Tuesday, December 28, 2004

Calls With Dr. Wells and Dr. Olsen

Yesterday I talked to Dr. Wells on the phone and he told me that after talking to Dr. Olsen he wants to make sure I have enough time to recover from the adrenal surgery before joining the study. That means that if we need to we will push back me joining the study by a week BUT I will definately be in this study. He said not to worry because he wants me in it. That helped alleviate so much tension and worry. I was so afraid it was going to be a repeat of NIH and I was going to be told sorry we don't want you if you can't do it NOW.

Dr. Wells is such a nice man and it just floors me that a doctor who is so accomplished, so sought after, and so respected would take such an interest in me. I hear he is like this to everyone though and that speaks volumes about him. I just wish the other doctors I've run across would learn a lesson from him.

Anyway- Dr Wells told me that he was going to call Dr Olsen (he is the surgeon who is going to do my surgery) and have him call me sometime soon. Within half an hour Dr. Olsen called me and started asking me questions so he could decide what route to take. He ended up telling me the best news I have heard yet.

He wants/hopes to do a Bilateral Laproscopic Adrenal Sparing Surgery on me. What this means is that they leave a part of the adrenal gland and remove everything around/inside it including the tumor. The inside of the gland is called the medulla and the outside is called the cortex. By doing this surgery they hope to make it so I NEVER have another pheo. By leaving part of the adrenal gland it means that I won't have to be on replacement hormones and worry about the things that come along with that. VERY good news. VERY VERY good. :) Of course, there is a chance that the pheos could still come back and I could end up having to have all of my adrenal glands taken out later on but to me this is a risk worth taking. Even if it just buys me a few more years of not having to be on replacements then I'm willing to take it. I think the fact that the laparoscopic surgery is so much easier to recover from than the open surgery Tampa wanted to do helps too. I don't think I would want to go through two open surgeries you know?

Dr. Olsen also said he wants to schedule me for an MIBG scan and an Octreotide scan. Looks like alot of fun doesn't it? Ugh. LOL. I will also meet with him and then most likely the next week I will have my surgery.

So hopefully this is the beginning and things will work out. On the dark side, I have finished my Living Will, and appointed Vin to make decisions for me pertaining to healthcare. Now I just need to finish my will and have it all go to a Notary Public to be notarized. I want to make sure everything is set and ready before any of this takes place. I've also already listed what is to be done with my remains as well and Vinny knows what I want done.

Sunday, December 26, 2004

What IS Men2a? Medullary Cancer? Pheochromocytomas? Hyperparathyroidism?

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 http://health.allrefer.com/health/multiple-endocrine-neoplasia-men-ii-info.html#definition .

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. 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 on 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. This cancer is malignant and goes through C cells and calcitonin. Here is a link to help http://health.allrefer.com/health/medullary-carcinoma-of-thyroid-info.html . 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 http://health.allrefer.com/health/pheochromocytoma-info.html#definition . 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." http://health.allrefer.com/health/hyperparathyroidism-info.html 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.

Saturday, December 25, 2004

Just A Line

I had emailed Dr. Wells to see what was going on and get an update on when they might schedule my adrenalectomy. I honestly didn't expect to hear back from him until after Christmas but today he emailed me back telling me that he would call me on Monday and some other stuff. The last sentence of his email made me smile because it's nice to know SOMEONE in the medical profession honestly cares.

"Hang in there. I am committed to taking good care of you and your children. Sam Wells"

To me what he said is a big deal. One of the most respected doctors in the endocrine field is committed to taking good care of us. It may sound stupid but it helps me to breathe a little easier now. :)

I'll update after I talk to Dr. Wells on Monday. Hopefully we can get things rolling soon even though in his email it sounded like I might not get to start the study at the end of January like we had hoped. I know I'm still going to be in it, just not sure on specifics now.

Wish us luck and thank you to everyone on Life With Heathens (my site), MD, Mamauniverse, Amitymama, and yes even you ladies on 20ish (wink) who have been keeping us in your thoughts and prayers. We appreciate it more than you know and it means the world to me.

Happy Holidays!

Jo and Crew

Thursday, December 23, 2004

Yet MORE Incompetance!

