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. 

California Dreaming

We have been very busy, but a good busy.  Actually, wonderful, magical, blessed time to be together as a family.  Lauren had her Make a Wish, which was to be in a Hollywood movie. She was willing to wait for it.  It was everything that she hoped that it would be, and then some.

Make a Wish is a terrific organization that tries to grant wishes to children with life threatening conditions, like a brain tumor the size of an egg or giant, throbbing osteogenic tumors.  We have been blessed and fortunate to have had two wishes granted in the past 5 months, although that is the ultimate "glass half full" statement.  The empty part of the glass is how we had two children with life threatening illness, simultaneously. I prefer to look at the blessings we have had, and this trip was definitely a blessing.

I must say that we packed a ton into the five days that we were in California: a day at Universal Studios and City Walk, a trip to Santa Monica Pier, a day of filming, a visit to the Hollywood walk of fame, an afternoon at Venice Beach, a drive up to the Griffith Observatory, and through the foothills of Hollywood for a photo op by the famous sign, an evening at the Getty Center which has stunning views from a mountain top...  It was all fantastic.

I think about the opportunity we had to be together, and for Lauren to have her dream of being in a movie, something that we couldn't have possibly done for her on our own.  I am so grateful to Southwest Airlines, those who donated airline miles and hotel points, the generosity of Disney and Universal Studios. We had everything that we could need, and it was all planned out for us.  It was an amazing gift to us all.



As I sat on the beach one afternoon, watching my children play in the surf, I enjoyed some quiet. Brent took his crutches into the water, delighted to be able to swim.  I watched the sun glinting off of the water, reducing the kids forms to silhouettes.  It was really beautiful.

I remember how my friend had shared with me a dream that she had very early in Brent's diagnosis.  She had dreamt of our children, all much older, playing together on the beach.  Monica is one of those people who have dreams that mean something, that seem somehow prophetic. In the very dark days of that fall, I couldn't fathom how her dream could be anything more than a fantasy.  Last week, I watched that dream unfold, right before my eyes on Venice Beach. 

The goose bumps I had could not simply be attributed to the cool ocean breeze, as this realization washed over me.

But as I watched my children splash in the Pacific, I thought about how far we had come.  Sure, we flew 2,000 miles to get here, but the road to this point was really much farther. It was physically grueling, emotionally exhausting, circuitous and perilous.  But, we had arrived together at the Pacific...meaning 'the peaceful' ocean.

Lauren was especially excited to be in the ocean.  She wave jumped, coming up and double pointing to the heavens as she stuck the landings.  Over and over, she took on the waves that came in, delighting in the challenge and celebrating in her own success.  She and Alex went pretty far out, and swam together.  I like how they look out for one another and enjoy each others company.

Olivia found a long stem rose in the surf, and planted it in the sand, reminding me of another dear friend who finds comfort in roses discovered in unexpected places.  Livvy began playing with the waves closer to shore, starting most sandpiper-like, being chased by them as all children do at that age.  Eventually, she too ended up wet, splashing around with Brent.  She isn't experienced about looking out to sea, and she got surprised by a wave, which completely knocked her over.  She came running in to have her face wiped and a sip of Gatorade to wash out the saltwater taste.  And then she was back in the ocean. 

They just pick themselves up, and get back to giggling.  Back to living.

So this morning I was waiting for yesterdays scan results, looking out to sea.  I was trying to be prepared, and not get completely knocked over by the waves.  At long last, the call came and the scans look generally good...only one lymph node to be followed up on in a month. I think we have stuck the landing and can double point to the heavens.

I know that the Pacific was named "peaceful" only relative to the stormy, tumultuous Atlantic.  There are still waves and sharks and other hazards out there.

But, today, I am most grateful for the relative peace that we enjoy as a family, and the good glow of mostly boring scan results.

 

Relapse

It has been a rough couple of weeks.  

The Ramers have been terrific.  Brent has perfect attendance at school.  We have been getting our lives back on track.  I might even get to see the inside of the kids school rather than the inside of their various hospitals.  It has been great.

We enjoyed a fantastic day on Sunday via Flashes of Hope, sponsored by the Browns in honor of pediatric cancer awareness month.  Dan and the kids got to go down on the field as the teams warmed up.  We watched the game with two other families from the corporate suite.  The Browns won.  The weather was perfect.  We parked under the stadium with the players.  Fabulous day.

But for me, there was something of a shadow lurking over this all, because in LFS land, it has been positively raining relapse.  A friend who messaged me frequently about her osteo relapse options, went into hospice.  Others have been struggling with their second and third cancers.  And most devastatingly, a dear mutant friend announced that they found mets in her lungs and bones.  Perhaps sarcoma, but likely breast cancer.  There has been some swearing in my head.  I have vile things to say about cancer.

