Carcinoid Syndrome Flushing

Flushing 

Not very pretty: melted mascara; mangled brows; sour-puss expression; no foundation, cover-up, or blush of any kind. But carcinoid syndrome ain't pretty. This is tell-tale flushing (which happened while relaxing on the couch - no rhyme or reason to it). My other symptoms are on high alert too. No one likes an ugly picture of them blasted on social media, but I made a commitment to be honest about my journey.

Carcinoid Syndrome Symptoms

Flushing feels hot and uncomfortable - like an internal, tingly sunburn. It starts on my cheeks by the bridge of my nose and spreads across my face and chest in a matter of minutes. For me, it usually happens in times of stress or when eating too much sugar. Lately, however, it happens for no reason at all. (I don't drink alcohol, but many have a reaction to that as well.) It is not menopause - got tested for that. Nor is it Lupus, got tested for that too. So it's not rocket science to put carcinoid cancer and carcinoid flushing together.

In the past, doctors have remarked how "good" I look... maybe now, with photographic evidence of clear flushing - everyone can get on the same page medically. Advice for zebras: gather as much evidence of fleeting symptoms as possible - you may just look too fabulous for doctors to consider you ill!

Admittedly,other than regular Sloan Kettering appointments, I've avoided my recent follow up visits with specialists. I feel a bit guilty about it, but sometimes you feel even more sick staring at yet another set of plastic chairs and fanned magazines on a faded coffee table, waiting to be called - only for the doctor to shrug and order more needles, more tests. But now it's time to get back on track and get my symptoms under control again

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The Power of Doctors' Attitudes

THIS IS MY FIRST BLOG IN MY 30 DAYS OF SUMMER PROJECT ... I WILL BE POSTING 30 BLOG ENTRIES BEFORE SEPT 30th STAY TUNED ...

     During my last CT, I forgot to take off my St. Anthony's medal, which hangs around my neck 24/7. Rookie move for such a non-rookie. The MSK nurse was so kind that she insisted on taping it to my wrist for good luck. Guess it worked because my latest scans and blood tests indicate that everything is stable. Thank God.

     Many have asked me why I continue with Sloan Kettering when the general noid community repeatedly hasn't had great success with them. That nurse's personal touch of boosting my confidence during a nerve racking procedure is the MSK difference.

      In my follow-up appointment later that day, Dr. Nash was a little flabbergasted at my lack of questions. Usually there are all kinds of painful revolts going on in my body, and I look to him to lead a forward charge against a slippery foe. He asked me if I wanted to redo my 5-HIAA test, which was a little higher last time... I declined. My weight has stayed stable and my symptoms manageable. That's the best anyone can hope for with chronic conditions. If any new tumors emerge, I'd probably go back to Dana Farber in Boston for treatment anyway. For now, I'm sticking with MSK.

     Doctors' attitudes have so much power over recovery.

     For example, my recent MSK visit was NOTHING like my ER visit at Hackensack Hospital two summers ago. My body was obviously shutting down ...I lost 25 lbs in 6 weeks and I couldn't walk. I was having uncontrollable muscle spasms, and was in so much pain that my friends couldn't bear watching me, so they took me to the local ER for help. The first two nurses were awesome. Then I got this doctor who saw my physical condition and incorrectly deduced I was a junkie in withdrawal.

     This dumb woman thought she was going to "call my bluff." Little did she know I still had 300 ... Yes 300 pills of oxycodone at home that I never touched during recovery because I'm against drugs. After my hemicolectomy surgery, nurses would push my morphine button because I refused to push it myself. They kept telling me that if I was in pain I wouldn't heal as quickly - I didn't care. I'm terrified of drugs. I've lost friends to drugs, and know their power to destroy lives. So this ER doctor couldn't have been more wrong.

     I told her I had Carcinoid and she asked for MSK's number. 10 min later she returned - with her back to me she announced to my friends, who were holding my hand, that I was a liar. My cancer was cured and there was nothing wrong with me. She smiled at me when they gasped in disbelief.