I called the Pediatric Endocrinologist in Florida yesterday to find out whether or not she ever referred the kids to Shands or not. I got the answering machine telling me that starting this past Monday they were on Christmas vacation until next Monday. Needless to say I was BEYOND ticked off! She was suppose to call me last Thursday and yet she could never be bothered. That just pisses me off! After calling Vin and ranting to him about it we decided that yet AGAIN we would have to do everything ourselves. So I emailed Dr. JS at Shands explaining things to her while Vin called her office and left a message for her to call me back.

Well of course she or her office never called but this morning I get this email...

"Dr. M at Shands is probably the world's expert on thyroid tumors and has had excellent results with one of the surgeons here at Shands. We can see you the first Friday after New Years or you can be seen by Dr. M. We will coordinate and determine the earliest time we can schedule an appointment and get the ball rolling for you. Dr. W did contact me, and I let her know what resources are available here. She is an excellent endocrinologist and may have already made the referral. We will proceed to get things done as quickly as possible."

Ummmmmm okay who the hell is Dr. M? What does a thyroid TUMOR expert have to do with my kids who have NO thyroid tumors? Was she talking about ME? Because if so no thank you, I'll stick with Duke thank you very much. Can she be anymore stinking vague??? This email doesn't tell me much of anything and it kind of ticks me off. Now if she planned on calling me and setting things up this would be fine but they can see "you" the first Friday after New Years blah blah blah and then she says they will coordinate and determine the earliest time we can schedule an appointment?? An appointment for who? With who?

I figured it would be better to post here before I email her back because I want to just go off on her completely. Please, be as vague as possible and run us around in circles and let US do all the work again. Please... we insist.

I also realize I was too hard on Vin before. I do feel he is thinking more with his head than his heart but it's how he is and I know this is hard on him as well. I'm afraid I'm quite moody thanks to not only my own illness but all the crap we deal with as well. I don't stop to think like I should that he might be stressed out as well. You would think after almost 5 years I would be better at this co-parenting thing but I'm not. I still go into defense mode when it comes to my kids and I still try to make all the decisions myself. *sigh* Things would be so much easier around here if we didn't have so many attitudes and thick heads. LOL

Tuesday, December 21, 2004

Kids' Calcitonin Results For Cancer

Dr. L called me today with BigB and Sis's calcitonin level results. Calcitonin is what they test to see if the medullary cancer is present/has started. Normal levels are 5 or under.

She said that Big B is at 28 and Sis is at 24 meaning that although the numbers are low.... the medullary cancer has started in them. She tried to make it sound like it's not a big deal but considering I've researched this well and know EXACTLY what everything means, I know that it's much better than if it was higher but still it isn't what you hope for. I mean, cancer is cancer no matter how much or little a person has. The fact that neither one has any lumps or swollen lymph nodes that we could feel in their neck though is a good sign. All we can hope for now is that it hasn't spread past their thyroids. If it has stayed there and not gone near any lymph nodes then the thyroidectomy will still rid them of it.

I just keep thinking over and over that this just SUCKS!! I think I made the mistake of getting my hopes up after the pheo results came back negative. Why I let myself hope I don't know. I guess I just wanted and needed to hope for SOMETHING.

So Vin now feels that I should get the kids' thyroidectomies at the same time I get my adrenalectomy. I just can't get through to him that I HAVE to be there for them when they get this done. This is the biggest thing to ever happen to them and I NEED to be there. It's my job. I'm their mom. Period! He talks about what's convenient but I talk about the heart. Something we also have problems with. *sigh*

The Pediatric Endocrinologist from Florida STILL hasn't bothered to call me to say whether or not Shands in Gainesville will take on the kids' cases. So I get to call her tomorrow and ask her what the hell is going on.

I just have a problem understanding why we're paying people to ignore us, blow us off, and screw up our stuff. What is so hard about them calling me when they say they will or even at least in that same WEEK??! I am thus far very not impressed with doctors.

