The 'gift' that keeps on giving - MEN1

I've been thinking about this blog post for a long time - it's a hard one to write because it talks about the fear, anxiety, and frankly, denial that come with being diagnosed with a genetic disease. This is *my* experience - your mileage may vary.

To start, a brief explanation of Multiple Endocrine Neoplasia (MEN1) from the Mayo Clinic website. Everything stated below hasn't necessarily been my family's experience but it's a good introduction:

Multiple endocrine neoplasia, type 1 (MEN 1), sometimes called Wermer's syndrome, is a rare disorder that causes tumors in the endocrine glands and parts of the small intestine and stomach. In MEN 1, the endocrine glands — usually the parathyroids, pancreas and pituitary — grow tumors and release excessive amounts of hormones that can lead to disease. However, the tumors are usually not cancerous (benign).

The excess hormones can cause symptoms such as tiredness, bone pain, fractures and kidney stones, as well as stomach or intestinal ulcers. Although MEN 1 can't be cured, if regular testing detects problems, doctors can provide treatment as needed.

MEN 1 is an inherited disorder, meaning people who have the gene mutation can pass it on to their children. Each child has a 50 percent chance of inheriting the disorder.

I can't remember exactly when I found out, or understood, that it was a genetic issue that caused the cancer that claimed my father's life. It may not have been until I was 11 years old when my mother took my brother, sister, and I to a local clinic to have a blood test done. I did understand that we were getting tested to find out if we had the same disease our dad had. Understanding that was pretty scary, but not as frightening as the prospect of getting a needle poke and blood drawn was. I had one vial of blood drawn and passed out cold - thus beginning a pattern that has continued into my adult life -  but that's a topic for another time.

Genetic testing for MEN1 didn't exist back in the 80s, of course. What we were being tested for were elevated calcium levels which would be a sign of hyperparathyroidism, typically the first presenting complication of MEN1. It would be unusual for a child to develop hyperparathyroidism but, sure enough, I had elevated calcium though my brother and sister did not. I had MEN1 - if you have a family member that was known to have MEN1 and present with one trait of the disease it is a confirmation of a positive diagnosis. I am not certain that I understood at this point what having MEN1 really meant for me in the long term. I knew more blood was going to have to be drawn from time to time which was scary enough but don't think I comprehended or was told much more than that.

My mother and I were referred to Shands Hospital at the University of Florida. This is where my father was diagnosed with MEN1 and where he sought treatment and surgery for his pancreatic neuroendocrine cancer. At age 13 we made our first trip to Shands where I was hospitalized for a few days and underwent numerous scans and blood tests. Frankly, this felt like torture for a kid who was anxiety prone on even a normal day. I was old enough to understand now that I had a disease that had killed my father but also had been told that the same thing might not happen to me. I also understood that I would have to have testing for the rest of my life to stay on top of issues that might develop. Based on the passing out, throwing up, and general anxiety I showed on that trip, I am also sure that my Mom also didn't look forward to the prospect of more testing. My roommate on that particular trip was a girl of about 16 that had terminal cancer. My mom and I both remember her cries of pain and her mother's anguish. While I may have felt bad for myself, I had that in my face reminder of how lucky I truly was. In the end it was determined that I could still wait for surgery. My calcium was elevated but not out of control and I had no other symptoms of hyperparathyroidism or MEN1 to deal with.

