A less traveled road

I spent most of September and a warm October in the hospital with Lauren and Brent.  I would see large families visiting Rainbow Babies and Children's Hospital on the weekends, sometimes providing improbable visuals: Amish men with their wide brimmed hats and distinctive beards walking behind a group of Muslim girls in full black burka. Witnessing these unlikely neighbors reminded me that pediatric cancer recognizes no boundaries.

While there is great focus on disparity, discord and discrimination between cultures, cancer is the great unifier.  We parents, regardless of any other differences, desperately want the same thing for our children.  Within the cocooned isolation of the oncology unit, we hold on to each other and to hope. We may pray in varied ways, but each of us utters the identical prayer.  Please bring health to my child.

The world, as presented in the media is a clanging ugly noise, something that is piped in via the television found in the nutrition room.  I generally turn it off, preferring the quieter expressions of love and kindness that we experience every single day.

While writing this, lyrics from some other lifetime, were called to my mind.

We share the same biology 

Regardless of ideology 

Believe me when I say to you, 

I hope the Russians love their children too
-Sting

I enjoyed my occasional days at home when I could to get Olivia on the bus. Upon waking, Olivia would always roll over to sleepily greet the sunrise, declaring its beauty and promise.  This morning when I woke her for school, it was dark outside, and cold.  Her cheer and enthusiasm are blessedly unwavering.

We return to warmer climate next week. Brent will formally enter a clinical trial at MD Anderson in Houston for his relapsed AML, a research study that just began recruiting in mid August, only a month before we sought options there.

I picked Lauren up after a rare day without chemo, one that permitted her to attend class.  We chatted over lunch about our family and the notion that "to whom much is given, much is expected."  The Ramers have been given so much. While we have been given far too much cancer in my opinion, we are also loved and supported in ways I never could have imagined. Lauren and I agreed that we each have a talent, and we should use it in service to others.

My daughter is comfortable speaking publicly and uses her voice to help the adolescent cancer community. Brent, she pointed out, has knack for getting out of incredibly tight spots. His talent seems to be providing Proof of Concept to enterprising folks in medicine, like the surgeon who successfully grafted skin from Alex onto Brent last November.

While I cannot deny the truth in this, I would much prefer that he take up juggling.

I reminded Lauren that I have been following developments in immunotherapy for years.  Emily Whitehead was a name I knew long before Brent had leukemia.  Her success with Car-T had dramatically changed the treatment options in ALL.  Reading about her and the brilliant researchers at Children's Hospital of Philadelphia brought me hope. I thought that using the immune system could transform all of cancer treatments, particularly for folks with LFS because it does not rely on our defective P53 function, as chemotherapy and radiation do.  I even said back in 2015 when Brent first developed leukemia, knowing that Car-T was still limited to CD19 /ALL, that I would gladly stand in line if a CAR-T trial for AML were available.

We now find ourselves next in that line for an early phase I trial for CD-33 Car-T to treat his relapsed AML.

I wonder sometimes if I unknowingly made a bargain with God. I alternate in my responses.  Should I be grateful about this, terrified, or completely pissed off?  I always lean toward gratitude, but in the interest of honesty, have to reveal my conflicting emotions. Faith, I have always said, is hard.

Our home smells of wood smoke and spiced cider when we return. At Thanksgiving, for three consecutive years now, Brent will attempt something unproven.  His daring is fueled by a mix of desperation and courage. As his mother, I choose to focus on his courage and his grace.

I wish we were simply taking a less traveled road, but Brent consistently steps into the wilderness with a medical machete, cutting a path for others to follow.

We welcome your prayers, however you form them.


   

Counting up the October blessings

I have had a busy month. I generally accept whatever lands in my lap, be it screwed up genetics or the kindness of strangers (or of loved ones.) While I do go out and make some things happen in this life, lately, I have been guided by what comes my way.   A lot has been put in my path recently.

As I look it over, it has been overwhelmingly positive. 

We did Lauren's Make a Wish.  Our trip to California was a huge blessing, and Hollywood couldn't have had a better experience. I am so amazed by the kindness of strangers.  My faith in humanity, if it had faltered at all, was renewed by the generosity of so many toward my children.  Having endured two solid years of 'medical nonsense,' as I euphemistically refer to this nightmare, I was glad to be together, outside of a medical facility, and for us to just have some fun as a family.  I was very grateful to not have to plan anything.  I feel lucky beyond measure.