Thursday, December 16, 2004

Another Talk With Dr. Wells

Tuesday night I emailed Dr. Wells about what my endo here had said. To be honest, I trust his word more than her's because he is a specialist and has 30 years experience with MEN, MTC, and so on. My endo is a general endocrinologist and although she is trying her best, I'm her first MEN case soooooooooooooo.

Yesterday (December 15th) I talked to Dr Wells on the phone and he said that yes I can have the laparoscopic surgery done on my adrenal glands and whether I'm in the study or not and whether I want to remove the tumors or not he would STILL recommend it to me because it is much easier. I asked him how he could be certain that the pheos are only on my glands and not what the spots in my liver and so on are as well. He told me (and this was new to me) that they have yet to see a patient with MEN2a who had malignant pheochromocytomas or pheos outside the adrenal glands. He has no problems answering my questions or calling me back anytime I call. I like that. Heck, he's been the Director of the American Association of Surgeons before and I can get ahold of HIM.

I told him I want in the study. I also told him that even if it does nothing more than shrinks a tumor or two I will consider it a success because it holds hope for my children. He told me "Oh no no we are working on a cure here. You will definately see more happen than a few shrinking tumors". He says this drug has shown remarkable results in Phase I of the study and seems to be doing well in the Phase II now which is what I would be in. He sounds really confidant... seriously.

We discussed me having the adrenalectomy at Duke University and he said he has already spoken to a Dr. John Olsen who "who is highly experienced in laparoscopic adrenalectomy. He is top class and you will like him". I asked him if I needed the Octreotide Scans again and told him Vin was wondering how they could know for sure that my pheos are pheos (which I told you how above). He said the Octreotide scan doesn't always tell you what they want to know and he doesn't know why they are doing it. It's pretty much a waste of money right now. He said that everything I've told him thus far is pretty textbook and he has seen plenty of MEN2a patients with exactly the same things I have going on and is pretty confident. They will of course look over my medical records and my CT scans that I had done. Oh and when I told him Tampa wanted to do another CT scan he said "why?". I told him they said to see how much growth there was. He told me these things wouldn't really grow much at all in that short of time so there's no sense in it. He talked to me about the alpha-blocker I would be on for the 10 days before the surgery. Most likely I will be on Dibenzyline for that.

Another thing he did was ask me what we had set up for the kids thus far. I told him as of today NOTHING and explained a little. He told me that if I wanted I could have the kids' thyroidectomies done at the same time I get my adrenalectomy done. I honestly don't know how I feel about that because I want to be there to take care of them when they have it so I might have to put it off until February (their surgeries). He said that we should be able to do my surgery the first or second week of January and that will give me about 10 days to two weeks of recovery before I start the study.

He told me they are very interested in me both because of this study but as a person as well and that they will take good care of me. That was nice to hear. I'm waiting to hear back from him and trying to get an actual surgery date set up. Wish me luck!!!

Wednesday, December 15, 2004

Kids' Pediatric Endo Appointment A WASTE!!

Well today was the kids' appointment in Florida(almost two hour drive), with the Pediatric Endocrinologist.

We got up at 5am this morning, got ready, and we were out the door by 6:10am. Lil Man was wide awake which surprised me. About an hour into the trip we were all worried that I took a wrong road and wondering if I should turn around and go back when I finally saw a sign that said we were on the right road. We got there an hour before the kids' appointment so we went back to the gas station so they could use the bathroom and then went back to the doctor's office. All three kids did wonderfully on the trip. Not a peep from anyone and the trip actually went pretty quickly.

Got into the office, signed in, let them make copies of the kids test results thus far, and a few minutes later we went back.

Big B is 5'6 1/2" tall, 114 1/2lbs, and his blood pressure was 100/74 if I remember right.

Sis is 55 1/2" tall ( four and a half feet tall I guess?), weighs 71lbs (YAY she's finally over 70lbs!!!), and her BP was 88/68.

Doctor came in and pretty much right away let us know that she had talked to my endo and they had decided that it would be more beneficial for the kids to be seen in Gainesville, FL at Shands which has a Pediatric center. This appointment was pretty much worthless because this ped endo will be turning over the kids' cases to Gainesville. So we made a trip for NOTHING and the kicker is that MY doctor supposedly knew that before we even left. So she sent me there for nothing. I will find out for sure when I email her later and get the whole story.

