Managing an "Orphan Disease"

"Carcinoid cancer and carcinoid syndrome are orphan diseases that do not garner as much attention as more common diseases, but we are trying to change that" - Dr. Richard Warner



There are about 11,000 to 12,000 people diagnosed with a carcinoid tumor each year in the United States. About two-thirds of all carcinoid tumors are in the gastrointestinal system. Specifically, appendiceal carcinoids account for about 26% of all carcinoid tumors, and out of these - only about 5% of appendiceal carcinoid tumors are greater than 2 cm.

Bottom Line (approximatly): less than 156 people are diagnosed with an appendiceal carcinoid tumor greater than 2 cm every year in the United States; I am one of them.

My friend Christine jokes that I won the very elusive "unlucky lottery" - and when you consider that 1,600 new lottery millionaires are created every year...she makes a darn good point.

If it wasn't enough to have a rare cancer, I also have a laundry list of very unusual symptoms:

* constipation after a right hemicolectomy (Dr. Nash teasingly pointed out during my last visit that this symptom has been conspicuously missing from my previous blogs... touche doctor). Usually, people without half a colon have the "opposite" problem with their bowels. Again, leave it to me to be the odd duck. It's rare, but sometimes the nerves in the colon negatively respond to surgery and the colon doesn't work as well afterward. Dr. Nash said he only sees this reaction in a few patients a year, and I'm on a daily dose of Colace (perhaps permanently).

* a heart rate that drops as my exercise intensity increases

* weakening muscle strength and increased joint pain

* exhaustion to the point of tears

* fevers and nausea

The most frustrating issue is that I'm getting progressively worse with each month - sometimes it's even hard to walk now. Is it related to the carcinoid? Since the MRI didn't show signs of the carcinoid metastasizing in the liver - probably not... but it's such a rare disease, how can we be sure? In search of answers, Dr. Nash and I have mapped out the following plan (including my annual colonoscopy since mid-gut carcinoid increases my risk of colon cancer):

Future Schedule

May 5th: cardiology appointment - Dr. Avendano (Cardiovascular Interventionalists)
June: rheumatology appointment (Hospital for Special Surgery in NYC)
July: colonoscopy - Dr. Maloney Patel (Robert Wood Johnson Hospital, CINJ)
October 7th: follow-up appointment - Dr. Nash (Sloan Kettering)

Thanks everyone for your continued support, love and prayers; I couldn't have gotten this far without you.

Today is the Day

It is always strange arriving at a highly anticipated day. Whether it's a joyous occasion like a wedding or a heartbreaking gathering like a funeral; weeks, months, years of planning are played out within the same short 24-hr time period, after which the sun rises on a "new normal" and we learn to adjust our hopes and sorrows to somehow account for the magnitude and consequences of that one highly anticipated day...

"Everyone who is born holds dual citizenship, in the kingdom of the well and in the kingdom of the sick. Although we all prefer to use only the good passport, sooner or later each of us is obliged, at least for a spell, to identify ourselves as citizens of that other place." - Susan Sontag

- Posted using BlogPress from my iPhone

The Final Countdown

A nice fellow by the name of Darrell called with my surgery time tomorrow: I check into the hospital at noon; surgery is scheduled to begin at 3:00 pm.

Memorial Sloan-Kettering Cancer Center
1275 York Avenue
New York, NY 10065

Since Darrell's call, Europe's great 80s rock anthem, The Final Countdown, has been running through my head (and making me smile):

We're leaving together
But still it's farewell
And maybe we'll come back
To earth, who can tell?
I guess there is no one to blame
We're leaving ground
Will things ever be the same again?
It's the final countdown

Pre-Surgery Appointment at Sloan-Kettering

On July 8, 2010 we traveled to Sloan-Kettering for my final appointment before my surgery with Dr. Nash in a week. They did the usual: EKG, chest-xray, blood work; standard stuff.

After we left Sloan-Kettering, I reflected a bit on the journey so far. In the last six months, I've had 16 doctor appointments, two lab work ups, one surgery, and six diagnostic tests: MRI (2/17); CT scan (3/18); endoscopy (4/2); anoscopy (4/28); sigmaoidoscopy (5/20); and colonoscopy (6/25). All of these diagnostic tests came back negative - even, unfortunately, the MRI and CT scans which failed to identify the large carcinoid tumor in my appendix prior to my first surgery.

Also, July 19th will be the fourth time I'll be put under general anesthesia in the last four months; however, it will be the first time that I'll also have epidural anesthesia. This will allow me to control my pain with the push of a little button for a few days following surgery, until I can eat and drink on my own.

What is Epidural anesthesia ?