I went to Boston last weekend to a genetics conference, which enabled me to meet some online friends.  I would try to write about what this was like emotionally, but I am afraid that I couldn't describe it any better than my dear friend Jen Mallory already has.  So, I won't even try to, rather just direct you to her beautifully written blog: 

http://lilykaymonkey.blogspot.com/2013/10/sisterhood-of-travelling-mutants.html

There was way more laughter than should be allowed...and hideously inappropriate cancer humor.  I am a firm believer in the notion of  'whatever gets you through.'  These ladies definitely are 'getting through.' I should mention that a song/chant of "I heard a little rumor, heard that you have a tumor, you have to have some humor, can't be a doom and gloomer" probably should not be what you lead with, approaching a newly diagnosed cancer patient.  However, this group is not standard, lets just say.

Further example of non-standard, when asked how many malignancies she had had, one mutant friend had difficulty answering, because she didn't consider melanoma a "real cancer." I think that we settled on five for her, but there was some debate, and also some beer.  So don't hold me to that number.

If the mutant convention was enjoyable, it was also enlightening. I met some researchers, and learned about the things that they are studying.  I am grateful for their work, and for the fact that we will gather together, researchers and subjects alike, every year now.

While some of the presentations were very "number-y,' there were encouraging things to be found in nearly every report.  My personal favorite was a researcher out of Utah, who serendipitously learned that elephants and whales, despite their much larger size and increased number of cells, oddly, most surprisingly, have a low rate of cancer.  There is almost no cancer among elephants.  He investigated and it was discovered that this is because elephants have not one pair of p53 genes, but 20 pairs.  20!!!  When I shared this with Lauren, she asked if she might borrow some elephant DNA, and giggled enthusiastically.  I would absolutely get her transfused with pachyderm blood, if I thought it would help.  It is coming, or something of the like, though, and I can feel it. 

It was birthday season, and birthdays are joyful days, particularly in our house where we do not take them for granted.  Cannot take them for granted.  The oldest three kids all have birthdays within 6 weeks of one another, and we have had our fair share of cake.  I keep saying that I feel that good things are coming our way, without any real justification for the feeling.  Out of the blue, Dan's dad offers not just his former car, but his pride and joy, to Alex.  On his 16th birthday.  Yes, we could never have done that for him. 

While Alex is understandably excited about this, I recognize that it is a gift to me as well.  He will not only be able to get himself to soccer practice and school once he gets his license, but will be able to help me get the other kids thither and yon...if I ever get it together enough to organize after school activities beyond physical therapy for any of them. Sigh...I will get there.

Upon our return from our Make a Wish trip to California, we did scans.  They were generally good, as I reported out.  There was one node to follow up on this week.  And I had slipped in the dermatology 'once over' this week as well, one that was supposed to happen over the summer, but had been pushed to the bottom of the priority list.  This is understandable, given that we had two New York surgeries to contend with.

So, when I returned from Boston, we had dermatology for the kids, which was not completely straightforward and will require several follow up visits. Sigh... Thursday, Brent had his follow up ultrasound for the concerning lymph node, which initially showed that it had grown.  Not a fan of this.  After dropping him off at school, I went to a friends house, to have coffee and catch up.  As I prattled on and on about my worries that afternoon, I fielded calls from the hospital.  Judy is a beautiful, dear friend with the patience of Job, sitting there as I sorted through the various hospital issues on my cell phone. Eventually, the CT was cancelled and it was finally determined that we are not looking at lymphoma.

"You are a mom.  I don't imagine that you ever stop worrying, knowing that everything can be cancer. I don't know how you do it." 

I explained it like this:  Lauren had headaches all week, a symptom of a virus that has been freely shared in my house.  Because she just had scans, I was not at all worried.  It was the only reason.  Usually, scans are on a single day, marked by mounting anxiety, which we try to mitigate, followed by emotional release with the hospital phone call.  Big worry, then giant relief and celebration when we get the all clear.  And peace, and confidence (oh, except for that time when they called about Christmas brain surgery).  The scans, which are stressful, are the cover charge for any ability to relax. Ever.  This time, there were follow ups...and so the worry drags out a bit.

The good glow of clear scans lasts about 2 1/2 months.  At the end of the day, we have been granted this blessing.  So, I will run with this.  There are good things coming our way.  Looking this over, I have no idea why I ever doubted it.  I am grateful for the further confirmation this week.

Brent and I leave in the morning for NYC, armed with some oncologic reassurances, looking for orthopedic encouragement.  We will again be relying on the kindness of strangers, taking an Angel Flight from Cleveland.

Good things are indeed coming our way.  The blessings from all sides keep landing in my path.

Knowing: The burdens and the blessings

Knowledge is power.  Knowledge is responsibility.  Knowledge is a call to action, to vigilance.  No wonder they say ignorance is bliss.  (Ha!)

On my LFS support page this morning, someone wrote about how they can now test embryos and using IVF, select for implantation only those that are mutation free at p53.  There are those who have successfully carried a mutation-free child.

