Tag: lynch syndrome

Is it Time to Kondo My Ovaries?

Guess what? I have a date for my surgery—April 7!

It’s a little more in the future than we’d originally envisioned, by about a month. Which gives me some time to do what I do best… over-think it!

Part of the reason for the delayed date is that is that we needed to find an available time for not just one, but two surgeons. One surgeon to remove my colon and another to remove my ovaries at the same time.

Wait, what? You’re thinking, if you’ve been keeping up so far, when did the ovaries get involved here?

It goes back to that fun Lynch Syndrome chart that tells us that while the risk of colorectal and endometrial cancers are the highest, the risk of ovarian cancer is also pretty high (between 8-38% according to the chart). When someone has a high risk because of a genetic mutation, the option is always on the table to remove the risky organ as a prophylactic (preventative) measure. If I have my ovaries removed, my chances of getting ovarian cancer are decreased by 95%. (Yeah, I know it seems like it should be 100%, but for some reason it’s not.)

For years, I’ve been doing ultrasounds and a special blood test for a marker called CA-125 to look out for signs of ovarian cancer, but both of these tests are felt to be very limited—definitely not as sure a bet as removing my ovaries. So for years, doctors have been checking in with me to see if I want to get rid of my ovaries, and I’ve been saying “Guys, not yet! Can’t you see I’m still using them!” This is because even though I’ve had my uterus removed (a hysterectomy), my ovaries continue to pump out hormones, which means I haven’t yet gone through menopause, and there are definite benefits to that.

But now… I’m probably within a few years of not using them so much (they are producing less hormones, approaching a natural menopause) and, of course there are going to be some holes cut into my abdominal region anyway… why not do two-for-the-price-of-one? (Note: I’m fairly certain I will NOT get two surgeries for the price of one! But it will be going under anesthesia only one time, getting cut into only one time and I’ll can maybe max out my insurance deductible just once.)

Given all this, it seems like it makes sense to, a lá Marie Kondo, thank my ovaries for their service, for the joy and protection they have provided, and let them go…

… Except that when I met with to my surgeon the other day, she said something in passing like “yes, even though mortality rates go up with ovary removal, in makes sense to blah blah, medical stuff…”

Wait… what was that about mortality rates?

Thus far, no one had mentioned this fact before now, but it pinged my radar enough to spur some internet digging, which turned up articles like this one, from 2017, called “Bilateral Ovarian Removal Associated with Increased All-Cause Mortality,” and one from 2013, called “Long-term Mortality Associated with Oophorectomy versus Ovarian Conservation in the Nurses’ Health Study.” In short, women who have their ovaries removed before their natural menopause die sooner, either from heart-related issues, or just… other stuff. As my doctor put it “we don’t exactly know why.” (However, there are also articles like this one, that say the difference in mortality is are less or perhaps negligible if the removal happens after age 50. Others say 55. One assumes that one’s age of natural menopause is a factor, but would be hard to incorporate into a study.)

So maybe, I’m thinking now, I should hang on to my ovaries a little longer — maybe they’re still sparking joy after all?

And maybe my overall risk ovarian cancer / dying from ovarian cancer isn’t quite as simple as the scary chart would indicate. My overall risk of ovarian cancer is lower because I’ve had my fallopian tubes removed in the past. The study sited in “Ovarian Cancer risk after salpingectomy” says the decrease in risk might be as much as 50%. This paper I kind-of-partly-undertand called “Features of ovarian cancer in Lynch Syndrome” seems to be saying that the survival rates for Lynch relate ovarian cancers are statistically higher than for sporadic cancers (not that I’m keen to test it and find out) due to being diagnosed at an earlier stage and also to having different properties.

Mortality aside, I’ve also discovered there are a number of more immediate effects to menopause — maybe exacerbated by being a sudden, surgical menopause—that I hadn’t realized. While we associate hot flashes to menopause, no one has ever sat me down and talked to me about potential hair loss, fatigue, anxiety, depression and brain fog. These symptoms are not a given, and they are not life threatening, but they are quality-of-life threatening.

Needless to say, I’m going to be ruminating…

Cartoon of Ovary on red carpet
Cute illustration totally stolen from someone’s old blog – credit at bottom.

First Try at Removing the Colon Cancer

(Note: If you notice some of these posts have more links and technical jargon than usual, it’s because I’ve become incredibly grateful to all the people (like Jim Sease, who I’ll reference below) who have shared their stories and information for people like me, and I want to pay it forward. I hope that if someone in my situation stumbles on this post during a late-night internet search, they might find the word or link they need to help them on their journey.)

Back in October, when my gastroenterologist at Kaiser told me I needed to have the rest of my colon surgically removed, in order to remove a small cancerous polyp, it sounded crazy to me. The polyp was less than two centimeters big. The problem, she said, was that it couldn’t be removed like a normal polyp because it had grown into the scar tissue from a previous troublesome polyp. The scar tissue was so rigid, it would be impossible to lift the polyp from it.

Still, the idea of removing my entire colon seemed extreme. Right? Doesn’t it sound extreme? If a polyp is only couple of centimeters, can’t you just take an inch or two of colon and call it a day? After that, I met with a Kaiser surgeon, and she explained that it wasn’t that simple, because when you cut out part of a colon, you can only reattach it at a point where there’s a good enough blood supply to keep everything working, so, at minimum she’d have to remove all the colon up to the next good blood supply.

I think that just the biggest arteries coming off that central one in this picture are considered “good” blood supplies.

Okay, I could see the logic. But, even as we began to schedule the surgery, I wondered, how could this be the only answer? Then, one late night on the internet, I came across a blog post by Jim Sease called Removing a Large Flat Colon Polyp by EMR without Surgery in which he gives a very generous and detailed report of having a five centimeter polyp removed by an alternative procedure called EMR. Like me, his doctors had recommended removing some of his colon, and as I was doing, he had searched for — and found — an alternative.