After a sterile prep and draping, local anesthetic is placed to numb the area where the Epidural needle will be inserted. The needle for the Epidural passes between the vertebrae of the Spinal column to the Epidural space. Once the position is verified, a very small catheter(tube) is placed via the needle. The needle is then removed and the catheter remains in the Epidural space. The catheter is then taped to the patients back. Local anesthetics and narcotics are given epidurally via this catheter. The procedure usually takes 10 - 25 minutes.

How do I feel about getting an Epidural?

Initially, I was upset. I always thought the procedure sounded scary and painful; but, one thing you learn with cancer is nothing is as bad or as easy as you anticipate. I didn't think I could ever stand a foley catheter, but after minor complications with my first surgery, when I had no choice but to get one, I realized it wasn't that bad.

One of my favorite artists is Kate Bush, and I'm reminded of her lyrics from "Walk Straight Down the Middle" that sum up the reality of anything tough in life, including my next surgery:

But he thought, he was going to die

But he didn't

And she thought, she just couldn't cope

But she did

And we thought it would be so hard

But it wasn't

It wasn't easy, though.

Memorial Sloan-Kettering (MSK) Appointment aka Chicken Wings

There is a great scene in "Tommy Boy" - one of the funniest movies of all time - in which Tommy (a sweet brake pad salesman who couldn't sell lemonade to a man dying of thirst) convinces a disgruntled diner waitress to re-open the kitchen so that he can have some chicken wings. His friend Richard watches in awe:

Richard: That 180 you just pulled on the waitress, why can't you sell like that?

Tommy: I'm just having fun. If we didn't get the chicken wings - so what? We still got that meat lovers pizza in the trunk.

Richard: No, you relaxed. That is what selling is all about.

After this pivotal scene, Tommy remembers "chicken wings" whenever he meets a tough client, and becomes a one-man brake pad selling machine.

Gary and I walked into Memorial Sloan-Kettering (MSK) confident and relaxed. If I didn't like what Dr. Nash had to say, so what? I already had a surgery date at Robert Wood Johnson (RWJ).

Analogy = RWJ: Meat Lovers Pizza :: MSK: Chicken Wings

My Sloan-Kettering appointment took about 4.5 hours, and basically consisted of a series of exams by a nurse, a surgical fellow, and Dr. Nash (including a sigmoidoscopy). After all of the exams, Dr. Nash sat down and answered our questions:

Questions Answered:

Why does the colon have to be removed? We finally understand why a portion of the colon needs to be removed - there are a series of lymph nodes close to the appendix that need to be removed, and in order to do that you have to cut the blood supply that runs to the right portion of the colon - and you can't leave a dead organ inside of you.

What is the difference between Carcinoid Cancer and Goblet Cell Carcinoid? The leading paper regarding Goblet Cell Carcinoid came out of MSK, they do not present the same way in pathology - and look quite different; so if the pathology from RWJ indicates Carcinoid Dr. Nash didn't see any reason to think that it was Goblet Cell...but their pathologists would look for it.

Why didn't my tumor show up on my March CT scan? Dr. Nash actually personally reviewed the CT film and confirmed what Dr. Maloney said - the appendix is obscured by folds in the colon.

How do you die from Carcinoid Cancer? There are several ways, but destruction of the liver is most common.

How soon do I have to have the right hemicolectomy and are there any other options for treatment? Dr. Nash confirmed chemo doesn't work on this type of cancer so it is important to surgically remove everything with a potential of cancer - starting with the lymph noids. Dr. Nash confirmed that a right hemicolectomy was absolutely necessary for any tumors over 2 cm big. However, Dr. Nash disagreed with RWJ on one major point - the timing of the surgery. Dr. Nash said he wanted to wait until I was more fully healed from the first surgery - and when I mentioned that I wanted to wait until after school was out, Dr. Nash said that was absolutely appropriate since the cancer was slow growing a few weeks would only make a negligible difference in metastasizing.

Do I need an Octreoscan? No. Dr. Nash does not feel this is a good test for my case because it is mainly used for confirmation of Carcinoid, and we already have that confirmation. The better tool for follow up testing is CT scans.

How long have I had this cancer? Years.

Is there any chance of developing Carcinoid heart disease? No. I do not present symptoms of advanced Carcinoid Syndrome.

What is my follow up care? Since there is no cure, and no other effective treatment other than surgery, I will have regular CT scans for the rest of my life, followed by surgeries should any tumors reappear. I could never get another tumor, or I could get one in a few years - or in ten years, it's completely unpredictable.

Dr. Nash scheduled surgery for July 19th, and I signed an intent form - which is not a contract - to reserve the operating room. He said if I decided to go with RWJ, that all I had to do was call his office and they tear up the paper.