I knew that this could be done because my geneticist told me about it when we learned that Brent had Li-Fraumeni Syndrome.  They also shared the new Toronto Protocol with us, which is a screening protocol to find cancer early.  The folks at University Hospitals, I think, were piling on the latest options and improvements to mitigate an otherwise devastating condition.  The lion’s share of research up until this point has focused on “How do you feel, now that you know?” and the psychological ramifications of living with this knowledge of a faulty tumor suppressor gene.  The failure of this gene often results in multiple episodes of cancer, sometimes simultaneously.   

I will not pretend that this is easy, either the knowledge or the attendant worry that comes with it.  As I have shared, a series of headaches in my 11year old begins with ‘rule out brain tumor.’  That is a tough way to live, but far better than sitting back and waiting.  Because after 3 episodes of pediatric cancer, we were going to worry anyway.  At least with the knowledge, we can take action and find things early.

Similarly, there is power in taking action, of being able to choose only healthy embryos, in being able to eradicate this mutation in the future.  Who wouldn’t want a healthy child?  This is a no brainer.  But as I considered this some time ago, about how I would explain this option to my children when the time is appropriate, I realized the ethical and existential morass that this would be.  Because this decision, in a way, denies the validity of my children’s lives.  If I present this option, it is implied that I would not (or might not) have chosen to have them if I had known.

It is kind of loaded.  Because we didn’t know. 

This would be prime time for calling in some of those psychological researchers and positing the question.  “Well, how do you feel, now that you know?”  What I do know is that there are many ways to form a family.  I will consider it an enormous blessing for my kids to have the opportunity as adults, to wrestle with exactly how they would like to accomplish this.  I will be very grateful for this particular problem.

 

Yesterday, there was a news release out of Memorial Sloan-Kettering, a hospital that we know well.  They were reporting advances in targeted cancer therapies, ones that use our own harvested T cells, which are infected with a virus and programmed to attack a particular tumor cell.  This is accomplished by looking at the genetic profile of the tumor.  And sometimes, by doing this, they stumble upon information that they weren’t looking for, like a BRCA mutation or LFS in the patient.  There are ethical conundrums that come with this data. Are researchers ethically obligated to share this information with the patient, which can have clinical implications?  Does the patient want to know?  Should they know?  Should you even pose the question to the patient, which all by itself opens a Pandora’s Box of worries? 

There are families with LFS that are torn apart, not just by cancer, which is difficult enough, but by the decision to seek genetic answers.  Because this is a family syndrome, even establishing the diagnosis for yourself has implications for everyone else who shares your bloodline.  So, in some families, having a p53 mutation established poses the question for other family members and by extension, their children.  For those who do not want to know, it can become an issue.  And they no longer bring the green bean casserole to Thanksgiving, let’s just say.

I am an avid proponent of research.  I celebrate advances in all areas of cancer, which you might contend is hugely self interested, given the likelihood of us dealing with more cancer.  I am no position to deny the charge.  But even with a dog in the fight, I recognize that scientific advances sometimes come faster than we can wrap our head around them.  This is ironic, because research seems to go slowly when you participate in it…or if you are waiting for a particular advance in a timely way.

I think of my friends, those with LFS, each fighting in their own way.  Battling the worry.  Being grateful for the worry, when it turns out to not be cancer.  Battling the cancer when it comes.  Or, when it comes back.  The fight is mental as well as physical.  It is all grueling, and it never ends.  But through this devastating and demoralizing disease, because of it actually, I have come to know some amazing people.  While I would much prefer that we had met at PSO function, this is the hand that we have been dealt and these are the people that have been placed in my path, on this path.

I am going on Thursday to meet some of these wonderful people in Boston.  The researchers.  The mutants.  I want to learn what advances are coming and how they might help my children.  All of our children, because we have become one big mutant family, bloodline be damned.  The blessings cannot come without the challenges….my new extended family is the blessing.  The challenges still suck.

We are fortunate that the Ramers are not waiting for ‘a particular advance in a timely way,’ nor are our children forced to wrestle with existential quagmires. 

Not today at least. 

Shared Parenting

My geneticist emailed me yesterday with the good news that she has figured out a way to pull the DNA from paraffin block of breast tissue, removed from me four years ago.  No, I do not understand, yet, what that all means.  I do know that this was a problem, getting the DNA out, and one that she has apparently solved (It is easier to achieve from a fresh or frozen sample, as I understand it.) I will get back to you about the details when she and I get together and chat in a few weeks.

Thinking about this, it occurred to me, that what we do here is complicated.  This could be explained by the whole cancer experience.  Heck, that statement could easily be justified by the simple fact that Dan and I have four children.  But I am talking about genetics.  Genetics and cancer are daunting things to try to understand, and even more so to explain. 

We can super-simplify things and say that we have a bad cancer gene and because of that, we get cancer.  But that really doesn’t explain it sufficiently, on any level.  Medical, emotional, relational…the list goes on.

