The timing of 'eventually' is perfect

They say that there is no such thing as coincidence.   I believe this.  I truly believe that there is a reason for everything.  And sometimes, it is not the things that happen, but the timing of those things that gives me goose bumps.

My son Brent was diagnosed with Osteosarcoma on his right pelvis nearly two years ago, and after 15 weeks of chemo, he had his entire right pelvis removed in NYC.  It was a giant surgery (18 hours) to remove a giant tumor. We were very fortunate to have found this surgeon, because he was able to reconstruct Brent's hip using donor bone, and save his leg.  (We are so grateful for the donor family as well)  There were 2 follow up surgeries back then, during the nearly two months that we were in New York, before we returned home to Ohio to finish another 15 weeks of punishing chemo.  The delays involved in the second half of chemo carried this process to nearly a year.

But, at the end of this year, my son was on the road to recovery.  He began physical therapy.  His marrow recovered.  He returned to school.  None of this was easy, but we were all happy to have active battle behind us.  There were still an ungodly number of visits to the hospital for scans, labs, PT, specialists... and at Christmas, a wound on his hip that simply would not heal sent us packing for New York again...a surgery whereby a muscle was moved from his abdomen over the donor bone, to help provide a better blood supply to the allograft and promote healing.

And he healed. It seemed to be a success, until last week's routine x-ray showed that the bone is disintegrating. Infection or cancer was the question of the day...because the cause of this deterioration dictated our choices.  If it were caused by cancer's return, there would be no choice. We would have to amputate and he would be on a very hard road of amped up chemo.

If it were infection, there might be some choice.  I prayed hard for infection, because my blood ran cold at the thought of cancer, both due to the orthopedic requirements as well as everything else that this implies.  Actually, amputation would be the least of our worries.  I am very glad to be able to put those thoughts aside for some time. May they remain there for all time.

We always knew that the donor bone would eventually need to be replaced.  'Eventually' is a very theoretical time, until you are looking at it face to face.  I am grateful that I am not smelling cancer's foul breath in this version of 'eventually.'

I would never have scheduled this, setting time aside for it.  But here we are.  And it will be ok.

Eventually, we will need to replace our roof...it is expensive and a very unsexy way to invest in your house, as I say. If you drop that kind of cash in your kitchen for example, you would have a party and invite your friends over to take a gander at your new cabinets when it was done.  There would be wine, and it would be worth the aggravation.  Which is why people ever embark upon such a construction project to begin with...one so inconvenient and disruptive, but with a big payout.

Most people, if they think about their roof at all, look at it and say "Eventually we will need to replace that."  But if it isn't leaking, or worrisome in some other way, you wait another year.  We have been in our home for 8 years...not sure how old the roof was when we bought it. I am grateful that we do not need to replace it this year. I hope to be grateful next year as well.  I hope to sell this house with the gratitude of many, many years of never replacing our roof.  If the eventuality of replacing the roof never came to me, I would be quite satisfied.

But, should we discover a leak, we knew that this was a possibility...a probability, in fact.  So, we call our contractor, and get busy.  Repair, or replace? 

I hope that the weather is dry, because we are doing a full tear off, exposing the rafters on this one. There are plans to rebuild though. We are thankful for the rebuild.  So very thankful.



Brent is happy, because he can finish the school year and enjoy all of the activities associated with the Kick-It fundraiser for pediatric cancer research that his school does every May.  He has been to a bake sale, a car wash, teacher dunk tank, a soccer scrimmage (boys vs. girls--girls won!) and next week has more events set up by middle school children.  If they make their goal again, they will have raised a total of $100,000 in four years, only working in the month of May. 

While there is some adult help, this is achieved mostly the effort and organization of children.  One of Brent's friends set up a website (heck, I couldn't do that) and a calendar of events.  There were scads of kids washing cars and baking cupcakes.  They are using their time and talent to make a difference.  They are using their collective numbers, with every little bit advancing the common goal.  It is a powerful lesson for these children, and one that I was impressed by, even before we were so personally invested in the cause.

