Telotristat study results for the rest of us

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Before telotristat

Octreotide and Lanreotide have been the go-to drugs for patients with NET cancer’s carcinoid syndrome for the last several years. They work by blocking the receptors on the tumors that cause them to secrete serotonin. Serotonin causes the symptoms carcinoid syndrome patients face: flushing, insomnia, diarrhea, bloating, and light-headedness, among others.

…I am not a doctor…

In some respects, the most immediately concerning of the most common symptoms are those involved with the digestive tract. The bloating leads to a loss of appetite; if you feel full, you don’t eat. If you don’t eat, you lose weight. When you do eat, the syndrome pushes the food through so fast there is no time to absorb the calories, let alone the trace elements, the body needs to function.

Syndrome creates social and physical issues

Frequent diarrhea–think several episodes a day, every day–also does bad things to the patient’s social life, as well as mental health. A life governed by how closeness of the nearest bathroom eventually leads patients to just stay home.

They work by blocking the receptors…

In the longer term, the excess serotonin causes a fibrosis of the intestines, destroying them the same way it destroys the valves in the right side of the heart. That fibrosis compounds the digestive problems by making it even harder for the intestines to absorb the nutrients the body needs to survive.

Somatostatin analog issues

Octreotide LAR and Lanreotide are long-acting somatostatin analogs. They were a big step forward over daily injections of the initial form of Octreotide. That form now gets used largely as a rescue drug for when symptoms return between the monthly injections of the longer acting drugs.

Frequent diarrhea–think several episodes a day, every day….

Unfortunately, for reasons that are not well understood, none of the somastatin analogs works for everyone. And they may eventually stop working even in those patients who have a positive response.

Telotristat: a different approach

Enter telotristat ethyl, a drug that comes in pill form that inhibits the production of tryptophan hydroxylase (TPH). Tryptophan–the infamous stuff in turkey meat that supposedly is the cause of Thanksgiving naps–is one of the things you must have in order to make serotonin. Slow–or stop–the creation of tryptophan and you slow the creation of serotonin.

…none of the somastatin analogs works for everyone.

A  of telotristat involving 135 patients showed that, “Among patients with carcinoid syndrome not adequately controlled by somatostatin analogs, treatment with telotristat ethyl  was generally safe and well tolerated and resulted in significant reductions in BM (bowel movement) frequency and urinary 5-hydroxyindole acetic acid (5-HIAA urine test).” (Items in parentheses added for clarity.) Doctors use the 5-HIAA urine test as a means to measure the amount of serotonin the body produces by looking at a by-product of it being metabolised in the liver.

Finding the right tool

We’ve known since 1967 that inhibiting TPH could improve symptoms for carcinoid patients. But previous drugs that would do that crossed the blood-brain barrier and resulted in patients becoming depressed. Too little serotonin in the brain is just as bad as too much elsewhere, it turns out.

…the infamous stuff in turkey meat…

Telotristat, for a variety of reasons, doesn’t cross the blood-brain barrier. Some patients did experience depression while using telotristat, but it was not clear, based on the results of this study, that the incidence was any greater than that of the patients in the placebo arm. It ?????????????????????????????????????????????????????????????????did not cause them to drop out of the study.

Telotristat impact

At the start of the study, the mean number of bowel movements per day ranged from 5.2-6.1. For the average patient, that number decreased by nearly three BMs per day–essentially cutting the number of BMs in half for many patients.  5-HIAA urine levels also showed a dramatic decrease: 78 percent of patients saw 5-HIAA levels decrease by more than 30 percent.

…doesn’t cross the blood-brain barrier.

The study did not look at fibrosis in either the intestines or heart but suggested, given the dramatic lowering of 5-HIAA levels, that a future study look at that.

Telotristat side effects

With any drug, there are potential unwelcome side-effects. We’ve already mentioned depression. Some patients experienced some increases in liver enzymes, while others experienced nausea. Neither was at a level that caused patients to drop out of the study, but longterm follow-up of these side effects is planned.

…essentially cutting the number of BMs in half…

Based on the results of this trial, the drug went the FDA for approval in the US and for approval in Europe.

A step forward

Having watched Jane inject herself with octreotide twice a day after her diagnosis–seemingly to little effect–I’m all in favor of oral medications whenever possible. Octreotide certainly did not control her symptoms. This absolutely looks like a significant step forward to me.

We’ve already mentioned depression.

But there are some other things to keep in mind: I am not a doctor and none of the above constitutes medical advice. I’ve read the paper and have run this by my friends at Dana-Farber to make sure I haven’t badly messed up in putting this in layman’s terms, but you really need to talk to your doctor about this, or any other treatment.

Matt Kulke, head of the Program in Neuroendocrine and Carcinoid Tumors at the Dana-Farber Cancer Institute served as the lead author on the paper that appeared earlier this month in the Journal of Clinical Oncology on telotristat ethyl, a new drug in the fight against NET cancer.
Matt Kulke, head of the Program in Neuroendocrine and Carcinoid Tumors at the Dana-Farber Cancer Institute served as the lead author on the paper that appeared earlier this month in the Journal of Clinical Oncology on telotristat ethyl, a new drug in the fight against NET cancer.

(Editor’s notes: The text of the original paper as it appeared in the Journal of Clinical Oncology can be found here. My thanks to Matt Kulke at the Dana-Farber Cancer Institute, lead author of the paper, for his help in making sure I’ve got it all right, medically speaking.  

(The last two posts in the ongoing 30 Day Challenge were not posted to many sites as they did not address anything patients or lay caregivers generally need to be concerned with. If you want to see them, they can be linked to from our Facebook page or on the NET Cancer News menu, where they appear in the order they were written.)

(This post is going up a day later than planned because of a commitment I had to take a friend to Boston. That ended up a 14-hour day and I was just too beat to want to do more than stare at a TV screen for a bit when I got home.)

(The post scheduled for today will go up sometime tonight.)

 

 

 

Quiet? Quiet? I wasn’t built for quiet

awareness, Grief, NET Cancer Awareness Day/Month

My day without NET

I had a quiet day yesterday. In the morning, a friend and I drove out to Cape Cod to see another friend inducted into the Hall of Fame of an organization he volunteers for. On the way out, we caught up on the little things going on in our lives. On the way back, we talked about another friend dealing with brain cancer. We are part of a group that gets him to his appointments and such.

