Every day, questions and realizations that frighten and strengthen me enter and exit my mind like a busy train station. As I write this, our house is filled with the sound of two little voices, singing, playing, and imagining together. I can’t help but helplessly wonder how long we will have that complete joy. As much as we live by the tenet to focus on today, it’s hard not to think about tomorrow.
March 5 is day #266. It’s also the day Piper goes back to Children’s Hospital for her fourth MRI. It has been 90 days since her last scan. The process will be familiar, but no easier. We will hold her hand as they put her under. We will kiss her baby cheeks before crossing the long hallway to the waiting room. We will sit breathlessly for an hour. When they call us back to her recovery room, her peaceful face will jolt awake, followed by 30 minutes of disoriented crying. When she calms down it will be time to take the elevator to the 7th floor – a sort of second-home for children with cancer and blood disorders. But that is where my assurances end. While this scan will be performed like every other, its results are far less predictable. Statistics tell us this is the part of Piper’s story where we begin to talk about the end. Statistics are what DIPG families are given when we should be given facts, treatments, answers, and hope. Statistics be damned.
At nearly nine months post-diagnosis, Piper has had some of her best days yet. She plays with her cousins, she pushes the kiddie cart at the grocery store, she twirls in the living room, and she belly laughs daily. Her body is still weaker on her right side, and her left eye skews her depth perception and affects her vision, but she persists. Despite fatigue, and the side effects of her chemo, she approaches her days like she always has… with curiosity, creativity, and happiness – albeit with moments of justified frustration.
People say Neddy and I are to credit for her resilience through this. But we know her strength is all her own, and was there long before her cancer. If we are to credit for helping her live, then we credit our phenomenal family, friends, and community for creating support so strong it feels like a tangible object. Something we can rest our tired heads on, or hide our faces and cry in. We have been carried forward through so many selfless acts of kindness. We have never known so much humanity, and we’ve learned that love often comes from where we least expected it to.
Strength-giving realizations like these, and fear-inducing questions like what comes next, will continue to occupy our minds regardless of these MRI results. But I have to believe we are not ready to talk about the end yet. Piper, like every other kid (with or without cancer) is her own person, with unique cells and an individual spirit. She is our only real barometer, and even at this stage, she’s chasing away the clouds and calling for sun.
Valentine’s Day is just around the corner, and Piper knows it. She calls it “Heart Day,” which is totally adorable in itself, but not half as much as her preparations for what everybody else considers to be a tertiary holiday. Hand-crafted receptacle for all the cards she plans on receiving? Locked that masterpiece down last month. Candy for you, me, and everyone we know? Got it three weeks ago. A royal selection of hand-curated Valentine’s Day cards to ensure a bespoke and timely holiday greeting? Got that too. In fact, it seems there is little left to be done but to spread the treasury of love she has saved up for us all.
While it might seem that such great lengths are simply the whims of a four-year-old girl who likes to shop and craft, I have come to realize they are not. Stop to consider what Piper has been through. She’s been under general anesthesia 39 times since June. She’s had brain surgery and lives with a port in her chest. Her left eye is permanently turned inward such that she sees double a lot of the time. Instead of going to the hospital once a decade like the rest of us, she goes weekly. She walks (indeed, runs) even though she struggles to lift her right leg anymore. She is losing more and more of the blonde hair she loves with each passing day. Nose bleeds of varying severity are a routine part of life. She was forced to become left-hand dominant when her right hand lost its strength and dexterity. In response, she has undergone 5 weeks of an occupational therapy program where we literally wrap up her left hand to remind her to use the right. She takes three kinds of chemotherapy drugs, endures regular blood draws, and has a compromised immune system and essentially no platelets. We tell her she has to get poked so the hospital can interview her blood in a laboratory and ask it what’s going on inside her body. This almost always wins a smile, before tears resume and we have to hold her down while she pleads with us to protect her. On top of this, she is nearly 8 months into the 6-to-9 months her doctors gave her to live. But for Piper, exceeding expectations is nothing new. So, as we have since the beginning, we hold tight to hope. Hope that her body continues to respond to the medicine, hope that she continues to be happy, hope that she is the 1%.
The fact that Piper has any love left to give is amazing. The fact that she has so much is deeply beautiful and full of meaning. It serves as a reminder that we all have goodness, and kindness, and love to give all the time. No matter what happens to us. No matter what is taken from us. No matter how bad it gets. Even when we think we can’t.
