My Story-Diagnosis and Treatments

My name is Donna and I am fighting Carcinoid Cancer.  I am a typical Carcinoid patient, which means, I went many years before I was diagnosed at 46 years old.  I had been misdiagnosed a time or two along the way as well.  The time it would take to accurately diagnose me, allowed the Cancer to metastasize from my intestines to my liver.

In the early 90’s, I went to the Doctor's office complaining of tingling above my kneecaps and exhaustion for 6 months.  She took my hand and asked me patronizingly, if I found my job and having 3 children stressful.  Well, I knew where she was going with that line of questioning, so I thanked her and left. No tests were done.

Next in 2000, more tingling and weakness on my right side and an episode of sweating and almost fainting at work, brought me to see Dr. Neurologist.  He did a scan and diagnosed me with MS, and also noted an 'MS' lesion in my intestinal area.  He told me the sweating, fainting and location of my lesion weren't a typical 'MS' presentation, but diagnosed me with MS anyway.

To his credit he did ask how my digestion was, and I told him I had a lot of gas, well, he said, “that is not typical of MS, but you may have problems in the future.”  So out the door I was with my 'MS' diagnosis, new medication, but no further testing of the 'MS lesion', which of course, now I believe was the start of the carcinoid tumor.  Not only did he not look further into the 'lesion', but he left me believing that, in the future, if I had digestion problems, it would be the old 'MS' acting up. 
That would be exactly what I thought in 2007, when I started having frequent irritable bowel symptoms.  I then started having frequent 'food poisoning' episodes.  I can’t tell you how many restaurants I would not return to because I thought they poisoned me.

In July 2008, my primary care doctor sent me to a gastroenterologist for an endoscopy and colonoscopy.  
When I woke up, the Doctor told me my intestines looked great, not even a polyp, but she took a biopsy for Celiac disease, a condition in which your body can not digest gluten, a wheat protein. She would call me three days later and tell me the biopsy for Celiac came back positive.  Remove gluten from my diet, and I would be fine.  Easier said than done, but that's another story.  I did not question this diagnosis, I needed an answer for the pain.  
In fact, the tumor was hiding where those two scopes don't meet, and the Doctor could not see it. As a patient, especially one that's never needed tests or had any illnesses, you think, "the tests say I'm fine, so I'm fine." That would be lesson number one, tests can be wrong.

By September, the debilitating pain and gastro problems were worsening, and frustratingly unpredictable.  Sometimes I would be fine for days or weeks, and then in an instant, I would bloat to the point that I had to open my pants. The pain was horrible, and felt like broken shards of glass filled my intestines.  As if I was in labor, the pains, would increase in frequency and length, until they peeked about 3 days later, then would slowly fade over 3 days.  During this time my only relief was to remain in the fetal position. Afterwards, I would be left like a wet, wrung out rag for about a week. I thought this was celiac disease.  What could I be doing wrong?  I had been meticulous with the diet, to the point of obsessiveness.

I called the gastroenterologist and let her know I was still experiencing pain, and diarrhea, causing me to miss work for days at a time.  She told me," to try to follow the celiac diet better.  Maybe I needed to make an appointment with a  dietician, and that it was awful funny I am having all these symptoms now that she diagnosed me," in a tone that said -HYPOCHONDRIAC.  Lesson number two- listen to your own instincts, doctors can be wrong.

Well, determined not to bother the gastroenterologist again, I did seek out all information on Celiac disease and went to Beth Israel's Celiac Center in Boston.  I was going to attack this problem and control it, not the other way around. 

The first question the gastroenterologist at Beth Israel asked me took me by surprise,  "How do you know you have Celiac disease?"  Well, I had a test, and a doctor told me so.  Now is the time to refer to the above Lessons One and Two, which I hadn't quite learned yet.
"Well, he said, we will have to do some more testing, this pain isn't normal." Somewhere inside, I heaved a sigh of relief, he is listening to me, and furthermore, he believes me!

But before the tests could be completed, I came home one day from work with yet another painful bout blooming.  This time, I was determined, things would be different.  This time, I would not waste hours on an uncomfortable emergency room chair in horrible, debilitating pain.  I would not be told, as so many times before, "well the ultrasound shows your intestines are swollen, we don't know why, probably a gluten exposure, you can go home now."  

No, this time I asked my husband to take me to Beth Israel, so the new gastroenterologist could see my reports, and maybe, I could get to the bottom of it all.
I must have been at death's door, not only did I NOT tell my husband once, how to drive, but as soon as we went through the emergency room door, I was whisked into the backroom.  Within three hours, I was admitted.  A team of five doctors and students came in and out of my room all night.  I had several scans and by that afternoon, they knew I had a blockage and the next day they determined it was Carcinoid.