My next trip to Shands was at age 17. I'm not entirely sure why there was a lapse of several years before this return trip. I would guess that it had as much to do with anxiety and fear on the part of my mom and me as it did with the busy schedule of a working mom and two teenage kids. And besides that, I felt perfectly fine and was a healthy, busy teenager according to anyone's standard. A blood test and ultrasound on the first day at Shands told a different story though, my calcium levels were very elevated and my parathyroids had grown quite large. Surgery was needed and there wasn't any putting it off. So I had my first surgery at age 17 (1988). It took 8+ hours for the surgeon to locate and remove 3 of 4 parathyroids and I was in the hospital recovering for a week. (Kind of funny to look back on those facts, now a parathyroidectomy only takes 1-2 hours and you may not even stay overnight.) I was old enough to understand that this wasn't likely the end of my MEN1 journey but that there was no telling when other issues (pancreas, pituitary?) may present themselves. I think it's also important to say again that I felt absolutely no symptoms of my hypercalcemia. Without testing we just wouldn't have known. I was also advised that if I ever wanted to have children that I would need to undergo genetic counseling. While I had never had an overwhelming desire one way of another about motherhood, this did make a big impression on me. I didn't see myself ever wanting to go through a selection process for healthy children knowing that I wouldn't have made the cut myself. I didn't like thinking of myself as 'damaged' and this pretty much put an end to any thoughts of having children.

From this point on it became my responsibility to care for my MEN1 and I was certainly old enough to understand what this might mean long term. I spent my college years fearful of what the future might bring but at the same time I was fatalistic in my outlook. After all, this was in my genes and not something I felt I could prevent or even minimize based on my behaviors. I didn't have the feeling of invincibility that some young adults have but also didn't see a need to avoid bad habits like smoking cigarettes.

Coming out of college, I wasn't fortunate enough to have employment that gave me access to health insurance. One of the first opportunities I had was once I joined Americorp in 1995 and got very basic health coverage. At that time I went to see an endocrinologist in the Atlanta area who screened me for high calcium, which was back, but there was no way I was going to be able to afford surgery or more advanced scans. My 20s were spent pretty much in this pattern. 

During this time, I brought up MEN1 with my family on occasion. By this time a genetic test had become available and I shared this info with my family. I didn't feel like it fell on receptive ears though. By this time I think we knew that my brother had exhibited some elevated calcium levels but since neither he or my sister had any symptoms I suppose we all figured what could be the harm. We were all still young - in our 20s and 30s - I suppose we thought there was still time. I even remember discussing with my sister what the symptoms of MEN1 were and she wasn't experiencing anything. I think we hopefully concluded that she didn't inherit the issue. 

In my 30s I think this same feeling - was it denial? - began to be my default  about MEN1 as well. By this time I had been to 3 different endocrinologists who didn't seem particularly concerned about anything other than my elevated calcium readings. In fact no doctor ordered anything other than blood work for me. No CT scans, no MRIs. Again, I felt very healthy and had begun pursuing cycling and seemed to be in great health. I certainly didn't understand that the doctors I was seeing weren't familiar with MEN1 and probably weren't familiar with the protocols for screening. There still weren't a lot of online resources about MEN1 at that time though a few were beginning to pop up. But by the time I was in my mid to late 30s I very rarely thought of MEN1 any longer as being part of my life. I knew I probably needed another parathyroidectomy but just couldn't be too bothered to schedule time off for a surgery on something that didn't even seem to bother me at all. I logically knew that there might be long term consequences for hypercalcemia but... well, I don't know. There just isn't a good excuse.

Of course if you're at all familiar with my story you know that this all came crashing down for my family when my sister was diagnosed with stage IV neuroendocrine cancer in 2008. Debbie felt fine like I said - until she didn't anymore. I will never forget the phone call I had with Debbie when she asked me what our father's symptoms were. I was still in such denial and want to believe that Debbie had hepatitis or some other liver issue - anything but that MEN1 had caused this but of course it had. Those feelings of guilt and regret over the next six months were very hard and they still nibble at me from time to time.

Looking back, there were actually small symptoms that Debbie and I shared. GERD that we both handled by taking a PPI or with Tums. TMI ahead... diarrhea that we probably should have realized was happening a little more often than normal. But I think we both just figured this was part of getting older. We also come from a family culture that didn't put up with whining. I still get a kick out of the fact that my Mom always told me that I was too young to have a headache whenever I asked for an aspirin growing up. A little of this attitude isn't a bad thing - but too much of it clearly isn't. Had we been a little more educated about MEN1 we also would have recognized that we were exhibiting classic dermatology symptoms of MEN1. Angiofibromas that kept popping up on our noses, collagenomas on on our chest and upper arms. A researcher could probably fill a career on our family MEN1 dermatology issues alone.

