Tag Archives: Alk mutations

Eleven beautiful and breathtaking years

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And counting. I’ve been so busy LIVING that I have neglected to note that we just zipped on past the eleven year anniversary of my diagnosis with lung cancer. That’s right–ELEVEN BEAUTIFUL AND BREATHTAKING YEARS that I didn’t think I was going to have.

Ever mindful of what a miracle waking up is, I continue to marvel at the fact that I AM ALIVE. Today, this day–and maybe tomorrow too. And you know what? It never gets old. The good, the bad and the ugly alike–it is a privilege to be here and something I shall never take for granted (and if you are reading this, neither should you).

Several days into year twelve (!), I vow to honor the memory of those who had to leave this party far too soon. Your departures hurt and anger–but death cannot tear apart the connections we made in life.

My personal goal going forward? To shift my focus from surviving to thriving. If you run the numbers, I’ve now known I had lung cancer for almost 1/5 of my lifetime–much of that lived on hold.

No longer. It’s full throttle from here on out as I plan to see and experience all that I can possibly cram into this life I call mine. To adventure!

Just doing it

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I had my six week scan review yesterday—twenty months of stability and counting. At these appointments Dr. Shaw always gives my lungs a listen and this time she kept her stethoscope pressed to my back a little longer than usual and I wondered what it was that she heard. Nothing, as it turns out. I’ve had a little wheeze in the bottom of my upper left lobe all along, but for the first time, she couldn’t find it. ‘Maybe I’m cured’ I said. ‘Maybe you are’ she replied. Or maybe that’s just what I imagined.

What she did say was that she was relieved as she’d been feeling a little anxiety as to what might transpire after my being off drug for a week. Me too. It might be coincidence but my progression on crizotinib followed a break from therapy when I had surgery on a shattered left ankle. And resistance to zykadia came in the wake of my second bout of liver toxicity and subsequent pause in drug.

My labs three weeks ago indicated elevated amylase. My liver enzymes have been only mildly elevated on PF-06463922 so I have continued to enjoy a glass of wine most evenings. Now alcohol was no longer an option.

And why the break in drug? Two weeks ago I began to feel really crummy. A virus, probably strep throat, and then several days later a sinus infection as well. I was also really constipated, an unfortunate side effect of treatment. But then I started to get really uncomfortable with a burning pain in my abdomen and super nauseous–combined with my elevated amylase these symptoms were highly suggestive of pancreatitis. I was in touch with Dr. Shaw, who had prescribed an antibiotic for the ear infection. She felt it would be wise to test my amylase again and as I had an appointment with my ear nose throat doctor on Wednesday we agreed to do it then. In the meantime I would fast and drink only clear liquids so as to give my pancreas a rest. By late Tuesday I was feeling so very bad that I texted Alice and asked her if I should consider going to the emergency room.

Alice called me back and when I told her I felt this represented pancreatitis her response was ‘Linnea, I really hope you don’t have pancreatitis because if you do, it would be grounds for exclusion from almost all further clinical trials.’ This was news to me. Just the day before I had spoken to a friend who’d been excluded from a trial for pancreatitis but I was under the impression that hers was a singular experience pertinent only to that particular trial.

So I told Alice I was not going to get my amylase tested again–that I hadn’t come this far only to learn that I had no more treatment options because of an elevated lab result. She said we’d see how I felt the next day. The following morning I received a text from her  asking me how I felt. My guarded and completely disingenuous response was ‘Hi. Better.’ She then gently but firmly urged me to come in for labs. And I responded with this message:

Alice, I guess I haven’t been paying attention but until I spoke to Margaret I did not know it was grounds for exclusion and it was only after speaking to you that I understood that exclusion meant virtually all trials. I have a very strong commitment to surviving and treatment options are part of my hope for the future. Under any other circumstances I would get that lab work done but as it stands, it is absolutely not in my best interest in the long run. I’m sorry. I would like you to advise me as to how long I can safely hold food for though. Thanks.’