You likely do not consider your genetics much, but they do influence the way that we move in the world, more than we think.  Aside from determining how much product I really ought to use in this humidity (but don’t) to tame the ‘snakes in my hair’ as Olivia likes to say, genetics in subtle ways define us, and nudge us in particular directions.   And we seldom think about it much, as our cells divide millions upon millions of times, even as I sit here writing this. 

If you are lucky, you find that your genetics combine with your passion, such that you can become a world class runner, for example.  When they match up, it is easier to find success.  If you have a passion for running, but have a less than stellar genetic make-up, you might have to overcome your genetics with really hard work.   My passion of late, my obsession perhaps, has been helping my children beat cancer.  I would be the latter example, that while this may be my passion, I certainly need to overcome my genetics.

Research indicates that children from two parent families fare much better, in a host of areas.  This is not surprising, given that when one parent gets tired, there is a back up to step in and keep the youths from acting up.  Once in a while, mom stays out late at book club, but Dad makes dinner, handles carpool and the kids still brush teeth.  If Dad works late or wants to golf all day on Sunday, mom manages to pack lunches, read bedtime stories, and set out clothing for Monday morning.  It works better this way.

Not that there aren’t successful single parents, but the odds certainly favor any children from two parent households.  This maxim continues to hold when you carry this analogy to genetics.

Genetically, we are half of each of our parents, who share the responsibility for what we become…blue eyes, brown hair, height…we all understand this basic part of shared genetics in some way.  But there are other more complicated jobs involved,  and monitoring cell division to be sure that our genetic code is copied properly is among the most important jobs. 

If you consider that there are countless codes and arrangements of proteins along our DNA that must be correctly copied each time we make a new cell (I imagine a billion tiny monastic scribes, scribbling away along the double helix), somewhere along the line, someone must supervise, because the monks get old, tired or might just be hitting the sacramental wine.  We are human and prone to error, after all.  P53 is the ultimate quality control, or parental control, so that shoddy product doesn’t get out there and replicate.  There are supposed to be two inspections of the work, each putting their stamp of approval on it.

With Brent and Lauren, genetically speaking, I have been a pretty horrible parent.  I never supervise their work, because I provided a non-functioning P53 gene, utterly ineffective, completely indulgent of their misbehavior and “creative license” in copying code.   I never correct them, or keep them in check.  I enforce no rules.  As far as I am concerned, they can run wild in the neighborhood, play music really loud, flunk out of school, cover themselves with tattoos and generally become less than model ‘citizen cells.’   And then reproduce.  Umm, yes, that is cancer.   

Dan, on the other hand, has been the genetic watchdog, the good P53 parent, reading over homework, correcting their spelling, making sure that they tow the line.  Only three times has his genetic supervision slipped up: once, in Brent’s 13 years, and twice in Lauren’s 10 years, despite billions upon billions of cell divisions. 

And I, for all of that time, have been gutter-drunk at P53, for more than a decade.  I failed to step up, and genetically ‘parent’ at these critical times.  Other moms do, which is why childhood cancer is so rare.  It may be irrational, but there is a guilt that comes with this understanding, and if not guilt, exactly, then certainly a sense of responsibility. 

Now, before you chastise me and point out that I didn’t choose this for them, I am well aware, in my head, that this was well beyond my control.  But, as I like to say, what you know and what you feel are not always in agreement.  And it would be dishonest to suggest that this isn’t something that I haven’t struggled with on some level at various times, this disconnect between head and heart.

And probably, subconsciously, I try to compensate for that.  Perhaps it is better said, I overcompensate.  If I failed to be vigilant from the inside, at the genetic level, I am hyper vigilant in their battle against cancer on the outside, attuned to potential signs of problems, checking over the labs and scans with the doctors.  I am pretty hands on, from the outside. 

Dan said the other day that I write sometimes like I am a single parent.  This put me into a funk, one that I couldn’t seem to shake, because he is integral to our story as a family, and I consider him indispensable.  I cannot imagine doing any of this without him.  It occurred to me that my rather emotional response was likely due to the irony that he has long been the single parent at the genetic level.  I am aware that there is no logic in this.  Because so much about this doesn’t make sense, I am not going to fight it, but simply acknowledge it.

It is all so complicated, but at the same time, quite simple.  We want the best for our children and we want to be our best for them.  Sometimes we have to overcome our genetics, sometimes other circumstances.  But we keep working at it.

That part is universal.

A week full of thoughts of surgery and support

We find ourselves quite unexpectedly at home.  Brent's first surgery went so surprisingly well that they decided that he might spend the next couple of weeks in Ohio, rather than the Big Apple.  There is an abundance of blessings to be found in this statement.  