So when all was said and done it turns out that all of us kids did have MEN1. Every single one of us hit that 50/50 genetic jackpot. I don't think a single one of us felt so much sad about this for our individual selves as we did for our Mom - that is more than a woman who already lost her husband to this disease should have to tolerate. My parathyroid issues were the 'strongest' in my family which is why I likely presented so early with elevated calcium. By the time I got around to my second parathyroidectomy in 2008 the surgeon said my tumor (benign) was the largest they had ever removed at Mayo and my calcium levels were through the roof. All 3 of us had pancreatic tumors that were malignant, though, thanks to my sister's diagnosis both my brother and I discovered ours before there was any metastatic spread. My brother also has thymic and pituitary issues he is dealing with that I have not yet exhibited. One of the ways that MEN1 is particularly unfair is that males are subject to a thymic complication that females don't seem to be. Looking back, it seems based on anecdotal evidence that my father may have had thymic carcinoid himself.

So why put this all out there? I'm hoping these lessons will help another family. Here's the conclusions I've drawn:

1) Find a doctor that knows what they're talking about when it comes to your rare disease

I shouldn't have wasted time on doctors that didn't specifically know MEN1. Even once I went to Mayo clinic for my screening I had an admitting doctor that said he was quite sure that I was fine since I was in such good health. That gave me a glimmer of hope that just didn't prove to be true. 


If you can afford it, go to one of the big centers that treats a lot of MEN1 patients; Mayo Clinic, MD Anderson, etc. Do a web search, there are resources out there. For example, amensupport.org.

2) Educate yourself and be your own advocate

There is a ton of info out there on MEN that wasn't available back when I was diagnosed. You can even get protocols on how often you should be screened and what tests should be performed. If you can't get to a doctor that knows MEN you can at least bring them a copy of a screening protocol. 

3) Be an advocate in your family

Ah, regret. I didn't want to be the doomsday speaker in my family and I just gradually bought into a hope that everything would be fine. If I had only known a little more, found out where genetic screening was available. Woulda, shoulda, coulda. Don't let that be you.

4) My MEN1 is not your MEN1


For that matter, my MEN is not my sibling's or parent's MEN. The spectrum of issues with MEN1 is going to vary from person to person. Some families, like mine seem to have more malignancies. Other families may go a lifetime with only parathyroid issues. My siblings and I had pancreatic tumors that produced different hormones for each of us. 


The thing is, you shouldn't read my story and assume things will be the same, or even similar, for you. Which brings me to my last lesson learned...


5) Being fatalistic doesn't work - you can help yourself

Being in great shape from cycling and triathlon likely played a big part in my speedy recovery from my pancreatectomy. Having training to look forward to day to day plays a big part in my mental well being. It turns out that cigarette smoking can exacerbate thymic carcinoid in males with MEN1. That is certainly something that we didn't know growing up that might have made a difference in the choices my siblings and I made.

It also doesn't help me to spend too much time looking at MEN1 studies online. It was overwhelming to see all the medical studies out there about MEN1 and shortened life span when I was getting ready for my pancreatectomy. I recognize that MEN1 has a place, sometimes quite a big place in my life, but I need to fill my life with lots of positives to make up for what has been so devastating for me and my family. So I try not to give it too much more thought than what it deserves. Sometimes it requires a lot of time and thought - sometimes it's very little.

I was very fortunate last year to be put back on a yearly maintenance plan at MD Anderson after three years of frequent checks. So far, so good. PTH and calcium levels are good, hormone levels and scans on my abdomen, chest, and brain are crystal clear. So time to keep on keeping on - crossing my fingers for positive outcomes and taking care of what is in my control. That's the best anyone can do and I'm really hopeful that good things are in store.

Remembering Joel

I just posted an entry on the 18th about a friend who had run out of options in his cancer care. When I woke up this morning I did my usual look through Facebook and saw that his brother had posted a couple of pictures of Joel in the very early morning hours. I immediately knew what that meant without having to look any further - Joel had passed.