I am grateful that Brent can participate in these things.  The timing of this surgery allows him to finish school, which is so important to him, and to attend 'Kick Bald' on Monday.  He challenged Lauren to raise $250 for the privilege of shaving his head in honor of kids in treatment, which has been a source of much teasing and fun between the siblings.  (I do not need to tell you how differently I felt about this as we worried about Brent's pathology last week, thinking he might soon be a child in treatment) 

Lauren baked up a storm, and made her goal...and then some.  I am so proud of her for thinking and planning.  She is grateful for the generosity of the donors...because while she makes a mean brownie, she recognizes that the charity was what was being supported. It will be a fun event, watching Lauren shave Brent's head at the school.  There is a sense of solidarity in the baldness.  Last year, when Brent had no hair, it was touching to see so many of his classmates sporting a shiny dome in his honor.  We are glad to be honoring other children this year, showing support in a visible way. 

We are looking forward to a time when baldness and cancer are not associated with children...or anyone for that matter.



Brent will be doing his Make a Wish next weekend. This is a wonderful organization that provides wishes for children with life threatening conditions.  Brent has dreamed for nearly a year and a half about what he would wish for, which has been a wonderful distraction from the many challenges that he has faced.  Many of his wish ideas involved sand or water, or a combination of both, which does not mix well with an open wound.  He wanted to wait until he healed, so that he could do this sort of wish. He waited, patiently most times. Eventually, we thought, he would heal and be able to do it.

But 'eventually' became far too theoretical for him last week.  He faces weeks in the hospital this summer and he knows that this surgery is not a sure thing.  He wanted to move forward with another wish.  He is going on a "shopping spree, " which mostly involves getting a computer.  He would like to learn how to design apps (?!?)  and feels that a laptop would be a good distraction while he is in the hospital.  He knows what this is like being isolated that way, and is using his wish, again, to make that difficult time better.


As I have been planning for this trip, I am struck with how different this is from the last time we removed his hip:

• There is no cancer to worry about, or chemo beckoning.  I will not be frantic about delays.  There will likely be delays.  I will be calm.
• Brent will be emotionally replenished and prepared.
• We know going in that it will be at least 4-5 weeks.  We can plan a visit with the kids in the middle of this because there are not restrictions for cold and flu. 
• The kids are out of school, so keeping things rolling here is simpler.  Alex is 15, and because he is home for the summer, can help with his sisters. 
• There are things that have fallen into place for us, like the fact that the chief of surgery from Bologna Italy, was recently picked up by MSKCC and put on staff...he has successfully done the surgery that Brent requires 12 times.  I am comforted by this addition.
• Dr. Healey, because he saw us just before his trip, has been consulting with the brightest in Europe about Brent's case.  We benefit from their experience and opinions, without needing a passport.
• In case I failed to emphasize it enough, we have no oncology pressure cooker.  I cannot say what a difference this will make going in. 

This series of surgeries is by no means a slam dunk.  It will be a long process and fraught with risk.  But there are things that I am encouraged by. Things that I am comforted by. Many things that make the challenge more manageable.  The timing of this eventually, is part of that comfort.

Only one problem, thank God

As I picked up my phone in the car yesterday, I saw the (212) area code, recognizing that Sloan Kettering was on the other end.  I answered, stomach dropping, and heard a familiar voice, Dr. Healey's nurse. The pathology was in, she said, and it was negative for malignancies.  She wanted to call me right away to put my mind at ease, which was an act of kindness that I will always remember with deep gratitude.

I could hardly speak, and only managed a tearful thumbs up to Lauren who sat next to me in a state of confusion, bordering on concern, as I blubbered incoherently.  Lauren has been with me before, when I fielded tough news.  She is an old soul, and mature far beyond her years. I am very grateful for the universal message that thumbs up provides, because I couldn't manage much else.

I immediately called Dan who was relieved beyond measure.  Then I called Brent, who gave an understated "woo-hoo" as though I said that we were having pizza for dinner. Really. He was more enthusiastic about hearing that I was taking Lauren for a date, than the fact that he didn't have cancer.  "You haven't had time together in a long time!  Enjoy!"

So, we went into the restaurant to have a date.  I was trying to see how she is doing with what Brent faces. I apparently will have to have another date with her, because while we had a joyful meal together, interrupted by many happy, relieved texts from friends and family, and my own emotional rollercoaster, I did not get a good gauge on where she is in all of this.

Lauren and Brent had cancer at the same time, which is long story for another post.  In short, she had a golf ball sized brain tumor removed here in Ohio only weeks before Brent had his entire right pelvis removed in New York. The abrupt change in his situation this week, including concern about his cancers return, and the likelihood of amputation, well, that is a bit much for any 10 year old to process. But, I wonder if she additionally worries about her own cancer status. It would be surprising if she didn't.