     She didn't even talk to Rachel or Dr Nash - she had some random person look at my chart that showed no new tumor growth. In her opinion, that meant no cancer. Carcinoid is often chronic, and many sufferers never even have their tumors found at all. MSK and Dana Farber both have in my charts that they "can't rule out" Carcinoid syndrome due to symptoms and initial tumor size. Cancer Centers of Hackensack has a positive diagnosis of Carcinoid Syndrome, as does my GP and dermatologist (who recognized my tell tale flushing during one of my visits - and controls flushing, as well as the swelling in my legs and arms with Spironolactone... He has other carcinoid patients he treats similarly. It works. Click here for an article about Spirolactone and Carcinoid Syndrome). Carcinoid is complex, and this simpleton ER doctor put her ego trip over an obviously suffering patient.

     Thank goodness I had Dr. Woltering's cell number, which he makes available to the Carcinoid community because of the general lack of knowledge in the medical field about this rare disease. Dr. Woltering calmed my friends and myself down as we drove - me writhing in pain - to RWJ, where I was first diagnosed. RWJ took care of me - turns out that in addition to muscle spasms in my legs and arms, I was also having intestinal spasms - the equivalent of a massive charlie horse in your stomach - every 10 min. They too explained Carcinoid syndrome to my friends, and we were again on the right track with new medicine.

     After the visit to the Hackensack ER, I was seconds away from giving up and having others give up on me too. Who knows how much weaker I would have gotten if I had let that ER doctor get to me. Thousands of Carcinoid patients face this reality every day, which is why most carcinoid patients are treated 3-7 years for the wrong disease allowing for the cancer to spread.

     Luckily for me, my friends know of my drug phobia and that I always downplay my pain ... So instinctively they knew the ER doctor was full of shit. With Dr. Woltering's and RWJ's sound advice, we kept hunting for answers. 

     For all the Carcinoid patients and caregivers out there - be your own toughest advocate. Don't let doctors bully you. They estimate my cancer went unnoticed for 5-6 years ... Partly because I didn't push for help until the symptoms were unbareable. Stay connected, stay positive and stay strong.

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Carcinoid Cancer Diagnosis Anniversary

April 27th was the 4-year anniversary of my Carcinoid Cancer diagnosis. If cancer can be described as a roller coaster ride, then these past couple years have had some crazy loop-to-loops, upside down turns, and gut-wrenching drops. There really isn't a lot that is stable anymore; it's just learning to breath during the times when the roller coaster slows down or is climbing.

This past summer I lost over 20 lbs in 6 weeks - and was down to 104 lbs (I'm 5'5). I couldn't walk; I was having palsy episodes; I was in excruciating pain all the time... my two best friends, Rob and Mike, took me into their home, and instead of enjoying their summer - they applied cold packs, created complicated pain medicine schedules, carried me to the car for ER trips, picked up my prescriptions in the middle of the night, and took turns staying by my side 24-hrs a day.

None of my doctors could explain why my body was shutting down.. the blood tests were almost indecipherable - anemia with high iron, blood counts and sizes in direct conflict with each other... my doctors ruled out all the immunity issues: Lupus, HIV, etc - finally, elevated 5-HIAA "increased their reasonable suspicion" that it was probably the cancer or after-effects of the cancer. Just as inexplicably as it came on, the process started reversing itself in November.

That's the thing about the neuroendocrine system - it affects EVERYTHING... and even when the doctors tell you it's not the cancer; if there really is no other explanation then you got to trust your gut. I'm back up to 118 lbs and eating again. My palsy is at a minimum and the pain is managed well without narcotics. So I'm in that breathing space right now... but weird symptoms and pain remind me that I'm still strapped into that roller coaster seat... locked in.

I'm grateful for all of the love, strength, prayers, and support that my friends, family, colleagues and the Carcinoid community have given me. I know this - even if I'm stuck on this roller coaster for a while longer, I've got amazing people in my life who have opted to take that ride with me, and others who are on the ground - waiving... cheering me on.

"Pee in this Jug" and Other Medical Updates

My Friend Mike's Abstract Photo Interpretation of Our Series of Doctor Visits

I just had follow ups with my GI specialist (Dr. Chamberlain) at St. Barbabas Hospital, and with my Internal Medicine specialist (Dr. Totaro). Both Dr. Totaro and Dr. Chamberlain ran blood work.

GI Specialist

Dr. Chamberlain said that if the Valium and Bentyl combination was controlling my intestinal spasms - just stay on them (forever if need be). I sheepishly told him that I had been supplimenting Advil for the Valium because I wanted to be able to drive, but it really didn't help the pain. Dr. Chamberlain reassured me that it was ok to drive on the Valium, and that my body would adjust.

