David Click's Blog, page 5

SOMEWHERE IN THE MIDDLE

You never know what to expect with Duchenne. You never really know where you are along the journey. Duchenne is predictable as much as it’s not. It’s bearable, as much as it isn’t. It teases with normality as much as it destroys. It draws others to you as much as it isolates. It blossoms with love, support, and perseverance, as much as it seeks a foothold with anger, resentment, and despair. It’s exhausting as much as it is strengthening. It's consuming as much as it's giving. But, mostly, we have found, Duchenne is a journey of love and acceptance that is beautiful as much as it’s fleeting.
 
Our Duchenne journey began in 2002. The diagnosis left us stunned, in complete shock and disbelief. Duchenne Muscular Dystrophy? What’s that? It does what!? Are you kidding me!? It can’t be! We were following a path we had always dreamed for years! This can’t be happening!
 
Welcome to your new reality, Clicks.
 
Sometime after that first shock, life settled down as we came to grips with the then immature beast. Alex still played in the yard, though waddled. He still swam in the pool, though weakly. His friends visited frequently, and life was good. He had no real complaints, nor did we. Sure, Alex was slower than most kids. But, that was something we could handle. It ain’t that bad, I remember thinking. We can tame this beast, I naively convinced myself. Just look at that smile on his face!
 
Despite our positive outlook, Duchenne settled in.
 
Soon, Alex began to fall…a lot. He would appear stable one second, and then face-plant the next. School became a dangerous place for him as kids moved swiftly through the tiled hallways or on the asphalt playgrounds and Alex struggled to keep up and avoid being knocked down. We noticed how his muscles were weakening, as did he. I remember many bedtime talks when he would ask “Why am I falling so much? Why can’t I keep up? Why won’t my stupid legs work?” That early stage broke our hearts. As parents, you do your best to protect your child and soothe any concern. We pumped him with confidence and told him we loved him no matter what. We did anything to keep his smile alive.
 
That semi-mobile stage was tolerable because Alex was still walking, though his freedom started to wane. We carried him often. I hoisted him on my shoulders like any proud Dad. I tossed him through the air in the pool and jumped off the diving board with him in my arms. We laughed and played and let our imaginations soar. Life was still somewhat normal as we still used the family car and took those beach vacations we’d always dreamed for our family.
 
Despite our support, Duchenne kept marching.
 
We soon realized Alex needed a power wheelchair. Although some may consider that milestone tragic or sad, it actually brought us relief and happiness, because it gave Alex freedom of movement and us peace of mind. It also brought new challenges. Accessible vans cost how much!? Our home needs what kind of modifications!? Holy crap!
 
The concerns snowballed. Will his school accommodate his advancing needs? Will they understand his limits? Will my employer understand absences to care for him? Will they allow me to adjust my hours? Will they understand Duchenne? Should we get a nurse? Should we care for him ourselves?
 
Our heads were spinning.
 
Somehow, we made it through those transitional times, and then entered what I consider the cruise-control phase where we adjusted to everyday life with Duchenne. We learned routines for dressing, grooming, and traveling. We learned to live with bumps and scrapes on our walls as he learned to handle his wheelchair. We learned where wheelchairs were welcome (malls, big box stores, parks, museums, zoos) and where they were not (old cities, water parks, hiking trails, friends’ homes). We learned grass, mud, gravel, and sand were bad, and that concrete and asphalt were good. We accepted Duchenne for what it was and realized our good fortune.
 
Duchenne could care less.
 
With time, Alex started having back problems with the onset of many spinal compression fractures. It became painful for him to sit comfortably and he missed a lot of school. Toileting soon became impossible, and we were forced to find other means to help with bodily functions. His torso weakened dramatically as did his arms. His weight increased from immobility.
 
As his body weakened and matured, we learned to adjust. We learned how to lift deadweight (note: Alex now weighs more than me). We realized the importance of our health in his daily care. Likewise, Alex encouraged our well-being, and knew we must stay strong to help him. I reconsidered physical sports (i.e., softball, basketball, racquetball, etc.) to avoid injury. I learned to appreciate the power of low-impact exercise (walking, swimming, hiking) to stay fit.
 
Still, frustrations never ceased and tempers often flared. Alex became increasingly dependent, and frustrated by his inabilities. He could no longer feed himself if sitting, and needed to lean back far in his chair to “climb” food to his mouth. He frequently dropped things (cell phone, Lego pieces, PS4 controllers) because of his weakening grip. He struggled to hold a menu or lift a simple drink. He increasingly became uncomfortable in his bed or wheelchair as bunched shirts, wedgies, and itches would drive him (and me) crazy. If he wanted to leave the house, dressing, grooming, preparing, and hoisting became major, time-consuming, productions. Often, the littlest of things sparked angry words, and arguments snowballed for no reason other than frustration, fatigue, and exhaustion.
 
