5th Anniversary

Today is my 5th TGversary. I had my stomach removed 5 years ago today.

It's been awhile since I've posted. I post less when I am doing great. 5 years seems like such a major milestone. I definitely wanted to post.

5 years ago I had so much fear and anxiety. I had no idea what my new life was going to be like. 5 years later, I can say life is good. It's actually been so much better than I could have ever anticipated. I won't lie and say it hasn't been without its challenges, but I have found a new normal...which is really close to the old normal. If someone meets me today, they have no idea I don't have a stomach. I do all of the things I did before.

I recently became a grandmother for the first time. My granddaughter will think it's normal for her grandma to not have a stomach, she won't know any differently. I'm so glad I will be around to enjoy her. And I am even more delighted that she will not have to worry about this horrible gene mutation. Her mother tested negative. My other daughter tested positive, but it unlikely to have biological children. So this is the end of the line. I don't have to worry about passing on the CDH1 gene mutation to my descendants. That is the best news of all!

CDH1 and COVID-19

2020 has been a year of graduations, not the high school and college graduation ceremonies that have been canceled, postponed, or made virtual, but leaving behind all things related to my cancer…except those that can’t be left behind (like not having a stomach).

This whole journey started in April 2015 when I was diagnosed with breast cancer. That May I found out I was positive for CDH1.  I had a double mastectomy in June 2015 and then a total gastrectomy in February 2017. That’s the background info. Now to the graduations. I’ve already written about my follow-up at MD Anderson in January of this year. At that appointment, Dr. Mansfield said I didn’t need to come back and see him anymore, that I could be monitored annually by my primary physician. Graduation #1.

Next up was the oncologist in June.  I was actually hoping I could skip that appointment, but I’ve always been one that if a doctor wants to see me, I go. This was my third oncologist in five years. I was going to the same cancer center, but the previous two doctors had left for one reason or another. My breast cancer was ductal carcinoma hormone-receptive positive, so I was prescribed a hormone blocker, letrozole, for five years. I had always assumed that once the five years was up, I’d be done with the oncologist. Since five years would be in June and I had enough drugs to get me through till past then, I called the office and spoke with the nurse to see if I needed to bother keeping my appointment. My breast surgeon who I saw in April, and will continue to see, actually suggested this. The nurse checked with the oncologist and he said he still wanted to see me, so I went. I met my third oncologist, he didn’t do any bloodwork or anything, we just chatted, and he released me. I don’t need to follow-up with him anymore. I did like him, and I did appreciate something he said. He said he would still be my doctor and was still part of my medical team, even if I wasn’t seeing him and that if I ever needed him, feel free to give him a call. I don’t expect to need him, but I still appreciated that sentiment. Graduation #2.

The oncologist told me to finish the prescription I had and then stop taking the letrozole. Since I was receiving a 90 day mail order prescription, that actually lasted me quite awhile. I just finally took the last pill a week ago. Graduation #3. The only regular medication I now take are vitamins.

As well as documenting my CDH1 journey, I feel compelled at this time to document the COVID-19 journey that we are all on and this blog seems as good of place as any to do that. This isn’t so much for those reading it now but more for the memories later of what we went through and for future generations that will read about 2020 in history books. I’m sure you, like me, never believed that we would be living in a global pandemic.

March started out normal enough. My Kansas Jayhawks were ranked #1 and were most people’s favorite to be National Champions. We were Big 12 Conference Champions again (after ending the 14 year streak the previous year) and were looking forward to the Big 12 Conference tournament and the NCAA tournament.

My best friend from college had ancestors from Fredonia, Kansas, the same small town in southeast Kansas that my parents were originally from, that I’ve had family in for generations, and where I still have family living. We had talked since college about going down there and never had. The last time we were together, we had decided that we were going to do it over her spring break this year. She had information about where the farm was near Altoona, cemeteries in Altoona and Fredonia she wanted to visit, and then we were going to do research at the Wilson County Historical Society museum in Fredonia. She is a professor at the University of South Dakota and her spring break was the week of March 9. We decided to go down Tuesday morning and spend the night, coming back Wednesday afternoon.

We had a great time. It was fun to get away and spend time together. She learned some information about her ancestors. In doing the research, I also came across names in my family tree multiple times and the trip reignited the love of genealogy in me. It was a great trip all the way around, but the best part was just spending time together. Little did I know how soon and how much things were about to change.