So after about 30 minutes there of the doctor asking the kids if they understood what was going on (to which they both said yes and Sis informed her that her mommy is very smart and knows everything about this. ) we left. She did say that neither one has any lumps in their throat or lymph nodes meaning the chances of either having developed cancer yet is pretty slim. She also said that only one in five people with MEN2a develops hyperparathyroidism so the chances of either of the kids getting it is slim as well.

Left the doctor's office, realized it was too early to get lunch, drove home, dropped Big B off at school around 11:30a, got some lunch, and then dropped Sis off at school even though she was considered absent for the whole day because it was after noon when we got there. I told them had I known that I wouldn't have wasted my time bringing her to which I got a shocked look from the secretary. Whatever lady... not in the mood.

So came home and read my email from Dr. Wells. Called him, he called me back, and... on to the next update eh?

Tuesday, December 14, 2004

Appointment With Regular Endocrinologist

Waited over an hour in the waiting room, went in, 5 minutes later Dr. L came in, told me that I can't have laparoscopic surgery, let me know considering the study wasn't really smart, told me to not worry about being cut open and that lots of her patients have no adrenal glands no biggie, told me to get daycare to take care of Lil Man after surgery, gave Lil Man dirty looks because he was misbehaving, seemed irritated that I told her to call Dr. Wells, gave me copies of the kids' tests, told me that my problems with focusing and remembering things (I can't go anywhere by myself anymore because I can't remember what I'm doing from one minute to the next) are most likely from stress and/or depression, and I left.

The kids 24 hour urine test came back and as far as that goes they aren't showing any signs of pheos yet. The rest of the test results are currently MIA. No clue where they are or why they weren't with these test results. So the good news is no pheos in the kids yet.

I hate doctors. I really really hate doctors. That is the suckiest part of all this. I hate doctors and the Fates KNOW I hate doctors so they stick me having to deal with them every month for the rest of my life.

The longer version of what I said before is that I guess they were running way behind (I waited an hour to see the doctor) and I don’t know if that is what made the doctor moody/distant or what but she was NOT acting how I expected. I had told her in an email that the way things are at home I just would rather not get treatment right now because I’m safer that way. It's just hard dealing with all the attitudes at home some days. She made the appointment with me because she said that worried her and she wanted to talk to me. Well from the moment she entered the room she was trying to rush me out the door.

I started to tell her about the study with Dr. Wells and she cut me off before I even got to start. Basically she let me know that she thought it was stupid of me to even consider the laparoscopic surgery. She even went so far to tell me that I couldn’t have it done because my tumors were too big. When I said we would be leaving the tumors in my liver and intestine she got this look on her face like she couldn’t believe I would even consider such a thing. *sigh*

I told her I’m having trouble focusing lately and that I can’t go shopping by myself anymore because I will forget what I’m doing and also end up in a part of the store and not remember how I got there. If the kids or Vin is with me it’s not a big deal but I went to Walmart the other night for what should have been a half hour trip TOPS and was gone for 2 hours. Ugh. So anyway- she informed me that it was most likely due to stress or depression and she would hate to blame EVERYTHING on the pheos.

Ummmmmmmmm no actually you can blame that on the hyperparathyroidism although she now says my calcium levels were fine. Okay then WHY are we saying I have hyperparathyroidism??? Funny, I’m staring at test results that show my calcium out of normal range and my parathyroid hormone out of range as well. It MUST be that I am just a depressed mess. *eyeroll* Why do doctors always think if a person goes through a rough spot then they MUST be depressed? Maybe some of us actually can handle bad things happening in our lives. I wish they would consider that.

This really sucks and I am stressed out and at times things seem depressing but I don’t have time for that crap! I have three kids to worry about so I will be damned if I’m going to get depression thank you very much!! Oh and for the record- I've been under WAY more stress before so stress is not causing my problems right now. It takes alot more than this crap to break me and if I was depressed I wouldn't be the one doing so much to try to get this stuff taken care of!!!!