Alice Shaw is a fabulous doctor and I consider her a friend as well. I admire her in so many ways and one of them is how she can just roll with the punches. She heard what I had to say and yet stood her ground. She advised me that if I were feeling better and merely had an elevated lab result it would not be considered pancreatitis. I needed greater reassurance–‘Would that protect my future options?’ She repeated what she had already said, that a lab test alone should have no impact.

So I assented. I got the lab work done and it came back completely normal. Only then did I acknowledge that I still felt absolutely awful. A quick reassessment of symptoms and Alice surmised that this was likely gastritis and possibly a peptic ulcer, which I could address with laxatives and anti-reflux medication.

We also talked a little bit more about what could have happened if I’d actually had pancreatitis. I told her that I felt these sorts of exclusions were patently unfair, and she said that the rationale behind the exclusions was that certain individuals were more likely to experience serious side effects.

I don’t buy it. When I entered my first clinical trial in 2008, I was taking on enormous risk–the only other person in the trial had died almost immediately, in large part because of the toxicity of the experimental therapy. Knowing this did not in any way deter me because I understood that if I chose not to enroll in the trial (my only hope and a thin sliver at that), I would be dead within a couple of months anyway. It was a no brainer.

My explanation to Alice was this: If I experienced life threatening side effects I might be fucked but at least I’d be fucked with options. But that having no treatment options meant that I was totally fucked as I would experience the most devastating adverse event of them all—I would be dead. And that it is my viewpoint that patient safety might be a secondary concern to getting drugs to market faster and without hitches (like adverse events).

I trust Alice implicitly and know that she is highly invested in my personal outcome. However, I hold no illusions about what it means to move from patient to participant in a clinical trial–the loss of autonomy can be really, really frustrating.

In the end, nobody loves this life of mine as much as I do and self advocacy is key to survival. Woody Allen once said that 80% of success is showing up and when it comes to tomorrow, I will not be a no show.

Down the rabbit hole once again

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I was off drug for seven days–a subsequent post will detail why. On day seven my brother John called and at the end of our long conversation he said ‘your voice is back to normal–you’re talking fast again’. So I was. One prominent side effect of PF-06463922 for me is that I speak more slowly. It’s as if I just can’t quite drag the words out and for some reason (maybe all that effort) my voice comes out louder too. In addition, I often sound a bit incredulous, as sentences sort of trail up at the end. It’s something that not everyone notices–my neighbors in the lofts have only known the slow speaking version of Linnea. However it is quite obvious to me, my old friends and family.

That was really sort of fun, talking fast. Better still, that evening I realized that I felt different as well. Kind of like the old me–sharper, calmer, more organized in my thinking. Clearly the drug had washed out of my system.

The next morning I started dosing again. Within forty eight hours my speech slowed and that sense of internal calm evaporated (emotional lability is a potential side effect of this drug, and was a big problem for me when I was on the higher dose).

Yesterday was one of those days that just keeps poking you with a sharp stick. This whole college application thing has been incredibly complicated and Peter let me know that some forms I was supposed to submit for his financial aid package were missing. This ramped up my anxiety as I am no longer able to retrace my steps, if that makes sense. I feel sort of like someone crossing a canyon on one of those suspended foot bridges, and as I run, the bridge falls away behind me. In other words, no easy way to go back.

My daughter Jemesii was having a bad day too, as a psych eval that she had waited a month for (and gotten up very early to go to before work) was cancelled upon arrival because the doctor had called in sick. She would have to wait yet another month for a new appointment. Insult to injury, the receptionist was rude and insensitive–someone who should not be working with a vulnerable population. This stressed me out some more.