So far so good; the drugs have kept my abdominal pain at a minimum, and being able to drive again has given me most of my independence back. I take my med combo every 6-7 hours.

Internal Medicine Specialist 

When I initially met with Dr. Totaro and explained my symptoms, including thrush  - he said, "well when I hear someone say 'thrush', I immediately think HIV. We better test for that."

Now, I didn't really think there was any real risk of me having HIV (I've only had a few long term partners); but I grew up in the 1980s and watched two friends die from AIDS in the 90s. Sadly, one of my current friends is HIV positive... living with AIDS and HIV is a lonely, painful existence since patients are forced to deal with both the relentless symptoms as well as the social stigma of the disease.

Truth be told, there is nothing medically scarier for me than HIV, and hearing Dr. Totaro suggest it as a possibility was a little like watching Jaws 6 times in a row and then going for a midnight swim in the ocean.

Luckily, the HIV test was negative.

The blood work also eliminated Systematic Lupus, Lymphoma, and Pernicious Anemia. Yet, it didn't reveal a cause for my muscle atrophy, shaking, bone pain, and weight loss (20 lbs since May). Additionally, a comparison of my labs over the course of the last two years revealed that I have become steadily and increasingly more anemic - despite having regular iron levels and a normal B12 count. There is absolutely no explanation for the anemia, nor for some of the other blood abnormalities on the reports.

Dr. Totaro wants me to see a Hematologist. His thought is that if we can figure out what is causing the blood abnormalities and unexplainable anemia, we might be able to find the cause for my other symptoms and declining health.

5-HIAA Test Results - Carcinoid Syndrome

When we realized that it wasn't the cancer causing this latest - and most pressing - health decline, I admittedly began to ignore the Carcinoid. "Why chase two illusive dragons at once?" I thought - one was enough.

Except there was the infamous 5-HIAA test - first ordered this past June - which I still had yet to complete. The 5-HIAA test is a 24 hour urine collection in a bucket:

The 5-HIAA is standard in the Carcinoid world for helping diagnose malignancy and Carcinoid syndrome. It really should have been ordered over a year ago, but that's of little consequence now.

In order to limit the chance of a false positive on the test, I had to stop Valium and avoid certain foods (plums, pineapples, bananas, eggplant, tomatoes, avocados and walnuts) both prior to and during the collection - which I did dutifully.

I just got the test results: the normal range is 0.0-14.9 .. mine was flagged "high" at 17.3. It probably is nothing serious, but I can't be irresponsible either. Dr. Woltering suggested a Carcinoid specialist on Long Island whom I'll investigate.

So What It All Means....

In my first meeting with Dr. Totaro, he summarized my medical condition perfectly; he simply said, "You're a complicated little girl aren't you."

My body is at war with itself, but no one knows why.  There are objective signs, symptoms and labs that just make no medical sense. It's one doctor after another, and when I wake up it's a question of what hurts, and how much. Not having a "name" nor diagnosis for what's doing this to me is probably the most frustrating thing of all.

In Romeo and Juliet, Mercutio described his fatal wound to his best friend this way: "Tis not so deep as a well, nor so wide as a church door, but 'tis enough, 'twill serve."

I am grateful for the health I have. I am appreciative that I can sit in this beautiful French bistro and type this blog. But the psychological toll of feeling like an unwanted stranger in your own body 'tis enough, t'will serve as one of the hardest aspects of conquering my latest medical mystery. Over this past summer especially, I have discovered that my greatest defense against this foreign, unknown enemy is the love and support of my friends and family - who are always at the ready for whatever new medical bombshells get thrown my way.

New Doctor - New Diagnosis

I don't have carcinoid syndrome.

At least that is what the oncologists at Sloan Kettering (Dr. Reidy) and Dana-Farber (Dr. Kulke) said. Well, one dermatologist in Morristown, NJ disagrees with them.

This past month, in addition to increased pain and my usual flushing, fevers, night sweats and nausea - my face broke out with deep, painful welt-like cysts. (Thanks body - you're really on a roll.) At first, I tried to ignore it - angry at myself for being so vain. No matter how I tried to "get over it" however, the new acne made me depressed... which in turn made me feel even more like a narcissist and less like a strong cancer survivor. I decided that there was only one thing to do. Get a dermatologist.

