is especially painful because every day brings me into contact, as well, with those who currently have NET cancer. To look into their eyes, to read their words, and to know what I know is a recipe for  a special kind of madness that is equal parts hope and despair.

...dying and taking it with them.

I know that if nothing changes each of those patients faces the same death I watched Jane struggle through. I know each of them will kill their NET cancer the same way Jane did: by dying and taking it with them.

NET cancer tears

Jane wept when I told her the night before she died that there was nothing left they could do. She could not talk–she did not have the strength–and I have wondered since if I did the right thing in telling her. I do not know if those tears came from pain at what I had told her or joy that she was finally going to be done with all the embarrassments. She was a proud woman–and the diarrhea she could not control had stripped her of every ounce of dignity.

There are few things I enjoy less…

I hate what NET cancer did to her. I hate what it will do to all the other patients I have met. I hate what it did to me. And I hate what it does to the families and their lives.

My hatred

I hate begging for money. But I have spent the week doing exactly that because I hate NET cancer even more.