Anyway- so I didn’t get to tell her about the study, she flaked me off, told me I need to get the scans in Tampa, and told me basically to forget the laparoscopic adrenalectomy. Like I said, I hope she was just having a bad day because I was really starting to like her.

Monday, December 13, 2004

December 13, 2004- Kids Bloodwork Redone

Kids ended up having to go back AGAIN to have blood drawn for a third time. This time the reason was because the doctor got so caught up in how sad she was for me when she told me the oldest two have MEN2a that she forgot to order the calcitonin test. It upsets me but at the same time, I can forgive her because she really DOES care alot about us.

So last Wednesday I was going to take them back again to have it done when they realized that they DO have the Elma cream (numbing cream) to use on Sis so she doesn't feel the needle stick when they draw blood. So we picked that up from the Pharmacy there and then went and got our Christmas tree. On Thursday, Big B's school bus broke down and he didn't get home until after 5pm so we didn't get to go then either.

I took the kids up on Friday and this girl I haven't seen before informs me that if they draw the blood it will go back because the people at Quest Laboratories aren't in on the weekend. I told her she has GOT to be kidding me! She says that she can go ahead and draw the blood anyway if I want but it would go bad and we'd just have to come back. I couldn't believe her stupid ass! I looked at her and said "Why in the hell would I have you stick my kids yet AGAIN for no reason whatsoever? It's bad enough we're here a THIRD time and two of those times are because of screw ups!" Then I told her to make sure they are ready for us on Monday and to make sure they don't screw it up again and know whether or not to freeze the blood or not.

So we showed up today after Big B got out of school and go back and it's the usual two girls that I like who are working the lab and drawing blood. Theresa (Lab Tech) asks me "Who told you they couldn't take blood on Friday?". I tell her that some girl with brownish black hair in a white coat that I've never seen before. She then asks me "Was she tall?". I told her "Hun, everyone is short to me". She laughed and said that was true and then I told her that the girl was younger and looked in her early 20s. I asked her why? She then tells me that had the girl bothered to look in the lab book she would have seen that the samples need to be FROZEN for the test they were doing and the blood could have been drawn on Friday just fine saving us today's trip. I was HIGHLY ticked off to say the least!! It looks like I will be filing yet another report tomorrow when I go to my appointment with my endo. *sigh* This crap gets real old real quick.

I haven't heard back from Dr. Wells yet but it could be because he hasn't been able to reach Dr. L yet. I'm going to make sure she knows he is trying to reach her and give her his phone numbers to speed things along a little bit. She wants to talk to me about some stuff and I plan on getting whatever results of the kids' are in and also copies to take to their Pediatric Endo appointment on Wednesday. I also have to talk to her about the fact that my problems with focusing and memory lapses are getting worse. I would also be willing to bet she hasn't heard a single thing from Tampa yet either.

Wednesday the kids have an appointment with Dr. NWin Florida. She's a pediatric endocrinologist who I guess will take over the kids' cases. I'm going to see about pushing for some surgery dates for the kids here real soon so we can get them done. I just don't want them to have to go through this too. Hopefully their pheo and medullary cancer tests come back clean and the surgeon here can do their surgeries. I like him and trust him and hope eventually maybe he can do one of my surgeries as well. Anyway, this ped endo will be the one who handles the kids' meds after their surgeries. The nice thing is she comes here once a month so that will mean we won't have to constantly travel there.

I think Vin finally took me seriously about the adrenalectomy today when I emailed him a link about it and then a link about adrenal crisis. He now knows exactly what we have to look forward to and exactly how sick I can get if things aren't taken care of right. It's about time is all I can say. I know this has all got to be really hard on him too but I need him to be there for me because if him and these kids aren't it could seriously cost me my life. That's so stinking scary! :( Anyway- I'll update again tomorrow after my appointment. Keeping my fingers crossed.

Thursday, December 09, 2004

Big News!!!

Where to begin?

I joined this medullary cancer board a month or two ago just so I could at least read on there what other people were going through. There are actually some MEN2a patients on there as well. One of them is named Mary and even though I only posted once on the board she has still been emailing me to get updates. VERY sweet lady.