I had been putting off seeing a dermatologist for awhile - Dr. Nash had suggested back in September that I seek one out for an alternative explanation for my "hot rashes" ... ( I began calling them "hot rashes" instead of flushing after Dr. Nash and Dr. Reidy assured me that it was highly unlikely that I had carcinoid syndrome.)

I did a lot of research, and found one of the most respected dermatologists in the state: Dr. Robert Marinaro.

As soon as I met Dr. Marinaro, I liked him immediately. I explained that I had been diagnosed with carcinoid cancer about 15 months ago, but that my tumor was surgically removed, and that my doctors were pretty confident that I don't have carcinoid syndrome. I explained that I wanted to discuss possible alternative causes for these "rashes" I was getting, as well as figure out a treatment for my cystic acne.

I was expecting him to ask me what carcinoid cancer was; he didn't have to - he knew. Over the next 45 minutes, he asked all the right questions, including what my 5-H1AA levels were (normal) and did I have bouts of diarrhea (no, because I have nerve damage from the resectioning of my colon). After an extensive dialogue about my condition, I was preparing for him to say either 1. he had no clue as to the underlying cause of my symptoms, or 2. I had some sort of an autoimmune disease, like Lupus or Hashimoto's disease.

"I think you have carcinoid syndrome" Dr. Marinaro said, without a hint of hesitation.
"But the doctors all say I don't" I replied.
"Yes, I know - but what you are describing is carcinoid flushing; I think it's carcinoid syndrome."

He went on to explain that if he had to guess, my body was just overly sensitive, and while the increased serotonin wasn't enough to spike my blood work - my overly sensitive system was reacting to the fluctuation as if the levels were highly elevated.

This explanation makes total sense. To say my body is "sensitive" is the biggest understatement of the decade. I can't tell you how many times a doctor has said "there's almost no risk to this - I see no likelihood of any complications" only for my body to prove them wrong, time and time again.

Dr. Marinaro proposed a new treatment for the cystic acne that was an "interesting option" for someone with carcinoid syndrome - a daily dose of 100 mg of Spironolactone, which is primarily used to treat high blood pressure (my blood pressure is on the low side 90/60), but can also be used to treat a hormone (aldosterone) imbalance.

I started the medication, and while I'm a little paranoid about some of the side effects - so far so good. My skin already looks and feels a lot better, and while I haven't noticed any changes in any of my other symptoms, I guess we will just need to give the medicine a little bit longer to kick in before I can really evaluate it. My next priority is to find a good endocrinologist and reschedule my heart stress test.

Update Dec 16th

My skin is miraculously healing and clearing on the new medication. My fevers and night sweats are still rampant, as is some flushing, but my energy level is much better - even though the nausea is back with vengeance. But hey, I'll take clearer skin and more energy for now!

I got the name of a few endocrinologists from the ACOR board - none of whom take my insurance. I did finally find one doctor in Hackensack with my insurance - I'm calling her, and the cardiologist, on Monday.

Gov. Christie Proclaims November 2011 to be "Neuroendocrine and Carcinoid Cancer Awareness Month"

Last Friday, Gary and I spent the day at Sloan-Kettering, which consisted of a CT scan, a follow-up appointment with Dr. Nash as well as a second appointment with my new oncologist Dr. Reidy Lagunas.

For CT scans, you have to arrive an hour early to drink 25ml of oral contrast and get your iv.


As I was sipping away on my artificially raspberry-sweetened concoction, I decided to send an email to Liz Ortiz in Governor Christie's office to see if any progress was being made in procuring a Proclamation for NET Cancer Awareness:

From: Marlena Johnston
Sent: Friday, October 7, 2011 10:31 AM
To: Liz Ortiz
Subject: Proclamation Draft Language: Neuroendocrine Cancer Awareness Day (11/10/11)

Hi Liz,

I am sure that you are very busy, but I was hoping to get a status update on my request for a proclamation from Gov. Christie declaring November 10th Neuroendocrine Cancer Awareness day in NJ.

Given the sad fact that Steve Jobs died Wed. from a neuroendocrine cancer of the pancreas, I feel the time is now for the Governor to help further raise awareness about this rare disease.

The following governors have already issued proclamations declaring Nov 10 2011 NET Cancer Awareness Day:

Gov Fallin (Oklahoma)

Gov Barbour (Mississippi)

Gov Hickenlooper (Colorado)

Gov LePage (Maine)

I sincerely hope Gov. Christie is soon to join them.