…it didn’t matter what cancer took Jane.

After we parted, I went to a local mall to do some shopping. I felt tired, so I got a cup of coffee and sat at a quiet table and listened to the Christmas music. I watched families coming and going. Some went to visit a lonely Santa in the middle of the mall.

Time without memories

Jane and I used to walk there on cold winter mornings or hot summer days when walking outside was more than either of us wanted to do. But I didn’t think about that as I sat there. I drank my coffee and watched and listened. I wondered if this was what normal was like when you’re a widower on a quiet Saturday afternoon.

 I watched families coming and going.

After a while, I got up. I had some things I wanted to get. I bought a new charging wire for my phone so I could have one in the car and not have to remember to take the one from the house when I went out somewhere. Next, I bought a dietary supplement to help with the still-unexplained neuropathy in my feet. I went into an antique store and saw the price on a table I like had gone down another $10.

Quiet distractions

I saw a store I like has begun a going-out-of-business sale. I’m friendly with the manager and staff there. They told me they all had other jobs waiting for them with other companies after the store closes. But I will miss them. They’ve become a part of my limited social circle–and a part that lets me forget for a few minutes about Jane’s death because they never knew her.

I drank my coffee and watched and listened. 

I came home and ate and had a glass of wine. The phone rang and I spent two hours talking with an old friend in Canada. His wife seems to have fought off breast cancer. He gets a piece of what I’ve encountered: he knows the fear when you hear those words about someone you love. He lives in western Canada now, and we rarely get to talk.

A quiet awakening

He hung up to have dinner and I read for a bit. I did a little writing and checked to make sure nothing existed in my inbox I needed to deal with. Again, I thought, this must be what the world is like for a widower who has lost his wife to a “normal” disease. Yes, the house is too quiet and the rooms too empty. Maybe he walks on a Relay for Life team or does the Jimmy Fund Walk. But maybe his life is not consumed by cancer.

I came home and ate and had a glass of wine.

And then, I woke up this morning. I thought about all the people I know fighting to survive other types of cancer. I thought about a friend going in for a biopsy next month. I thought about the news I got Friday night that a former student had died of breast cancer last week. At that moment, I remembered who I am and the way I deal with things.

Grow where you’re planted

The truth: it didn’t matter what cancer took Jane. In a sense, it was better it was something rare that almost no one was doing much about. Breast cancer, pancreatic cancer, lung cancer, prostate cancer, all have legions of people working on them. NET cancer needed me–and, perhaps, I needed it.

…I remembered who I am…

Otherwise, I might be sipping coffee in a mall somewhere, watching other people go about their lives. It would be a quiet life–but not a very productive one.

Sometimes I need a quiet hour or two where I don't think about NET cancer. Working on a landscaping project, going on a trip, or sitting in a mall can sometimes create that space. And sometimes. I need that quiet more than others.
Sometimes I need a quiet hour or two where I don’t think about NET cancer. Working on a landscaping project, going on a trip, or sitting in a mall can sometimes create that space. And sometimes. I need that quiet more than others.

Challenge sometimes is just keeping up

awareness, Boston Marathon Jimmy Fund Walk, Fundraising, Marketing NETs, NET Cancer Awareness Day/Month

What this challenge is teaching me

I think I need some help. This challenge has reminded me how difficult–and how undermanned–our fight against NET cancer remains. I write pretty quickly, but each of these posts represents at least three hours of writing time. Then I spend another hour putting in links, break-heads, and pull quotes before posting it to various pages and support groups. And often, before I write, there is research that needs to be done.By the time even one of the shorter pieces is ready for your eyes, it has 4-5 hours of work in it. I’ll spend another hour or two fielding comments and thanking people for sharing pieces every day.

If you’re interested, let me know.

I needed to update this website this month, as well. In reviewing what we have, I realized that most of the information on the other pages here is over a year old. I need to find the time to look at more up-to-date videos and check to see which links to other pages still have value–or still work. And lists of foundations and blogs and cancer centers all need updated as well. Gradually, I’m beginning to understand why most foundations and organizations have at least one person–and often more–doing nothing but social media and website stuff.

The fundraising challenge

But maintaining this website and keeping up a social media presence are not all I have on my plate. Walking with Jane tries to average one fundraiser every month. Most of those require a press release for local media outlets. Most also require I design and print posters and get them up in local businesses. Many events require tickets get designed and printed as well. Money gets counted and taken to the bank. The bookkeeping required even for an organization as small as Walking with Jane, makes me crazy.

I needed to update this website this month…

Then, we do three mailings major mailings a year and three smaller ones. Our holiday mailing works purely to bring people up to speed on what we’ve done over the course of the year. The other two major mailings prospect for individual donations to our Jimmy Fund Walk effort. The smaller ones recruit crafters and others for our annual yard and craft sale–and sponsors for events like our Jimmy Fund Walk t-shirts and our miniature golf tournament. I craft every letter and every envelope gets stuffed by hand. More bookkeeping follows.

Other WWJ challenges

Next, we go prospecting for grants to help fund the awareness piece. Next year, we hope to send out copies of two pamphlets to every primary care doctor in New England about NET cancer. If we can find the funding, I’d like to reach every primary care doctor in the US and Canada. I’d go beyond that if I could find the money and someone to do the translations.

…we do three mailings major mailings a year…

Throw in Form 990, an annual board meeting, several craft fairs, a summer gathering for volunteers, and you begin to see the scope of the challenge on just the Walking with Jane front. But I have other NET cancer irons in the fire.

The Dana-Farber challenge

I also chair 3-in-3:The Campaign to Cure NET Cancer for the Dana-Farber Cancer Institute’s  Program in Neuroendocrine and Carcinoid Tumors. I meet with doctors and staff there once a month to plan and execute the various parts of that effort. That has included designing a presentation aimed at businesses, civic and fraternal organizations. We are also creating brochures and public relations materials. A series of podcasts may happen next year. We also look for other ways to raise awareness of NET cancer and the latest research.

…we go prospecting for grants…

That work also involves serving as group leader for the #cureNETcancernow group and captaining our NETwalkers Alliance team for the Boston Marathon Jimmy Fund Walk. Next year, I will also take on at least part of the Pan-Mass. Challenge as we try to start a Program-focussed team for that biking event. Fortunately,  some overlap exists there since much of the fundraising we do goes to support Walkers on our teams.

