Keeping up with the Jolies...or the Joneses

After hearing what we have to do, people often say that they feel that their worries or problems are not worthy of mention. The Ramers have it way worse than they do.

I was thinking about this today as I drove home from dropping the kids off at school and Olivia was still sort of waking up, so not actively talking my ear off as she customarily does.  Without having each American flag along the route pointed out to me, I quietly said my prayers and counted my blessings, as is my habit in such rare moments. And I thought about this notion of comparing our lives to that of others. 

I remember once, shortly after we moved here, Brent went to play with a friend who lived in another neighborhood.  When he returned, he exclaimed that they had an enormous house: beautiful and so big, that he got a little lost at one point.  Would we be buying a house like that someday?

I told him that I had no intention of moving for quite some time.  Our home was perfect for our family, with room for my mom, who has since moved in with us.  We have a wonderful neighborhood and live in a fantastic school district. I pointed out that there would always be someone who had a bigger house.  There would always be those who made more money.  We cannot measure our worth on this scale.  You cannot gauge your happiness in things, or in comparative terms.

I think that this idea translates beyond material things, actually.  I think you cannot find happiness comparing your life to others, your problems, your blessings…any of it.

I wrote recently about how I felt that Angelina Jolie might help bring some understanding of genetic predispositions to the general public, which then might translate to a better understanding of LFS…a place we could start our explanation from, without going all the way to the beginning.  (This often results in looks of confusion, disbelief, or best yet, a glazed over, vacant stare.) Given her giant celebrity status, she could bring attention to genetics and cancer, the way that Michael J Fox brought a public awareness and appreciation of the challenges of Parkinson’s disease.

It was interesting to hear from my LFS friends, and my friends that are breast cancer survivors (and for the record, there is some overlap here).  I was surprised at how aggravated some were with this whole business.  To me, it was as simple as someone famous, taking a strong preventative stance and providing some good PR, for lack of a better term, in sharing her personal story about cancer and giving some attention to genetic predisposition syndromes.

I suppose that because I seldom listen to the radio and never turn on the news, I probably missed a large portion of the ignorance out there (confirming the wisdom in keeping my TV off). So, I can understand the frustration of my friends with every idiot who failed to understand Angelina Jolie’s decision, or of what breast cancer brings. And further, by the fact that they felt compelled to confirm their ignorance by opening their mouth or commenting on the internet, denouncing her decision, one that most folks in this position happen to agree with.

But even if we didn’t agree, it is her body, her health, her decision…not public domain.  That, to me, seems pretty obvious.  And I could easily understand how difficult it might be to listen to such comments, and want to defend against such nonsense…maybe in violent frustration.

But what really surprised me was the reaction of some to the facts of BRCA, and Jolie’s decision to have surgery, thus nearly eliminating her risk.  Apparently, this was received with much public support, lots of “brave” and “courageous” accolades.  I personally do not have a problem with these adjectives, but some breast cancer survivors found this objectionable, which I found curious, until I listened a bit more.

I believe it is the opportunities that Jolie has been afforded that makes it difficult for some to drape her in laurel leaves as a ‘champion.’  Because she certainly has blessings in this life:  Financial independence such that she can hire help with laundry and kids; a platform from which to speak and a voice that people pay attention to; access to the very best doctors and the money to pay them without needing to  duke it out with an insurance company; genetic knowledge in advance of a diagnosis, one that afforded her the opportunity to chose a mastectomy, and careful reconstruction, when others had the same procedure forced upon them with a diagnosis, further insult to follow in the form of a chemo and/or a radiation chaser; Angelina’s ability to overcome her ‘genetic liability,’ (the way those with LFS cannot) and likely avoid cancer altogether. 

Let’s just say that there were strong feelings about all the gushing and hoopla among some LFS friends as well.  LFS is like playing in the major leagues of cancer.  Jolie just got called up and these vets were not necessarily impressed with the rookie.  She hasn't faced a fastball, the screwball...the curve. Does she have any endurance?  She hasn't got the experience, and some out there are making her MVP.

I was bewildered. 