Brent is managing amazingly well with all that he faces.  He wants to focus on the good things the next few weeks will bring before surgery...the Kick-it for Cancer fundraising events that his school has every year to support pediatric cancer research, the end of school bonfire, his Make a Wish.  He says that he does not want to talk or think too much about surgery. I would worry about this, concerned that he is not preparing himself, but this is how he has always done things, focusing on the good that today offers and dealing with the difficulties when he must, regardless of the challenge: chemo, surgery, distant hospitalization...

In addition to checking in with Lauren and preparing Brent, we have the other kids to think about. Alex is a very physical, athletic kid.  He and Brent, only 2 years apart, have always been close.  They played soccer together and rough and tumbled outdoors their whole lives.  Over the last year and a half, Alex has struggled with the change in their relationship, in the ways that they can relate.  He wants to fix it, to fix Brent.  He wishes to return to how it was before cancer.  I wish I could do this as well, for both of my sons. 

Brent is happy for Alex and has a very generous spirit. I have never seen any jealousy or resentment from him about Alex's ability to continue along a path that isn't as physically restricted as his own. While Brent hasn't gone to but a couple of Alex's games, this is mostly because sitting so long is uncomfortable for him physically.  I think Brent will continue to adapt well emotionally, but this is going to be a very tough thing for Alex to come to terms with.  I recognize how strange that seems.

Olivia, well, I do not know what to do to prepare her. At 5, I think that this would best wait until we are much closer to surgery.  But I am at a loss at how to make all of this right for any of our kids when it is so difficult for us to wrap our own heads around.  I do not know exactly how to help them.  Even if there was a manual for raising kids, I doubt that there would be a chapter that covers this.

While I still feel the heavy weight of our limited orthopedic options for Brent, without the oncology bearing down on us, it doesn't seem quite so overwhelming.  It is unbelievably hard, but not soul crushing.  I will go with "not soul crushing," and be grateful.

Very, very grateful.

And try to find our way to the next step. As serious as the problem is, and as difficult the solutions, we know that we are blessed in a dozen different ways, starting with the clean pathology.

Brent had cried out in Dr. Healey's office that it wasn't fair, which really struck me.  This is a statement that he has made very, very rarely, in the course of this 21 month ordeal. (I believe only twice)  All of the chemo, all of the surgery, all of the rehab...and he has complained very little.  I think he recognizes that while it cancer is not fair, this is what is.  Fairness is immaterial, so why dwell on it?

So we try to focus on what we are given, rather than what we have lost.  What we are given, the things that we count among our blessings, might not occur to others.  It is probably a given that your children to not have cancer. I name this daily as a blessing, but likely take other things for granted.

We are all different.  We all have blessings, and challenges, each unique. I think that our happiness stems from where our focus lay. (lie? lays? lies?--I can never get that one right)  You understand what I am mean, crappy grammar aside. 

Not to be all rainbows and unicorns, but I am going to focus on what we have been given, as we move past the shock of all of this. Like Brent, I am not going to dwell on the immaterial, unchangeable facts, but pick a solution and march on.

That is my plan at least.

Walking in Manhattan

After starting the linens in the washing machine, I thought that I would let Brent sleep a bit longer at Ronald McDonald House. (One advantage of him finding sleep on the couch more comfortable than on the bed, is that I do not need to wake him to do the 'exit laundry'). I decided to return the wheelchair to Memorial Sloan Kettering that we borrowed after Brent's needle biopsy yesterday.  I threw my handbag into the empty wheelchair and set off, alone with my thoughts in the city.

This has been an emotional trip, and I am finally alone, without things that I actively need to do like when we were in the hospital.  There is anonymity in the city, especially one this large, and with the understanding that I do not know anyone here.  There is real temptation in screaming right there in the street...wailing to the heavens.  A plea for some mercy, a frustrated cry for insight, a bit of anger at God.  I think that it is ok to be angry with God once in a while.  I do not rage outwardly as I walk, but I am grieving.

My son Brent, who only a day or two ago (could that possibly be right?) expressed to me how he couldn't wait to be able to run again, how much he is looking forward to the freedom to play with Olivia in that physical way almost required of 5 year olds, will never be able to walk normally. We learned that he is in all likelihood, going to lose his leg, after a long year and a half of surgeries and rehab in an effort to save it.  

And that may not be the bad news.  We are now concerned about local relapse of his cancer.