The training challenge

Of course the Walking–I do 26.2 miles every year–and now the biking, require year-round training at my age. I do more in the spring and summer than I do in fall and winter, but I try to get some vigorous exercise every day. In the summer, I try to do at least one 20 mile hike a week, leading into the Marathon Walk at the end of September.

I also chair 3-in-3…

I also try to spend some time every week talking individually with NET cancer patients and monitoring the various support groups. I try to read a few NET cancer blogs each week as well. And, of course, I have the scientific papers to wade through.

The life challenge

Meanwhile, I somehow find time to have something that looks like a life. I work on the landscaping, spend time in my garden, read what Jane used to call “mind-candy,” and take some photographs. I like to cook and bake my own bread. And I write things other than NET cancer posts and try to keep up with what is going on in the outside world.

…I do 26.2 miles every year…

So it’s no wonder I always feel like I’m running behind. I’m clearly trying to do too much. And yet, I can’t see any way to drop any of this. It all needs doing and most of the people I know who could help have equally daunting schedules in front of them. We all do what we can.

Can we get a little help here?

Now if you’d like to get involved with any of this stuff, I need whatever help I can get. Some people have written pieces about their experiences with various therapies. Others have sent me their crafts to take on the road to the various craft shows I attend. All the money from those goes to NET cancer research. I have friends who come in to stuff envelopes a couple of times a year.

I’m clearly trying to do too much.

Interested? Let me know.  You can reach me at walkingwithjane@gmail.com. As one of our ministers used to say, “Many hands make light work.”

We try to average one fundraiser every month. That is just one of the challenges involved in ou fight to fund NET cancer research.
We try to average one fundraiser every month. That is just one challenge involved in ou fight to fund NET cancer research.

Caregivers have different set of problems

awareness, Caregiving, Death, Grief, Jane, NET Cancer Awareness Day/Month

The weight of caregiving

We focus most of our energy–and rightly so–on the needs of patients. Their lives are at risk and we need to find ways to help them. We need treatments to ease their symptoms, slow the course of the disease and, ultimately, cure it. Many of us, when we hear the word caregivers, think of doctors and nurses and technicians–at least I always did until Jane got sick.

They are fighting for their lives.

But the bulk of the day-to-day care of someone with a chronic or lingering or debilitating illness falls on ordinary people. Lay caregivers get no specialized training. You simply wake up one morning and discover your entire world has changed. Suddenly, you need to learn the details of a disease you may have heard of but know nothing about. Sometimes, you draw a disease like NET cancer about which no one seems to know anything. You have to juggle a job while taking on all the chores a significant other either did or helped with–as well as your own.

The emotional and physical toll

In an instant, you become both the emotional and physical support of someone you care deeply about. Their pain becomes your pain. You bottle up your own pain and negative emotions because the last thing the person who is sick needs is to see what their illness is doing to those around them.

Lay caregivers get no specialized training.

People tell you to take care of yourself so you can keep helping the patient, but no one really tells you how to do that in a practical sense. And even when you figure out how, you feel guilty because the person you are caring for never gets a second off from their disease.  And sometimes you watch as every day the person you care for gets weaker and weaker–until you have to dress them and change their soiled underclothes and bathe them. It’s not that you mind the work, it’s how awful it is to watch someone you care for become an infant.

Personal impact

From the day of Jane’s diagnosis to the day of her death, I lost more than 20 pounds. Emotionally, I was–and still am–a mess. When I was with her, I was generally patient and gentle and kind. There were times, though, I failed. I carry the guilt of those times with me, even now, nearly six years after she died.

People tell you to take care of yourself…

When we were apart and I didn’t have to be perfect, I could fly off the handle at the slightest miscue. I was walking across the lawn one afternoon. I was buttoning up the outside of the house for the winter. My foot went into a hole. Every ounce of repressed anger came out in that instant. I couldn’t yell at Jane, couldn’t yell at her cancer–but I could pound my fist into the ground over and over again.

Outcomes

And if you get lucky, the person you love survives. They regain their strength and become someone similar to who they were before. They are not the same person–you don’t look death in the eye and return unchanged. But love abides because of what you’ve both been through. Neither of you is unchanged–and it makes you both stronger.

…I lost more than 20 pounds.

And if you are not lucky, you pick out a coffin and plan a funeral service. You choose a burial plot and a headstone. You put on a brave face and thank people for coming to the wake or the funeral service or the burial. And you cry yourself to sleep at night until you find a way to deal with the empty chair at the table that represents the void in your life.

Tears on the road to Boston

I’m still crying. The tears sneak up on me at the strangest moments. I drove to Boston late yesterday afternoon. I pulled into a rest area to check a phone call. I’ve stopped there before without incident–but suddenly I’m crying, remembering our last trip to Boston together the day before Jane’s surgery. We’d stopped there because Jane needed to go to the bathroom.

You put on a brave face…

I’ve done grief groups, had counseling, and I still get ambushed by grief like that. Nearly everyone I know has largely moved on from Jane’s death. For me, the numbness has just begun to wear off–and the real hurting has just begun. The pain is a good thing, though. It means the wound has healed enough to hurt.

The unanswered need

We have support groups for patients. We have support groups for grief. Caregivers lurk in both. Too often, they have nowhere else to turn for the solace and support they need. But a caregiver cannot really talk about what they experience in either place. We’re determined not to let patients know what we are feeling in our darker moments. They are fighting for their lives. And grief groups aren’t quite the right forum for that either. They are about the pain of loss, not the pain of having failed.

I’m still crying.

Support groups for caregivers do exist, but they are too few and too far between–and often focussed on a particular disease. NET cancer caregivers need such a group, but we are so scattered that getting together–other than online–is difficult. But we really do need such a group where we can let out hair down without inflicting our pain on the patients who are our loved ones.

On the surface, caregivers may appear serene when taking care of a loved one. that serenity comes at a price.
On the surface, caregivers may appear serene when taking care of a loved one. That serenity comes at a price.

 

Birthday NET cancer thoughts on Jane’s Day

awareness, Fundraising, Jane, NET Cancer Awareness Day/Month

Another missed birthday

Jane would be 62 today. She would have awakened to a birthday card, presents and breakfast in bed. That breakfast would have included tea and chocolate croissants. Later, flowers would have arrived and we would go to lunch at a favorite restaurant.