Thank you very much,

Marlena Johnston

channelingjackieo.blogspot.com

Yesterday, I got a call from Jeanne Ashmore, Director of Constituent Relations at Gov. Christie's office, with great news - Governor Christie signed a Proclamation declaring November 2011 as Neuroendocrine and Carcinoid Cancer Awareness Month.

In addition to my badgering, the NJ Carcinoid Cancer Network also helped to get this important Proclamation issued. Below is a PDF version of the Proclamation; Ms. Ashmore said she would put a hard copy of the Proclamation in the mail for me today.


I wish I could say that my latest doctor appointments yielded similar concrete results. Basically, I've been going through more of the same - no one seems able to pin-point the cause of my "constellation of symptoms".

The Oscan results came back pretty much normal - some uptake in thyroid (to be expected) and right abdomen (probably scar tissue from surgeries). My blood work has the same flags - but nothing too alarming.

New blood work confirmed that my night sweats, fever flashes and flushing aren't due to pre-menopausal hormone changes. Dr. Nash discovered that I've developed an incision hernia, and the CT revealed that I have a new ovarian cyst which needs further testing. (My mother's first question - is it the same ovary that I had surgery on last year? Good question mom. Didn't even ask which ovary it was.)

Next Tests:
Oct - follow up with GYN on ovarian cyst and sonohysterogram
Nov - heart stress test
July - regular CT scan and follow up with Dr. Reidy Lagunas

Next Steps:
Research homeopathic remedies for pain
Research Essiac (alternative cancer and immunity treatment)
Make an appointment with a rheumatologist for bone pain
Attend regular yoga classes and other meditative exercise

Octreotide Scan Recap

August 16, 2011
On the way up to Boston (for my Octreotide scans), we stopped in Mystic, Conn. for my "meet and greet" with a beluga whale. Afterward, we ate at Sea Swirl, a seafood and ice cream stand which is housed in an old, converted Carvel Ice Cream storefront.

Mystic Sea Port (where Humphrey Bogart and Lauren Bacall spent their honeymoon)

I Love Sea Swirl's Logo: a Fish Eating an Ice Cream Cone

Sea Swirl's clam strips are simultaneously flaky and chewy - in a good way.

Sea Swirl has both soft serve and hard ice cream (including black raspberry); I was especially excited to see watermelon soft serve - but discovered it was vanilla ice cream with a "flavor blast" of watermelon... still delicious.

After we left Mystic, Connecticut, we went to my aunt's place in Wakefield, Massachusetts - where we stayed for a few days in preparation for my Dana-Farber appointments.

August 17, 2011
We arrived at Dana-Farber at 8:00am, and after we wandered around the wrong building and the wrong floor for a while, Gary and I finally found the nuclear medicine department. Perhaps the coolest thing about the entire experience was the crazy-looking metal container that my radioactive iv shot came in - the nurse explained "this container is to protect me, not you." Ok, that's comforting I guess.

After four hours, the scans started. I hated it. I thought I'd be fine after conquering the MRI, but I was wrong. While the Oscan machine is open on both sides, a massive, flat camera gets lowered down until it's right above your nose (for much of the time). Even with a blindfold, meditation music, and deep breathing - I spent nearly every moment fighting panic.

At first, I couldn't figure out why I was having such a problem with the tests (which are a couple of hours long) until I remembered that in my early 20s I went through a phase of having really vivid nightmares of being buried alive. Worse than the nightmares themselves, however, was the fact that somehow I'd manage to crawl UNDER the bed during my sleep... so I'd actually be touching the underneath of my mattress while dreaming that I was feeling the inside of a coffin. I would scream and scream until someone in the house would pull me out from underneath the bed and wake me up. I think having a large flat surface over my face triggered those memories. Hopefully, I won't need to get another Oscan for a long, long time.

My Radioactive "Non-Warning" Label


Octreoscan Machine (the flat screen lowers down once you slide into that doughnut looking hole)


Gene Display at Dana-Farber Cancer Institute

Post Oscan Activities
Since my first Oscan was an all-day event, Gary took in an afternoon Red Sox game at Fenway. I had some time to kill in between my testing and the end of the ballgame, so I went to the Gardner Museum, which is one of the most eclectic, fascinating, beautiful museums I've ever been to. (Travel note: Fenway Park and the Gardner Museum are both within walking distance of Dana-Farber, and totally worth a visit.)

