What IS Men2a? Medullary Cancer? Pheochromocytomas? Hyperparathyroidism?

*I previously posted this in December 2004 but with all the people who still visit this site on their search for MEN2a, I felt it was important to post again WITH corrections because we all know I've learned alot more about this in two years. ) As always, thank you for reading and please remember that I have had two surgeries since this for the pheos and medullary cancer.*

I figured that I would post some information about MEN2a and the things that come with it as well as links for anyone who is interested to read.

"Multiple Endocrine Neoplasia II (MEN II) is a hereditary disorder in which patients develop a type of thyroid cancer accompanied by recurring cancer of the adrenal glands. One type of this disease (MEN IIa) is also associated with overgrowth (hyperplasia) of the parathyroid gland. The cause of MEN II is genetic -- a mutation in a gene called RET. Multiple tumors may appear in the same person, but not necessarily at the same time. The adrenal tumor is a pheochromocytoma and the thyroid tumor is a medullary carcinoma of the thyroid. The disorder may occur at any age, and affects men and women equally. The main risk factor is a family history of MEN II." Excerpt from AllRefer.com- MEN2a.

Basically all that means is that I inherited a mutation in the RET gene (codon C634R) that causes medullary thyroid cancer, pheochromocytomas, and in my case hyperparathyroidism too. Ninty seven percent of people with MEN2a develop medullary thyroid cancer, 50% develop pheos, and 20% develop parathyroid problems. Lucky me got all three. WHO I got this from I don't know. My mother and I are not on speaking terms so she won't tell me the results of her testing and she has denied me my birth father's true name my whole life. So I get to be considered the first in my family history from here on out to have this. We believe it came from my father's side though because I have 6 aunts and uncles and there was never word of this before now. It's hard not knowing for sure though and had my mother been a little less selfish I might not be as far advanced in my cancer as I am now. :(

Symptoms of MEN2 can include severe headache/migraine, heart palpitations, rapid heart rate, profuse sweating, chest pain/angina, abdominal pain, nervousness, irritability, loss of weight, diarrhea, cough, cough with blood, fatigue, back pain, increased urine output, increased thirst, loss of appetite, nausea, muscular weakness, depression, and personality changes.

I've had problems with some of these things over the years but I feel that I am one of the lucky ones. I am not in alot of pain and have only dealt with some things here and there over the years whereas some people have felt poorly and this has completely changed their daily life. For awhile I just thought I was a lazy person and just a weinie to be honest because all these little things would go on. I'm also a very stubborn person though so I have always just gone on and not complained about any of it.

There is no "cure" for this unfortunately although there is hope for one in the future. I am keeping my fingers crossed because my two oldest children have tested positive for MEN2a as well. Only my youngest son tested negative. Had I known about this when I was younger then I could have had a thyroidectomy and been saved from ever having the medullary cancer. We would have known to watch for the pheos and my bones and teeth could have been spared from the hyperparathyroidism. All of this could have been so simple yet someone's selfishness has made it so complex. Of course, I never would have had my children either sooooooo...

Since we've found this fairly early in my children they will have the thyroid surgery and hopefully be cured of the medullary cancer. We will unfortunately always have to watch for the pheos and they have a 1 in 5 chance of developing the hyperparathyroidism. At least they will never have to have my life and go through all I'm about to. For that I am very thankful.

What is Medullary Thyroid Cancer? There are four different kinds of thyroid cancer- papillary, follicular, medullary, and anaplastic. There is also recurring thyroid cancer as well. The first two are the ones most people get and can be treated and possibly cured with radiation therapy and chemotherapy. Anaplastic is a very fast moving and very deadly cancer. It's the one you just don't want to get. Recurring I honestly know nothing about and I apologize for that.

Medullary cancer is thankfully slow moving but it can not be cured by radiation therapy or chemo. In fact, neither one of those really does much of anything for my kind of cancer. People say to me "Well at least you won't have to go through chemo" and I am thankful for that BUT what they don't get is that I will also never know what it's like to be cured or in remission. You might think not having to endure chemo is a great thing but for someone who wishes for a cure and hope, it's not.

Anyway- this cancer is malignant and goes through C cells and calcitonin. Here is a link to help - Medullary Thyroid Info. As you can see there, around 700 cases are diagnosed per year in the US of MTC. They give people like me who it has spread in and who have tumors over 1cm less than a 15% chance of survival after 5 years. Does that bother me? Some of course, but I know of people who have had it worse than me for 20 years so I say "Pppppfffffffftttttt" to their 15%. ;)

What are Pheochromocytomas? Pheos are adrenal tumors that are usually bilateral in MEN patients. Only 10% of the time are they malignant so the cure for them is to have an adrenalectomy. Some people only get one gland out and the tumors never come back. Others battle them constantly until both glands are removed. I have a tumor on each gland and will have to get both glands removed. My children thus far have tested negative for pheos but we will always have to watch and wait.