I met Joel many, many years ago when he was just a little guy. His brother Jason had befriended me in our first year at our local junior college. Jason was a life saver at that point because most of my friends had left for out of town schools and I was floundering because I knew so few people. Jason brought me into his group of friends and made my two year of junior college a lot of fun. I wouldn't be nearly the geek I am today without the influence of that sci fi and comic book loving gang. Jason helped me get a summer job as a youth director at his church and there I met Joel and a lot of other great kids. Joel was cute as a button with a funny voice and a great sense of humor (it runs in the family). I only spent a summer working there and then went off to finish my last two years of college in Alabama.

Fast forward many years to the time when my sister was sick and I was going through my own treatment. Joel reached out to me via Facebook to let me know he was available to talk. I had stayed in touch with Jason a bit throughout the years and knew Joel had thyroid cancer at 15 but I hadn't realized the extent of the cancer at that time or that he had to have continual follow ups since his teen years. Turned out that Joel knew the ins and outs of endocrine cancer much better than I did. This is Joel's story from the MD Anderson website.

We exchanged a lot of facebook messages and a couple of long phone calls. There is something about talking to someone else that has had cancer that strips down your walls in talking about the disease. I could be honest with Joel about how weary I was - for me and my family. He listened to me talk about the grief of losing my sister and my frustration with my brother's diagnosis. I was mostly struck by Joel's unwavering optimism and faith despite the difficult therapies and treatment he was enduring in never ending succession. I told him that I honestly couldn't seeing myself enduring that. There were some other topics as well - but it's a little too real to talk about - particularly today.

In time, I got dramatically better and rebounded stronger in some ways than I was before. Joel and I actually switched up treatments centers and teams right around the same time. He moved back to Florida and went to Mayo and I started going to MD Anderson in Houston. During my checkup last year I got excellent news - all my hormone levels were stable, all scans were crystal clear - I was now clear to go to annual check ups. Joel was one of the first to congratulate me. I was looking over our facebook messages this afternoon and want you to see what he said to me:

Susan, Glad things went well at MDA. You're setting the bar high and an inspiration to me. I just got back from Mayo today, they have a couple drugs in mind... Just so happy for you. Pop is staying with me right now and we've been praying for you and your brother. God is working on you. I'm being wordy at this point but I'm just so joyfully tearful for you. Love you so much, Susan.

I can't be sure exactly what kind of news Joel had gotten at Mayo that day but it surely wasn't as positive as mine was. Tell me what kind of caring, great person sees that a friend got great news when maybe they didn't and gets in touch to let you know how happy they are for you? I wish that was the kind of person I was but I am not sure it is. 

Now, of course, comes the regret. Why didn't I reach out to Joel more often in the past year? I heard from him in September that he was going to be in Atlanta in October and we were going to meet up but I never heard back. Maybe he had a decline and couldn't make it? What was so important in my life in October that I didn't make time to get in touch? Logically I know this is a normal part of loss but that doesn't make it easier.

When I saw the post from Joel on the 18th I knew that he would be gone very soon. He was so relentlessly optimistic and him saying that it looked like the end of the road was a sign to me that it surely was. I sent him a message via facebook but can't bring myself to tell you all the content but do want to share one thing:

I will be keeping you in my thoughts as I train this year for Ironman. I know a lot of people think I'm crazy for doing this training - but you know what I know - this is EASY compared to the journey we've been on and you've been on it longer than me. This training gives me a sense of peace and joy. Your spirit also give me a sense of peace and joy Joel.

Even if he didn't get to read or hear this - I know that Joel knows how I feel now - and I very much doubt he would want me to dwell in regret. So on the way to my ride this morning I thought about Joel and put on the most painfully happy song I could think of, Jonsi's Go Do. (I warn you - Jonsi is a character and you may not love the video. Just listen to the music and lyrics.) One of the odd things about me is that a sad song doesn't generally make me cry, but a joyful happy song? I cry buckets. So I cried and sung along:

You wish surprise, will never stop wonders
You wish sunrise, will never fall under
We should always know that we can do everything

It Doesn't Get Easier

I woke up this morning and started perusing Facebook on my mobile phone. The first status update I saw was from a friend that has been fighting thyroid cancer for many years. It appears that he has been told that there isn't anything more that can be done. 