Duchenne smiled with pride.
 
Thankfully, such eruptions are short-lived and burn out quickly. Afterwards, we laugh and love as always, and push back against the stealthy beast. We share knowing looks, smiles, yawns, and belches. We talk about life’s joys, sorrows, worries, and fears. We laugh about the past. We talk about the future, but focus on the present. We hold hands every night while falling asleep, and our last words are always love you.
 
As our journey advances, if there is anything positive about Duchenne it’s that Alex and I have become close…very close, like-an-old-married-couple close. We finish each other’s sentences. We know each other’s quirks, habits, and needs. He gets my jokes even before I say them, because he’s heard them a thousand times before. He looks to me for wisdom and direction, and he trusts me completely. I enjoy his outlook on life. We appreciate what we have and try to forget what we don’t. We keep marching onward, together and unafraid, as we watch the beast grow.
 
We’ve been through a lot over the years and know it’s only just begun. Knowing those living with Duchenne in their advanced years, like Ian, Paul, and Ricky to name a few, I am fully aware that we ain’t seen nothing yet. Life with Duchenne never gets easier, only harder.
 
Oh joy. Can’t wait.
 
Until then, we simply just do the best we can and take each day as it comes.
 
So, this is where we are right now. Somewhere in the middle of this incredible journey. This is where I take a deep breath, reflect on how far we’ve come, and buckle down for the long and challenging road ahead. This is when I walk into Alex’s room and sit near him while he plays a game or watches a movie. I listen to his laughter and join in his smile. I talk Star Wars or Disney because I know he enjoys it. He may ask me about the Reds. Oh, I still roll my eyes, every now and then, and grumble when he asks for yet another water bottle. That’s every parent’s prerogative. But, I try to remind myself where we are in this journey, and appreciate all we have right now.
 
Because with Duchenne, each day is the best it will ever be. That fact alone smacks me with life perspective I never would have imagined otherwise.
 
For that, I strangely say thank you, Duchenne.
 
But, don’t get a big head, because I really don’t like you….at all.

THE BRIDGE REVISITED

Sometimes in life you face difficult conversations. When they happen, you can confront the discomfort or delay the pain. You can use words you’ve rehearsed for weeks or think fast on your feet and hope for the best. Either way, the conversations are never easy, for anyone. They are often painful, raw, and emotional. They require strength, perseverance, and sensitivity. They also demand patience because follow-up questions often emerge and must be answered no matter how tired you may be. Ironically, these conversations also have a positive side, which can make all the difference.
 
With Duchenne, you know the hard questions about life and death are coming, you just don’t know when. You know they’re inevitable, you just don’t know to what degree. You envision the moment of their emergence. You imagine the time, place, and general setting of their asking. You imagine yourself rising to the occasion, standing tall, tear-free, and delivering with love, patience, and understanding. You imagine your son listening, nodding, and smiling with acceptance.
 
With Duchenne, however, we have learned that life rarely happens as you imagine.
 
The bridge I had feared for years first appeared last October after a fun day watching the Cincinnati Bengals beat the Kansas City Chiefs, when Alex asked me if he would die before the age of twenty (http://www.davidlclick.com/blog/the-bridge). The question caught me off guard and I did my best to answer him honestly. It was a tender moment between father and son (in a dusty parking garage, of all places), as Alex shared his innermost thoughts and concerns. While we drove home that day, I told myself that although the bridge had been crossed, more questions about Duchenne, life, and death were certain to follow.
 
They didn’t, and life with Duchenne continued onward along its familiar, unwelcome, and unwanted path.
 
Then, nearly eight months later and after a fun-filled weekend of relatives, a Dave Matthews Band concert, and an Angry Birds movie, the questions returned with a vengeance. It was as if Duchenne had been starved for attention and wanted to renew its havoc in our lives.
 
Hello, Clicks! Did you miss me?
 
My cell phone rang at 4:30 a.m. early Sunday morning, waking me from a deep sleep.
 
“Dad,…I need you! Something…weird…is happening to me!”
 
I immediately tossed the covers aside and raced into his room. Alex explained that as he played a video game, he could breathe in, but he could not push air back out. With a panicked expression, he described how air would only seep out of his mouth when he tried to exhale! I immediately recalled how Alex’s Pulmonologist, Dr. Sawnani, warned us that Alex’s lung strength continues to slide as does his ability to push and pull air in his lungs. Mixed feelings of concern, worry, fear, and guilt filled my brain as I calmed Alex and then positioned his BiPAP over his face and turned on the air. Once his breathing stabilized, the onslaught of questions began after their long hiatus.
 