And, amongst all of this, Lauren has been getting headaches. They started out fairly sporadic.  They happened only at school.  But there has been a definite uptick in the frequency and the intensity of them. Dan and I were not thrilled about this.  We were nearing on scans, and were thinking between us that the brain MRI in two weeks couldn't come soon enough.  

As Lauren had more headaches over the weekend, I made plans to email her oncologist.  But first thing Monday morning, my phone rang.  The local Make a Wish chapter had a possible opportunity for Lauren, which felt both exciting and ominous.  While I had hoped to move her MRI up for my own peace of mind, now I really needed to, for this wish opportunity.  I emailed her team, advising them of her new and concerning symptom.   They forwarded it on to neuro oncology. 

When she came home after school on Monday in tears over a headache, I put her to bed, and called oncology. Phone calls back and forth...and by the end of the day, she was scheduled for an MRI first thing Tuesday morning. 

So, I checked Lauren several times overnight, but she slept soundly.  Lets just say, I did not.  Tossing and turning.  At 2am, I woke to discover a recent email from my geneticist.  My friend had also emailed.  I had too many thoughts swirling in my head. I couldn't sleep, but I couldn't do much else either.

We battled morning rush hour traffic to a satellite of University Hospitals. Lauren was great.  I was just simply jumping thought the next hoop, grimly, because we had to know what we were dealing with. We checked in at radiology and began to wait.  

Make a Wish called while we sat there to confirm the dates for her wish.  I knew that we would do her wish regardless of the scan results, and am grateful for the opportunity.  But it would feel very different, if we were traveling with the carry on baggage of another brain tumor, and a return trip to oncology on the itinerary.  

Lauren changed into a gown, and I was allowed with her in the back, to watch her scan.  The tech stopped the MRI to stick her and give her IV contrast, then put her back in the tube. He was a one man show in this small outfit. I resumed my waiting, trying not to think ugly thoughts.  Make a Wish called again to confirm more details.  I wanted to throw up, completely distracted by the fact that they were making images of my daughters brain, and how that information would determine the course of our lives.

I shared the details of Lauren's wish with the tech, in an effort to distract myself from awful thoughts.  I imagined how excited she will be to hear this news, having been patient so long.  He wished her well, and finally went in to pull Lauren out of the tube.  She quickly changed back into her clothes and we left.  

Before we could even get on to the highway, my phone rang, a number I recognized from the hospital. Dr. Sterns, the neuro oncologist said that he was looking over the scans, and that they appeared unchanged from her last ones.  I think that he must have been looking at them live from UH Main Campus during her scan.  I am so grateful for technology.  I am grateful for the clean scan.  I am grateful for the phone call.

I could breathe.

Lauren texted Dan to inform him of the good news while I was driving: "Hi daddy, its Lauren.  Just wanted to tell you that there is nothing wrong with my totally awesome cancer-free brain." We had breakfast together, at what she now calls our 'good news restaurant,'  and I took her home to get some rest.  I headed out to the main hospital for my own appointments and errands, now very happy with my day.

The very first person I lay eyes on upon arriving at UH was Lauren's neurosurgeon.  I swear, if I didn't already know her MRI was clean, I would have thrown up all over him.  Instead, I giggle to myself and text Dan about it.  These things feel so different without the uncertainty. 

I know, in my head, that cancer isn't contagious.  But the series of sad and troubling announcements from my friends had the universal effect of sending everyone in LFS world scurrying to "get their shit checked out," forgive my language.  Because, while we might laugh inappropriately at cancer, at oncology, and the horrible things we are required to do, we all have a very healthy respect for LFS (Ha! Isn't that an ironic turn of phrase?) and the potential for cancer. Neither LFS, nor cancer, is anything to mess with.

The relapse storm just serves as a reminder.  An alarm bell to anyone who might be tempted into complacency, which we all are. Everyone is busy with life, and everyone with LFS wearies of time in hospital. Furthermore, even for us, there are normal things, not just cancer.  It is just hard to know what to worry about.  

A great deal of the research to date with LFS centers on the psychological implications of knowing your status, what I refer to with much irritation as the "how do you feel, know that you know?" research.  It is a balance, having due diligence, and living.  With LFS, we start with 'rule out brain cancer' and work our way back to  'adolescent onset migraines.'  We have to, given our genetic circumstances. 

And we high five migraines in this house.

So at the end of my long day at the hospital, I returned home, and Dan and I gathered together the kids to talk as a family.  Our talk is different than other talks that we have had.  The talk that I worried about having.  A talk that my friend was having with her four beautiful children.  We celebrate migraines in this house, as cancer averted.  And we get back to the business of living, and embracing the blessings that each day offers us.

And we shared that Lauren has been granted her wish. Hollywood is going to Hollywood.  She and her totally awesome cancer free brain are going to be in a movie.

We leave in a week.