 Before our trip there had been some news about this coronavirus that had started in China and there were a few cases in the US, especially in Seattle, California, and New York, but nothing close to the Midwest. As a Department Chair, my friend started receiving texts on Tuesday about being prepared for possible online learning after spring break. That was my first indication that maybe this was turning into something bigger than we had initially thought. Then, just before the first games in the Big 12 tournament Wednesday night, the Big 12 announced that the games starting on Thursday would proceed, but with no fans in attendance. Then on Thursday, just before the games were to begin, they canceled the tournament all together. Many other conferences did the same thing, some in the middle of a game. Later Thursday, the NCAA also canceled the NCAA tournament meaning the Jayhawks would not be able to compete for the National Championship they were favored to win. Disappointing for sure, but this was the reality check that this virus, now called COVID-19, was much bigger than we had realized. The World Health Organization declared it a pandemic on Wednesday and a national emergency was declared on Friday, March 13.

When I returned to work at the church on Thursday, everything went quickly into COVID-19 mode. At first things seemed normal, but that all started rapidly changing. Every hour was like a day with things changing so fast, including the changes in sports mentioned above. My email inbox started filling with articles about the coronavirus and how to prepare. We had a funeral scheduled for the following Monday. The family had come to town to meet with the pastor to make final plans. They ended up deciding to postpone the funeral as they had family traveling from Seattle and other areas more affected. They thought they would wait till June when it would all be behind us. (It is now August and we haven’t yet scheduled the funeral.) We had a youth group from Oklahoma planning on coming to Kansas City and staying at the church for a week of mission the following week. Early Thursday afternoon, I had an email they were still coming. By late afternoon, they had canceled. That’s how rapidly things were changing.

We still had in-person worship on Sunday, March 15, although some area churches didn’t. We did make modifications such as no Passing of the Peace, etc. Our attendance was half what a typical Sunday would be. By Monday, the decision had been made to cancel all in-person meetings, events, and worship at the church, so much of my time was spent getting that communicated to our members and other groups that use the building, as well as assisting with plans for online worship the following week. The original plan was to keep the church office open.

That changed over the weekend when Johnson County declared a Stay-at-Home order effective Tuesday, March 24, followed by the State of Kansas on March 30. We had one day in the office on Monday to prepare to work from home. I did occasionally go into the office to collect mail and other needed items as well as to do some essential work that couldn’t be done from home. That continued until May 11 when the Stay-at-Home order was lifted and the church office reopened.

When everything was being shut down in March, we thought it was all temporary and things would return to normal soon, at least by May or June. The more time went on, the more we discovered that life wasn’t returning to normal anytime soon. Things started reopening, not because anything was better, but because there was pressure to open the economy. We had to make adjustments and find the “new normal” – social distancing, wearing masks, all these were part of the new normal. No one knows when, or if, life will return to what we think of as normal.

While we couldn’t have in-person worship, we developed other ways to reach the congregation, mainly though email and social media. One of the things we did was send out a daily devotion via email and it was also posted on Facebook.

When I reflected on the devotion I would write, I realized life being uncertain and developing a “new normal” was something I was familiar with. When I was facing my gastrectomy a little over 3 years ago, life on the other side was very uncertain. I knew things would never be “normal” again and didn’t really know what the “new normal” would look like. Sounds a little like how many of us have felt the last 5 months. Life on the other side of COVID-19 is uncertain. We’re not sure that our life will ever be “normal” again and are wondering what the “new normal” will look like.

We returned to in-person worship on June 21. It has very little resemblance to worship before COVID-19. In the summer, we always have Worship in the Park, an 8:00am outdoor worship service. It has been the most popular worship service this year. It had 51 people the first week and has consistently had in the 30s or 40s, a significant increase over last year’s numbers. The two worship services in the sanctuary have each been having less than 20 people, that’s 1/3 to 1/4 the number pre-COVID. People are obviously more comfortable worshiping outside than inside.

The two sanctuary services are also live streamed. Congregational singing is not allowed at any of the worship services, the hymns are sung by a soloist or a small group of no more than 3. At the park, people are asked to allow at least 6 feet between parties and they usually allow even more. In the sanctuary, every other pew is roped off and families are spaced out. Many churches have made the news by being a place the virus has spread. We don’t want to make the news.

