The Mechanics of It All

I’d like to begin by saying how blown away we have been by the response to our last post. Every single comment has been read and taken to heart and we are beyond grateful for all the prayers being said for us and our families.

We have had so many questions about surgery and life post total gastrectomy, so I wanted to take some time and explain the procedure, recovery, and what we are expecting afterwards.

Total gastrectomy is the complete removal of the stomach organ. The term prophylactic is attached to our procedure because it simply means it is being done as a preventative measure.  We spoke with three different surgeons before deciding on Dr. Mansfield and realized there are several different methods that can be used to achieve the end goal. For the purposes of this post, I will focus on Dr. Mansfield’s method for performing the operation. 

Here is what our stomachs currently look like:

Our doctor uses kind of a combo approach to this surgery. Laparoscopically he will go in and prep for the big separation, he mobilize the stomach, divide the duodenum, and work on the blood supply. Nicole will also be having her appendix removed during this procedure (mine was removed 10 years ago) and we imagine that will be done laparoscopically as well. Dr. Mansfield will then make a 6-7 centimeter incision at our breast bone and this is where the stomach will physically come out and the reconstruction, known as Roux-en-y reconstruction, will take place. This reconstruction involves attaching the esophagus to the small bowel and one end of the duodenum will be attached to the small bowel as well.  Here is an after shot of what it will look like after the surgery:

So what are the major complications/concerns?

• The most major concern is a leak at the anastomosis (the place where the small bowel and esophagus are connected), which could actually be fatal if it occurred. One thing that really made us like Dr. Mansfield is that while in the OR he fills the abdomen with saline, submerges the new connection and blows air through to check for leaks before closing us. By doing this he has a leak rate of 0% doing this surgery. 
• Stricture at the anastomosis, which means the passage there becomes very narrow is another concern. This causes difficulty and pain when swallowing and makes it hard to eat or drink. This would have to be treated with a procedure to dilate the area. Luckily the rate of stricture with a hand sewn anastomosis is much lower than one that has been stapled.
• Bowel obstructions are a common complication as well and unfortunately can occur even years after surgery. All we can really do is be mindful of our bodies and pay attention to any signs of discomfort. Most small bowel obstructions can resolve themselves, but some do require surgery to repair.
• A hernia in the Petersen space (the extra space left due to the absence of the stomach) is another concern. This would mean the intestines could herniate in the space and surgery would have to be done to repair the defect. The major concern with this space is also the intestines twisting which would result in blood flow being cut off and the intestines dying, which could be fatal. This complication is incredibly rare and our surgeon will take extra precautions to “close the traps” where this could occur. 

The biggest long term concern is of course nutrition. Most people lose between 10-20% of their body weight in the early months following surgery. We will be unable to eat or drink substantial amounts at one time. We will be eating very, very small amounts pretty much around the clock during the early days. Coming out of surgery we will have a feeding tube (J-tube) that will help us keep up with our caloric requirements. As time passes our bodies will adjust to the new mechanics and we will be able to eat larger portions less frequently. Similar to people who've had gastric bypasses or gastric sleeves, there will be some types of foods that will be hard on our system.  Sugar seems to be the #1 food to avoid. Also on the bad list are fried foods, raw veggies and fruits, and for some people lactose is a problem. Over time we may be able to add small quantities of these foods back into our diets, it's just a matter of letting our bodies adjust first. One consensus from people who have had this surgery before is that at some point we will experience what is called dumping syndrome. This occurs when food, namely sugars, empty into the small bowel too rapidly. Symptoms of dumping syndrome are nausea, vomiting, abdominal cramps, sweating, dizziness, and a rapid heart rate. One great piece of advice we've been given is to keep a food journal and track how our bodies respond to different foods and to try adding new foods to our diet one at a time so we can easily track how we tolerate each thing. We've talked to several people who have gone through the total gastrectomy and it seems like things really start to level out around 6 months and by a year you're settled into your new normal and eating larger quantities and a variety of foods. 