After a lot of conversations with family and friends, going over pros-cons with Gary, and trying to figure out where my emotions were re: comfort level, I have decided to go with MSK. In short, it makes sense that if they are going to do my follow-up care that I have the surgery there. Also, if anything goes wrong, they are the premier institution in the country for Carcinoid Cancer treatments.

New Time Line:

Cancel RWJ Surgery
Cancel CT (Dr. Nash said it's unnecessary)
June 25th - Colonoscopy (reschedule to another date in June so that I can attend graduation of my 12th graders)
July 8th - Pre-Surgery Testing at Sloan-Kettering
July 19th - Surgery by Dr. Nash at Sloan-Kettering

Sloan-Kettering Appointment: Thurs May 20th

Just got an appointment with Dr. Nash at Sloan-Kettering for Thursday, May 20th (see post below). Dr. Nash only sees patients on Thursdays due to his surgery schedule, and May 13th is already booked (they will call me if there's a cancellation). I don't need a colonoscopy (yay)- but I do need to get my glass pathology slides from RWJ... I wonder how difficult that will be.

Time Line for a Cure

Time Line
I thought it would be useful for everyone, including myself, to have an overall time line of doctors, tests, results and surgeries:

October 2009 - after 10 years of off and on abdominal pain, I begin to experience extreme pain and pressure in my lower right abdomen. I am unable to keep food down due to nausea, and am extremely fatigued

November 25, 2009 - appointment with OBGYN Dr. Pinzon to discuss condition, she orders an ultra sound

December 21, 2009 - t/v ultra sound reveals a complex ovarian cyst and two major fibroids. Dr. Pinzon orders an MRI.

February 17, 2010 - MRI with and without contrast completed at University Radiology.

February 27, 2010 - appointment with Dr. Pinzon to discuss MRI results, which revealed a thick cell wall around the ovarian cyst that contained internal bleeding and complexity. Pinzon recommends consulting with a gynecological oncologist. She also orders a CA-125 test.

March 8, 2010 - CA - 125 test completed at LabCorp (results negative)

March 11, 2010 - first meeting at CINJ (Cancer Institute of New Jersey) with Dr. Alison Wagreich, who reviews MRI and orders a CT scan and endoscopy; she indicates that ongoing health issues could be caused by a necrotic fibroid leaking fluid into the abdomen. Because of my symptoms and the complexity of the ovarian cyst, she recommends surgery.

March 18, 2010 - CT Scan with and without contrast completed at University Radiology

April 1, 2010 - second meeting at CINJ with Dr. Wagreich; small new cyst discovered - CT revealed complex cyst is still potentially cancerous. Surgery is scheduled to remove cyst and fibroids and test for cancer - hysterectomy pre-authorized depending on the biopsy results.

April 2, 2010 - endoscopy performed by Dr. Kastuar (results mild esophagitis, mild gastritis, no bacteria, duodenal diverticulum)

April 5, 2010 - RWJ (Robert Wood Johnson Hospital ) Pre-Admission Testing (EKG, chest x-rays, blood work)

April 21, 2010 - laproscopic surgery preformed at RWJ: removal of fallopian tube, ovarian cyst, two fibroids, and uterine palap - cross section of cyst reveals it's non-cancerous. During the course of the surgery, a general surgeon is called in for a consult to look at my "deformed" appendix. Surgeon gets familial approval to remove appendix.

April 22, 2010 - released from RWJ after slight complications

April 27, 2010 - biopsy of the appendix reveals a 3cm carcinoid tumor - referred to Dr. Maloney Patel for right hemicolectomy surgery

April 28, 2010 - meeting with Dr. Maloney Patel to discuss surgery. She confirms that right hemicolectomy is the next course of action, and orders a CT and colonoscopy prior to surgery. Surgery is scheduled for June 8, 2010. Additionally, she explains that my case will be presented to the RWJ Tumor Board later this month.

May 5, 2010 - Dr. Maloney Patel presents my case to RWJ Tumor Board

May 6, 2010 - follow up appointment with Dr. Wagreich

May 17, 2010 - signed out pathology slides from RWJ

FUTURE SCHEDULE

May 20, 2010 - appointment with Dr. G. Nash at Sloan-Kettering

May 28, 2010 - pre admission testing at RWJ

June 1, 2010 - follow up CT scan at University Radiology

June 7, 2010 - colonoscopy

June 8, 2010 - right hemicolectomy surgery - 3-5 days hospital recovery; 3-6 weeks outpatient recovery

Not Yet Scheduled
Octreoscan Test (if needed)