Prophylactic total gastrectomy. After deciding whether to have one, you will need to decide when to have it. For many medical conditions, a sense of urgency for surgery exists because of existing symptoms. In those instances, the question oftentimes is “how soon” can we do surgery. However, with prophylactic total gastrectomy, the patient does not have symptoms. The sense of urgency, therefore, is mostly based on the uncertainty of whether stomach cancer has already developed to an advanced stage before surgery.
In view of this symptomless situation, patients are left to ask themselves when the “right time” is to have surgery.
In 2015, a team of nearly fifty of the most informed CDH1 healthcare professionals from all around the world published guidelines for the clinical care of CDH1 mutation carriers. In the 2015 guidelines, the authors addressed this exact issue. What they said is that no perfect time exists for having a prophylactic total gastrectomy. Rather, each patient will need to pick what works for them. In their words: “The optimal timing of prophylactic gastrectomy is unknown and is usually highly individualised.”
For me, deciding when to have the surgery was fairly easy. My mom had just died from hereditary diffuse gastric cancer, and I couldn’t think of any reason to delay. So, I coordinated with my family and work and made sure to be in good physical shape.
I made sure the date worked with my dad’s schedule because I wanted him there, and he wanted to be there too. He and I live in different cities, so we found a three-week period when he could come stay with me. He stayed with me at the hospital and then at my place afterwards. I also coordinated with my brother and sister to make sure they and their families would be available. I wanted them nearby too.
I’m so thankful that my dad was able to spend so much time with me. This may sound weird given the magnitude of the situation, but it was a very fun and special summer.
I also coordinated with work. I wanted to make sure my office was prepared for me to be gone for at least six weeks. Although I started working from home during the fourth week after surgery and was in the office by week six, I recommend CDH1 mutation carriers find at least six weeks or longer to not even think about work. Ideally, in my opinion, you should have at least two months of no work pressures. When you do resume work, I recommend easing back into it rather than jumping back.
Finally, my physical fitness was good. I had regularly been running, stretching, and practicing yoga. It was important to me that my physical fitness be good because I thought it would help me recover faster and easier. I was slightly overweight (about 10-15 lbs) before surgery, but my cardiovascular health was good. I lost about 20% of my weight within the first six months, so being slightly overweight beforehand might have given me a little extra cushion.
Everyone’s situation, however, is unique. Other things to account for are your spouse’s and kids’ schedules, whether and when other family members are having the surgery, out-of-pocket healthcare status, at what age family members (if any) developed diffuse gastric cancer, whether you prefer to have total mastectomies before total gastrectomy, whether you want to have children before surgery, the current status of surveillance and drug development, potential surgery-related complications, whether the patient is young and healthy enough to recover, and other medical conditions of yours that might be affected by the timing of your surgery. Those are just a few things to consider. I’m sure more exist.
Despite there being no universally applicable time, the general consensus is that CDH1 mutation carriers have a prophylactic total gastrectomy in early adulthood. One paper recommends between the ages of 18 and 40 years: “total prophylactic gastrectomy, performed between the ages of 18 and 40 at a high-volume cancer center with low perioperative mortality rates, is advised in CDH1 mutation carriers fulfilling the clinical criteria for HDGC.”
The 2015 guidelines recommend a slightly shorter window. They recommend that doctors inform CDH1 carriers and offer them the option of prophylactic total gastrectomy between ages 20 and 30. In their words: “The current consensus is that the procedure should be discussed and offered to pathogenic germline CDH1 mutation carriers in early adulthood, generally between ages 20 and 30.”
The importance of being well informed should not be understated. In the short term, prophylactic total gastrectomy will dramatically interfere with your day-to-day. For instance, during the first three weeks after my surgery, my main focus was staying on a good schedule of eating, walking, and relaxing. Doing anything more than that will be fairly challenging and could distract you from your main objective: healing. The 2015 guidelines recognize this: “Since this surgery has major impact on the quality of life, the decision to undergo prophylactic gastrectomy should be well informed, balanced, prepared and timed.”
If you need help deciding when to have your prophylactic total gastrectomy, talk with your doctors, family, and friends. In my opinion, it should be a decision heavily influenced by your inner circle. Although, you should have the final say. Indeed, the 2015 guidelines say that whether and when to have the surgery is essential: “Decisional counselling, outweighing the pros and the cons of the intervention is essential.”
Some CDH1 mutation carriers may prefer to not have their surgery shortly after learning of their mutation. That may be okay depending upon the circumstances. Although most CDH1 mutation carriers have at least some cancerous cells in the stomach at the time of their total gastrectomy (regardless of age), that does not necessarily mean they need to immediately have a total gastrectomy.
The gastric cancer cells, although malignant and present in the stomach tissue, appear to not necessarily spread or progress immediately after forming. Rather, a dormant period seems to exist during which the cancerous cells have developed but not caused any symptoms or other chaos. This makes sense based on the anecdotal evidence. If over 80% of total gastrectomy patients had at least one early-stage cancer cell, yet none or few had actual symptoms or metastasis, then the cancer cells must form but not necessarily spread right away. Otherwise, some or all of those 80% of patients should have had more advanced cancers that had spread to other organs. Many of those patients likely wouldn’t have even qualified for total gastrectomies due to the cancer being already too pervasive.
The 2015 guidelines address this: “There is likely to be a dormant period in which the signet ring cell adenocarcinoma does not spread or progress since they have a low proliferative index and the age of prophylactic gastrectomy is generally lower than that of overt cancer. This may explain why so many individuals are found to have T-1 N-0 stage tumours after prophylactic gastrectomy.”
However, nobody knows when the cancer cells form, what causes that formation, when after formation they will spread, and many other important details. Therefore, the 2015 guidelines recommend that patients have surgery early in adult life. Indeed, they say: “it may be possible to safely postpone prophylactic gastrectomy in some patients, but until such time it is safer to recommend surgery early in adult life.”
Being diagnosed with a CDH1 mutation is a scary thing. The fear of not knowing whether the cancer has spread can be paralyzing. Know, however, that finding out you have the mutation is just one step. It doesn’t mean you have pervasive disease. My grandfather, a CDH1 mutation carrier, lived into his mid-80s without developing pervasive stomach cancer. One of my aunts who is a CDH1 mutation carrier is over 70 years old and is without signs or symptoms of stomach cancer. Another aunt of mine had her prophylactic total gastrectomy in her early 60s. My cousin had his prophylactic total gastrectomy in his early 40s. I had mine in my mid-30s. My other cousin had hers in her late 20s. Another cousin will have hers in her late 20s several years after finding out she carries a CDH1 mutation.
Therefore, everyone’s situation is unique. Please don’t freak out upon learning you are a CDH1 mutation carrier. Rather, gather information. Talk to doctors, family, friends, and other CDH1 mutation carriers. Join the Facebook group for people with a CDH1 mutation. Search Instagram for other mutants using #cdh1 #totalgastrectomy and #nostomach. Then, develop your plan on whether and when to have a prophylactic total gastrectomy.
Also, watch the 2015 Spotlight on Gastric Cancer, featuring the doctors who discovered the connection between gastric cancer and CDH1 mutations, CDH1 carriers, and others.