I am responsible for giving him IV antibiotic three times a day (with a 2 hour drip for each dose) until we return for his next surgery.  I am praying that this antibiotic regimen clears the infection.  None of his cultures grew anything, despite the graft being obviously infected, so we are hoping that this measure of going broad in spectrum, as well as deep in coverage (6 weeks) will wipe it out.  Whatever 'it' is, which is unknowable at this point.

I finally sorted out things with MSKCC, and our plan it to return to NYC in a bit over 2 weeks for the definitive surgery.  It is unclear at this point, exactly what form that will take, but the surprises have been good for the past month.  We are praying that they continue to be good.

As I waited for the appropriate time to hook Brent up to his IV (or unhook him...I forget which) on Tuesday night, I checked in with my LFS friends online.  The Internet is a blessing, especially for support in something like Li-Fraumeni.  With an estimated total of 400 people in the US with this genetic disorder, I would not have likely had the opportunity to meet anyone like us, with the same challenges and similar worries.  We would be essentially alone.

I remember when we initially learned about LFS, back when Lauren was first sick, some 8 years ago now, I had read about families with LFS in medical journals, but those were written for clinical use. There are no names, of course, and details about the patients are limited to age, sex, diagnosis and outcome.  The stories of these families are devastating from a medical perspective.  It was a very tough read, with little encouragement to be found, especially with the worry that we might be just such a family.  There was no sense of warmth, of hope, of connection.  It was written for a researcher, not a patient... or a parent.

When we discovered nearly two years ago that we in fact had the p53 mutation, initially, I went back to reading articles in medical journals, when I had the time. Fortunately, (??! ...that doesn't seem quite right!) I was pretty busy just helping Brent through chemo and then surgery...and follow up chemo, dealing with Lauren's brain tumor (which by comparison, seems like an acute issue)  and generally trying to keep the wheels from falling off.  I didn't have much time to seek further information or support...or offer it.

The omnipresence of Facebook and social media has been something that I have been very slow to embrace.  I am not sure why some folks find it necessary to share photos of their lunch...when it is utterly mundane fare from Taco Bell. But, it is not all bad.  In the last 6 months I have stumbled into a private support group of similar "mutants" as we refer to ourselves.  And consequently, this rather Amish girl has become grateful to Mark Zuckerburg, for indirectly making possible these connections with fellow LFS patients and, by extension, this support.  

Talk among my LFS friends on this particular evening centered on the various reconstruction techniques and options after a mastectomy, with much advice being offered.  I must confess that the technical talk is all a bit beyond me, having zero experience with breast cancer, and in all hope, I should like to keep it that way.  But several things struck me.  First, should I ever find myself in Alabama, for example, I absolutely know who I would stop and have a drink with, knowing that all of my cancer and genetic jokes would be appreciated.  These ladies have a wicked sense of humor.

Second, if I found myself in California, I know who I would rely on for advice on hospitals or doctors. This group has a wealth of information and medical experience (so wish that were not the case). But, they are willing to share their personal trials in very generous ways, in order to help others find the solution that is right for them.  

 

Reconstruction, for example, while it might seem like an obvious choice if available, might not be right for some.  Or the timing might not work, given recovery requirements.  How it is achieved technically, if chosen, is varied as well...and makes a big difference.  These considerations can be sorted through, with folks who have a practical understanding of what it all means.  That sort of advice is invaluable.

With LFS, we have a host of considerations that others do not, and likely cannot fathom...in medical treatment, in family dynamics, in finances, insurance, life perspective...the ways that cancer invades your life is not so different from the way it can take over your body.  I really try to keep it in check (umm...as I blather on and on about it here).  I clearly have varying degrees of success with this. These ladies have similar understanding of this effort at balance, and I am grateful to them for their openness and honesty about all of the consequences.

Besides, in having them scattered across the world means that at 3 am, should I find myself awake and worried about something, someone is likely to be awake somewhere.  And support, understanding and reassurance is easier to reach for, when you know that you are not disturbing someone's sleep.  I have been glad on more than one occasion, that Hawaii has a 5 hour time difference.

I woke up Wednesday morning to hang Brent's antibiotic, thinking about a dear friend who was to have surgery that day.  I was hoping that she had clean margins and was praying for clear nodes. This was for her second episode of breast cancer, not relapse, but rather a different kind of breast cancer than her original, nearly 10 years ago.  Double primary...it makes me want to swear. Kind of a lot.  And she does not have LFS, incidentally, not that it even matters.

My own surgery had been scheduled for Thursday (as if I could afford the time for such recovery) I cancelled it when we thought that we would be in NYC for the summer.  My ovaries will keep for a bit longer, and my geneticist currently has some other tissue to work with for a while. I hope to keep her busy this fall with more samples, and come up with some answers for what I should be doing. 