Piper may be young, but we can all learn something from her Heart Day fervor: There is never a bad time to care about another person, and there is no circumstance which can bar us from elevating the human experience with compassion and solidarity. For as long as we are able to breathe, we can say “I love you.” For as long as we are able to hear, we can listen. For as long as we can lift our arms, we can embrace somebody who needs embracing. And for as long as we can put Hello Kitty tattoos into cheap, tiny Valentine’s Day cards showcasing cute little puppies, we can and should cover them with princess stickers and affix them to a box of chalky hearts in an expression of the goodness we have to give. We will all be the better for it.
Happy Heart Day.
We Walked Off To Look for America
Before DIPG, we wouldn’t have considered taking two young children to New York City in the winter with absolutely no plan. But I suppose we can thank DIPG for approximately one thing: it pushes aside unimportant hesitations, makes you consider more than just what’s sensible, and creates a space where you can act on a dream. One of Piper’s big dreams was to see what she calls “The Statue of America”. And that’s how our adventure started.
Despite hurdles like weekly doctor’s appointments, having to pack a full arsenal of medications, and the holiday travel rush – we said yes. We knew it would be physically exhausting and mentally challenging. But it didn’t matter more than telling Piper something exciting was going to happen, instead of just when she was due for her next chemo dose or blood draw.
Serendipitously, we were contacted by a kind stranger named Jennifer Majuta on the day we finally resolved to book a flight. As it turns out, in addition to being a seasoned flight attendant for Southwest Airlines, Jennifer is also a children’s book author and lives just up the street from us. She generously offered us guest passes to fly standby, and spent countless hours researching routes and walking us through the process. The day of our trip, as many as six Southwest employees greeted us at the gate, bearing gifts and smiles, and offered to carry our bags on the plane as Piper boarded first. Among the gifts was a Southwest teddy bear Piper aptly named “pilot bear,” and a book titled “Come Fly with Me” written by Jennifer. It was the perfect way to get her excited about the magic of travel.
Thanks to C. Roese Ramp, the talented documentary photographer we were introduced to early in this journey, we were put in touch with Tova Friedman, a NYC-based photographer, just days before arriving. On our first afternoon in the city, Tova graciously offered to meet us at the American Museum of Natural History so we could have the greatest of gifts, photos of the four of us together.
The next day we embarked on a chilly ferry crossing to see our muse, Lady Liberty. The expression on Piper’s face when she finally looked up to see the statue was worth every. single. hassle. Pure awe. It was as if we took her on a rainbow ride to see a unicorn. If only we could give her that too.
We also made stops at the 9/11 Museum, which reminded us that great tragedy is part of a shared human experience. Bryant Park brought us holiday cheer, The New York Public Library warmed us up again, and the sky brought us snow just in time to see the tree at Rockefeller Center.
It wasn’t the trip we would have taken before DIPG, instead it was the trip we took in spite of it. It was hard at times. It was unforgettable. It was all for Piper, but it was a dream come true for all of us.
A Girl Worth Fighting For
The power of community should never be underestimated. You see it all across the world, every day. People helping people overcome natural disasters, violence, disease, the list goes on. Our community is something we always had but never understood until Piper’s diagnosis. But every day since then, our community has shown up, for her, and for us. Now we understand.
When we were strapped for a venue, my sister used the power of community to ask for help. She posted our request on local forums and within hours we had people at the ready, offering help although they had never met us.
United Elite Cheerleading offered their spacious gym. The Enchanted Paintbrush offered time and great talent to paint faces. MICI Handcrafted Italian prepared 20 delicious pizzas. Jenny’s Haute Cakes created a work of art I never imagined possible. C. Roese Ramp lent her unparalleled photography skills to document the day. Victory Love + Cookies baked up 80 amazing treats to send home with guests.
All of these people, all of this help, was given free of charge. Given by good people because they care; because they saw our plight and wanted to support Piper. But help is such a small word – what these businesses gave and what we received was so much greater than that.
The side effects of Piper’s chemo may have kept her from fully enjoying the day like she would have pre-DIPG, but nothing can keep this ray of sunlight down for long. Having this party meant the universe to her. That nearly 100 people made her celebration part of their Saturday was pure magic.
Additionally, this month we were able to raise just over $4,000 dollars for The Cure Starts Now, so they may continue to fund critical cancer research. On Giving Tuesday, we started a campaign in Piper’s name that generated $2,535 dollars in four days. That figure included 20% of Warrior bracelet sales to-date, which amounted to $1,000, including the largest single order from Meadows Family Dentistry who, in addition to being great people and a great practice, generously purchased bracelets for their staff. Victory Love + Cookies donated 100% of store proceeds last Saturday, raising an impressive $689. They saw more traffic that day than they did before Thanksgiving. And for Piper’s birthday party, another $778 was donated.