August 18, 2011
After my second day of testing, Gary and I headed home - and hit Frank Pepe Pizzeria in New Haven, Conn. for a clam pie. Gary thought it was absolutely pizza hall-of-fame material; I thought it was solid, but not mind-blowing.

We finally got our clam pie (See previous blog post: Not So Clamtastic aka Shell Shocked)

Muddled Hope: Oscan Test

Editing Note: The above picture has been changed from the original post because THIS is really what an Oscan machine looks like... before that big block looking thing lowers down until it almost touches your nose.

After a lot of invaluable input from family, friends and fellow noid survivors, Gary and I decided to go ahead and keep my appointment at Dana-Farber tomorrow for an Octreotide scan (aka Oscan). After we made our final decision, Gary turned to me and said, "I don't even know what to hope for anymore... I'm not sure if we want the test to come back positive or negative."

I know what you're thinking... how can you want the test to come back positive for cancer? Well, here is the problem - regardless of whether the Oscan test shows that I have carcinoid syndrome, or it's another phantom disease making me sick - there's still a silent mutiny going on inside of my body. If it's related to the carcinoid, at least the enemy has a name... and we have a couple of weapons in our arsenal to fight it. If it's not the cancer, we have no immediate course of action - just more specialists and tests, pain and frustration.

The other tricky thing is that you wouldn't know by just looking at me that I'm sick, so a "positive" result would make me feel... well a little less crazy. Every time I'm sitting across from a new doctor - he inevitably looks at me skeptically after I describe my symptoms - like he just can't believe that this athletically built, happy looking girl (with all her hair) can really have anything all "that" wrong with her. It's like I'm at my car mechanic's garage, trying to explain a weird sound my car is making, and my mechanic is looking at me like I'm nuts.

One of the blogs that I follow is The Cancer Culture Chronicles, which details the journey of a very brave (and talented) author in her battle against breast cancer. Recently, she wrote a post "Look at Me" about this very phenomenon:

" 'You just wouldn't know it to look at you,' clucked Nurse Lovely as she drew my blood and I was explaining the excruciating pain I was experiencing in my left arm and shoulder area. Pain so strong it had awoken me from my sleep several times that week.

I've heard this expression many times, and I'm never quite sure how to respond. The thing is, pain for the most part is invisible, until it causes our facial features to contort, and our eyes and bodies to grow weary with exhaustion." The Cancer Culture Chronicles

So much of what is wrong with me (including a lot of pain) is also invisible. Every day activities - such as climbing my condo stairs, have become difficult mini-battles.



These few little steps used to be a non-issue; now, they are my nemesis. Other small "changes" I've gone through in the last 8 months - that are invisible to the outside world - include:

- sleeping in two towels to try to sop-up some of the perspiration from my severe night sweats
- scarfing down nausea pills when the cold sweats and spinning come on
- regularly taking my temperature to monitor a never ending series of low-grade fevers
- constantly checking my heart rate at the gym to make sure it doesn't suddenly drop to 50 bpm (usually it does this after I hit 140 bpm)
- having to stop and rest - a lot
- making these little whimpers of pain every once and awhile... that just squeak out.

While most of the world aren't privy to this new reality, my friends and family are - and they tell everyone (including me), "I just can't explain it - but she's sick and weak." Their confirmation is comforting, as if they are sitting next to me at the garage with my mechanic saying, "yup- I know that car really well too, and something isn't working right."

So my big question is which girl will the Oscan detect tomorrow: the one who at first blush seems absolutely fine, or the one whose body is slowly deteriorating a little more each month? Will the Oscan come up with some answers, or just tell us to "go fish" again? Whatever it shows, I guess it's good news: negative = no more cancer; positive = a couple of treatments that might make me feel better. I suppose we will just have to hope for "the best"; whatever that may be.

MRI Results

Dr. Nash called yesterday evening to let me know that the MRI scan looked normal. YES!! Now, as long as the cardiologist doesn't find any leaking valves on my echo, we can safely eliminate carcinoid syndrome as the cause of my other symptoms.