She told me about a study that is being done at Duke University by Dr. Samuel Wells. Dr Wells has 30-40 years of experience working with the RET gene (the mutation we have), MEN, and medullary cancer. He is one of the doctors who designed the test the kids and I took to find out out results about this. VERY knowledgeable man with a list the length of your arm of credentials.

Anyway- I emailed him a few days ago and just gave him a short history and asked if he would please keep me in mind for any future studyings he might do. He almost immediately emailed me back saying I match the criteria for the current study he is doing and he would like to talk to me. I gave him my phone number and the very next day (yesterday) he called me.

The criteria is that you have to have a calcitonin level over 1000 (my last one was over 24,000) and you have to have medullary tumors of 1cm or larger (all mine are 1cm or larger). It is also of course preferred if you have MEN, FMTC, or a genetic reason for having medullary cancer.

The study is on a drug that has been shown to shrink and totally destroy some medullary cancer tumors. They first tried this drug on lung and breast cancer patients with wonderful results and found that the drug basically attacks the RET gene meaning it should do wonders for people like me. The HOPE with this study is to shrink or get rid of tumors and stop the medullary cancer from spreading or coming back. In other words, this is the prelude to what someday could be the cure for my children and their children.

From what I understand the study is 8 weeks long and takes place at Duke University in Durham, NC. The first two weeks you check into the hospital as an inpatient and they do a CT scan on you, bloodwork, EKGs and so on. They start giving you a pill once a day and check your calcitonin levels to see if they are going down (your calcitonin levels only go down if you have tumors removed or cancer cells are reduced). They also do EKGs to check how your heart is doing.

The doctor said that we would work something out so I could see my children (aka all three when/if Vin brings the oldest two and Lil Man daily) and do what I need to do for my family. That was such a relief!

It starts January 28th and as I said I have to stay there for two weeks and then can commute (8 hour drive one way) back and forth the rest of the time. After the first two weeks you have to be there weekly for half a day and stay close for 24 hours so they can draw blood and do an EKG and monitor you. If the drug is working then after the study you would continue on it with routine follow ups. You would also continue to get the drug for free. For being in this study you get the hospital stay for free whenever you are there, the drug is FREE (believe me, you do NOT want to know how much something like this costs!), and they give you a living expense so you and/or family can afford to be close by and travel for all/part of the study.

They have one patient now that is in her third week and is 77 years old. She has shown definate marked progress so that is HUGELY encouraging! The second patient starts next week and then more of us come in January.

That alone was great news but there is more. So on the phone Dr Wells asks me what I have set up in Tampa. I explain to him that they want to do an open adrenalectomy on me so they can remove the adrenal glands, the tumors in my liver, and the one in my intestine. He says I definitely need to have the adrenalectomy before I can be in the study because one of the possible side effects of this drug is hypertension (high blood pressure) and that’s not good in someone with pheos. He tells me that he is NOT trying to change my mind but if I want to be in this study they would of course need me WITH my tumors.

What that would mean is I could get a laproscopic adrenalectomy instead of the open. The laproscopic is MUCH easier. Less surgery time, three small incisions on each side of my back, less recovery time, less time in the hospital, less bleeding….. much better and what is usually done. It would mean that I would only spend a few days in the hospital for the surgery, get out, and be able to move around better after about a week. The thing with the adrenaline would still be the same but I wouldn’t be cut from side to side and have to worry about trying to heal for two to six months. They would leave the tumors alone so that this med has a chance to do it’s work on them. I would still need surgery later on for my throat even if this med works because I have two calcified nodules and I still have the hyperparathyroid problem. It would mean less work on my neck though and that helps too.

Dr Wells said they can do the adrenalectomy there at Duke if I would like (I would still have to pay for it of course) and that I don’t need to do all the scans and tests that Tampa wants me to do. Basically I would just start taking the one medication to shut down my adrenal glands 10 days before the surgery and then show up to have it done.

Let’s see what else? So the plan we are working on at this point and driving up to NC and having my adrenalectomy done VERY soon. Then we drive back, I recover for a week or two (or more depending on when I can get this done), and then drive back up to NC for the study. Rent a hotel room for 2 weeks while a friend or two stay in it with Lil Man and I get all my testing. Go back and forth taking care of Lil Man and then after two weeks come home. Make the trip up there once a week for the routine testing and then come back home. Hopefully by this summer have the thyroid surgery done and then be finished HOPEFULLY with surgeries for a very long time.