Happy Birthday, darling.

Instead, I’ll visit her grave. I will leave flowers, a card, and three small stones–one for her, one for me, and one for us. My life has a gaping hole in it I think sometimes no one sees but me. I work on NET cancer, I comfort the afflicted and afflict the powerful. I live the best life I can. But there is too little joy in it.

The birthday present I wish I had for her

What I really want to give Jane for her birthday next year is a cure for NET cancer. I want us both to smile at that reality. It hasn’t happened this year–but we’ve made some progress that gives me some small hope. Our understanding of the disease gets better every year, though it seems we will need to find some new approaches if what we are seeing be true.

Instead, I’ll visit her grave.

The money we have for research continues to grow with each passing year. The year Jane died, we spent less than $2 million on the basic research that creates cures. This year we have $15 million to spend in the US. A year ago, we were preparing the launch of 3-in-3: The Campaign to Cure NET Cancer at the Dana-Farber Cancer Institute. Between now and early December, we should reach our first $1 million in our effort to boost funding for the Program in Neuroendocrine and Carcinoid Tumors at that institution.

Birthday presents in my heart

On November 1, thanks to that effort, Dana-Farber created a new lab for gastrointestinal cancer that will have NET cancer as its primary focus. Our Boston Marathon Jimmy Fund Walk teams raised over $156,000 as part of that campaign. Riders in the PMC Challenge raised nearly another $50,000. And next year, we will do better.

This year we have $15 million to spend in the US.

Private donors outside those two groups will have contributed another $800,000 when all is said and done. Some of those donations are big; some are small. Every one of them counts because every dollar brings us closer to the understandings we must have. Patients deserve to live full and normal lives without NET cancer hanging over their heads.

The next birthday

At Dana-Farber, we need to build on that success. By Jane’s next birthday, I hope we’re talking about a $1.5 million addition–or more–to what we raised in this first year. I’d be thrilled if the three-year goal were reached in two. But it won’t happen without help from lots of other people who are not yet involved.

…a new lab for gastrointestinal cancer…

And we need to replicate the success of 3-in-3 at other NET cancer centers, both in the US and abroad. We need to turn that $15 million we raised for NET cancer research nationally this year to $30 million next year. To do that, we need every support group and small foundation to do whatever it can. For some of us, that will be as simple as writing a check.  Support your local cancer center’s program or  larger umbrella groups like the Carcinoid Cancer Foundation or NET Research Foundation.

Creating the NET future

For others, it may be organizing a NET cancer Walk or piggybacking onto a larger and more publicized event with a team or group of teams that earmarks their money to NET cancer research. Still others may run craft fairs or dinners or other events aimed at raising money for NET cancer and raising awareness about the disease in their community. And still others may find other ways, like selling candy bars or going door-to-door to share their story with their neighbors.

…we need to build on that success.

We need both patients and caregivers to understand the importance of taking part in trials–not only for their health, but for the health of others. Jane said, “I want to beat this–I want to be the first person to beat this. But if I can’t, I want doctors and researchers to learn everything they can from me about this disease and how it works.” There are times I wish she had let herself go earlier. But I know what we learned because she kept fighting was worth the extra pain she went through to get it. She was–and is–my hero because of that.

In our hands and in our hearts

What we do and how much money we raise as individuals matters less than doing all we can to take control of our own disease. After five years of working in this vineyard, I know how much–and how little–we can expect from the federal government and the major cancer charities. We have to realize that we are MS, we are ALS, we are Cystic Fibrosis–and take our future in our own hands whenever–and wherever–we can.

She was–and is–my hero…

The future of NET cancer research, the future of NET cancer treatment, the future of a NET cancer cure rests in our hands. If we don’t lobby for increased government spending, no one else will. If we don’t do the spade work to raise private money for research, no one else will. If we don’t volunteer for trials, no one else will. We are all walking with Jane–and none of us walk alone.

Happy Birthday, darling.

One of the things we might have done on Jane's birthday is go watch the waves come in on the beach. But we need to build a different kind of wave to find a cure for NET cancer.
One of the things we might have done on Jane’s birthday is go watch the waves come in on the beach. But we need to build a different kind of wave to find a cure for NET cancer.

Crisis and calm in the cardiac ICU

awareness, Death, Jane, NET Cancer Awareness Day/Month, NET cancer experiences

Before the first crisis

I was sitting just to the left of Jane’s hospital bed six years ago today. She’d arrived in the cardiac ICU just about midnight and they’d let me in to see her, briefly, about 1 a.m. I’d talked to her doctor about the surgery. He’d seemed pleased, despite the complications they’d encountered, with how things went. There’d been no sense of crisis.

…she kept paying it forward.

They’d warned me what I would see when I entered the room for the first time. Sometimes the pasty complexion and all the tubes and wires cause people to faint when they first encounter it. I spent half an hour there, just holding her hand and whispering sweet nothings. Then they sent me back to my hotel.

Jane wakes up

I slept a few hours but arrived at her bedside a little before eight the next morning. The nurse stood behind his panel of dials and meters, monitoring every vital sign, every drug going in and every fluid going out. I liked him. There was a calm about him I wanted to emulate. I saw that same calm in all her nurses over the next few weeks, even when things collapsed into chaos and crisis. That happened four times before death claimed her.

They’d warned me what I would see…

Now, I held her hand and talked to her. The nurse told me she was unlikely to wake up before noon, but her eyes flickered open within an hour of my arrival. They didn’t stay open long, maybe five minutes, and she dozed off again. But within an hour she was fully awake and alert.

We had a plan

She couldn’t talk with the tube going down her throat to aid her breathing. She couldn’t write with all the tubes and wires she was wrapped in. But we could hold hands and I could tell her what the surgeon had told me. It very much looked like she’d be in the step-down unit by Thursday night–as planned–and I’d be back home and out of the nurses’ and doctors’ way as she began to rehab her heart.

There was a calm about him…

It didn’t happen that way, but we had 30 hours that certainly looked like clear sailing into a difficult but workable future. Yes, her liver was a mess. Yes, her intestines were likely lined with tumors. But we had a plan–and the first part of that plan was going well. Tomorrow, we’d celebrate her birthday as best we could when she couldn’t eat, confident there would be more in the future when she could.