But presently, I am just very happy to be back home.  I am glad to be with my family, and to step away from the hospital scene, as much as is possible, with a fridge stocked with bags of Vancomycin rather than bottles of summer Corona.  I am trying to have normal conversations, about other peoples vacations, or children's activities, remodeling projects, because our take on these subjects is a bit different:  We vacation at Ronald McDonald House, my daughter is at oncology camp this week, and our remodeling project this summer is in my son's hip. 

I find that I make OR reservations more often than dinner reservations these days.

We will get closer to normal, but just not this summer, apparently.

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.

.

.

.

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.

In the Begining...

So, Dan has been saying for a couple of weeks now that I need to give sort of a background of our story…a thumbnail of how we got here, because some on this blog are not on Caringbridge.  In truth, I have been dodging this task (but in fairness, it is not like I have been eating bonbons and watching reality TV or anything so frivolous)

So, how did we get here, in 10,000 words or less?  Uggh….Bonbons and reality TV is sounding pretty good about now. 
 



Our cancer story began in late 2003, once upon a time, when Dan and I were younger, much less medically experienced and simply enjoying our family.  We were busy, I thought, with three small children, but a ‘good busy’ and we had a blessed life. 

That whole frame of reference shifted, just changing Lauren’s diaper a bit before Christmas.  I noticed that she had begun to get some hair…down there.  I had a 15 month well visit on the calendar for her and so I planned to mention it to our pediatrician when we went in. 

We didn’t see her regular doctor that day at the practice (it was nearing  on Christmas), but the substitute pediatrician blamed it on hormones passed  to Lauren from my birth control pills, because I was still nursing.  Lauren was my last baby, and Dan still brought her in to me first thing each morning.  Feeling guilty and responsible for harming her, I never nursed her again.

I did, however, call my OB, to let him know (after all, he had prescribed the pills knowing that I hadn’t completely weaned her yet).  He asked me to bring Lauren in to see him that same day.  After examining her, he encouraged me to see a pediatric endocrinologist by the first of the year and to get some answers (What?  Getting into a specialist might take months, even without the holidays) He was emphatic that my nursing her had nothing to do with this, and strongly suspected some endocrine issue, maybe with the pituitary gland. He warned that Lauren might continue to develop, even start menstruating (What??!!! She is in diapers!!)   He told me not to worry, but to get some answers.  He also offered to help me get the consult, if I had any problems getting in.  First of the year…

This whisper of urgency was a message from God.

The specialist we saw initially examined her, but just wanted us to come back in 6 months.  He wasn’t even going to draw any labs.  My OB’s words stayed with me, and I pushed.  Finally, the endocrinologist agreed to draw blood, but assured us that he had seen this before, not to worry, and that we would see him in the summer. 

He called a few days later, with results that he did not expect, but that he failed to explain, despite our many questions.  He ordered another test, again, with assurances that knew what it would likely be…although he never explained what that precisely was. We repeated this pattern several times, for several different tests.

Finally, by early March, I took Lauren in for the latest test, an abdominal ultrasound.  Dan and I had decided that after this test, we wanted a new doctor, because this man, while very smart, was not a good fit for us.  We needed to understand what he was looking for, and what he was ruling out. 

We decided that Dan would take half a day off of work, to be with the boys and I would run in to Rainbow for the ultrasound, which was non-invasive and easy.   In the darkened room, I chatted with the tech, asking questions about what we were looking at, because when I was pregnant, I could never tell the difference between a foot and a face, much less determine who the child looked like.

“  … so, that is her adrenal gland?  Interesting…”  On to the other side.

“Is that her other adrenal gland?”

No.  You apparently are not supposed to be able to see it. This information was starting to take root in my mind.  But the tech then said that we needed a full bladder to look at her uterus or her ovaries, I forget which.  Could I maybe get her to drink some juice and fill her bladder over the next half an hour and she would be back? 

Alone in the room, we looked at the colorful mobile that hung above the examining table, read a book to Lauren, plying her with juice (I doubted that this would really work…in my experience, a full, uncomfortable bladder for a baby means an empty bladder and a full diaper).  I considered the two adrenals, the discrepancy in size and thought about how Dan would laugh at me for the fleeting thought that it was a tumor.  How ridiculous, because she was a baby.   We would certainly be laughing at this mama by dinnertime for being so silly.

But half an hour later, when the tech came back, she was not alone.  There was a full posse of medical backup…oncologist, social worker, radiologist…I forget who all else, but from my current perspective, I suspect the rest were underlings in tow (until we were well into this journey, I did not understand that there were levels of doctors, and doctors in training…all white coats are not created alike.)

There was a mass of people in the room. 

There was also a mass, in my daughter. 