We are living in a time I never thought I would experience in my lifetime. I still have a hard time believing it, but here we are. We do what we can to be cautious and stay safe, while not being fearful. In his sermon last Sunday, our pastor talked about the difference between caution and fear. The scripture was Matthew 14:22-33, the story of Jesus walking on the water and how Peter got out of the boat with faith, but then started to sink when fear overcame him. We need to remember during this crazy time we find ourselves in that God always walks beside us and guides us, if only we let him.

We don’t know when this will all end, probably not till after there’s a vaccine, which could be a year yet. In the meantime, we try to figure out what the new normal is and to stay safe and healthy. That is my prayer for anyone reading this blog.

Goodbye Houston

We flew down to Houston on New Year's night for my follow-up with Dr. Mansfield at MD Anderson on January 2.  It was surreal walking into the room at the Rotary House, the hotel adjacent to MD Anderson.  We've stayed there for previous follow-up appointments, but I don't recall feeling the same way I felt this time.  Maybe it's just that I am doing so well, I feel so removed from when we were there nearly three years ago for my surgery.  I flashed back to then, being there the night before surgery and facing the fear and the unknown. I flashed back to being there recovering after surgery, not knowing what to do, not knowing how to begin life without a stomach, throwing up in the bathroom sink....it all came rushing back to me.  Keith had some of the same emotions as he commented that he saw me as more fragile in that space. 

We went over early on Thursday for my blood draw appointment to increase the likelihood of them actually having the results before my appointment.  Dr. Mansfield was also running behind so they had all the results except one at the beginning of the appointment and got that one before we were done.  I was within normal range on everything. My B12 count was normal, but at the low end of normal.  They suggested I increase my dosage to two sublingual tablets a day instead of just one.  They also recommended getting a bone density scan done as they like to do that around 3 years after total gastrectomy, which is coming up soon.  But that can be done in Kansas City with my primary care doctor.  I will also want to continue having blood work monitored annually, but that can all be done up here.  I'm done with follow-up visits to MD Anderson.

Four years ago I had never been to Houston.  Now, 9 visits later, it was time to say goodbye.

I was excited to learn that Dr. Mansfield and a team from MD Anderson is doing a research study to put together a CDH1 Gene Registry to track those with the gene mutation and their medical information, family history, etc.  Hopefully, through this research, they will be able to learn more and eventually people with the mutation will have more information and more options when determining whether or not to have a total gastrectomy.  One of the most frustrating things when I was first diagnosed was that there was very little information available.  I am thrilled to be a very small part of changing that.  With this study, they will continue to be updated on my medical history and be in touch with me yearly for up to 11 years, but there is still no need to return to MD Anderson for follow-up.

So, GOODBYE HOUSTON!

More About CDH1

The E-Cadherin gene or CDH1 is a tumor suppressor protein that maintains tissue integrity as it mediates cell-cell adhesion. It helps to regulate cells so that they don’t grow out of control or grow too rapidly.  For those who care, the CDH1 gene is located on chromosome 16q22.1 and it contains 2.6 kb of coding sequences with 16 exons.

In 1998, Dr. Parry Guilford first linked inherited mutations in the CDH1 gene with familial gastric cancer in three Maori families in New Zealand.  Many subsequent studies have been done and published papers can be found online.  Depending on your biological and medical knowledge, some are easier to read than others.  Because this is such a rare mutation, many of the studies have very small sample sizes and with each new study, the results change, sometimes considerably.

My gene mutation is c.2287G>T.  It is a nonsense truncation in Exon 14.  My results, dated May 22, 2015, indicate a 80% cumulative risk of diffuse gastric cancer by age 80 for both men and women, as well as a 39%-52% risk of lobular breast cancer for women.  There also may be an increase risk for colon cancer, but no percentage was listed in my test results.  In updated clinical guidelines, found in several articles online published February-May 2015, the cumulative risk for diffuse gastric cancer by age 80 is listed as 70% for men and 56% for women and the risk of lobular breast cancer is listed as 42%.  According to those guidelines, there is currently no evidence that the risk of other cancers is significantly increased in individuals with a CDH1 mutation.

Whether the risk is 80% or 56%, or something in between, the risk is high when you are talking about your life.  The recommendation of all the studies is that individuals who tested positive for a CDH1 mutation should consider prophylactic gastretomy regardless of other screening.  However, if the individual chooses not to have the gastrectomy or delays the gastrectomy, annual upper endoscopy is recommended with a minimum of 30 random biopsies.  However, the literature goes on to say that because of the very focal nature of these endoscopically invisible lesions, it is quite possible that they will not be detected by random biopsies.  So have the endoscopy, but more than likely, it won't find anything.