Piper may not entirely understand this at four years old, but that contribution to research is her gift to other children. None of this would have been possible without our community-turned-Warriors working together toward a common goal. One that presently brings a smile to Piper’s face, but in the future, brings about long-ignored change for children with DIPG.
Thank you with everything we have for supporting Piper, a girl certainly worth fighting for.
Party Fit For A Princess
As we prepare for Piper’s 4th birthday party, one I refuse to accept could be her last, I have been thinking a lot about how to celebrate in the context of cancer. We have more to celebrate than ever before, but this year our joy is indivisible with DIPG. The two cannot be separated. But just like you need dark to appreciate light, our unimaginable circumstance allows us to distill what matters and appreciate the magnificence of life.
The party will take place three days before Piper crosses the six-month mark of her diagnosis. We didn’t know what this point would look like, but thanks to her determination and access to precision medicine, she fights on. She has good days, even great days, where it is possible to forget cancer for a moment. Her encouraging last MRI gives us every reason to keep hoping, something we truly thank God for. There is still much to battle in the months ahead, but we do it with renewed determination.
When we talked about how Piper wanted to celebrate her birthday this year, her response was “a big party.” So, a big party she’ll get. But this year, we had the help of a village. Thanks to the compassion and generosity of people in our community (many of whom we have never met) Piper’s day will be special beyond her wildest expectations.
I sincerely look forward to standing beside her band of Warriors to celebrate the gift of this birthday.
Lately, Piper has been entertaining us by reenacting the story of the three little pigs and the big bad wolf. Adorably, she’ll say, “I’ll huff, and I’ll huff…” before blowing down our pretend house with all her might. Each time it resonates with me. As she plays, she is unaware that there is a big bad wolf threatening her too. And perhaps what her house is made of will matter. Our house is made of love. It is all we had in the beginning, and it is all that matters at the end of the day. As Piper says, it’s “true love forever.” What could be stronger than that?
But as we’ve said before, DIPG is not a fair fight. It’s more vicious than any hungry wolf, and it has been left to roam for too long. When faced with something like DIPG, you have no choice but to brace yourself and fight. You hold on to each other through the gusts. You remain vigilant. But it’s not enough to be strong. You have to believe too. You have to believe that good will prevail somehow, and that breakthroughs are on the horizon.
Fairy tales like the three little pigs and the big bad wolf are used to convey lessons in an easy-to-digest format. The fact that kids are dying from brain cancer every day, and will continue to die, is not a palatable topic to most people. But as we prepare to close out 2017, a year that saw little more advancements to DIPG than the last forty, we have to find a way to face this hard truth.
My hope is that Piper’s smiling, innocent, brave face can convey an important lesson the same way a cautionary tale can. Right now, pediatric cancer research and treatments are nothing more than a house made of straw. We already know that outcome, so let’s do the right thing and grab some damn bricks already. May this great need tug at hearts until we’ve collectively built something stronger for our children, and fast.
Hope is a precious commodity to the parents of DIPG children. It must be pursued and discovered, and then cherished and defended once found. For us, hope came from a team of doctors on the cutting edge of DIPG research.
As mentioned in the last post, one of the biopsy samples from Piper’s tumor was sent to doctors and researchers at the University of Michigan so they could determine the tumor’s full genetic profile. The doctors sent us swabs to obtain Piper’s normal DNA from the inside of her cheeks, and they compared the profile of Piper’s normal DNA against the DNA found in her tumor to identify the tumor’s specific mutations. Performing this task took many weeks but resulted in the discovery that Piper’s cancer is driven by two genetic mutations. These mutations, in turn, are targeted and treated by two existing FDA-approved medications called Panobinostat and Everolimus.
Armed with the knowledge that Piper’s tumor may well respond to these medicines, and that they would probably get into her brain, taking them was presented as the best option to prolong her survival. At last we had hope.
That hope, however, was quickly dashed. Although months of science had gone into discerning which drugs would work to treat Piper’s disease, our health insurer refused to pay for them on the grounds that they were experimental and investigational. Our doctors appealed, but the insurer automatically maintained its denial since the drugs had not been FDA-approved for the treatment of Piper’s specific disease. As a reminder, there are no drugs FDA-approved for the treatment of DIPG. We were crushed when we learned this, followed by the news that the drugs would cost $35,000 per month if we were to pay out of pocket.