I had an appointment at noon for a new general practitioner–someone who works specifically with oncology patients. I was super excited but didn’t know how to get to MGH West and just assumed Siri (on my iPhone) could help me find the way. Well, she couldn’t parse the difference between 40 2nd Ave. and 42 Ave, which apparently doesn’t exist. And Waltham just didn’t compute for her. I turned my car on to warm it up and noticed three lights were flashing but I decided to get on the road anyway. As I pulled out I placed a call to the doctor’s office for directions but the switchboard ended up putting me through to billing where I was on hold for almost ten minutes. That individual wasn’t able to help me but said they would connect me to someone who could–but all I got was ‘We are unable to answer the phone but you can leave a message at the tone’. Damn. I tried Siri one more time, with a little less specificity. This time she understood, I got my exit, and was almost on time.

After the appointment I looked through my vehicle’s manual to see what those flashing lights meant—next to the icon that looked like an engine it said ‘take to Toyota dealer immediately’. Well fuck.

So this morning I drop my car off at the mechanic. I had scans in Chelsea so the plan was to take the train to Boston, walk to MGH and catch a shuttle to Chelsea. As I walked the half mile to the train station in Lowell our property manager called and told me that I had mistakenly sent my alimony and child support checks rather than those for rent. Oh boy. After I boarded the train I reached for my wallet and it wasn’t there.

Sometimes a girl just has to say uncle.

So home I went. Made myself a cup of coffee to calm down/warm up again. Called Chelsea to cancel my scans and sent my scheduler an email. This is one of those moments where my own challenges feel, well, just a little too challenging. That’s code for ‘and then I feel sorry for myself’. However, it never takes me long to gain perspective. Life is hard for most, and almost impossibly difficult for some. This drug that muddles my brain is also keeping me alive. And I can’t argue with that.

Miss March

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Don’t let the title fool you—mine was merely a supporting role.

The MGH Fund puts together an annual calendar, and I was asked to pose with a slide of my tumor (!) and March’s featured star, Dr. John Iafrate. John is a pathologist at MGH and also someone who knows me rather intimately–that is, on a cellular level. Those of you who are ALK+ are probably familiar with the Break Apart Fish Probe Kit–used to identify ALK+ cancers–well, that was developed in Dr. Iafrate’s lab. My continuing survival has been based on so much luck (a lot of it of the right place/right time variety). Had Dr. Iafrate not developed that diagnostic test when he did, well, I really would be history. As it was, my ALK+ status turned three to five months left to live into eleven and counting, come April (it was ten when we took this photo last year).

Pathologists are sort of the unsung heroes when it comes to the treatment of cancer despite the crucial role they play. When I was initially diagnosed with NSCLC in 2005, I wrote a thank you note to the pathologist. My general practitioner couldn’t see the forest for the trees, and had attributed more than two years of symptoms highly suggestive of lung cancer (cough, shortness of breath, clubbing of fingers, and even hemoptysis) to adult onset asthma. Even though my diagnosis was devastating I was also relieved to finally have a plausible explanation for my symptoms and that gratitude was directed toward the pathologist.

So anyway, here’s to Mr. March and pathologists everywhere. You know us better than we do, and we couldn’t make this journey without your guidance.

 

Linnea live (I like the sound of that)

This video was recorded at the annual LUNGevity Hope Summit in 2014 and fits in perfectly with the theme of clinical trials. When I refer to starting a new trial, it is for PF-06463922 or lorlatanib (which has kept my cancer stable for 18 months now–woohoo!). I’m a little breathless and hoarse as my cancer was advancing again. I noticed immediately how fast I am speaking– markedly slowed speech has been a side effect of PF-06463922.

Slow, fast, hoarse or not, the most important message here is one of hope (thank you LUNGevity). When I mention going from cure to living longer I am talking about accepting the fact that I would never be cured. That’s a difficult concept to embrace but in order to make it even remotely acceptable I found I needed to replace cure with a potentially obtainable goal—becoming an outlier. At ten plus years (eleven, in April) I am there.