When right turns wrong

By the following day, everything was ready for the move upstairs except locating a bed for her on that ward. No problem: we started taking out the lines and doing the physical therapy as though the move had already happened. And Jane was a more than enthusiastic participant. She kept doing the simple exercises long after the PT people left. They’d told her she could–that it would speed up the process. And Jane wanted to be home by Christmas.

…we had a plan…

And then it all went sideways. At the shift change the new nurse ran the checks every new nurse makes when taking over a patient’s care. They’d taken off the pulse-oxygen monitor in preparing to move Jane to the step-down unit. The exam showed Jane’s blood oxygen levels crashing. They put her back on oxygen but the levels stayed lower than was good. They ran tests, did scans–but could not figure out what was going on.

Carcinoid crisis

I spent that night sitting with her in her room in the ICU. I didn’t sleep–and neither did she. She was in the midst of her first carcinoid crisis, though we didn’t realize it at the time. It was minor, compared to the ones that came later. Those would put her in a coma–and the last one would kill her.

…Jane wanted to be home by Christmas.

Jane never got to the step-down unit. I didn’t sleep at home again until December 10, several hours after her death. I curled up in a fetal ball in my corner of the bed and cried myself to sleep. Then I put on a brave face and went to work planning her funeral.

What we learned

We learned a lot from Jane’s struggles over those four weeks. Doctors rewrote the protocols for heart surgery aftercare on patients with carcinoid syndrome from what Jane went through. People are alive today who might not be because of that. I’m glad about that–a small brightness in an otherwise dark firmament.

Jane never got to the step-down unit.

Jane wanted good things to come from her fight with NET cancer. She wanted her doctors to learn all that they could from her. And she wanted future patients to benefit from her battle with the demon. Even dying, she kept paying it forward.

Walking with Jane was born out of my personal crisis after Jane's death. She always wanted to keep paying things forward. I still do.
Walking with Jane was born out of my personal crisis after Jane’s death. She always wanted to keep paying things forward. I still do.

Serotonin as an example of what NET cancer can do

awareness, Jane

What serotonin does

Serotonin is an important hormone for human beings. In the right quantities, it balances our moods, aids our digestion, regulates our blood pressure and respiration, and a bunch of other things as well. But if we have too little of it–or too much–the results are nasty.

…it has all filled me with a terrible resolve.

Some NET cancer tumors produce serotonin. Jane’s did. I’ve seen the impact of a steady overdose of that hormone from the outside. What it feels like to be on the inside of that situation is beyond my ability to imagine. But for lots of NET cancer patients, they don’t have to imagine it because they live it every day.

Why NET isn’t ‘the good cancer’

And they can live with it for decades. That fact lets some people–including too many doctors–think NET cancer is a good cancer to get. The patient looks healthy. And in a culture obsessed with weight, some of the women may even be envied for their ability to stay slim. Too many people count the years and forget about everything else.

…the results are nasty.

No one thinks about the quality of life in those decades except for patients and their families. They live with what the disease does to the quality of a person’s life in ways no doctor, no insurance or drug company rep., no average person does. They see the reality of the disease and what it does.

The beginning of Jane’s journey

Jane and her family dealt with her cancer for five years before I met her. They didn’t know that cancer was the cause of Jane’s sudden weight loss in 1980–they just knew she stopped eating. Her mother thought her daughter might be dealing with anorexia. But Jane only knew that every time she ate more than a few bites, her stomach filled up with painful gas that made her look three months pregnant.

The patient looks healthy.

Then the diarrhea would arrive and she’d feel better for a few days. She discovered if she stopped eating after 5 p.m., she had fewer problems. Slowly, she figured out that some foods were less of a problem than others. She adapted her behavior to minimize the impact of the disease–at least where eating was concerned.

Hidden symptoms

She stopped losing weight. Over time, she gained back a few of the pounds she had lost–though nowhere near what she had lost. People around her thought whatever she had had vanished. But it hadn’t. It was still there. She’d just learned to cope with the food part of it.

Then the diarrhea would arrive…

The insomnia was a different story. No one really saw it. When she couldn’t sleep, she got up and made her family fancy pastries for breakfast, graded papers, or prepped lessons. No one connected her sleeplessness and her digestion issues.

And then there was me

And then she met me. I didn’t make those connections either. At first, she kept her dietary issues from me–until I took her out for pizza after a movie one night. When I called the next day, she told me she wasn’t feeling well. The next time we went out, she told me she couldn’t eat after 5 p.m.–that it did bad things to her digestion. She was embarrassed by it and hadn’t wanted to spoil our date. She’d spoiled the rest of her weekend, instead.

The insomnia was a different story.

I come from a family that doesn’t need much sleep. I chalked her hours of sleep up to the same genetics we have that wakes us up after 6-7 hours of sleep. If I get much more than that, I stay groggy for the rest of the day.

Day-to-day realities

It was only after we were married I realized the extent of her insomnia, which gradually worsened over the course of our life together. I’d wake up at 2 a.m. to find her using her earbuds to listen to the tiny radio she kept on her nightstand. I functioned on 6-7 hours of sleep. She worked on 3-4–and sometimes on none at all.

…she kept her dietary issues from me…

Diarrhea was her constant companion. At first, it was a couple times a month. It would be proceeded by a couple of days of boating and gas. She’d trace it back to something she’d eaten or the pressure we both faced in the classroom. A doctor had told her teaching was stressful and the most likely cause of what ailed her. The first symptoms had come on just after she’d started teaching. It seemed logical.

Getting through the day

The number and frequency of diarrhea episodes increased gradually over time. She was embarrassed by them and, for a long time, would not admit even to me that they were happening. Her last year teaching, every morning started with diarrhea followed by an Imodium tablet that would get her through the day.

Diarrhea was her constant companion.

I tried to get her to the doctor, but her response was always the same: “They’ll just tell me it’s IBS, again; that it’s all in my head.” She thought the job was the problem and, in another 18 months we’d retire, and that would solve everything.

Mood shifts

Jane’s moods were also mercurial. We could be laughing and joking or snuggling in each other’s arms and, out of nowhere, she’d be so angry she couldn’t speak. It could be a compliment or a well-meant tease in response to a tease of her own. She would suddenly go cold and silent. By morning, she was herself again, as though nothing had happened.