For months, there has been starts and stops with physical therapy.  Concern and frustration nagged at me about the fact that we have not managed to establish a head of steam in his progress.  But, it seemed last week that we might have just turned a corner.  His wound was improving, and I was going to set up PT again for him, beginning tomorrow, upon our return to Ohio.  We turned a second corner here in New York however, when routine X-ray showed that the donor bone is dissolving.

I never saw that coming.

So now, the worry that I have had about infection for a year, has been transformed into my wish.  My fervent prayer is that we have been tamping down an infection for months, despite massive doses of antibiotics and antifungals, and that the infection has damaged this donor bone, degrading it to the point that it must be removed.  That is my new hope.

From an orthopedic perspective, the problem is severe, and the solutions are all unappealing.  But if the causative agent is infection, we have one sort of problem.  If it is cancer, we have much bigger challenges and more difficult "fixes."  

I walked, looking at the flower vendors, and food trucks setting up for the day. The constant movement of people, like a pulse, even in the early hour carried me along. I really love the city, for a country girl.

Along another line of thought, I spoke at length to my oldest son last night.  He, quite understandably, is struggling with this.  He offered up a string of ideas, solutions for this orthopedic problem that do not involve amputation.  "Couldn't you go with metal?  Could you use his fibula?..." He told me that he had loads of ideas, as he wrestled with the changes that Brent faces, that we face, as a family.

I explained to him that while it is not completely decided yet, amputation is what he should really be prepared for. I told him that Brent is not defined by his physical form.  But that this change, which is troubling to Alex, might serve as his own personal inspiration, especially if he has ideas.  Go into biomedical, and make the world better.

We all have hard things. It takes no effort to lie there, curled up in life and bewail our challenges.  Our job, I believe, is to turn those hard things into something good. 

I challenged Alex, that if he has ideas about how to fix this, he should write them down, explore them, find the problems that come with with the solutions (they are always there) and try to fix those problems too. If he feels passionately about this, he should do something about it, and try to help others.  I am trying to do that through my writing, using the hard things we have, and bringing good things to others, making it easier, in all hope.  

I drop off the wheelchair, having borrowed it overnight.  There is a family feeling about this, sort of like when I would take my parents car when I was a teenager.  The discharge nurse had encouraged us to borrow it (He had a needle biopsy only hours before, after all) shouting to the secretary at the desk as we left that we were taking it to RMH, feeling like like a sister of sorts.  Will I ever feel differently about this place, like when you step into your parents house after living on your own?  A bit of a guest, rather than like you belong there?  Should you knock?

I head back to RMH, up First Avenue.

A third line of thought, which I try to sort out...the people that have come into our lives.  I had shared with Brent that I believe that things happen for a reason, that there is a plan.  While God sometimes gives you hard things to do, he also sends you help along the way, guidance and support.  I see too many coincidences to think otherwise.  Lately, there have been people, who visit, and revisit my life in odd ways, at serendipitous times.  

Looking back, I sometimes understand what someone's purpose was in appearing at a particular time. But sometimes, I worry about the future when someone shows up.  I don't want the "help" that they might be offering, or the problems that they are best suited to assist me with. I fear what it might mean, and really wish that they would just go away.

For example, a doctor that has been particularly helpful to us in Ohio is looking for permanent position elsewhere, and unfortunately, has not secured anything as of yet.  He is extending his stay with oncology as a night hospitalist for another 6 months.  Until yesterday, I simply wished good things for him, and left it at that.  Now, that information carries a tinge of anxiety.  I do not want that sort of help, or those sorts of problems.  And I have a half dozen examples of this ilk to worry at me.

It is best not to look ahead in that way, I think. I remind myself that it is not all about me, or my family. And these sorts of things are not prophetic.  There are a multitude of purposes, and reasons, none easily seen or understood from this perspective. 

I have racked my brains, trying to figure out what all of the labs, and symptoms of the past 5 months add up to, in advance of the pathology which will take a week.  How would the CRP behave from infection, from cancer? But I am no doctor, and the doctors don't know.  We all wait.  I wait impatiently. Anxiously.  

I step into a coffee shop, my regular stop on this route. Brent and I were here only 2 days ago, grabbing a bite while people watching, and resting as the 5 block walk to Sloan was a lot for him to crutch. We didn't bring our own wheelchair to New York, and the thought had flitted through my head that day that perhaps I should arrange to return it, as we seldom use it anymore. Brent was looking forward to showing Dr. Healey how he could walk.  We had no idea how different that meeting would be from what we had imagined in that moment.