Secondly, the importance of options. When diagnosed in 2005, the first hurdle I hoped to jump was qualifying for surgery. I had nineteen lymph nodes and most of my left lung removed followed by four rounds of adjuvant chemo and yet my cancer returned almost immediately. Two strikes, and I’d been informed that treating lung cancer was basically three strikes and you’re out. My first clinical trial in 2008 was a long shot. I was thrilled beyond belief when I responded to crizotinib, but also understood that it represented a temporary fix and that there was nothing else out there once it stopped working.

Thankfully, that’s no longer true. Sadly, there aren’t viable options for everyone with lung cancer. Medical research got me to where I am today (alive!) but we can’t stop now.

Taking the leap into clinical trials

I was initially diagnosed with non small cell lung cancer in April of 2005. My tumor was large (5 centimeters); a poor prognosticator. However, it had not spread beyond my lungs and I was staged at IB. One week after diagnosis I had the lower lobe of my left lung removed. As I was recovering from surgery my new oncologist introduced himself; Dr. Tom Lynch. I had no idea back then, but I was incredibly fortunate to have Tom select me as a patient. The reason behind his coming to me rather than the other way around? As a young (age 45) non smoking woman, I fit the profile of someone who might be EGFR+ and Tom was an early innovator in the investigation and treatment of EGFR+ patients.

I tested negative for an EGFR mutation but in the process acquired arguably one of the best oncologists in the world; someone who was always forward thinking and cutting edge in his approach to treatment. In fact, I was offered a chance to enroll in my first clinical trial soon after surgery. Because of the size of my tumor, adjuvant chemo was indicated and a trial that would include the addition of avastin was proposed. I was having a difficult time even being convinced to have chemotherapy (Tom was adamant) and I couldn’t wrap my head around the possibility of also being a medical research subject. As it turned out I would not have been a good candidate anyway–I am a bit of a bleeder and I was coughing up blood for weeks post lobectomy (there is a small but significant increased risk of serious pulmonary hemorrhage secondary to avastin).

Fast forward to September of 2008, two months after I’d been restaged to IV and advised that I likely only had three to four months to live. At my scan review after two months of tarceva–tried as a last ditch effort even though I was EGFR-, there was nothing but bad news from the radiologist. However Tom shared that a sample of my biopsy had been submitted for further genetic screening and had come back positive for an EML4-ALK translocation (another example of how ahead of the pack he was—the ALK mutation had been identified as a driver in NSCLC only months prior to that).

As we discussed the significance of this finding we also reviewed my options going forward. The way Tom saw it, there were four possible scenarios. I could stay on tarceva, return to traditional chemotherapy, do nothing (that option only underscored how serious my situation was) or attempt to enroll in a phase I clinical trial that targeted ALK mutations such as the one that was driving my cancer.

Would you believe me if I said I never hesitated but instead leapt at the opportunity to be in a clinical trial? Why now but not back in 2005?

This is why.

This image of my scan says it all. The upper lobe of my left lung was now almost completely clouded with consolidated ground glass tumors which had spread to my right upper lobe. And I had been told that I might have three to four months to live two months ago. The math was easy—I had almost run out of time and out of the four options I’d been provided with, only one seemed to offer a glimmer of hope.

And glimmer is the operative word. There was no precedent for me back then—no reason to believe that this trial might actually prove effective. All Tom could offer me was the fact that I had been preceded by one other participant at MGH. ALK+ like me but so debilitated by disease that they were confined to a wheelchair. Their initial response had been extremely encouraging, to the point where the wheelchair was temporarily abandoned. But then they had died, in part due to the toxic effects of the trial drug on their liver.

So this is what I knew. One before me with a promising response who had succumbed both to disease and to the toxic effects of therapy. That the experimental therapy could in fact prove fatal to me as well. But that my cancer would certainly kill me if I did nothing. An easy choice, after all.

And so, on October 1 of 2008 I had my lead in dose of the drug that would eventually be known as Xalkori.

 

Love story

Day 28: for my final post devoted to Lung Cancer Awareness in the month of November I am going to talk about my superhero: Dr. Alice Shaw.