While Jolie is genetically cursed with a mutation (BRCA) that she was wise to both respect and address as she has done, those same genetics blessed her with extraordinary beauty, which is partly why we are still talking about this…she is in the movies and we are Americans.  Forgive me for stating the obvious. 

And that twist sort of hacks some regular people off, especially those who have the yin of crappy genetics, without that yang of that exceptional beauty…which has led to her status, her platform, her financial ease, all of which are enviable.

But if I had had cancer, had this surgery thrust upon me, fought my way through chemo, through radiation, learned of my devastating genetic diagnosis which cannot be surgically mitigated, and struggled to care for my children while fighting with the insurance company like a superhero, or madman…I might not have warm and fuzzy feelings about this Johnny-come-lately who had done none of this, and who people pay attention to because of her beauty.  I can see how that would be irritating.

Well, now I get it.

From that perspective, she might not seem so much 'brave,' as logical, pragmatic and resourceful…using whatever is available to her.  And she has much available.  I do not personally begrudge her the resources.  Believe me when I say that I use whatever I can find to get through.  I count my blessings.  I know that I have many.

But ultimately, I think that it comes down to understanding the fact that there will always be those better off than you, and there will always be those worse off.  Using a dear friend’s description, LFS is 'the mother of all cancer syndromes.'   And thus, genetically, we are the top of the heap, cancer-wise.  We are the most exclusive club, but the sorority that no one rushes. No one has greater cancer risk than this small, scattered group of families from all over the world.

They struggle, anonymously for the most part, for generations, losing significant portions of their family.  They want help.  They want solutions.  They want a voice.  They want research dollars.  They want to be heard.  Mostly, they desperately want a cure. 

I am a newbie, without any sort of LFS perspective, or heavy oncological baggage in my family.  I lost my father to kidney cancer, although I have been assured that this was not LFS related.  It counts toward my cancer fear factor, this loss.  But it is not in the same league, not even close, to the stories of my friends, who have lost so many…children, siblings, cousins, parents, aunts, uncles… 

I have a different perspective.  A different story.  We all do.

I am grateful that Angelina Jolie shared her story.  She still garners my sympathy and admiration, because she seems to be a pretty private person when it comes to her family.  In sharing this, she has helped others, and I view it as a measure of generosity, this giving of her privacy, a treasure that she seems to hold dear.  The same anonymity that others find despair in, might be exactly what she craved as she went through her procedures, skulking around and hiding from the paparazzi.  You have to take the bad with the good, and we all have different challenges. 

I try not to compare my life, or situation to others.  This is simply my story.  And I try to do the best with what I have been given, and relate compassionately to those who cross my path.  I look with much gratitude to those who have helped me, which includes the insight and support from a beautiful group of strong women from all over the world, each rivaling any character that Angelina Jolie might play on the silver screen.  They are bad-ass in real life and deserve equal time, even if you do not know them and they never happen to grace the cover of People Magazine.

Just saying.

Angelina Jolie and LFS

Angelina Jolie made all kinds of news yesterday.  I am not a tabloid reader, and I have been far too busy this week to do more than a mad dash into the grocery store, where I otherwise might have stumbled across the latest Hollywood gossip while waiting in line.  

But even I, in my nearly Amish status, am aware that she had a double mastectomy, and breast reconstruction. (My FB page is littered with breast specialists and geneticists, representing every hospital and cancer organization that I am "friends" with...and there are many.  They are all talking about it.) She announced this fact in the Op Ed section of the NY Times, giving details of her family history, her genetics, and the surgical options that she selected, understanding the implications of the former two.

In my world, which has been electronically expanded to include folks with LFS from all over the world, such a drastic and radical decision is not that unusual.  I am acquainted online with many women who, given their family history, devastated by breast cancer, and their LFS status, have taken the same aggressive and strong preventative position that she has. Many more among them are contemplating this same measure. I applaud them all. It is fierce, and beautiful, regardless of common ideas about beauty and femininity. 