At first, Alex asked me to explain why he had difficulty breathing. But after the technical questions were addressed, Alex quickly zeroed in on his primary and most vital concern.
 
“Dad, does this mean I am going to die soon?”
 
Immediately, I was filled with anger though did not show it. Damn this disease! No kid should have to think about dying! No kid should have to worry! Do kids with Duchenne not suffer enough!
 
“No, Alex, this doesn’t mean you are going to die soon.” I reassured, hopeful the sincere recipe I applied in October would suffice again today.
 
“Then what does it mean?” he demanded. “Why can’t I breathe normally?”
 
“Well, Duchenne, unfortunately, affects all muscles….” I began before he interrupted.
 
“Even my lungs?”
 
Deep breath. Choose your words wisely. Although Alex has heard Dr. Sawnani speak of breathing concerns before, Alex has been convinced for years that Duchenne only affects his legs and maybe his arms.
 
“Yes, even your lungs.” I answered honestly.
 
Say something quick to lessen the impact! NOW!
 
“But, we can help that by using your BiPAP more frequently.”
 
Alex expressed immediate concern, his voice sounding nasally as he spoke over the air being pushed into his nose.
 
“Does that mean I have to wear this stupid thing all the time?”
 
Hear him, but guide him. “No, but we need to wear it as often as possible when you are in bed…when you watch movies or play games.” Do I explain his likely future with ventilation? Do I share with him the strong possibility of a tracheotomy? .....No, not now. Those discussions will come with time. Focus on what he will understand. Focus on the positive. Focus on the….
 
“But, if I can’t breathe…will I die?” he cut to the chase. The hard questions were getting harder.
 
Oh Lord.
 
“No, if you need help breathing, doctors have other ways to help you.”
 
Alex grew silent as he searched my eyes for the slightest expression.
 
Am I saying the wrong thing? Am I saying too much? Am I causing worry?
 
“I don’t want that, Dad. I don’t want to be hooked up to a machine and tubes the rest of my life!”
 
You can do this. Acknowledge. Foster hope, yet prepare him for what will likely be his future, because it is clear he is seeking answers. Be gentle.
 
“It doesn’t necessarily mean that, Alex.”
 
“But, boys with Duchenne will need it, right?”
 
“Yes, some do.”
 
“Will I?” he pressed.
 
Although the likelihood is high, I deferred. “Maybe. Maybe not.”
 
Alex looked towards a window, clearly thinking. I could tell he wanted answers. He looked back to me, directly, and changed his line of questioning.
 
“Dad, does Duchenne kill?”
 
“Well, everybody dies of something, Alex. You just never…” You could immediately see his dissatisfaction as he rephrased.
 
“Will Duchenne kill me?” he asked point blank.
 
Still searching for a soft landing, I said, “Alex, nobody knows how they will die. We could be killed in an accident, we could be…”
 
“Dad, CAN Duchenne kill me?” he wanted the truth and would settle for nothing less.
 
Looking him directly in the eye, I then uttered the hardest, yet simplest, most honest word I ever have had to say in my lifetime.
 
“Yes.”
 
I immediately wondered if I went too far. Late hours and fatigue tend to expose your core thoughts, no matter how you try to control them. But, I also could see that Alex would not accept dancing. He wanted truth. Still, I needed to qualify my response…and quick, so as not to lose him.
 
“But, Alex, with Duchenne you never know. Remember my friend, Ian, who I’ve told you about? He has Duchenne and is 34 years old! He’s doing great despite his challenges. He is an author, a digital artist, and has a website! He’s an uncle! In fact, when Ian was your age, he worried about his lungs, too! But that was 17 years ago!”
 
Alex calmed slightly, but I could tell he was still leery of everything and needed more.
 
“I also have friends named Kitsune, Ricky, and Paul. They all have Duchenne. You should see all they do! Kitsune is 34. Ricky just turned 35. And Paul is 49! So, you see, Alex, Duchenne affects everyone differently. You’re only 17. You have your whole life ahead of you. Don’t worry about your breathing. We can help that! You can do the things you want to do and live a long time!”
 
You could see Alex relax a little. You could see him understand and reluctantly release his fear because he considers people in their 30s and 40s to be ancient. But, you can also see his concerns were still there, brewing and processing. The questions continued.
 
“Does everyone with Duchenne live that long?” He probed.
 
Again, I resorted to honesty. He deserved as much for living with the monster.
 