I remember so well, the young student doctor, with tears of concern in his eyes, as he researched pain medicine late into the night, until he found one that could ease my pain.  His determination to diagnose me so clear, as he spent hours with me going over and over the symptoms I had, until his suspicions were confirmed with tests that I had Carcinoid Cancer.

"If you have to have Cancer, this is the one to get," my well meaning, very sweet, nurse said, the day after diagnosis. Well, how many of us have heard that one?
I say a very loud and resounding, "NO", to that.  I suppose, back when Carcinoid Cancer was considered  to be mostly benign tumors, this made sense for a nurse, to comfort a new patient with.  It is definitely more accurate to say, they are slow growing tumors, and yet more accurate still, to say, not enough data.

Even after diagnosis, I continued to refer to the tumor as the obstruction, or the blockage.  I either did not want to acknowledge the disease, by saying aloud that heart stopping, gut wrenching, paralyzing word- -CANCER- or, I was just blissfully, ignorantly in denial.  Either way, I do believe it allowed me to have a very positive, rosy colored outlook, which is a big part of recovery.  Little did I know, how much I would need it.

I called work and told them I would not be in that week.  Yes, I really did that! Clueless, I was.

I never had to stay in the hospital before, and it was quite an experience.  I felt horrible still, and the team busily worked on the 'plan'.  It did not matter what time it was, there was always noise, lights and chatter.  Talk of everyday life, plans for vacation, new babies, BBQ's, new shoes, shopping bargains, new home purchases, divorces, ect. all could be heard from my bed.  All little stuff to me now.  How did I get here, I wondered to myself?
I was so uncomfortable with IVs in each arm, a tube down my nose, and an oxygen tube on my nose, a foli and all connected to a pole if I had to walk.  I was very weak.  Several different doctors, each with a different role to play in my care, and their students came in and out at all hours.  Nurses and aides, along with daily phlebotomists poking me for tubes of blood.  I hadn't been able to shower in two days, and all the jokes about the hospital gowns not covering your backside, well, they are true.   Not sexy, definitely not cute.

It was March 6, 2009, and now with a diagnosis in hand, the team of Doctors would put their minds to work on a treatment plan.   I was in a very weak state and had lost 25 pounds since November 2008.  I had an NG tube down my nose to remove the fluid build up from the blockage,  and a PICC line was inserted to provide TPN nourishment, this an IV inserted in your arm, threaded through your vein and stops near your heart.  The scans had revealed that the tumor had metastasized to my liver.

Now begins what I would come to think of as the roller coaster ride.  The surgical team decided to wait for one of their surgeons, with the most carcinoid experience, to return from a medical conference in Europe.  The surgery would be delayed for a week.  I was grateful that my best interest was put first, but it did mean that I would remain without food and on IV's, PICC, NG, and Foli tubes.  The wait would allow my body to gain strength but, if it proved necessary the team could and would be ready to operate.
Everyone praised the surgeon we were waiting for, as did my family and I when we met him.  His eyes were very comforting.  His nature was warm, friendly and he thoroughly explain the plan for the operation.  I felt all my fears and concerns shift instantly to a complete sense of positive hopefulness.  I thought the operation would be scheduled for the next day, but no, I would have to continue the TPN to be as strong as possible.   Roller coaster.

On March 16th the 6 hour surgery would remove along with the tumor, 140 cm of small bowel, 17 lymph nodes(10 positive) and a gallbladder.   I was very lucky, no colostomy bag would be needed.
I had never had any major illnesses, never needed any procedures, much less surgery until now.  I was not prepared for this and the recovery to come. You just don't realize how much you use your intestinal muscles to balance, walk or even sit, until you go through a surgery.  Wow.  But the most difficult part of all of this remains the inability to remove the look of anguish from the faces of your family and friends.  You can talk yourself into dealing with it.  You can put one foot in front of the other and stay positive, but you can't erase concern from your loved ones faces or hearts.  You can't calm their fears in the middle of the night.  That is, and will always be the hardest part of all I have been through.

I left the hospital on March 21st, with plenty of loving family and friends that took such good care of me, and a great hospital staff, I always felt lucky.