Following surgery we will be in the hospital for 5-7 days. The first four days we will have nothing to eat or drink by mouth, but they will start giving us nutrition via the J-tube the day after surgery. After the first four days we will slowly start with liquids, then advance to soft solids. After we are released from the hospital we will stay at a hotel close to MD Anderson for about a week, then we will have one more follow-up with our surgeon before flying home. In total, recovery should take 6-8 weeks. Nicole will be out of school for the summer and I plan on taking 6 full weeks off work then work half days for two more weeks and return to full duty after 8 weeks. 

Other developments in the past few weeks:

• Nicole had her first appointment, MRI, and mammogram with the breast health center at UAB and everything came back clear! So she'll be checked again in 6 months and my first set of breast tests will be May 16th. 
• We both met with a nutritionist at UAB to start preparing for the road ahead. She gave us some helpful information on adding calories after the surgery and some protein powders and supplements that night be helpful.
• I met with a fertility specialists at UAB to discuss the possibility of pregnancy with no stomach and doing IVF with pre-genetic screening to eliminate the CDH1 mutation risk for any future child.  

Over the next few weeks we just want to focus on spending as much time as we can with our families and friends and eat as much delicious food as possible. We continue to focus on the positives of this situation, like totally eliminating a major cancer risk, but that doesn't mean we don't have days where we are scared or anxious about what's to come. So thank you for continuing to pray for us and our families, because that truly helps us get through those days. 

Wait, We have WHAT?!?!

If I had a dollar for every time I was told how much I’m like my mother…well, let’s just say I’d be a wealthy woman. To me it has always been almost freakish how I look so much like her, how my voice resembles her, even how I make the same facial expressions. It turns out my sister and I are more like her than we could have ever even imagined.

We are “the Jackson sisters.” Nicole is the older sister. She is married to Heath, has two little boys, Caden and Liam, and teaches elementary special education in Hartselle, AL. I am Jessica, the younger sister. I’m married to Steven, have one son, Andy, and I’m a data analyst in Birmingham, AL. Our mother was diagnosed with aggressive breast cancer at the age of 36 and passed away almost two years later (we were 13 and 10). For most of our lives we have feared cancer. We were both tested for BRCA years ago with a negative result.  We felt a sense of relief over those results; we would still be monitored closely, but it wasn’t like there was some biological force working against us. Or so we thought.

After my son was born I swapped OB/Gyn’s and when I hit 30 we started discussing how to tackle my breast cancer chances head on. At the time she suggested I start annual mammograms and she really pushed meeting with a geneticist to discuss new genetic breast cancer connections. I had my first mammogram and everything looked great, so I went on with life. When my annual exam came up in 2016 we discussed meeting with the geneticist and this time an appointment was made. To be honest, beyond my mom and her brother (who had kidney cancer a few years ago) I had no real idea about my family history. We discussed what all I knew, she drew blood for a 32 gene panel, and then the six week wait started.

On Thursday, March 3^rd the geneticist called and said “unfortunately your panel came back with a mutation consistent with an enhanced risk for breast cancer.” I will never forget pulling into a Dollar General parking lot to take notes and hearing the term CDH1 for the first time. I knew it was pretty serious because I asked her if this diagnosis would qualify me for the advanced screening measures we talked about and her response was “yes, but we need to discuss other preventative measures based on where you are in life.” We set an appointment for me to come in and discuss the findings the next Monday and she warned me to be cautious Googling this mutation because it would frighten me. And man was she right.

Here is what we learned. The purpose of the CDH1 gene is to code for a protein called E-cadherin, which is found in the membrane of epithelial cells, the cells that line the surfaces and cavities of the body. E-cadherin helps cells stick to each other to form organized tissue. It also acts as a tumor suppressor protein by preventing cells from growing and dividing too rapidly or in an uncontrolled manner.  When a mutation of the CDH1 gene is present is causes production of a non-functional version of the E-cadherin protein or production of a protein with an altered structure. The loss of this normal function prevents E-cadherin from acting as a tumor suppressor and can lead to uncontrollable growth and division of cells. That’s a whole lot of scientific talk, but basically, the lack of normal function associated with CDH1 mutations leads to a 53% chance of lobular breast cancer (the cancer that took our Mom) and an 83% chance of hereditary diffuse gastric cancer (HDGC), a rare, virtually undetectable form of stomach cancer.  Everyone has two copies of the CDH1 gene, one from your mom and one from your dad. While our Dad’s two copies are totally normal, our Mom had one good copy and one not so good copy. Based on the basics we all know about genetics, that means Nicole and I had a 50/50 chance of inheriting the crummy CDH1 gene from Mom.