Fortunately, I am an attorney specializing in insurance coverage and litigation. Having secured health and disability coverage for many others in similar circumstances, I knew how the internal appeal process worked and what documentation was necessary to get approval of the medicines. I scoured through our 128-page policy to find that while it potentially excluded experimental and investigational drugs, it covered off-label use of FDA-approved medicines. Against this policy basis, I called the insurance company every day for a week to gather and present information, I conferred with other lawyers to discuss strategy, and I met with Piper’s doctors and nurses to obtain medical articles and letters supporting that the medicines were safe and effective. After a second appeal, the insurer agreed to pay for the medicines.
A relief no doubt, but the process cost nearly a month of Piper’s precious time.
Last week Piper began using Panobinostat, taken in the form of two capsules three times a week, every other week. No small feat for a 3-year-old who practiced for an hour with M&Ms before she could learn to swallow pills. Pills that each cost $1,450, so it was a nervous undertaking for us all. But like everything, she has tolerated the strange new routine remarkably well. She will start Everolimus in the coming weeks, and we pray they will be the breakthrough she deserves.
We hope that Piper’s fight, and in turn, our struggle to obtain science-based treatment for her, paints a picture of the difficulties pediatric cancer families face every day. There are simply too few medicines for the treatment of cancer in children, and even when they exist, insurance companies will not willingly approve them.
So, in addition to caring for our child by day, and trying to maintain hope and normalcy, we were forced into a secondary fight in the off hours. Without the specific knowledge we were fortunate to have, we might not have this opportunity. That needs to change. Because in the world of DIPG, hope must be nurtured. Once you find it, you can never let go of it.
We still have ours, and for that we are grateful.
The Road Ahead
Nothing in this new reality makes sense. It’s a place where opposites rule and decisions – what few we have in this mess – must be made quickly.
Where else can words like “progression” be words to fear? Where else can phrases like “enjoy your time” be the polite equivalent of “sorry we can’t help you”? Where else can children go from full of life, to a life celebrated in memory in the same year?
Every year there are new technologies and slick advancements – money poured left and right for mindless entertainment. But the world of pediatric cancer research remains sluggish and unchanged. Yet in our experience, people DO want to help and WISH they could do more. Trouble is, there seems little to be done until more doors open up, or alternatively, too much to be done with too few resources.
Bottom line – it’s an impossible place, our reality. This is DIPG. But as parents, our instinct to keep trying, hoping, and fighting for Piper is as strong as our instinct to breathe, no matter the odds.
After Piper was diagnosed, we were presented with two up-front clinical trial options. We elected to participate in a phase zero study which entailed giving her one dose of Gemcitabine, a well-known chemotherapy drug, just before her biopsy, to see if the molecules could pass the blood/brain barrier (BBB) and reach her tumor. The BBB has proven an impenetrable fortress in countless trials before, but to everyone’s surprise the drug got in and was equally dispersed throughout her tumor tissue. But, given the early stage of research, we were told this wasn’t a viable therapy we could consider for Piper, as factors like dosing and side-effects were still untested.
Next we were presented with an opportunity to send some of Piper’s tumor collected at biopsy to the University of Michigan to undergo extensive genetic testing. This is something researchers have only begun to do now that DIPG biopsies are considered safe and routinely offered. Previously, tests like these were limited to post-mortem tissue when elected by the family. The goal of this study was to illuminate the specific biology of Piper’s tissue, and use that information to identify effective treatments.
After agreeing to those initial trials we focused on Piper’s six week radiation regimen (AKA the same and only standard treatment for DIPG unchanged in 60+ years). We were repeatedly told that radiation was palliative, and that it was not a cure for Piper’s DIPG. We were advised to seek second opinions only after this point, as radiation represents the “gold standard” in treatment and would be suggested by any/all reputable neuro-oncologists.
When the time came, we sought opinions from all the top pediatric cancer institutions including: Stanford, Dana Farber, St. Jude, and Memorial Sloan Kettering. This exercise resulted in nothing ground-breaking, only unanimous agreement of Piper’s diagnosis. It did give us the opportunity to consider a few additional trials that were not previously available, however we resolved to try a different path altogether.
With the genetic information obtained from the University of Michigan study, Piper’s care team will pursue a personalized treatment plan. We reasoned that if obtaining biopsy tissue and genetically testing it represents the newest technology in the world of DIPG, we should use what we learned. While this is not presented as a “cure” (a word decidedly avoided in these cases) it goes beyond just controlling symptoms. It aims to fight Piper’s tumor using a duo of chemotherapy drugs believed to represent her best chance. Unlike trials, this gives her the freedom to continue on Avastin in lieu of steroids (greatly improving her quality of life) as well as stay in Colorado for her care.
If we are successful in our insurance negotiations, Piper will start treatment this month, along with physical therapy to encourage her body to reclaim its independence. We are in uncharted territory. But it’s what we have – and its more than some families ever get. For our part, we will continue to “enjoy our time” and use it to laugh together, research like hell, advocate in between, and hope and pray that this treatment works for Piper.