…every morning started with diarrhea…

Those sudden shifts in mood became increasingly frequent over time. Often, I could ascribe them to the pain in her bloated belly. Sometimes, I knew I’d said something or done something that set her off. But sometimes, they came out of nowhere. Knowing what I know now about serotonin levels and moods, it was one more signal of the cancer that I could not see.

Another masking agent

Menopause came early for Jane. Her menstrual cycle, always irregular at best, went completely haywire in her late 30s. She had the hot flashes and night sweats we associate with menopause start up at about that point as well. Her libido died before she was 45. We still snuggled and kissed each other good night; still woke up in each other’s arms and held hands everywhere we went. We still loved each other deeply. But the sexual piece of our time together was over.

But sometimes, they came out of nowhere.

And, of course, we both ascribed a chunk of her moodiness to the emotional struggles many women go through during the “time.” It made perfect sense. But in the background, those tiny little tumors were churning out their deadly little drops, poisoning everything–or trying to.

Other things we might have connected

Jane had chronic low blood pressure. Her resting heart rate was sometimes under 50. We ascribed both of those things to her excellent physical condition. She never smoked, rarely drank and exercised vigorously virtually every day.

Menopause came early for Jane.

But sometimes she would get dizzy if she stood ups too quickly. Sometimes she fainted getting up off the toilet. She confessed later, there were times she felt like the world was coming to her from far away–as though she were having some kind of out of body experience.

Damage we couldn’t see

Serotonin is a caustic substance. In the amounts we’re supposed to have in our intestines, it doesn’t do more damage than our bodies can repair. But in the quantities NET cancer tumors can produce, they can slowly induce a fibrosis that makes the intestines increasingly brittle. In that state, they have greater and greater difficulty absorbing the nutrients the serotonin is pushing through them at faster and faster rates.

Jane had chronic low blood pressure.

All of this conspired to increase the rate at which Jane was starving to death. She needed bathrooms more and more often. It made travel of any kind more and more difficult. There came a point where just going out for groceries meant a pit stop in every store. And then, even that was too little.

Into the liver

At some point, long before the events of that last paragraph, Jane’s tumors hit her liver and exploded. The serotonin the body produces is supposed to be metabolized in the liver. This prevents it from reaching the heart in all its destructive power. But when the hormone is manufactured in the liver, all that serotonin shoots up through the valves in the right side of the heart, doing to them what has already begun to happen in the intestines.

…Jane was starving to death.

Jane was a physically very active woman. She played tennis every day in the summer–singles with her sister in the morning, often followed by doubles in the afternoon with three men, all of whom were good players. At school, we paced the halls at speeds that left even teenagers trying to keep up with us breathless.

Destroying the heart

As the valves in her heart deteriorated, stairs became increasingly difficult to navigate. Her feet would swell up with the fluid leaking back into her body from those leaking valves. Then her legs filled with that fluid. Then her belly. Without surgery, she would have died from congestive heart failure–essentially drowning in her own fluids as they squeezed her heart to the point of shutdown.

Jane was a physically very active woman.

She gave up tennis that last summer. We tried to go for walks and hikes, but they became shorter and shorter. Sometimes, she would send me on to finish what we started while she sat and read the newspaper or stared out across the water to the farther shore.

Every story is different

Not every NET cancer patient whose tumors are producing serotonin goes through everything that Jane did. Some go through more–or a different mix of events and symptoms. Nor does every NET cancer tumor produce serotonin. Some produce other hormones and peptides that create very different symptoms and experiences. Some appear to produce no hormones or peptides.

She gave up tennis that last summer.

It is a truism among the doctors who have become experts on this form of cancer that, “When you’ve seen one case of NET cancer, you’ve seen one case.” Every NET cancer is different, seemingly, from every other NET cancer.

The impact crater on my soul

I can’t say what any of this felt like to Jane. I can’t imagine what it was like for her to watch her world closing in on her every day until all that was left was what she could see from her hospital bed or the chair in the room she had to be hoisted in and out of with a crane.

 Every NET cancer is different…

I only know the damage it has done to my own mind–my own soul. And I find I can’t describe that in any way that makes people understand the depth of either her suffering or my own in having had to watch her go through it. But it has all filled me with a terrible resolve: NET cancer dies.

 

Part of what gets me to walk 26.2 miles every September is my memory of watching Jane fight her way through the serotonin overdose her body was delivering her on a daily basis.
Part of what gets me to walk 26.2 miles every September is my memory of watching Jane fight her way through the serotonin overdose her body was delivering her on a daily basis.

Awareness creates knowledge and change

awareness, Goals, Marketing NETs, NET Cancer Awareness Day/Month

The emotions of November

I’ve had a mind-shattering kind of weekend. I’ve cursed and shouted and cried, both publicly and privately. I’d like to say I feel much better today, but that would be a lie. I have a tough enough time in November, given all the memories it conjures up. Adding the angst of NET Cancer Awareness Month/Day into the mix only further complicates things.

I sell a piece of my soul for that…

But while moving NET Cancer Awareness to some other part of the calendar would be good for me–and getting it away from the elephant in the room that is October might be good for all of us–it’s not likely to happen. Truth be told, we lose more than 1000 patients to NET cancer every month in the US alone–and diagnose even more. No month or day is a good day when that happens.

Why we need awareness every day

As Ronny Allen argues, “Every Day is NET Cancer Day.” Every day, patients around the world hear some variation on the words, “I’ve never heard of this cancer before.” For new patients and their doctors, the first time they hear “NET cancer” they are launched on a quest for information that raises their awareness–and the awareness of the people around them.

I’ve had a mind-shattering kind of weekend.

But it’s not enough. In fact, I’d argue, that awareness comes too late. The doctors need awareness before they see that first patient. That way they would be more willing to consider the testing that will lead to that diagnosis sooner–or at all. As the people at the Carcinoid Cancer Foundation have said for years, “You can’t detect it if you don’t suspect it.” And you can’t suspect something you’ve never heard of.

Doctors need NET knowledge, too

We have patient conferences where both the newly diagnosed and the long-suffering can find out the latest about treatments and support systems. But we have nothing similar–no real in-service training–for experienced primary care doctors. They can go online, as Jane’s doctor did, and try to make sense of the disease that way. But by the time they have a new NET patient sitting in their office, it is too late.

‘Every Day is NET Cancer Day.’ 