After reading all of this and picking my jaw up off the floor, I thought perhaps I just wasn’t seeing this right and that when we met with the geneticist she’d tell me I was in a different group and the risks I kept reading about wouldn’t be the same for me. Wrong again! Knowing Nicole had the same chance of having this same mutation, I had her and her husband come with me and my husband to the meeting with the geneticist. I don’t think I will ever forget the opening exchange with the geneticist. She asked me to tell her what I had read about CDH1 mutations, so I proceeded to tell her everything from above, including the 53% and 83% chances of the cancers associated with this mutation. I waited for her to say yes, but that doesn’t apply to your specific mutation. Instead she responded “you’re absolutely right.” Talk about having the wind knocked out of your sails. Since Nicole was there, she went ahead and started the process of screening her for this mutation. Two weeks later, in the middle of the Central Park Zoo, Nicole got the call that we were all dreading, she too tested positive for the same CDH1 mutation. 

Now let’s talk about screening and prevention. On the breast cancer side we can do alternating MRI’s and mammograms every 6 months, we can take Tamoxifen, an oral chemotherapy drug that can help prevent the cancer from developing, or we can opt for a total mastectomy if we felt we wanted to be that aggressive. On the gastric side here are the options we were given for screening: absolutely nothing. HDGC accounts for 1 – 3% of all stomach cancers and because it spreads out within the submucosal layer of the stomach and doesn’t form the typical mass associated with other cancers it is not picked up on an endoscopy. Even having frequent endoscopies and taking regular biopsies is like finding a needle in a haystack.  Like I said earlier, it is virtually undetectable until it’s in a late stage.

So what can be done to prevent HDGC? You can remove the stomach entirely through a surgery called Prophylactic Total Gastrectomy. I know, you’re probably thinking WHAT?! That was our thought too. Here’s the thing, there are a lot of people who have had this procedure done because they too had this genetic mutation and they are living normal, healthy lives. You can live without a stomach, you can still eat without a stomach, we’ve even connected with an awesome lady who is running marathons without a stomach and another lady who had her stomach removed 30 years ago due to cancer and has never missed a beat. It may seem like an extreme measure to some, but an 83% chance of developing an undetectable form of cancer is way too high of a risk to take. We have three little boys who need their mom’s and we know firsthand how hard it is to grow up without a mom. To us, having this surgery is a no-brainer. After much research and talking with three different surgeons (I’m sure we’ll write a whole post on selecting a surgeon), we have decided to have the Prophylactic Total Gastrectomy with Dr. Paul Mansfield at MD Anderson and surgery is set for both of us on May 24^th. We have already undergone our baseline endoscopy and colonoscopy screenings. About 40 biopsies were taken from each of our stomachs and all tested clear of cancer. Unfortunately, that really doesn’t mean anything. After the stomach is surgically removed most places will run detailed pathology on the entire stomach and 95% of the time some degree of cancerous or pre-cancerous cells have been found. Are we scared? Absolutely! But we have chosen to see this as a blessing, not a curse. We have a chance to save our lives, a chance our beautiful Mother never had. We would much rather hear “you have a genetic mutation and need your stomach removed” than to hear “I’m sorry, you have stage 4 cancer.”

Nicole and I have decided we want to tackle this together. My husband has said we’re like Thelma and Louise driving off the cancer canyon together. We will each be updating this blog with news on our progress and CDH1 for people who may be newly diagnosed and looking for information. Please feel free to ask us any questions, we know this is a lot of overwhelming information. Thank you for reading this and supporting us on this journey. Please know we are genuinely grateful for and covet your prayers for us and our family during this time.