The liver tumors would not be operable due to their placement in the liver, another set back.  After a few weeks of recovery, I would undergo chemoembolization, a procedure in which the radiologist inserts a liquid chemo through the femoral vein directly into the tumors and then plugs them with a gel or bead.  You have a local anesthetic, there is no pain, but you must remain completely still and you will become nauseous.
Unfortunately, the Chemo liquid, leaked out and I became ill for weeks and didn't fully recover for months.  The next two procedures to control the liver tumors, would be whats called, Bland Embolizations, there would be no chemo to leak out.  The beads would be threaded through my artery and plug the blood supply to the tumors. These worked wonderfully, the tumors shrunk and some died.  I was so excited.
The liver surgeon now felt not only could he operate to remove the liver tumors, but if I remained tumor free, I could be a candidate for a Liver Transplant.  I was over the moon.  I felt that I had fought this battle back from the brink of death and despite everything, we would remove the last of the tumors and I would receive a new liver and life would go on swimmingly.  Sounds good, right ? Wrong.  Damned roller coaster.

My insurance company refused to cover the liver transplant, due to the fact that I had metastatic cancer. Several requests from both my liver surgeon, and myself were met with denial.  I was infuriated at the time.  How dare the insurance company think that their doctors knew better than my doctors about my illness, and how to best treat it.  What a rude awakening for me.  I never had an insurance company not cover my medical costs before.  Then again, I had never been sick with a major illness before.

This was a blow, I started thinking about life and death.   Wondering why, I, didn't have the final say in the treatment of my illness.  It was such a hard time emotionally, and really brought me down low. In the end, the tiger in me would come back.  

I started to look through the internet.  Again, I came across an article about Ruth Gerdes.  She had twenty two tumors in her liver, and was operated on at the University of Nebraska Medical Center. Many carcinoid experts around the world did not believe she would be a good candidate for surgery. However, her primary care doctor sent her to Dr. Botha and he agreed to operate on her. First, removing the left lobe and when it regenerated, the right lobe would be removed. This would be the first time that this surgical method was used in the United States. Today, Ann is still tumor free, five and a half years post surgery.

My surgeon didn't believe this would be the operation for me.  He didn't actually believe it possible to have a long term, good outcome.  He would be wrong about Ann's surgery, but none of us would know that until now.  

On June 11, 2010, I had the left lobe of my liver removed to reduce tumor load.  The surgery went well and no other tumors, could be seen.  The plan was to watch me with CAT scans every four months and as soon as we saw activity, the tiny tumors would be treated with Radio Ablation.  

We knew there were tiny microscopic cells left behind, and future treatments would be needed. What I didn't know was that scans don't show everything, and lots of activity was going on, without being detected by the scans or blood work.  Roller coaster.

For the next year and a half, I went regularly once a month for blood work and an Octreotide injection.  In addition, there would be  CT and MRI scans every four months, a separate appointment to review all of the tests with the Oncologist and then again, with the liver specialist. Often, the scans and blood work would reveal some concerns, followed by a call that another test is needed.   I have had to come in for additional blood work, scans and ultrasounds, and a Colonoscopy to have a polyp removed.  Strange lumps developed on my legs and those were removed, as well as, a curious looking mole.  Everything would come out fine in the end, but from the moment you receive a call that starts with, "I am not too concerned, but we should follow this up with, ..ect", you are suddenly on that roller coaster taking a hard, fast, steep dip.

December of 2011, was a turning point.  I had always done everything I could to be proactive and treat my disease aggressively.  Even though I had multiple surgeries and constant treatments, I felt relatively well.  I kept my emotions in control with positive thoughts and felt that simply, it is what it is, and I will keep fighting and stay on top.  The plan was if no new tumors appeared anywhere else in my body, we could hopefully proceed with a liver transplant.  We would fight the insurance and go to the state for a review.

You learn to read the faces of Doctors and Radiologists, even when they are smiling.  It's a sixth sense that develops.  This visit wasn't going to be good, I could feel it.

"The scans aren't good, there is a lymph node that has lit up," my doctor said with sad, concerned eyes. "We will need to biopsy it, to confirm if the carcinoid has spread, but because of its location, this will require a major surgery." she let the information sink in.

We scheduled the surgery for February 3, 2012, and I had a PET scan prior to the surgery.  Only the lymph node and some tumors we were aware of showed on the scan.  I was hoping that there was some other benign reason to explain the swelling of the node.  On the day of the surgery, I was prepared to go and get this over with, hoping that we could remove this problem and the liver transplant would still be an option.  All the treatments we have done so far was with the liver transplant as the goal.