Maybe what we need is a traveling road show/conference about rare diseases aimed at primary care physicians: Six diseases you’ve never heard of in six hours. NET cancer is not, after all, the only disease out there that makes a doctor go, “Hmmmm.”

Awareness in the general population

We need the general population to know about the disease before the first time they encounter someone who has it. They are where the money for research has to come from–whether through donations or government funding. Currently, virtually the only people donating to research are those who know someone who has NET or who has died from it.

Six diseases you’ve never heard of in six hours.

Getting awareness of NET cancer to happen among the general population is hard. It’s why I’ve never turned our Walking with Jane Facebook Page into a private group. I understand the need for a place patients can let their hair down. I’m part of several such groups. But my purpose is somewhat broader than providing emotional and intellectual support for patients. So Walking with Jane exists as a public forum to educate non-patients and non-caregivers so we can create a broader base of support.

Purpose of public pain

Of course, the vast majority of the people who read what I write on NET cancer are patients, caregivers and people who have lost someone close to them to the disease. I know some of what I write is as painful for them to read as it is for me to write. It is a reality none of us wants to think about. And it will remain a reality until we find a way to drive out the demon that is this disease.

…we can create a broader base of support outside our own ranks.

I hope, in showing my pain, I can ease the paths of many who are patients. Despite my depressive tone, I want everyone to know that I am making progress toward dealing with the most horrible loss a spouse can experience. I want both patients and caregivers to know there is hope, as well as sadness, on this path.

Engendering compassion

And I want those who have no experience with NET cancer–or any other rare or fatal disease–to gain some understanding of what it means to face a disease without a cure. I want people to understand what it is to be a widow, widower, or orphan who has had to walk someone to the end of life too young. I want people to understand the emotional cost of such a loss. I want them to know what it does to change your view of the world and the people in it.

It is a reality none of us wants to think about…

As my Grandfather used to say: “Intelligence is learning from your own experiences; wisdom is learning from the experiences of others.” I hope, in addition, my posts will encourage some compassion to take root in the hearts of those fortunate enough not to be where too many of us are. I sell a piece of my soul for that every time I write something.

People talk about the beauty of a blue sky. But awareness of what clouds do can change our perspective on everything.
People talk about the beauty of a blue sky. But awareness of what clouds do can change our perspective on everything.

 

Promises and forgiveness in the name of love

awareness, Death, Grief, Jane

Forgiveness in the face of life

Jane and I forgave each other for the mistakes we had made over the previous 21 years, two months and 11 days on this date six years ago. We also forgave–or tried to–each other for the mistakes we knew we would make in the next few weeks as we prepared for her heart surgery, scheduled for November 15. We knew she would be in the hospital for at least three weeks. We both expected she would be home for Christmas. But we wanted nothing left unsaid, just in case. And we made each other promises.

…I have promises of my own to keep.

I brought her body home two weeks before Christmas and put it in the ground next to her mother–fulfilling one of those promises. Hard as this is for me to say–hard as this is for some of you to hear–my life has been empty ever since.

Promises to fill the void

I’ve tried to fill that emptiness by doing good in the world. I’ve worked on cancer funding–both generally and specifically for NET cancer. I’ve worked on NET cancer awareness by creating brochures, writing articles, making videos and talking to anyone who would listen–as well as those who didn’t want to. I’ve worked to raise money for research, helped patients try to find the resources they need to live better lives, tried to console and inspire others who have lost what I have lost.

Jane and I forgave each other…

Beyond that, I’ve written about the power of love, the power of forgiveness, and the need to embrace those things in our daily lives. I’ve tried to practice those beliefs every day in everything I do. Some days I’ve been better at it than others, but the desire is always there.

Broken promises

I envy those of you who find solace in prayer and simple faith. I equally envy those of you to whom those things have no meaning. Believers and unbelievers alike have certainties I do not share–and cannot share. I have seen too much to deny the existence of some higher power–or powers. But, equally, I have seen too much to believe in their benevolence–at least toward me and those I care about.

I’ve tried to fill that emptiness…

I have seen too much of death–too much of suffering. I’ve watched a man gouge grooves in the arms of a maple chair against the pain of end-stage lung cancer; watched a woman drown for three agonizing days as pulmonary fibrosis slowly choked her to death; watched my wife suffer for decades with the NET cancer that slowly destroyed her heart and shredded her bowels before it killed her. I have seen too much other hideousness to remain on speaking terms with divinity.

The empty sound of a shattered heart

Something snapped in me while I slept last night. I’ve felt it building in me for weeks–for months–for years, really. I’ve taken, as Peter, Paul and Mary sang, my “place on the Great Mandala, as it moves through your brief moment of time.” I have made my choices and live the outcomes of those choices every day–as well as the promises.

I have seen too much of death…

But I feel empty–empty in the way this house too often feels empty–even when it is full of people. I feel abandoned–the way I abandoned–and Jane abandoned– her cooling body after her death. And I’m tired–tired of the daily effort, the daily lifting and turning, and the slow, almost invisible progress that some days seems as much illusion as reality.

Dancing our promises

I danced with Jane to the end of her life. It was a dance filled with love. And since her death, I’ve tried to keep dancing that dance, even with my partner gone and the music fading like the sound of her voice at the end. We believed in love and forgiveness–and once you’ve heard that song, you can never dance to anything else–nor stop dancing.

Something snapped in me while I slept last night.

I made Jane promises before she died: I promised her a cure for NET cancer; I promised her I would not stop living; I promised her I would never stop working for the things we believed in. Those promises were not mistakes–but they are proving really hard–and often very painful–to keep.

Promise me this

Someone said to me this week that I am a person who goes where angels fear to tread. I said, “It’s easy to do when Heaven doesn’t want you and Hell is afraid of what you’ll do next.” I said it lightly, and the other person laughed. But it is how I feel–how I’ve felt for a long time.

I danced with Jane to the end of her life.

So don’t pray for me. Instead, love one another and forgive one another, even when you feel some people don’t deserve it. They’re probably struggling, just like you are, with something you can’t see. The hurt coming from their mouths and actions is evidence of that battle–a battle they sometimes can’t see or even acknowledge.

And I, I have promises of my own to keep .

 

For me, promises can have the power of a vow, depending on when and how they are made. The promises I made to Jane at the end of her life have that power.
For me, promises can have the power of a vow, depending on when and how they are made. The promises I made to Jane at the end of her life have that power.