Brent is still sleeping when I return, an untroubled sleep. I put the linens in the dryer and suddenly it all washes over me.  I need to breathe.  And take one step.  One word.  One moment. One breath.  Then another of each.

I need to take the hard things and make them good.

We came here in solid clouds, that made the landing a leap of faith.  We leave in fog, which obscures the path right in front of us.  I am so grateful for the fact that we have an Angel Flight, so that I do not need to drive for 9 hours and focus on the immediate responsibilities of driving for that long, which are both too much and too little at the same time.  I miss my husband and my other children, and long to be all together.

We wait a week for pathology, to find out what we are doing.

We wait.

Trust from dizzying heights

Greetings from 7,000 feet.  It is beautiful today and Brent and I are on our way to NYC, courtesy of Angel Flight.  I cannot say enough how blessed we are to have this kind of help.  We are going to change planes in Williamsport rather than in University Park, so that another patient can get a ride home.  It is amazing to me, the coordinated generosity of so many pilots.   

With this bit of time, I thought that I might tell you about my plans.  I met with my OB on Thursday to talk about surgery.  He is putting in for pre approval from my insurance company, which I am hopeful will go through. (Although, if it doesn't, I have programmed my case manager's phone number into my phone...I have someone on the inside who might help me with the appeals process if need be). If denied, I will appeal.

I really, really like my OB, who delivered Lauren and Livvy.  I actually have a piece that I wrote some time ago, that I will post later about picking doctors.  I feel like I have to run it by both Drs. OB and and Ortho before I do, as I refer to them by name, and out of deep respect, would like their permission before I post it. But I digress.  

My point is, that I would really, really, really like to use my OB for the surgery...it would be our last hurrah together, as I will have no parts left for him to annually check when it is done.  I trust him, and well, he is my guy for such things.  Should insurance deny coverage, I would need to have surgery at UH, where incidentally, my guy does not have privileges.  I would have to use someone else.

Because, given that the Ramers are sort of the goose who laid the golden egg for UH, I would be in a better position to work out something financially with them for of all things, removing my eggs.  If I didn't have irony and snark, you might as well cut out my tongue as well, because I would have nothing left to say.  You have been warned...this is going downhill from here.

So, if I get my wish and have the surgery at Southwest, which is affiliated with UH, but not in fact owned by them, I will need to get my girl parts shipped across town.  Last time that I needed to do something like this (who ever needs to do something like this?!!) Dr. Peters, my kids oncologist, was kind enough to drive across town with a box of dry ice and swing by FEDEX on his way back to UH, sending one biopsy off to Toronto.  How's that for service above and beyond the call of duty?

As my local geneticist is taking the lead on this one, having developed an essay that will detect our p53 mutation to within 2%, international shipping will not required this time, thankfully. So, I need a local medical currier...which we are thinking might end up being Dan.  "Honey, can you pick up the dry cleaning, and drop off my ovaries?"  Seriously, who makes arrangements for the transport of their own organs, outside of their body?  

I need to be sure to get a doctors note for my dear husband, in the off chance that he gets pulled over.  (I know more than a couple of doctors...which one is the best for such a thing?)  Because "What is in the box?"  could be a very awkward question to answer, when it is in fact, pieces of your wife.  Probably should rework the phrasing of that answer.

In case you missed the memo, I have a weird life.

So, the actual surgery...  You will remember that I was looking to have multiple biopsies done.  I went in to this talk with my OB with a particular idea...kind of a buffet or smorgasbord of my organs...take a little sample of each while you are in the neighborhood.  So, after a little geography lesson...there is sort of the continental divide in you belly (diaphragm) which makes loads of things out of easy reach if you happen to be doing a pelvic surgery.  Damn. 

But, there is the liver and spleen in the area (ooh, goodie!). However, given their vascular nature, they have the tendency to bleed ...and not stop.  I was advised that this was a very bad idea. Usually, such biopsies are done with CT guidance, which is a bit more than 'just taking a bit while in the neighborhood.' 

I have said that I would never presume to tell a pilot how to fly or a surgeon how to cut. (Writing of a surgeon as sit next to a pilot)  While I really am committed to finding some answers, I am not wanton.  I do in fact listen.   I may be crazy, but I am not stupid.   But I was bitterly disappointed, nonetheless.  

Bowels will be biopsied by GI in a separate procedure, the details of which I will be sure to keep to myself, and for which you will thank me, heartily.

Which leaves my tubes and uterus.  

"Are you planning on having more children?"