It immediately took me back to hearing the same news for my sister. You know what it means, but it's so harsh and hard to hear. At one point when I was talking to my sister during this time and I started to cry. She looked at me in a concerned and worried way and said 'I thought we were used to this?'. But the truth is, there is never a point at which you get used to this no matter how many times you have been through it with family and friends. 


So I am thinking of my friend today and for many more days to come. He has been a source of strength as I have gone through my own journey. He has fought long and hard, in a way that I don't think I have the strength for. Believe me, training and doing an ironman is *nothing* compared to the constant grind of surgeries, scans, shots, chemo, radiation, and on and on that someone who is fighting cancer for years goes through. 


Much love and peace to you always JD.

About Those Missing Organs

The title of my blog refers to not one missing organ but a few. This post is about my pancreatectomy and those other missing organs.

I should tell readers that one thing I committed myself to when doing this blog is talking about some topics with honesty. If you’re finding your way here due to a search on MEN 1, pancreatectomy, etc., I want to give the real scoop on what it’s been like for me. And really, it’s been quite good all things considered. I have had a fantastic recovery that has made me able to continue with my active lifestyle.  Not everyone that has this surgery is that lucky, there is a lot of room for complications and the chances are high that one will crop up. So while I’ve had a great recovery and it’s been much easier than most – it’s not always as easy as it may seem to the casual observer.

Technically, what I had was a laprascopic pancreaticduodenectomy, but that’s too much of a mouthful to say. Checking out during this procedure were my pancreas, duodenum, gall bladder, bile duct, and spleen. The last three were mostly obliged to say goodbye due to the removal of the first two. Dr. Asbun at Mayo Clinic in Florida did the procedure along with his surgical team and completed the surgery in about 9 hours. Pretty good considering we were told it could be 14 hours. Since the surgery was done laprascopically I only have a few scars. The longest one which is only a couple of inches runs into my belly button. (I assume that’s the incision where these organs came out but perhaps you are like me, and prefer not to think about this level of detail.) There are a few other small incisions around my abdomen adding up to 5 small cuts where the cameras and instruments were positioned to make the cancer go bye bye. If you looked at my belly today you wouldn’t have any idea that I had such a big surgery. I’ve even received some puzzled looks from doctors when I tell them my medical history and they then do an exam. “Oh, it was laprascopic”, I say. “Yeah, not many surgeons do it, he was awesome”. I had great experiences with almost all my doctors at Mayo and Dr. Asbun was one of the stand outs. Funny, caring, and personable in addition to being experienced with this surgery.

I have blogged several times about what the diabetes after effects of the surgery have been like and how I manage them but the piece I don’t often talk about and that you can’t visibly see is what it’s like to live without the exocrine function of your pancreas. In addition to producing insulin your pancreas also produces several digestive enzymes. These enzymes are primarily responsible for digesting fats and proteins. Since I no longer have a pancreas I take a supplement to replace these enzymes, in my case it’s Creon 24. I tried a generic for a while after my surgery but this was definitely a case of generics not working as well as a brand name drug. I take 2-3 Creon with each meal depending on how much I’m eating and how fatty the meal is. God forbid I forget to take my Creon with a meal, particularly if it’s a fatty one. It’s extremely uncomfortable for me for up to 48 hours and unpleasant for everyone in my general vicinity. I think that’s as detailed as I will get. I think you catch my drift. (Get it? Hardy har har!)

In the short term I lost a LOT of weight. I had purposely put on some weight prior to surgery knowing that I would drop a lot. I think the grand total ended up being close to 40 pounds. For about a year there was no amount I could eat that would put that weight back, but gradually I did start putting weight back on and am now capable of gaining weight just like the good ol’ days. The experience did make me appreciate weight gain as a good thing rather than the negative association I had always had. 