I woke up from the surgery, and saw the Doctor and smiled, in my heart I believed this all was going to turn out well, to have just been a lot of worry for nothing.   He smiled back, but that look, that unmistakable look, I have come to dread was written all over his face.  The words just came tumbling out of his mouth all at once; "it doesn't look good, you have several, too many to count, tiny tumors all over the outside wall of your intestines.  Of course, I left the node and closed you up right away.  They weren't there in 2010 when we did the liver surgery.  They didn't show up on the PET scan.  They took us all by surprise.  I'm sorry, the liver transplant is off the table for good."
I was shocked and couldn't quite grasp what he was saying.  I said, "But, but, but what about the radioablation?  What about the surgery Anne Gerdes had?   This was not at all what I had planned on hearing, at all.  He just shook his head slightly, and said he would leave me with my family now.

Now what?  I was trying to wake up and let this sink in.  I had another major surgery opened from sternum to pelvis, a big recovery.  My mind was going a million miles an hour, looking for some answer, a way out of this.  You know, if you get pushed over a cliff, you reach out and grab whatever you can, and try to stop your fall.  If you are lucky enough to grab a branch or land on a ledge, you will start looking around and making a game plan to get back to the top or a least to a better position.  Not many of us start falling and say, "yeah, I'm just going to go with it and let myself fall."  Not human nature.  Such a roller coaster.

The next couple of days went by in a blur of numbness, and going through the motions with my mind and body not quite connected.  A part of my brain would, forevermore, be dedicated to going over the results of my scans, the surgery results, my options, how I felt, my symptoms, no matter what else I might be doing, or saying.  A brain divided, and on a roller coaster with apparently no stops. One thing I just could not figure out, was that I felt so good.  I know I have more tumors, but I feel fine.   The fact that so much about Carcinoid's prognosis is subject to 'a best guess', due to just, not enough data, makes me want to fight harder and not hear what I can't do.
I am on a search for what I can do.

Back to the internet and research.  I came across the ACOR website, (Association of Cancer Online Resources).  This was a beacon of hope, a wealth of knowledge and experience here.  My husband and I began reading all the emails from patients and a special, kind Doctor, who answers many emails.  I read about people with similar histories to mine, as well as, others that were much sicker. Everyone was so kind and helpful, this gives you a lot of hope and helps you to stay positive.  The greatest thing is you can read or post what is on your mind twenty four hours a day. That is so helpful on those sleepless nights.
I found out about an international Carcinoid Cancer Conference being held in Ottowa, Canada in May,2012.  It was being hosted by the Carcinoid NeuroEndocrine Tumour Society Canada, CNETS, and would include carcinoid specialists from all over the world.  It provided an opportunity  for patients to interact with the Doctors.  All of the latest information on treatments would be available.  My husband and I made the decision to go.  I wasn't to sure what to expect, but I felt drawn to go, as if, the decision was made as soon as I read about it.

In March, I went for a follow up appointment with my oncologist.  She went over the options as, traditional chemo, Everolimus, a pill form of chemo, or wait and see.  We needed to treat the tumors with systemic therapy, as surgery was no longer an option, and it appeared that Octreotide alone was no longer working.
As she talked, I felt myself floating away.  I tried to stay focused on her, but most of my brain was screaming, what?! She has always told me chemo doesn't work on my type of carcinoid.  Always.
My brain was busy with thoughts ricocheting off my consciousness, faster than I could comprehend them, much less find a solution.
I just kept thinking, that chemo is so toxic, and may do nothing to the tumors, but certainly will harm me.  I want to live at least twenty more years, and I don't know anyone that is on chemo that long.  So scratch that off the list.  The Everolimus is also chemo, in pill form, harmful and while promising, also not proven to be effective.  Then, there is wait and see, which, this is cancer, so, we know cancer is going to keep on doing what its doing, which is not good.  What to do?  What to do?
Initially, we opted for the Everolimus, but I tell her that there is an International Carcinoid Convention in Canada in May, and my husband and I will be going.  We put off the decision to use the medicine until after the Convention.
Although I was holding up my end of the conversation, shaking my head up and down, and keeping eye contact, it was all a false bravado.  I take the pink papers and listen to the directions of which labs I needed to have done.  I try to stay focused on the Doctor, however, this time, it is all just too much.  Carcinoid dealt a good blow, but I had no intentions of going down.  I just needed to get my balance back.  I floated down the hall to the treatment area, and as soon as my nurse walked in and cheerily said, "how are you doing?", I just collapsed into tears.  In my husband's arms, and comforting back rub of my nurse, I was reassured that, it was all going to be alright.  I believed them, after all, I felt fine, no matter what showed on the scans, the truth is, I felt fine.  What a roller coaster, please let me off.
May couldn't come soon enough for me.  I had been going into work and living my daily life with what felt like a ticking time bomb.  I tried to keep my mind off my worries, but they crept in and invaded my thoughts.  A mind divided, no matter what else I was doing or saying, part of me was always analyzing my situation and options.  During rides home from work, I never failed to notice the beauty all around me.  No matter what the weather, I enjoyed my quiet rides home.  Drinking in every last drop of the scenery before me, that I once was so oblivious to.  Fully conscious and  aware of all the simple, daily interactions with family and friends.  Vacation snapshots, sights, sounds and smells tucked away in my head.  Appreciative and absorbing it all like a thirsty sponge.  These are the pictures, that I had locked away in my brain, and brought out to enjoy during hospital stays, tests, scans, and prior to surgeries before I drifted off.  That is how I deal with all of this.  The truth is, love is all we have, all we can give, all we leave behind, all we take with us.