 

They were admitting her, because one type of adrenal tumor causes dangerously high blood pressure and she could stroke out.  Did I need to make some calls?  The social worker would mind Lauren, and guide me over to admitting.  We would then be going to the oncology floor. She was going to be sedated for a CT scan.  I was informed of these things, told of this plan, not asked about it.  I think that this was probably a very good thing, having it all decided for me, because my brain had entirely stopped functioning.

This was the moment that I realized that I would never be going back to Kansas.

The social worker went with me and Lauren to a bank of pay phones, where I called Dan ‘…come meet me in pediatric oncology.  Yes, that means cancer.  Get someone to stay with the boys…my sister, or a friend in our neighborhood…but you need to come.  Now.’   It became a blurry nightmare…

Horrible call.  Horrible day.  To this day, I cannot walk past that bank of payphones without being in that moment, stomach lurching.  And they remain there for me to walk past, nearly every time I am in the hospital.  It never gets better.  Those pay phones take me there, every single time.

As it turned out, Lauren did not have the blood pressure issue with her tumor.  She had another, rarer, kind of mass (3-4 cases in 10 million as I understand it).  We were discharged after a sleepless night on RB2, one that I have next to no memory of, except that I left with a pounding headache, likely from not eating, not sleeping, and crying an awful lot.

While we waited a week for surgery to remove the tumor and entire gland, I thought about how fortunate we were that this happened at her age.  Because, had she been a bit older, she would have been potty trained, and I would not have noticed her symptoms so easily.  Her ravenous appetite, I chalked up to an impending growth spurt.  Many children get vaguely moody.  The one thing that really got my attention was the pubic hair.  And had I not seen it, especially when my OB would have reason to weigh in on it, well, we might have been in a whole ‘nuther boat.

A week later, when we came in for surgery, I remember changing her diaper and looking at her belly.  She looked so pink, so happy, so perfect.  I could not imagine handing her over to the surgeons, for them to cut her open, because she appeared so healthy.  It was hard to remember that she was sick, and it was very serious because she was a happy child, in no pain.  I really wished that a bikini wax would have fixed it. 

The surgery took several hours.  It felt longer, because as I now understand these things, it always does.  But Lauren did well.  Within days, we ended up chasing her around the unit with an IV pole on Rainbow 2, wheeling her around in a wagon.  She played in the playroom incessantly, partial to a kitchen set that they had there.  We were especially encouraged by her bounce, probably because we were absolutely spent with worry.    

Clean margins, completely resected.  When the pathology came in mixed, they explained that there were several factors that were encouraging…but the most concerning, was that it was pleomorphic, meaning under the microscope, there many different looking cells, which suggested an aggressive tumor. 

For this rare type of cancer, Adrenal Cortical Cancer (ACC), there was no effective chemotherapy available.  We could only wait and watch.  They would scan her, and do blood tests, measuring the testosterone level.  If the cancer came back, wherever it raised its ugly head, it would produce testosterone, which was what caused the pubic hair to grow, and the appetite.  This was something that we could detect at very low levels in her blood, likely before it produced any symptoms.  This is how we would know.  And then we would go looking for it.

When we came home, Dan turned to me, and said that he had to leave for a bit.  I was surprised, given that we had just returned home from a weeklong stint through the bowels of an emotional hell, the likes of which I could not have imagined.  I was personally ready for a nap. 

When he returned, his mission was clear.  He had been to Toys R Us and picked up a kitchen set for Lauren.  He noticed how much she enjoyed the one in the hospital, and really wanted her to have that same enjoyment at home.  It was a celebration of her recovery, and reward to her for being such a trooper.  This is just how my husband is.  I love him for it, and weep at the thought of how much he cares and looks out for our children.

When we came in to RBC for our first outpatient visit after surgery, the oncologist had a long talk with us.  She let us know that given Lauren’s rare tumor and young age, we should consider genetic testing.  The overwhelming odds, given the ACC, favored her having a genetic predisposition to cancer which was even more rare than the very rare tumor.  It was called Li-Fraumeni Syndrome.  It is a failure of the tumor suppressor gene p53, so she would more likely develop other cancers, different cancers.  All cancers.

 And if she had it, we should consider testing the boys.  And ourselves.   There was a 50% chance that we were also carriers.  Would we mind sending her tumor to St. Jude’s for testing?  ‘What were we going to do with it?  Put it in a jar on the mantle?  Of course, send it, by all means.’   

This, incidentally, might have been the very beginnings of my stress driven snark.

So, that day, we went from being cautiously pleased with Lauren’s surgical recovery and the mostly positive news out of pathology, feeling that we could beat this, to learning that we were likely only beginning our troubles, which could be vast. 

The genetic testing takes time, and for a month, I could only look at my children and instead of entertaining typical maternal thoughts, imagining the people that they would become (What college would they go to? What will their spouse be like?  Would they have children of their own?  What career might they take up?) I worried about a life in hospitals and the cancers that the three of them might develop.  It was a very long time in limbo. 