“Well, unfortunately, no. As I said, Duchenne affects everyone differently. Some die young. Some live a long time. You just never know, Bud. You just never know.”
 
Alex reasoned thankfully. “So, if I wear my BiPAP and take my pills, I’ll live a long time, too, right?”
 
“Yes, Bud. We must do everything our doctors tell us so we can live a long time!”
 
Alex then relaxed enough to not outwardly worry any further that night, seemingly satisfied with our discussion. Over the past several days, however, he has asked many follow-up questions about life and death. Each question seems to come with a new level of understanding and awareness. Each question also leads me to believe there will be more.
 
A difficult aspect of Duchenne is that as Duchenne’s physical destruction advances, your son matures emotionally. As his body weakens, his awareness strengthens. It’s an awkward passing. He no longer will accept measured and tailored explanations typically given to a child. He will no longer accept redirection as a means to change or avoid the subject. As he matures, he wants answers and he wants the truth. He wants you to speak to him as a young adult and no longer as a kid.
 
You’d want the same at seventeen, wouldn’t you?
 
As tough as it is to cross the bridge of facing our own mortality, I know his questions will only get deeper as we move forward. This current bridge is long and, from this vantage point, I see many more on the horizon. Strangely, the bridges seem to appear after happy and so-called normal times of life, if there are such things.
 
As you can imagine, these are difficult conversations to have with your teenaged son.
 
They are emotional.
 
They are surreal.
 
They are life with Duchenne…and they’ve only just begun.
 
So, about the positive side of this nightmare, because there is one…always…if you open your eyes to it. The breathing scare last weekend has reinforced to Alex that he must do everything he can to combat this unforgiving disease. Alex now wears his BiPAP every night, and often while playing video games or watching a movie. He takes all his prescribed medication without taking his usual “days off” he has been prone to demand. He realizes his sleep schedule may need adjusting, and asks to go to bed “earlier.” Hopefully, these positive behaviors will continue forward as his new routine.
 
The bridge revisited has also been bonding. We look to each other for support and cherish the many moments we live together, all things we should never take for granted though sometimes do. But, perhaps the most powerful outcome of this latest Duchenne episode has been both emotional and physical, as Alex now frequently asks for a hug before I leave the room. It’s almost like he is worried we may never see each other again.
 
It goes like this.
 
“Dad, can I have a hug?”
 
The question usually comes as I finish whatever parental task and then prepare to leave his room. The question accompanies his hands open and resting by his sides because he cannot lift his arms high off the bed.
 
I smile and reply, “You bet.”
 
I then bend down, put my head next to his, our chests together, and position my arms around him and near his hands so his wrists can then curl enough to complete the embrace.
 
I’m telling you there is no better feeling!
 
After several long seconds, we part. Alex then looks me directly in the eye and tells me he loves me…
 
…I smile, place my hand on his chest, and tell him the same.

SIMPLE ACTS AND FLYING PIGS

Sometimes the simplest of human connections make all the difference in our fast-paced world. They can produce smiles and spread joy. They can promote inclusion and brighten a rainy day. And for a boy with Duchenne Muscular Dystrophy watching 20,000-plus runners race by him, such simple acts can lift you up even when some try to put you down.
 
This past Sunday, Alex’s sister, Kaitlyn, ran her first Flying Pig Half Marathon. She raced as part of Muscular Dystrophy Association (MDA’s) Team Momentum to raise funds, create awareness, and support research for Duchenne Muscular Dystrophy. She ran to help kids like her brother who cannot run for themselves.
 
After dropping off Kaitlyn among a sea of runners and wishing her well, we parked downtown and worked our way towards the marathon course. Along the way, and per his usual behavior, Alex fist-bumped good-luck wishes to several runners and tossed good-morning nods to many coffee-carrying spectators. We settled near the corner of Seventh and Sycamore where Alex then befriended a man sitting next to us. The man had just moved to Cincinnati a few weeks ago and was sitting alone. The pair talked and cheered while the fastest marathoners sprinted past seemingly without effort and ahead of the endless wave of runners soon passing in all shapes, sizes, and bright-colored shirts/shoes. Alex and his newest friend talked superhero movies and Star Wars while we continually swiveled our heads looking for our daughter.
 
At one point, I overheard a conversation that warmed my heart. Team Diego (a Dad pushing his 11-year-old son who lives with Duchenne) had just passed in front of us, prompting Alex to share with the man that he, too, had Duchenne just like Diego. I continued to watch the mass of runners, but listened with interest to hear how the man would respond to Alex’s openness.
 
The man didn’t miss a beat.
 
“Well, I’m sure that’s no fun,” he said sincerely. The man then added. “But, it looks like you’re doing great! Keep up the good work!”
 