Angelina Jolie has a genetic predisposition to ovarian cancer (which her mother suffered with) as well as breast cancer. A  BRCA 1 mutation will significantly increase the likelihood of her developing breast cancer, somewhere between 54 and 87% over the general population, as I understand it (especially in rare disorders, numbers and studies vary, kind of a lot) and ovarian cancer between 40 and 50%.  These are some giant, scary numbers put in front of anyone. Even if you look only at the low end of that risk, it is significant.  Losing her mother to ovarian cancer makes this information much less theoretical. Given her personal experience with it, I suspect that there were some sleepless nights involved for this poor woman.

There was something in Ms. Jolie's statement that I read, that suggested to me that the double mastectomy was perhaps not the end of her medical to do list, but rather, just the beginning. She 'started' with the higher risk and more complex surgery, which sounds to me like an oophorectomy might be next, to take care of the ovarian risk.  I would love for her to get in line, right behind me.

I have very recently had my research approved by the IRB, to determine the extent of the mosaicism of the p53 mutation (LFS) in my body. It is known that the mutation is at least in my ovaries, and as they have completed their usefulness (I have four children and am over 40) I believe that it is time that they go, before they go bad, go rogue, misbehave...pick your favorite cancer euphemism.

Then, by this research, we can begin to learn if my risk is limited to germ cells or if I have a global risk.  This is an important bit of information, not just some idle curiosity about my innards. I need know if I should be screening, like my children are. It sort of matters. A lot.

I do not want to, in any way, minimize the seriousness of a BRCA mutation, but LFS is like BRCA...on crack. While LFS carries a similar elevated risk for breast cancer, we also face increased risk of brain, lung, adrenal, colon, bone...every cancer out there is fair game...every cancer out there is our game, and the odds are higher for us to play in every single one of them.  The female LFS carrier has a 90% chance of developing cancer by 60... Ninety percent.  

Stew in that for a minute.

The difference with LFS is that there are few risk reducing measures to be taken.  While BRCA patients can choose to live without breasts or ovaries in order to reduce cancer risk, (again, not minimizing the difficulty in arriving at such decision, or in taking such measures) but for LFS, bones and brain cannot be eliminated proactively.  My kids have parted with a bit of both, but that, naturally, comes after the oncological fact.  We scan, and we screen.  We do what we can to catch it early, our experience being that a small cancer is more easily managed than giant throbbing tumors (And we have had both).

So, aside from the red carpet, the international humanitarian missions, the galas, and waking up to Brad Pitt every day, Angelina really isn't so different from me.  (Ok, that is snarky). But in truth, if you peel away all of that, which really doesn't matter to me (except for the humanitarian stuff), she is a woman who knows that the genetic odds are not treating her kindly, and she really wants to be there for her 6 kids.  She is willing to go to some extraordinary lengths to take care of herself, so she can do just that. I believe that I can relate to this.

And also, I am sure, because she is a smart woman who has surrounded herself with some smart doctors, she is thinking about whether to test some of her children. Certainly, her love for them does not take into account biology...half of her children are adopted, I believe.  But while her love is blind to personal genetics, in this case, the biology does in fact matter.

And I can certainly attest to the angst that comes with wondering if you unknowingly gave your child the crappiest gift ever...and wondering if should you find out?  And what would you do about it, once you did find out? My prayer for her, or anyone who decides to test their children, is that they have the reassurance of a negative result.  Because, a positive one brings other difficult decisions...many of them.  And worries.

But also, as Angelina Jolie publicly proclaimed and demonstrated, some power. And that power to act, to decide, even when the choices are tough, that is the blessing we have in this day and age.

I return to that 90% number for ladies with LFS...and what we might do to make Lauren's life the fullest, in every way.  She has had two cancers by the tender age of 9...and while we feel that this has been more than enough, the research indicates that she might not be done.  What will Dan and I do with this information? I will let you know when we decide, but I can assure you that we have thought about things, been forced to think about things, things that the average parent of a 5th grader never contemplates, and likely cannot remotely understand.

So, with their giant stage, I am glad that the Jolie-Pitts have shared some of their story, if only because the public at large will now have some notion of what genetic predisposition syndromes like BRCA are, and consequently, might have a better chance of understanding LFS a bit better.