Umm.. No.  (Thinking, I would remind you that you are taking out my ovaries....and we talked about this you when you tied my tubes several years ago)

With extreme patience, and a hint of irony, "Do you plan on carrying a child for someone else?" (In my spare time?  with my spare energy?  With my 44 year old parts and sketchy genetics hovering around in the background?  Not likely.)

At which point he explained that really the only thing that my uterus could bring to me in the future was cervical or uterine cancer.  He could biopsy them, but really, it makes infinite sense to remove them.   

Dan will need a bigger box.

As I left to schedule it, an opening was available for Thursday.  As in this Thursday.  I feel odd, having been anxious and prepared to do this surgery for 6 months now, but Brent is doing hyperbaric oxygen treatments every day....and this would be the day after we return from New York.  Seems like a lot, and so waiting until the next slot in June makes more sense.  We go in June at this point.

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Flight number two...chatting with our pilot, I learn that he is from Maryland and works for NIH.  I kid you not.  We exchange IRB stories, naturally.  

The weather has changed dramatically, and we fly in solid clouds, which I have never done before.   I think that he said to within 800 feet of the ground, which he preferred was much higher, naturally.  I had no idea, sitting next to him, how disorienting it could be.  You must completely rely on your instruments, as instinct will help you not at all.  I have no doubt as to how JFK Jr got into trouble, because what you feel and what the instruments tell you do not jibe.  If you are accustomed to following your instincts, this sort of flying is a recipe for disaster.  

I am trusting in the experience of this pilot.  I trust the experience of my doctor, and of the doctors that we are traveling to New York to see.  Hoping that we all get on the ground soon...and safely.

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.

 

A Delicate Gift

It was a remarkable day.  A beautiful one, but one laced with fear, punctuated with shock, celebrated in jubilation, in disbelief…in grief. 

The day began as my mom and Alex left to attend a funeral.  One of Alex’s soccer coaches, a young man, only 20 years old, died very suddenly and unexpectedly.  I could not go with Alex, because I had appointments with Brent at the hospital, and am grateful that my mom could go with him.  Alex spent his day celebrating the life of a friend, and grieving the loss of him.  This family, who I do not know, is close in my prayers, and heavy in my heart.

Dan has been keeping me updated on the status of a friend’s newborn baby, the joy experienced at his birth less than two weeks ago has been replaced with fear as this little one battles meningitis.  He has had multiple spinal taps and they expect several weeks in hospital.  Even from the tiniest humans, there is the spirit to survive.  I think of this family throughout my day and send them strength.

Brent and I were at the hospital all day.  There was nothing earth shattering…a mammogram for me, and clinic visit for him.  We stepped out for a walk at lunchtime, meeting Dan in the sunshine.  Brent and I toured Case’s campus, enjoying the chalk scrawls of anxious students approaching finals.  The encouragement and humor displayed was a good thing to see.  The mix of flowers, and mix of old and new architecture on campus was beautiful, and somehow calming.  We went into the Peter B Lewis building, designed by Frank Gehry, which Brent really enjoyed. 

At clinic, I called about the new treatments that Brent is to start, but learned that we cannot until insurance approval has cleared.  They said that it can take up to 10 days.  I am a little frustrated with this, but I know that there are bigger problems to have.  We have had bigger problems.  Others currently have bigger problems.  I try to balance patience with persistence, accepting what I cannot change, doing what I can, and finding the ever elusive “wisdom of knowing the difference.”

Dan texted me and said that Brent and I should go have a date, one that does not involve a hospital.  So, we did.  We went to a movie, on a Monday night, which is unheard of, and was fantastic.  Brent had dreamed of seeing Iron Man 3 that night, and woke up bitterly disappointed in the ending, which was one of the fun things we talked about as we walked.  So we went to see it together, just the two of us.

When we returned home, Dan told me the shocking news that Jina DeJesus, Amanda Berry and Michele Knight were all found alive after a decade of being missing.  Amazingly, joyful news.  It is unimaginable, both in that they are all alive, but also because they have been hidden here in Cleveland all along.  Unimaginable to think of what those years must have been like for those young girls, what they lost, how they will have to mend and rebuild a life.

Life is a gift, albeit a fragile one.  Even a broken one sometimes.  But out of the brokenness, out of the striving for survival, out of the rebuilding, and even out of the loss, we show that it matters, that it has value.   

And there is beauty where there is love...from the encouraging chalk scrawls on the sidewalk of the university to the hug of a neighbor-savior, and everything in between.