Tuesday, April 12, 2011

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.


  1. Isn't it amazing....how rare these carcinoids are? I'm glad your scans went well and that you have a plan. That always helps and it sounds like you are in great hands!!

  2. Well Marlena, guess what? For the longest time after my surgery I was also constipated and the doctor couldn't understand this. You and I both had a right hemicolectomy and an appendiceal carcinoid tumor (mine was 2cm.)In my case I'm still dealing with severe digestive problems currently linked to a rare defect with my pancreas. After the surgery I kept losing weight (ever since I was diagnosed I've lost 42 pounds) and I am still unable to eat properly, to gain weight and to go back to work.
    I am happy for you that the MRI is negative for any carcinoid tumors or syndrome! Keep writing, I know it helps me and inspires me!

  3. I forgot to add that you are in my thoughts and prayers.

  4. Wow Annie! You and I are both rare birds in the appendiceal cancer world. I'm sorry to hear about your digestive issues...have they suggested any special diets? What about a low-residue diet - like the kind after the hemicolectomy? I get a lot of strength from connecting with other carcinoid patients through my blog - and I really appreciate your kind words :) You're in my thoughts and prayers too.

  5. Hi Marlena,
    My Name is Lisa Lambert and I was diagnosed in 2006 with Carcinoid Cancer in my stomach. I also have started a blog. Still learning how to add all the links and make it pretty. But it is a start I am sure It will get better as I continue. I wanted to share with you I am going to join you (hopefully with my own team) At the relay at Paramus Catholic. Thank you for continuing to raise awareness. This is now my goal also! Zebra HUGS!

  6. Hi Lisa! It would be GREAT if you join us at PC! Let me know if you need any help in registering. Blogging has been a great relief for me - and has connected me with so many wonderful people in the carcinoid community. Zebra HUGS back to you!!

  7. Great post Righ hemicolectomy and hemicolectomy also helpfull