Last Days of Normal Life
The warning signs were not what you might imagine with such a weighty diagnosis. Piper’s symptoms presented in such a way that simple, rational explanations were enough to satisfy our early concerns.
After Nelson and I returned home from a 3-night trip to Chicago (our first travel without the girls) we began to notice changes in Piper. It started with her speech. Minor pronunciation issues that we attributed to her wanting to talk like her baby sister, Harlow, or simply acting out because we had been away. A couple days later we noticed she also seemed to have difficulty swallowing and chewing her food. Her saliva would gather in the right corner of her mouth and if she was laughing or looking down, it would drip out. But still this was all minor. Then came the calls from her school. Always watchful, they described a lack of energy and engagement uncharacteristic for Piper.
Friday, June 9, six days after our trip, we had an appointment with our family pediatrician in hopes of finding answers. The visit put all our concerns at ease… “it may be an ear, nose, throat issue” … “perhaps she has strep or swollen glands” …. “no neurological concerns”…. Piper had also been having night terrors and, paradoxically, sleep laughter over the previous six months (also early signs of DIPG) so the doctor felt that sleep deprivation might be playing a role. She scheduled a sleep study and told us to go to Target to get nasal spray to calm down what she thought was a swollen tonsil.
Then, late Sunday, June 11, Piper’s symptoms suddenly worsened. No longer nuanced changes, but clear signs that something bigger than an ENT issue was at hand. She lost her energy, began to trip and stumble, and her speech became so slurred we could hardly make out her words. Although she was talking as much as ever, she was clearly laboring to do so. Alarmed, we began to Google her symptoms but her eventual diagnosis never once came up in the search. At that late hour, reassuring ourselves it could not be neurological like the doctor said, we decided to let her rest and take her in first thing the following morning.
Monday, June 12, 2017, will forever be etched in our memories as the day the sky fell. The day our seemingly perfect world evaporated. At 6:30 a.m. it became clear Piper was not improving. Although she had slept comfortably in our bed all night, she was slow to wake and couldn’t even really get out of bed. Rather than wait for her doctor’s office to open, we rushed to the ER at Children’s Hospital South Campus. They wasted no time getting a CT scan in process. Even then, worried as I was, I believed the explanation would be something I’d at least heard of, or could be treated in the age of modern medicine. We waited for what seemed like hours, and although we had been given no information, we began to know that something was wrong. The nurses and staff who were so cheery when we arrived were now avoiding eye contact and conversation. After about half an hour, the ER doctor asked us to meet her in an empty room. The short walk to the next room was silent and foreboding. A box of tissues had already been placed on the table in front of where we were to sit, then the doctor bravely, and with obvious heartbreak, told us that the scan showed a mass in Piper’s brainstem. Everything echoed. “What kind of a mass?” we both asked. The doctor said nobody would know until Piper had an MRI at the Children’s Hospital Main Campus in Aurora. After we pressed the issue, she said the mass appeared to be cancerous. Before the conversation was over, an ambulance was ready for us.
Minutes have never felt so long. After we arrived, Piper’s condition began rapidly deteriorating. She couldn’t walk or even move much at all. She couldn’t talk, but just whimpered and uttered half-formed words. She wanted to tell us something, but she just couldn’t. The doctors explained they would image Piper’s brain and spine and that it would take about an hour and a half. We held and kissed her as they put her under general anesthesia so they could obtain a clear image.
Before the procedure was even complete we were asked to come to yet another ominous room. They had seen enough to make a diagnosis. Waiting for us was another pre-placed box of tissues, two neuro-oncologists, a medical student, and a social worker. It was there that we first heard the words Diffuse Intrinsic Pontine Glioma, or DIPG for short. These words came alongside others like “inoperable,” “incurable,” and “universally fatal.” The doctors said our beautiful 3-year-old would die from her brain tumor and that she might live 6-9 months if we elected palliative radiation, or just 7 weeks if we did nothing.
We held hands and cried until there were no tears left. Then each of us broke the news to our parents. Life has never been the same since. We spent the next week in-patient with Piper, encouraging her to talk, chew, and slowly walk again. Her tumor was biopsied (something they only started doing in the last five years) and confirmed it to be grade IV DIPG.
Every day since June 12, 2017, has been far from normal – even in the happiest of moments. Although our reality has been irreversibly altered, some changes have been positive. We understand as we never could have before that we should be grateful for each day. To take struggles one step at a time. To live for today. To love like there may not be a tomorrow.
Arvada, Colorado, USA