UPDATE April 12th: I spoke to Dr. Nash, who called me in between his surgeries today. (This guy deserves a bronze statue in Sloan Kettering's hall of fame.) He explained the CT and MRI results a little more in detail. I have two small cysts on my liver that were not in my previous CT scan. This is not unusual, because CT pictures are taken in intervals - so they can miss things. He just wanted to make sure they weren't new growths or changing, and the MRI delivers a much clearer image for monitoring purposes. (To read more about diagnostic CT and MRI imaging of benign liver cysts, click HERE.)

Syndrome or Disease?

I don't know if it's the cancer or an unrelated ailment...but I'm in real pain. If Dr. House were creating one of his famous "white-board lists" of my current symptoms, it would read as follows:

* history of carcinoid tumor
* fevers and night sweats
* joint pain and stiffness in hips, fingers, toes, ankles, knees, lower-back
* muscle weakness and aching
* radiating, burning pain in shins, thighs, arms
* extreme fatigue and shortness of breath
* abdomen discomfort, cramping, pressure, bloating
* nausea and vomiting (infrequent)
* hot rash over face and chest that comes and goes
* heart palpitations
* low blood pressure
* heart rate that drops with exercise

I just finished a series of RX Augmentin for a lingering ear and throat infection...but it did not have any affect on my other symptoms.

So here's the million dollar question - is it possible that the collection of symptoms could be a result of a syndrome and not a disease? The difference between the two is a little confusing.

A "disease" is a term that indicates a health condition that has a clearly defined reason behind it, while a "syndrome" is a kind of medical mystery - and can produce a number of symptoms without an identifiable cause. To muddy the waters even more, certain diseases can cause specific syndromes.

Carcinoid Cancer v. Carcinoid Syndrome

Carciniod cancer is an extremely rare disease, and a very small percentage of carcinoid cancer patients develop carcinoid syndrome, which have the following symptoms (% of patients affected)

Flushing (94%)
Diarrhea (78%)
Heart Valve Lesions (53%)
Cramping (51%)
Telangiectasia (25%)
Peripheral Edema (19%)
Wheezing (19%)
Cyanosis (18%)
Arthritis (7%)

March 22, 2011 (4 pm) - iphone pic

March 22, 2011 (4:10 pm) - iphone pic

Out of all of those symptoms, I only have flushing (see above), cramping, and arthritis. In a few days, I'll have an echocardiogram to determine if I have heart valve lesions. I don't think I have carcinoid syndrome because usually if you have a midgut carciniod - the liver filters out the excess seritonine, which is the cause of the syndrome. If I do have the syndrome, that means that the carcinoid cancer has metastisized to my liver.

One of the things I don't understand is why none of my doctors have ordered a 5-HIAA test, which is used to determine if the carcinoid cancer has metastasized to the liver, most likely causing the syndrome. I guess that's another test I'll have to ask Dr. Nash about when I see him for my CT scan in April.

New Medical Guidelines for the Diagnosis and Treatment of NETs

The North American NeuroEndocrine Tumor Society (NANETS) has issued a comprehensive set of guidelines to assist medical professionals with the diagnosis and treatment of neuroendocrine tumors. The guidelines are organized into eight manuscripts, each one addressing key aspects of NET diagnosis and treatment by organ site. I focused on the manuscript pertaining to NETs of the appendix, and I was genuinely impressed by its clear synthesis of competing and complementary evidence-based research on this rare disease. The guidelines are not only an important resource for medical professionals, but also a way for carcinoid patients to feel more empowered about their own treatment options.

Surgery Notes and Pathology

I had my post-surgery appointment with Dr. Wagreich today, who was pleased with my progress but indicated that it would still be a couple of months before internally I was entirely healed. When I asked her about my appendix having to be cut away from my abdomen, she said that she had already spoken to Malone about it - and Malone would have to go in and biopsy that entire area since Wagreich, unaware of the potential for cancer, left some "scar tissue" that was connected to my appendix attached to my abdomen wall.

Wagreich also gave me copies of her and Dr. Davidov's surgery notes (he is the general surgeon who performed my appendectomy), as well as my pathology reports. I also went to University Radiology to pick up my barium drinks and CT / MRI reports.

I don't want to brag, but I've passed a couple of pretty tough tests in my day, most notably the California State Bar Exam and English Praxis Test. The feeling is always the same when you see that envelope, and today's little white envelopes were no different. The butterflies still fluttered even though this time I already knew, for the most part, my results. Unlike the CA Bar and Praxis, however, I didn't pass my pathology tests on my first try. (Warning to the medical world of rare cancers: I'm bringing my A-game to all future exams.)