Long term I am on a proton pump inhibitor (Omeprazole) and a lot of supplements. I have deficiencies in a lot of electrolyte and vitamin deficiencies due to poor absorption. Primarily magnesium, calcium, iron, vitamin A, vitamin E, and vitamin D. Check me at meal time and you’ll find me swallowing a lot of pills trying to make up for these deficiencies. It took a year or so but those deficiencies seem to be pretty much worked out at this point and there’s always a blood test around the corner to make sure I’m on track.

1 day of pills and supplements

One issue that continues to bug is some occasional stomach pain. From time to time I get a ‘burning’ sensation when I eat that can last for a few days with each meal. It’s not a killer pain but there is enough discomfort to make me wince my way through it. More of a pain, both literally and figuratively is a very occasional ‘attack’ that seems to be textbook bile duct/gall bladder blockage. It’s only happened a handful of times in the past couple of years but is enough to get me doubled over on the floor. Fortunately Ellen has got it timed to not lasting for more than 8 minutes and she counts down the time for me until it’s over. As best as anyone can tell, there may be a little fragment of a bile duct left and bile may be trying to make its way out. Too bad bile – you’re stuck there and it would seem I’m likely stuck with the pain. Crossing my fingers and knocking on wood that this remains a very occasional experience.

All in all, pretty darn good. Better than the alternative!

My (Long) Story

The about me section doesn't give nearly enough space to explain the journey that has led me to do this blog. Here's the full scoop...



I was diagnosed with a genetic disorder, Multiple Endocrine Neoplasia Type 1 (MEN 1), at age 11 after my father passed away from metastatic pancreatic neuroendocrine tumors.  In 1988 I had a subtotal parathyroidectomy due to continued elevated calcium levels and kidney stones that showed up during ultrasound. All other tests pointed to normal pituitary and pancreas functions. 


Life went on very normally for the next 20 years. I had occasional blood test monitoring for MEN 1 every few years with different endocrinologists in the Atlanta area and I took my good health for granted. My calcium and PTH levels continued to be elevated but all other hormone levels were normal and other than some osteopenia I felt no other complications.


In July 2008 my sister was diagnosed with metastatic pancreatic neuroendocirne cancer. This was a shock to my entire family since Debbie didn't demonstrate the elevated calcium or PTH levels that I, or eventually, my brother did. We had thought that she had escaped the genetic disease and, unfortunately, we hadn't as a family taken advantage of the genetic tests that had become available since our childhoods. Debbie fought hard and, as she did throughout her life, she taught me a lot of lessons before she passed away in February 2009.  


Debbie's diagnosis was a wake up call that sent me searching for a doctor or facility that was familiar with MEN 1 and neuroendocrine cancer. I underwent testing at Mayo Clinic in Florida between September and December 2008 and was found to have new parathyroid tumors and several neuroendocrine tumors in my pancreas. I had a total parathyroidectomy and auto-transplant into my forearm in October 2008 and after a lot of thought on my part and advice from the staff at Mayo, I had a total pancreatectomy in December 2008.     


Since then I have returned to triathlon. It's been a big challenge as I have had to learn to train and compete again as the equivalent of a type 1 diabetic. Now I'm ready to take a stab at Ironman and am training to compete at IM Canada in August 2012.


So this blog will be about a lot of stuff... primarily I want to record my Ironman journey - that's for me more than anyone else and it will help me avoid posting all of it to Facebook. I don't think a good deal of my FB friends care whether I ran, bike, or swam that day and are hardly impressed. :)


But I also want to talk about what it's like to do this as a diabetic. What it's like to survive and be fundamentally altered by cancer. What it means to have a genetic disease that has so drastically affected me and my family. How I'm getting through the grief of losing my sister - I'm sometimes shocked that is still such a work in progress. Maybe some of that would be helpful to the reader - we'll see. 


Thanks for checking in!