Best case scenario of this study is it rids me of tumors, kills any cancer cells, and stops the gene mutation meaning I never get medullary cancer again. It’s a long shot but at this point I will take ANY improvement or even hope for the future. I really don’t care what it does for me. I want it to have a chance to work for my kids.

Monday, December 06, 2004

Misc Stuff

Figured I needed to update a few things. Let's see- the kids had to get more blood drawn because the day we went the regular lab girl wasn't there so another girl handled the specimens. She was suppose to put the blood in a purple top tube so that it could be sent to one lab and then to another for various tests. Instead she put it in a tube that is frozen meaning only one lab could use it. In other words, she royally screwed up. I was NOT happy to say the least.

The only funny thing is that now that I have gotten people fired TWICE since this all started they are all VERY respectful and about fall all over themselves. I find it funny because if they had just done their jobs in the first place we would all be much happier.

So anyway- the kids' results from the 24 hour urine should be back hopefully this week and the blood tests should be back soon after. I'm afraid to hope but crossing my fingers just the same that they come back negative for the cancer AND the pheos.

I obviously made it just fine to the airport to pick up my MIL. It was much easier than I thought it would be to get there. She was honestly alot of help and alot of support while I was down there. I couldn't tell her enough how thankful I was. Of course, I also have this nervous habit of talking nonstop and proceeded to blab to the poor woman constantly. How embarrassing is that?!! If you ever read this Nancy... I am soooooo sorry. *blush*

So now we wait. Hopefully I'll be able to make the appointments at the end of the month for all the testing but who knows right now. This is one of those times when having family would really be helpful. A bunch of my friends are trying to help and it means the world to me but even they can only do so much. I would never expect them to disrupt their lives for me.

Sunday, December 05, 2004

December 02, 2004- Tampa Trip Day Two

Got up and did the same routine as the day before after being up all night with a 2 year old who was running a 102 degree fever. One of his mystery fevers again of course. The fever finally broke around 2am and he slept okay after that. I was awake trying to figure out how to do everything, feeling more and more like I should just cancel all this, and having "bone" pain.

Vin called a few times (after 8am) over a fiasco with Big B at school which stressed me even more. Seems my oldest child was trying to raise the bus window and dislocated his thumb (come to find out it was only "jammed" a little and the nurse wouldn't listen to him).

After breakfast Nancy and Lil Man dropped me off again at Moffitt. Check in was easy this time and I got my pager and sat down to wait. TWO AND A HALF HOURS LATER the pager finally went off and I went back to be weighed, BP taken (103/71), and wait for the doctor. Let me just say that while I was waiting that two and a half hours I had a lady teach me how to do a crochet stitch or two. That’s how bad it was.

About 15 minutes after I sat in the room a med student/resident came in to get my history again. I’m glad that I’m such an oddity that I make a great learning tool and all but after making me wait over two hours do NOT screw with me! I was in no mood to play guinea pig anymore. Put up with him (he really WAS a nice kid just wrong day was all) and then waited to see the surgeon. Dr BC finally came in and started talking to me.

This is where everything changed and my head started to swim. Basically he said he wanted me to understand just how serious and complicated this surgery was so I wouldn’t have any surprises afterwards. He said we are going to remove both my adrenal glands, burn out all the tumors in the liver, and remove the tumor in the small intestine all in one surgery because the more they go in the higher the risk to me is.

For 10 days prior to the surgery I will take a medication that basically shuts down my adrenal glands. If I roll out of bed like a normal person- I will faint. If I get up too fast out of a chair- I will faint. If I bend over and come up too fast- I will faint. Then a day or two before the surgery they will start injecting me with steroids to level off my blood pressure so it will be safe to go in for the surgery.