From reading his article, I gained enough knowledge and vocabulary to start investigating the possibility of either EMR (Endoscopic Mucosal Resection) or a related procedure called ESD (Endoscopic Mucosal Dissection). Although my doctors at Kaiser didn’t feel I was a good candidate for these, I found a doctor at UCLA who specializes in these procedures along with a newer one called EFTR (Endoscopic Full Thickness Resection) and was willing to take a second look. There were a number of science-y things to consider, that are outlined in this video:

To do the procedure at UCLA, I had to change my health insurance provider during the open enrollment period and wait for that to take effect in January. Then I had to navigate through the new system to get referred to the specialist and then wait for an available date. I was lucky with a cancellation and got a date on January 27th.

On January 26th, I stopped eating and drank my too-familiar bowel-cleaning prep solution, and early the next morning, arrived UCLA. My doctor explained the three things that might happen: 1) He could remove the lesion, and if it was shallow, then I would heal and that would be it. Or, 2) he might remove the lesion, but the pathology would show the cancer was deep, and later I would still need to get a surgery. Finally, 3) he might look and decide I wasn’t a good candidate at all, for some of the reasons outlined in the video.

I was hoping, of course, for the prize behind door number one. I was bracing myself for door number two. Because he had (I hoped) looked at the images taken by my gastroenterologist, I didn’t think he’d pick door number three…

But he did. When I woke up in the recovery room, the doctor visited to let us know that he’d looked, and didn’t think my my case would be helped by any of his techniques. It was a little too big (he measured it at three centimeters) and had concave features. All he’d done was take a few more pictures and another biopsy. In the end, he agreed with my doctors at Kaiser, that surgery was going to be the route for me.

No lie, this news was disappointing. I went home and got straight into bed — both because I’d been up much of the night doing the prep, and because, in general this is my favorite way to deal with disappointing news. After a long nap, I woke up and went for a walk outside. The weather was very nice.

Did I jump through a lot of hoops for nothing? Maybe? But, I’m someone who needs to feel like I’ve really gotten all the information before committing to something life-changing. Now that I’ve been through this, I’m feeling ready to take my next steps… which you’ll get to hear about soon!

I Have Lynch Syndrome

Most people have heard of the BRCA genetic mutations that predisposes its owners to breast and ovarian cancers, especially after Angelina Jolie disclosed she carried the gene in 2013.

Fewer people are familiar with Lynch Syndrome, which causes a predisposition to a number of cancers — primarily in the abdominal region — and is comprised of mutations to a handful of genes that mostly start with or contain the letter “M”, like MLH1, MSH2, MSH6, as well as PMS2 and EPCAM. These genes are involved in a process called mismatch repair. According to the Dana Farber Cancer Institute, they “act like spellcheckers to find and correct the “typos” made in the gene copying process.” When the typos don’t get fixed, the mistakes can replicate and accumulate more and over time this can cause cancer.

Growing up, my father was diagnosed with cancer several times, and we just assumed he was very unlucky. But then, in 2003, I was diagnosed with colon cancer at an unexpectedly early age. After a surgery removing half of my colon, the doctors sent my tumor to a genetics lab where they discovered a MSH2 mutation. Then they tested my father’s blood, and found the same mutation. That’s when we learned we both had “hereditary non-polyposis colorectal cancer (HNPCC),” also known as Lynch Syndrome. Depending on which genes are affected, people with Lynch Syndrome have a higher risk than the general population of getting certain cancers:

General populationLynch syndrome (MSH2)
Colorectal4.20%33-52%
Endometrial3.10%21-57%
Ovarian1.30%8-38%
Gastric0.90%0.2-9%
Bladder2.40%4.4-12.8%
Biliary tract0.20%Up to 1.7%
Urothelial<1%2.2-28%
Small bowel0.30%1.1-10%
Brain/CNS0.60%2.5-7.7%
These are statistics for MSH2, which pertains to me. I took it from this more complete chart by the Jackson Laboratory.

After my first diagnosis, I made lifestyle changes to improve the risk factors that I could control. I stopped drinking alcohol, eliminated or cut down on meat and sugar while increasing my vegetable intake, re-committed to yoga and meditated to keep my cortisol levels down. Maintaining this lifestyle while returning to grad school wasn’t always easy, especially in Los Angeles, where socializing and networking is “part of the job.” When I graduated from Screenwriting school in 2011, I got an offer to be a writers PA on a television show where I’d been interning—a dream scenario, except the job didn’t have health insurance, and pre-ACA, there was no individual coverage for someone with my history. It was hard to pass up that job, and I’ve often wondered how different my career might be now if I had been able to take it…

But I made the practical choice, and, as it happened, having health insurance soon came in handy. In 2012 I was diagnosed with endometrial cancer and my uterus was removed.

Then, after was nine fairly peaceful years, last October (2021), a scan revealed colon cancer. Again.
It felt kind of like getting struck by lightening twice. What are the chances? Turns out, with Lynch Syndrome, they’re pretty high.

Estimates say that about a million people in the United States have Lynch, but that 95% of them don’t know their status. If you have questions about Lynch Syndrome, you can check out the websites of advocacy groups like Lynch Syndrome International, AliveAndKickn.org and FORCE. They are a good source of information as well as encouragement. Many people with Lynch live long and full lives!

(This is a kind of nuts-and-bolts rundown. If you’d like to read a longer essay with more personal and philosophical thoughts, you can check out something I wrote for The Colorado Review awhile back called “Luck, Statistics, Magic.”)