Interestingly, since most of my medical tests and reports were so focused on my ovarian cyst and fibroids, little to no mention of my appendix appears. It is a little scary that no one seemed alarmed until pathology came back. Perhaps the most unsettling report concerns the CT scan because Dr. Malone said that she couldn't even see the appendix on the CT films, yet the CT report indicates the appendix looks normal.

The relevant report findings appear below:

3/18/10 CT Scan Report: "the terminal ileum and appendix appear unremarkable"

4/21/10 Surgery Notes from Dr. Davidov: "appendiceal tip slightly swollen and edematous in a clinical picture that might be consistent with a history of possible appendicitis that had improved, and now has recurred."

4/22/10 Pathology Report: "the tip of appendix is enlarged measuring 1.2x0.8x0.8 cm. sectioning of the tip reveals an area with soft yellow mass measuring 3x0.5x0.5 cm and extends to 1cm from the appendix base. Final pathological diagnosis of appendix, appendectomy: appendiceal carcinoid (3 cm in greatest dimension). Tumor infiltrates through the muscularis propria of the appendix into periappendiceal adipose tissue. Perineural invasion is noted."

Preparing for Appointment: Dr. Maloney Patel

Tomorrow is my appointment with Dr. Maloney Patel, who is a colon-rectal surgeon at RWJ. Hopefully, some of our questions will be answered:

First, starting with what I do know:

Cancer Type: Gastrointestinal Carcinoid Tumor aka Neuroendocrine Tumor (NET)

Date of Diagnosis: April 21, 2010

Tumor Size: 3cm

Primary Origin: Appendix

Now, what I don't know:

What stage is my cancer in?

Stage Determination based on Point of Origin and TNM

T - size (cm)

N - extent spread (regional)

M - extent spread (distant)

Staging for Appendiceal Carcinoid Tumor according ACS:

Stage I: (only for tumors less than 2cm)

Stage II: T2 or T3, N0, M0: the tumor is either larger than 2 cm OR it has grown into the cerum (T2) or ileum (T3). The cancer has not spread to nearby lymph nodes (N0) or to distant sites (M0).

Stage III: either
T4, N0, M0: The tumor has grown into nearby organs or tissues. The cancer has not spread to nearby lymph ndoes (N0) or to distant sites (M0).
OR
Any T, N1, M0: The tumor can be any size and may or may not have grown into nearby structures (any T). It has spread to nearby lymph nodes (N1), but not to distant sites (M0).

Stage IV: Any T, any N, M1: The tumor can be any size and may or may not have grown into nearby structures (any T). It may or may not have spread to nearby lymph nodes (any N). The cancer has spread to distant sites (most often the liver).

NET Tumors: When Size Matters

Because carcinoid tumors are "slow growing" cancers, the likelihood of whether or not the tumor metastasized (spread) is directly proportional to its size. Typically:

If the tumor is less than 1 cm, then there is only a 5% chance that it has metastasized

If the tumor is 1-2 cm, then there is a 40-45% chance that it has metastasized

If the tumor is over 2 cm, then then there is more than a 50% chance it has metastasized.

About 50% of carcnoid tumors occur in the digestive system, 30% in the lungs, and 20% in other organs.

The carcinoid tumor, found in my appendix, was 3cm when it was removed. My gynecological oncologist explained that the treatment for a carcinoid tumor of this size was a right hemicolectomy, and she arranged for me to meet with a doctor she's worked with at RWJ in the past (Dr. Maloney Patel).

Carcinoid Cancer Awareness

Zebra stripes are used to symbolize rare or “camouflaged” conditions. The Carcinoid community wants med students to flip the old adage, and think: “When you hear hoof beats don't assume it's a horse, it might be a zebra.”







Wearing a zebra pin or a zebra-striped bracelet or ribbon shows your support to promote awareness and to fund the education and research needs related to carcinoid cancer.

TWO GOOD WEBSITES:
www.caringforcarcinoid.org
www.carcinoid.org

Carcinoid Cancer Survivor

Someone once said that the minute you decide to fight your cancer after diagnosis you can call yourself a survivor. I like that. On the morning of Tuesday, April 27, 2010, I was diagnosed with appendiceal carcinoid cancer. On the evening of Tuesday, April 27, 2010 I became a carcinoid cancer survivor.