Alice and I met under what then felt like sad circumstances. It was the spring of 2009 and I was several months into my snatched from the brink of death fairy tale; aka crizotinib. As far as I was concerned (and I still feel this way), my original oncologist Dr. Tom Lynch walked on water. However, I woke up one morning only to read in the Boston Globe that Tom was leaving MGH to become the head of Yale’s Smilow Cancer Center. I was devastated and sent him a quick message saying I felt like he’d broken up with me via email. In my head I was already thinking I’d have to move closer to New Haven as I viewed my continuing survival to be inextricably linked to Tom Lynch–as an oncologist he was always on the cutting edge, having tested me for an EMLK4-ALK translocation in June of 2008, long before most of the world had even heard of an ALK mutation.

Tom replied quickly and with assurance; he had hand-picked my next oncologist and he was certain I would adore her.

I was at MGH for a long trial day (PF-02341066) when Alice introduced herself to me. We chatted for more than an hour as I had soooo many questions regarding my cancer (she was the lead investigator for PF-1066 as she was for ceritinib, the next agent I would go on trial for). She listened carefully, compassionately and answered with honesty but also great detail. The treasure chest that was my own personal medical information had finally been opened and I was smitten.

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Dr. Alice Shaw (thank you MGH The One Hundred)

So what makes Dr. Alice Shaw so special? I had some fun researching her online in order to write this post and it amused me when I’d run into something I’d written when googling Alice Shaw (I have been rather vocal in my adoration). She was honored as a caregiver at MGH’s The One Hundred celebration in 2012 and at that time I said this: “Alice is an uncommon blend of brilliance and compassion. My relationship with her has greatly advanced my understanding of lung cancer while validating my personal experience–when coping with a serious illness that validation is empowering.”

That’s sort of Alice in a nutshell. Harvard educated (B.A. biochemistry, MD, PHD) she is an associate professor of medicine at her alma mater in addition to being a clinical oncologist at Massachusetts General Hospital. She has been awarded numerous research grants and awards and on November 2nd was appointed as the inaugural incumbent of the Paula J. O’Keefe Endowed Chair in Thoracic Oncology. I was fortunate enough to be in the audience, as was Dr. Thomas Lynch, my original oncologist–he has returned to MGH in the position of Chairman and Chief Executive Officer of the Massachusetts General Physicians Organization. Dr. Jeff Engleman, no slouch himself in this crowd of crowds, gave the introductory speech and noted that Alice is simply the best at everything she does. But that she is also incredibly humble and down to earth and places the utmost importance on patient care–that she is fully invested in securing the best possible outcome for every single person she treats.

When Alice came to the podium that evening, she expressed her gratitude to all those who had supported her. Mentors, colleagues, fellows, research assistants, members of pharma, benefactors, family–including her Husband Stan and two lovely sons who were all in attendance. But she also thanked her patients.

Dr. Alice Shaw is a rising superstar in the field of thoracic cancers. I recently heard her husband Stan make the humorous comment that he knew her before she was famous. The beautiful thing is, the only way in which fame has changed Alice is she’s slightly less accessible due to demand. However, as I noted in my blog about my friend Christian, although he is no longer getting his care at MGH, she still calls to check up on him. Somehow, some way, she finds the time.

In 2012 I had this to say about my oncologist and I wouldn’t change a word today: “Alice is my super-hero. She is contributing to the future of cancer research and treatment. And she is doing her best to make sure I have a future as well.”

Love you Dr. Alice Shaw!

…..

I am the lowest common denominator when it comes to instructions/rules/general compliance etc… This post is intended to be part of a blog chain this month (along with my blogs about Christian and Diane) but I failed to list the blogs prior to and following. I shall this time, however!

Yesterday: By Craig Blower about Dave Bjork found at http://craigblower.wordpress.com

Tomorrow: By Dann Wonser about Genevieve Wonser found at http://www.dannwonser.com