The surgery itself will take 6-8 hours. After the surgery they will screw with my adrenaline some more for a few days. I will spend about 5 days in the ICU unit because of this. They will cut me from one side of my abdomen, under my ribcage, and to the other side and then open me up and do the surgery that way. LONG way from the three little cuts I thought I was getting in my back. :(

It will take me two to six MONTHS to recover from this he says. I will not be allowed to pick Lil Man up, vacuum, or do anything that has to do with lifting. It takes so long to heal because they have to remove things and cut through so much muscle.

After the surgery, I will be on two medications for my adrenaline for the rest of my life. I will have to wear a medic alert bracelet as well because if I am ever in a stressful situation aka childbirth, car accident, etc then the paramedics at the scene have to give me an injection of adrenaline so my body will deal with it or I can die. Since I will be on medication I won’t be able to have adrenaline rushes like normal people do whenever I’m in a stressful situation so that is why they would have to give me an injection. If I’m under stress here at home I will have to raise my meds in order to deal with it. He says expect to feel like complete hell for at least 6 months.

So that is where we are at right now. No surgery date at this point. The surgeon said it would be sometime after the holidays and with the way they have things set up I’m thinking the middle to end of January IF we go ahead with treatment right now.

Friday, December 03, 2004

December 01, 2004- Tampa Trip Day One

Nancy, Lil Man, and I all got up early, took turns showering, got ready, had breakfast, and then left for the hospital. Aidan honestly seemed happy to have a "grandma" around.

We arrived at Moffitt around 9:40am (my appointment was at 10:15a) and Nancy dropped me off at the entrance so her and Lil Man could go do things while they waited for me. I went up the elevator to the second floor, checked in, fifteen minutes later was registered, and sat down to wait.

While I was waiting I called Nancy (Vin’s mom) to check on Lil Man. He was crying and mad at her because they were at the zoo and she made him sit in a stroller. LOL. He really loved looking at the monkeys though which was good.

I ended up sitting in the waiting room for an hour and a half! I was getting really ticked off and was about to start complaining when my pager went off and I was finally taken back to a room.

Got into an exam room, was weighed, had my BP taken (98/62), answered some questions, and then waited for the doctor. A few minutes later a med student/resident came in and asked me questions. She checked for swollen lymph nodes, examined me, asked more questions, and couldn’t seem to grasp the fact that I had TWO calcitonin tests done and my levels were at 24,000 NOT 16,000. She said that her and Dr LK would be back.

A few minutes later Dr LK came in and let the student/resident give him the rundown on the info she had gathered. Again she said my calcitonin levels were 16,000 and AGAIN I corrected her, she argued with me, and I let her have it! I told her “You DO know that after those first four pages there you have my WHOLE medical file from the beginning of this including ALL my test results and everything I’ve had done INCLUDING the second calcitonin test which was higher than the first". She looked a little upset at me but I was too busy looking at the doctor to see if he understood how ticked off I was getting.

They had my medical records in their hands for a full WEEK before I got there and do you know that not a single solitary person even bothered to look at them? They knew NOTHING about me when I sat down with them. NOTHING! If they had bothered to look at my records they could have scheduled all my testing while I was already there. Instead…… NOTHING!!

Dr LK asked me a question here and there and then showed me two of the CT scan films that showed my adrenal glands. He showed me where the tumors on my glands were and said he feels that those are the pheos. After that he examined me and then told me that they would remove both adrenal glands to get rid of the pheos, then they would cut or burn the tumors in the liver, and then do something about that one in the intestine. Of course, I will also still have to have the thyroid surgery as well.

He then told me that he treats to cure not for a quick fix so he expects me to be cured when we’re all done. From what I understand, you can’t be cured of this because of the MEN2a but it’s nice that he thinks so. He ordered another CT scan, Octreotide scan, and ALL the same bloodwork I’ve had done over again. Of course, our insurance won’t pay for another CT scan nor will they pay for all the bloodwork to be done again. :(

They wanted me to come back December 6, 7, and 8th for everything and a follow up appointment but I just couldn’t do it. There was no way I could have come back this week because Vin doesn’t get paid until Friday for one and there was no one to watch the kids while I was gone ANOTHER three days. I eventually had to settle for them to set up the testing on December 21 and 22 and then a follow up appointment on January 4th.