On May 4, 2012, the Ottawa Carcinoid NeuroEndocrine Cancer Symposium, held at the Fairmont Chateau Laurier, was to be a major factor for me in understanding my disease and treatment options.  Patients, caregivers and Carcinoid Specialists from around the world had come together to share information and concerns. Patients were invited to pick the brains of all these Doctors.  It was truly a wonderful event.  Although, my husband, and I would hear many interesting speakers during the four day conference, two Doctors and one treatment would leave a lasting impression.
Dr. Eugene Woltering, of Ochsner Medical Center, Kenner, Louisiana,  made me realize how important it is to include a specialist in Carcinoid Cancer in my treatment.
Dr. Eric Liu, of Vanderbilt University in Nashville, Tennessee, had studied the PRRT treatment in Basel, Switzerland and spoke of how this treatment targeted Carcinoid Tumors that had spread throughout the body.
PRRT , Peptide Receptor Radionuclide Therapy, simply put, a radionuclide is attached to a peptide of Octreotide, which then is absorbed by the receptors on the tumors, and the radiation is left behind to destroy the tumors.  Read more about this on the information tab.
This seemed fascinating to me.  I was even more intrigued, when I questioned the other specialists at the Conference, about my condition and options.  All of them responded with, "why haven't you had PRRT?"
It was information overload, but in a good way.  I was excited to bring this to my Doctors at home in Boston.
In the States, Oncologists, and even many Carcinoid Specialists that have not been exposed to treatments and research from other countries, are reluctant to have their patients treated since they are not able to assess the risks.   If PRRT is considered, the American Oncologists view is to wait until disease progresses, which is opposite of the European Oncologists view.  In Europe PRRT has been used, with much success for the last fifteen years.
This is the block in the road I met.  My Oncologist had not heard of PRRT, and the specialist I saw in June, felt we needed to wait until my disease worsened.
In July, I felt a lump in my left breast.  After a biopsy, it was proven to be carcinoid.  It was spreading.
In August, as my disease progressed, I reached out to my Oncologists, to contact Dr. Liu and give me her opinion of PRRT, since I trusted her, and her view.
On my Birthday weekend, and at the end of my emotional rope, my Oncologist called me at home and said, "I think you should go to Nashville and meet with Dr. Liu, while I still have concerns about long term effects on your bone marrow, you need to follow through with a treatment that has been important to you for a long time now."  I just love her!  She has always been there for me, and I know if her concerns were many, she would sit me down and go over them.  She gave me strength and hope.

I went to Nashville in September of 2012, and after some testing and review of my records, Dr. Liu sent a request to Basel, Switzerland to admit me for PRRT.
On October 16th, I received my invitation to receive treatment in Basel, Switzerland on Monday November 19th.  The cost for all three treatments would be about $30000.00, not covered by insurance and not including airfare and hotel.  That's a big nut.

My mother and her dearest friends, wasted no time organizing a fundraiser.  They also worked themselves ragged, organizing and creating baskets, pounding the pavement daily.   Donations poured in from so many of my mother's friends and past coworkers.  Family donated generously, my husband's and my coworkers not only donated, but created fabulous baskets, that only love can make.  Complete strangers to me, but if they loved someone that cared about me, they gave, they came, they cared and they made a difference.
November 9th was a wonderful night.  A beautiful outpouring of love, from family, friends and strangers, and I am honored to have spent the night with each and everyone of them.
How do I repay them?  I can't.  I am so humbled.

November 18th 2012, Sunday, Basel, Switzerland, here I am.  Treatment starts tomorrow.  I will try to post daily as long as I am doing well.   Here we go, on the roller coaster once again.