In almost 100% of the cases where a prophylactic total gastrectomy has been performed because of a CDH1 mutation, signet-ring cell carcinoma has been found in the pathology after the surgery, meaning that the patients are considered to have Stage 1 cancer.  However, because it is early stage and contained within the stomach, the surgery is curative and no further treatments are needed.

Telling the World

This is what I posted on Facebook this morning:


Two weeks from today I embark on the scariest journey of my life. When I was diagnosed with breast cancer in April 2015, I had genetic testing done. I tested positive for a very rare gene mutation, CDH1. While it is affiliated with breast cancer, it has an extremely high correlation with Hereditary Diffuse Gastric Cancer, cancer that forms in the lining of your stomach and is undetectable until stage 4 or 5 and it is too late. The only recommended course of treatment is to have a Prophylactic Total Gastrectomy, ie have your stomach removed as a preventive measure. After much research and agonizing, I have decided to have this surgery completed at MD Anderson in Houston on February 21. I appreciate your prayers for a smooth surgery, uneventful and swift recovery and for a lifetime of learning to live a normal life without a stomach, it can and has been done.

Making A Decision

It was November 12, 2016 when I shared this with family:

I have made what I believe is the hardest decision I have ever made in my life.  On February 21, I am going to have a prophylactic total gastrectomy, ie. I'm going to have my stomach removed.  As much as I don't want to do it, all evidence indicates that it is better than the alternative.  I think what really made me decide to do it was not so much the not wanting to die from stomach cancer (which I don't want to do), but more the not wanting to live the rest of my life in fear.  If I don't have the surgery then every time I don't feel quite right, I would be wondering "is this the cancer, is this the cancer".  I don't want to live my life like that.  And I have always taken doctors' advice about surgeries or procedures to have or whatever, so why was I questioning it this time and thinking I somehow knew better than the doctors.  This will be life changing, no doubt about it, but I have read several blogs online and talked to one person on the phone who is 4 years post surgery and there are plenty of people that have had the surgery for this exact reason and are living a "normal" life.

The surgery is on a Tuesday, but all of the pre-op appointments (surgeon, anesthesiologist, cardiac, x-ray, lab) are the Thursday before, so we will actually be headed down on Wednesday, February 15.  We are going January 5 to have another endoscopy done as it will be almost a year since the one we had down there last April and the surgeon wants a more recent one than that.  If we had it done at the same time in February, it would have to be the Tuesday before so they would have time to get pathology back and I just couldn't see being down there for a whole week before the surgery.  Also, Keith's vacation resets January 16, so by doing it in early January, those two days can be on this year's vacation time instead of next year's.

For more details about the actual surgery, I recommend this blog by two sisters who both had the surgery done in April by the same surgeon I will be using at MD Anderson.

Endoscopy

I had an Endoscopy done by Dr. Patrick Lynch at MD Anderson on April 1, 2016.  Like Dr. Mansfield, he has lots of experience dealing with patients with the CDH1 mutation.  As I said before, if you are opting not to have the surgery or delaying the surgery, they recommend annual endoscopies with at least 30 random biopsies.  Whereas my GI doctor in Kansas City had taken 3 biopsies when I had the endoscopy done last June, Dr. Lynch took 50.  Even with 50 biopsies it is unlikely they will find anything as there could be cancer right next to where they biopsy, but it wouldn't show up.  But it was still satisfying knowing he had at least followed, or actually exceeded, protocol.

Searching for Answers

My oldest daughter, Jesi, also tested positive for the CDH1 gene mutation. She had genetic testing done as soon as I was diagnosed with breast cancer because she actually had risk on both sides of the family.  She had her blood drawn the day before mine.  Hers was sent to a lab that was supposed to take two weeks longer than mine, but she actually got her results back a week before I got mine. However, she didn't tell me until I had my results as she was hoping it would be from her late dad's side of the family and I wouldn't have to worry about it.