The most common symptom of pheos is high blood pressure. Other symptoms can be found here along with more information Symptoms of Pheochromocytoma. All three of us have normal blood pressure which in my case the doctors find interesting. With my pheos as big as they are I guess I should be suffering from hypertension but I have always had normal to low BP... thankfully.

What is Hyperparathyroidism? "Hyperparathyroidism is excessive production of parathyroid hormone by the parathyroid glands." Excerpt from AllRefer-Hyperparathyroidism. As you can read at that link some of the symptoms are a royal pain in the butt. For me the itching, "bone" pain, ulcers, just over all achiness, and fatigue get to me. The fact that this has sucked most of the calcium from my teeth upsets me because my teeth are horrible and in a bad state of decay that no dentist could have ever saved me from. I've also had more kidney and UTI infections than I can count and blockages to my right kidney as well. The hypercalcemia that results from it has caused a slight curve in my spine (scholiosis), memory loss, bone and muscular pains, and flank pain as well. I think I hate the hypercalcemia more than anything.

Hopefully what I've posted and the links help you understand this more. You can also Google for MEN2a, pheos, or any of the other stuff to get more information. Thank you for reading.

So Much For That

I guess the correct way to give the CT Scan results is kinda of inconclusive? I need to get a copy of the radiologist's report and read it myself before I can figure out what's what. Right now there's just too many questions left unanswered. Sorry but hopefully things will get worked out and I can come back here and give a good report.

Other than that, I'm back on Ultracet for pain (we're starting from the bottom up again on the painkillers because I'm just not willing to get into the heavy duty stuff) and back on Neurontin for the peripheral neuropathy. I also have to make an appointment with a new endocrinologist, my rheumatologist, and wait for my referral to an oncologist. Busy busy me.

Until then....

Just A Quick Update

aka So I Don't Forget! :) I finally have a date for my CT Scan! I'm happy that I can finally get it done but at the same time, having a reaction to the barium shakes makes me want to hide under my bed and not go. lol.

The scan will be done on March 12th and I'm hoping to get the chest x-ray earlier that same day so everything can just be done and over with. We'll be doing a scan of my neck, chest, abdomen, and pelvic area specifically. What we are looking for is medullary cancer mets in two of those places, check the fibrosis and hemangioma in my liver (bigger? smaller? more? less?), and then the elusive "tumor" in my small bowel. We already know that the cancer is coming back in my neck but we're hoping it's not forming in the lymph nodes in my chest yet or anyplace else.

If we don't see a tumor in the small intestine this time then I will finally breathe a sigh of relief and consider it nonexistant. In 2004 I had my first scan done (with barium and iv contrast) in Georgia which came back showing a tumor in my small bowel. About a year later I had another scan done here(with something else since the barium made me extremely ill and iv contrast) that came back not showing anything. My endocrinologist (at the time- FIRED) decided that their scan was right, Georgia's was wrong, and blew everything off. That did not sit well with me at all so that is why I'm having it checked again and doing it with the barium despite my reaction to it.

As I said, if it comes back not showing anything then I will happily consider that chapter closed and finished with a YAYYYY! ;) If it does show up then I will be having surgery this year to remove it. As anyone with no adrenals/adrenal insufficiency knows... surgeries are just tons of fun! *thick on the sarcasm* but with MEN2a and metastatic MTC it's a necessary evil.

So as soon as I get the results I will update again. Nothing exciting going on besides that. I have to see my rheumatologist soon and restart medications for the fibromyalgia and etc, I need to hire a new endocrinologist, and I need to find out if I should add an oncologist to my "team". Fun stuff I tell ya! Woot Woot. lol.

Also, I'm sorry for so many downbeat posts. I hope the people who read understand that after two years all this has worn on me and this is finally my "depressed" phase I guess you could call it? Being in pain everyday is hard and sometimes it comes through in my typing. I apologize for that. I don't mean to bring the folks who are nice enough to read this down but I have a feeling that most of you do understand and for that I am thankful.

I'm still hanging in there. Sometimes a finger slips off the ledge a little but I'm still holding on. ;)

By the way- tomorrow is my *gulp* Thirty-Second birthday (yep that 3 and 2). How can I be in my 30s when at least in my mind I still feel like I'm in my 20s? Then again, my body feels like its in it's 80s so maybe that evens things out. LOL.