 

Craft, artistry required in NET cure search

awareness, Jane, NET Cancer Awareness Day/Month

Craft fair prep, travel prep

The alarm woke me at 6 a.m. today. I had a craft fair to get to by 8 a.m. and I needed to shower, shave, eat and pack the car before I could leave. Fortunately, the site was only about 15 minutes away. I didn’t sleep well last night.

Jane and I were lucky.

I didn’t sleep well six years ago either. Jane and I came back from Dana-Farber late in the day. We’d had appointments with her oncologist and a nutritionist, as well as her regular labs and a Sandostatin injection. When we got home, for the first time, I had to carry her up the stairs.

Craft items

I got set at the craft fair pretty quickly. I’ve been at this long enough that I have a basic design for the table pretty well down. I’ve simplified some things, so I carry fewer boxes. I’ve started a new line this fall: I’m selling limited editions of photographs I’ve taken.

I didn’t sleep well six years ago…

I’ll add some zebra-themed hats, scarves and other items as soon as I get the time to get over to Rhode Island to pick them up from the group that is making them. I also have some nice hand-crafted earrings and knitting items.

Medical craft

But there was nothing routine about November 12, 2010. Jane and I slept in because I didn’t have to work. I had an in-service day at school, but my principal had strongly suggested she would not object if I spent the day with my wife instead. I hadn’t told her that was my plan in any event. Jane’s health was declining rapidly.

I got set at the craft fair pretty quickly.

We were sitting together in the living room when the phone rang. Her heart surgeon had talked with her oncologist and cardiologist. Apparently, he didn’t like what he heard. He wanted to schedule Jane’s surgery for Monday or Thursday.

Decision point

We wanted a few minutes to talk about that before deciding which day. We both felt sooner was better than later. She was worried about a cold she thought she was fighting off, but we both remembered me carrying her up the stairs the night before.

…he didn’t like what he heard.

We called back and told them Monday. They told us to arrive on Sunday afternoon.

Photographer’s craft

There are three kinds of photographer. The most common is the person simply trying to capture an event so they can remember it later. The second is concerned with sculpting an image made of captured light. The best photographers do both simultaneously. I’m not that good.

We wanted a few minutes…

On a good day, I’m capable of being the second, but I have to think about what I’m doing. I don’t do well with moving targets even then. If I get one good image out of every 200, I’ve done well. I have just six photographs I am pleased enough with to hang in my house or sell to others.

Our last trip together

I knew Jane was in pretty serious trouble the day before the operation when we drove to the hospital in Boston. I’d had to help her off the toilet that morning before we left the house. I had to go into the Women’s Room at a rest stop on the way up to help her again.

I’m not that good.

I helped her out of the car when we arrived and when I came back from putting the car in the parking garage, she was in a wheelchair, almost weeping. She apologized for me having to carry her bag and push her to the ward she would stay on that night. She just couldn’t walk that far.

The craft of craft fairs

It’s 10:15 a.m. and the craft fair isn’t going very well. I’ve talked with one person about NET cancer. Three or four more have drifted past. Mostly, it’s been crafters talking to crafters. There are three people here I know from other shows. We’ve checked in with each other and will talk as the day goes on. We’re all hoping things will pick up.

She just couldn’t walk that far.

Sometimes they do. Sometimes they don’t. You hope the people running the show know what they are doing—that they’ve done the advertising and the public relations stuff. Even if they have, you are at the mercy of the weather and who else has fairs where that weekend. Walking with Jane has run one in June for five years. Traffic is different every year.

The surgeon’s craft

Even an average heart surgeon needs to be better at their job than I am as a photographer or an event organizer. Jane’s surgeon was brilliant. Jane’s heart was so damaged he had to sculpt the muscle to craft a new seat for the valves he installed. Despite his skill, Jane would need a pacemaker to keep her heart beating for the time she had left.

Sometimes they do.

Jane’s surgery was supposed to take 3-4 hours. It took 12. I paced the waiting room. I walked up the hill to a church not far from a place I lived as a student. I tried to distract myself with television shows we liked to watch. I stared out the windows at the lights of the city. Nothing eased the waiting.

The craft of waiting

This morning, I deal with another kind of waiting. Nothing is at risk beyond my ego. And it’s not taking any serious damage. Someone asked, again, if my photographs are watercolors. I take that as a compliment. It’s part of what happens for me when I am seeing light and capturing it the way I want to. A few others have whispered, “Beautiful.”

Nothing eased the waiting.

Of course, there’s always the person who thinks the camera is more responsible for the quality of the picture than the photographer. There’s this much truth to that: the quality of the lens and the quality of the sensors do make it possible to more accurately capture the light we see. You can take a good picture if you have the skill with a point-and-shoot camera. I’m not sure you can take a great picture with one—though some of what I’m seeing from the latest phones is pretty impressive.

The surgeon’s tools

A surgeon has to have the right tools to do the fine work that heart or brain surgery requires. Hand them a Swiss Army Knife or an Exacto knife and they can certainly do more with it than the average person can. Serous work demands serious tools. But it also requires more knowledge and practice than most of us can imagine.

I take that as a compliment.

Hand me the best scalpel in the world and you still don’t want me doing surgery on you. I can paint with words, I can paint—sort of—with light. But I am more craftsman than artist. Jane’s surgeon was an artist—and Jane’s condition required he create the medical equivalent of the Mona Lisa. You don’t pay a person like that based on the tools they use.

The art and craft of medicine

Jane and I were lucky. At every step we encountered people who knew what they were doing at the very highest levels of their art. When we encountered people who were not, they were smart enough, and wise enough, to pass us on to those who were.

Jane’s surgeon was an artist…

That doesn’t always happen. If the nearest NET cancer specialist works a day or two away—as is too often the case—the patient is stuck. We need more NET cancer doctors, more NET cancer centers, more NET cancer research. Otherwise, it’s like a craft show with no crafters for people looking for something that works.

Photography is both a craft and an art--and I am more craftsman than artist. Surgery is also both art and craft. Jane's surgeon was both. Without that combination, Jane would have died on the operating table.
Photography is both a craft and an art–and I am more craftsman than artist. Surgery is also both art and craft. Jane’s surgeon was both. Without that combination, Jane would have died on the operating table.