Internet research, for the record, is not a good pastime at such a point in your journey.  LFS is no picnic, I rapidly discovered.  It runs in families, and is devastating.  There is no cure, and only recently was there a way to test to see which family members would be affected by carrying a mutation in their p53 gene.  I cannot imagine how, in the absence of science, families processed this, making sense of the frequent and returning cancers in their family, of every variety.  They must have felt cursed by God.  They might still.

When we returned to RBC a month later, the genetic results still were not back.  The oncologist, knowing our anxiety, kindly called down to St Jude’s and got results faxed over.  She excitedly told us that we were somehow on the skinny side of the odds…Lauren did not have the mutation.  This was a fluke…  Bad luck…A lightening strike…  Pretend that this never happened.  Carry on.  Congratulations!

Jubilation and disbelief doesn’t begin to describe how we felt.   In retrospect, we probably should have stuck with disbelief, but who doesn’t grab on to good news when it is offered?  No one gets a second opinion on good news.

And as we progressed, drawing labs every month, with reassuringly low testosterone numbers and scanning her every three months with clean CT scans, the likelihood of the adrenal cancer returning decreased.  My anxiety on scan day never did, but statistically speaking, if the cancer were to return and spread, it would have likely done so within the first year.  We scanned her for 18 months and followed her labs, mostly for my own peace of mind, for several years at increasing intervals.  We were moving on.

But despite this generally positive trend, I frequently wrestled during this time with odd things that happened with Lauren.  Changes in her appetite had a sinister feel to them, a potential symptom of the cancer’s return. (And what child doesn’t have these sorts of changes as they grow?)   I tried to tamp down my worries and reminded myself that we had it beat.  And it was only a lightning strike...  A fluke...  Bad luck...

I am ashamed to say that more than once, I panicked as I changed her diaper…spotting a black hair or two on her nether parts.  The sick feeling, as my stomach dropped and my mind raced to very ugly places, fearing that the cancer was back…well, it fortunately disappeared upon closer inspection. 

I cursed our black dog Max, and my apparent lax housekeeping skills.  It was only stray dog hair down there, hair that easily wiped away.  Yes, this happened more than once, but my reaction was always the same.  I can laugh about it now, but in that moment, you know deep in your soul, that it can all change just that quickly.  And while the hair is easily wiped away, the fear is not.

So, I vowed to vacuum more diligently, to enjoy my blessings, and to try, with much effort, to relax.  I do recognize the paradox in that statement, but it was an effort…one that was required in order to make a new habit. To relax.  I was determined to not allow this episode to change our happiness.

Back in the day, we didn’t have cell phones and voicemail.  We waited for important calls, chained to our home, paralyzed with the wait.   When there were lab results, or scan results, this didn’t help me in my vow to enjoy my blessings, or offer any assistance with my effort  to relax about the results.

 And while my time on the phone with doctors and nurses naturally had to be a priority, Lauren was oblivious of this because of her young age.  The boys, and Alex in particular, were not oblivious.

Alex had questions, as all children do.  Especially on occasions when I was talking on the phone to various medical folk, and I would scold him and Brent impatiently, for squabbling over a toy, interrupting and distracting me from a rather significant conversation.  (They were 4 and 6, so, it is not surprising that they would occasionally need outside intervention, a peacekeeper, or negotiator)  But, I felt that I had to explain to them what was happening in our family and why they had to be especially good while I was talking on the phone to doctors.

Many people would try to protect their children from the hard reality of this all.  If I could, I probably would have tried.  But, for me, it seemed that Alex’s little world would make most sense if he understood what was going on.   He would understand me, and trust me.  It was a philosophical position, one of honesty, despite his young age.  I have never backed down from that decision.  And it has not always been easy.

After I explained that Lauren was sick, and that I needed to talk to her doctors, who were trying very hard to fix her up, Alex asked me if she was going to be all right.  He asked me, point blank, if she would die.

It was quite the loaded question out of a six year old. 

I was honest.  I told him that I didn’t know.  And it was perhaps the hardest sentence that I have ever been required to spit out.  It broke my heart saying it.  Almost in tears, I nearly choked on the words, and the awful reality of what those words represented.  Speaking the truth to him, I could no longer deny the possibility in my own mind. 

Not to spoil the ending or anything, but I have had to say these words more than once to Alex.  And it does not get any easier; you only think less about cushioning the truth in some fluff.  Because, God forbid, if it didn’t work out, which I could not control, I could not have that absolute travesty compounded by Alex’s mistrust of me and my words.  I knew that if I lost Lauren, I would also lose Alex, unless I was very clear and very honest in that particular moment. 

This was instantly distilled in my mind on that day, sitting on the steps of our little home, looking into his questioning eyes.   

I have never looked back.  On that point, at least.