 After spending several months researching ourselves and talking with genetic counselors, medical oncologist and gastric surgeon in Kansas City and not talking with anyone that had ever seen a patient with a CDH1 genetic mutation, we decided to go someplace where we could talk with people that have actually dealt with this gene mutation.  Through a friend of a friend type referral (not from any of our doctors), we connected with Dr. Paul Mansfield at MD Anderson in Houston and Jesi and I both met with him January 7, 2016.  We also met with a genetic counselor at MD Anderson. Both Dr. Mansfield and the genetic counselor have several patients with this gene mutation.  In some ways, we didn't learn anything new, but it was still helpful to hear it from someone who has seen it before.  

I had tried to convince myself that our family didn't fit the "typical" family with the mutation and HDGC (Hereditary Diffused Gastric Cancer) because we didn't have as many incidences of gastric cancer as most families; the genetic counselor said that actually we are a typical HDGC family.  They are finding more CDH1 mutations since it is now in the breast panel gene testing, but it is still a rare genetic mutation.  Because of the rarity and the small sample sizes, it is hard to determine the actual risk of gastric cancer, but they still believe it to be somewhere between 50-80%.

We heard the same answer that we had heard before, but it was helpful to hear it from someone with experience.  The only preventative measure is to have a prophylactic total gastrectomy.  The only recommended screening if you choose not to have a gastrectomy or before you do, is annual endoscopies with random biopsies.  Because the cancer develops in the stomach lining, it rarely shows up on an endoscopy, but that is the only screening available.  Jesi and I both had endoscopies done the previous summer because of the mutation, but rather than the 30 recommended biopsies, I had 3 and I think Jesi had 5.  Thus, the problem with seeing a doctor with no experience with the gene mutation.

The gastric surgeon we saw in Kansas City assured us that even though he hadn't done gastrectomies for CDH1, he has done plenty of them and "removing a stomach is removing a stomach."  What we learned at MD Anderson, is no, there is a difference in how you need to do the surgery so that you make sure you get every stomach cell, etc.

What I decided in January was that as much as it scared me to have the surgery, it also scared me not to.  At that time, I was leaning toward having the surgery, but waiting a year to do so.  I wanted the time to get myself emotionally ready, physically ready, ready at work, not miss any events around two weddings in 2016 (my youngest daughter and my nephew), etc.  I did of course run a risk by waiting, but I felt like I would be much more at peace with the decision in a year, so was willing to take what I thought/hoped was a small risk in waiting.  I decided in January, that if I did the surgery, I would definitely do it at MD Anderson rather than KC.  I learned that it would be approx 5 days in the hospital and then they want you to stay in town for another week in case of complications.  I felt that as much as I would rather be near my family and friends during that period, if I was gong to do this, I wanted the best and that was in Houston.  There would still be plenty of recovery time at home as the anticipation was to be off work 2-3 months.  Thus, part of the reason of wanting time to prepare.

My word to family in January 2016: "I don't like it, but that's where I am."

How it All Started

It was April 9, 2015 when I heard those dreaded words "you have breast cancer".  The cancer was found through routine mammogram and was in the very early stage so prognosis was very good.  The plan was to have a lumpectomy and then intense radiation called brachytherapy.  Since I had a family history of breast cancer, I decided to have genetic testing done first because of statistics like 80% of people with the BRAC1 gene will develop breast cancer again.

On Friday, May 22, two weeks after I was supposed to receive the results, just before leaving the office to come home for our Memorial Day weekend plans, I got a call from the genetic counselor.  She had the test results.  I tested positive for a very rare gene mutation, CDH1.

I learned from the genetic counselors and from doing my own research online, this gene mutation has a 39-52% (I've seen as high as 60%) risk for lobular breast cancer.  Interestingly enough, what I had was ductal breast cancer, so what I had may or may not actually have been caused by this gene, but the risk is still there of future breast cancers.  The scarier part is the 80% risk of Hereditary Diffuse Gastric Cancer (ie stomach cancer) by the age of 80.  What makes HDGC even scarier is that it develops in the lining of the stomach  and is undetectable by any early screening methods.  The only recommended course of treatment is to have a prophylactic total gastrectomy (removal of the stomach as a preventative measure).

All of this was a lot of info to take in. Even though I eventually would have to make a decision about the stomach cancer risk, the immediate question on the table was what to do about the breast cancer. After thinking about it, talking with the genetic counselor, the breast surgeon, the reconstructive surgeon and the medical oncologist (all in a short Memorial Day week), we decided to go ahead and do a double mastectomy on June 18.  By having the mastectomy, there was no need for either radiation or chemo.