I turned around enough to see Alex beam with pride that his new friend understood how he felt about Duchenne and welcomed him for whom he was - a kid just like any other.
 
What struck me is the man didn’t have to say anything. He could have dodged the question or conveniently cheered passing runners in supposed distraction. He could have nodded a simple “oh, really?” and then turn away focused on the race. But, he didn’t. He engaged Alex, gave him his full attention, and pumped him with assurance that he was no different from anyone else. I smiled, equally impressed by the man’s kindness, and then turned fortunately in time to catch a glimpse of Kaitlyn as she raced eastward towards the hills of Mount Adams.
 
We then wished the kind man well and headed towards the riverfront to find a spot at the Finish Swine (I’m telling you, Cincinnati puts on a great race!). There, we jockeyed for position while Alex roamed the area behind us. Being in a wheelchair, Alex could not see beyond all the standing bodies, but he didn’t let that bother him. From a distance, I watched as Alex nodded hello’s to everyone he could, talked with several new friends, and pet many thirsty dogs. But, when I looked back and saw him talking with a man dressed in black…ALL BLACK…I did a double-take!
 
Not for fear, but amazement. There was Alex chatting with a man who wore full body armor and carried an AK-47 assault rifle among other weapons! I’d have worried, but the man was smiling and laughing with Alex like they were already old friends. We approached and told the man to haul Alex away if he is causing any trouble.
 
“DAAAAAAAADDD!!
 
The SWAT officer smiled and said Alex is not causing any trouble and then graciously posed for a picture. He shared a fist-bump with Alex before moving on to more official duties.

Not much longer, we watched Kaitlyn finish strong and under her goal time of two hours! We moved to find her in the post-race party zone, where both the bad and the good of humanity soon revealed itself.
 
The party zone receives runners funneled from the finish line, and includes tents, booths, merchandise, music, and fun! It's also packed with people as exhausted runners stagger about looking for friends, family, food, or drink. Though some lie collapsed in fatigue on the lawn, most runners are euphoric and smiling for completing the race. Family and friends are supportive and full of praise for the incredible efforts. For the most part, the party zone is a festive atmosphere.
 
But, there’s always that one ignorant person.
 
“Wheelchairs don’t belong here where the runners are!” A man said with annoyance and directly to Alex as he pushed passed his wheelchair and into the crowd.
 
Excuse me?
 
Just as fast as the man spewed his hateful words, he was lost in the mass of people.
 
Fortunately, Alex took the rude comment in stride. This wasn’t the first time he had heard such ignorance, and won’t be the last. But, come on! Does that man honestly believe Alex enjoys gridlock, ever-worried of clipping heals with his foot tray, and equally worried others would bump his extended feet (note: twisted ankles are painfully common in crowds). Did the man think Alex wasn’t equally entitled to enjoy the party zone? Did he even think about his implication? Alex would gladly exchange his limp, still legs for the man's tired, achy legs...any day!
 
We dismissed the man’s ugly words as we made our way through the crowd and listened to Kaitlyn recount her journey.
 
That is when Alex received an incredible act of kindness that made our day and quickly restored our faith in humanity. Out of nowhere, a gray-haired man in a navy blue t-shirt, shorts, and running shoes, stopped Alex among the throng of people. He placed a caring hand on his shoulder, smiled warmly, and then gave Alex his race medal.
 
“Here, buddy. This is for you!”
 
Alex lurched in surprise, but then gradually beamed with pride. Standing alongside, Kristy and I couldn’t believe what just happened. We looked up in amazement, but the man was already gone, disappearing into the crowd just as quickly as he had appeared.
 
Alex looked to the medal resting on his chest.
 
“Dad! Did you see what that man just gave me!?”
 
I did, but didn’t know what to say. It seemed the man knew what just happened. It seemed the man knew Alex would run if he could. It seemed the man knew Duchenne.
 
“Whoa, this thing is heavy!” Alex continued with excitement. He then tossed a sideways, teasing, glance towards his sister as if to say…and I didn’t even have to run 13.1 miles to get it!
 
Kaitlyn responded kindly, “Whoa! That is so cool!”
 
Kristy and I beamed with happiness for our kids, who were BOTH champions!
 
Sore legs and all, we eventually made our way through the crowds, back to our van, and then back home. We reflected on the eventful day. Kaitlyn raised Duchenne awareness, challenged herself, and beat her goal time. Alex made friends, avoided arrest, and got a medal. Kristy and I simply enjoyed our time together as a family. We smiled as we remembered the friendly man sitting near Seventh and Sycamore. We laughed thinking of the caring SWAT officer. We smiled with joy and gratitude for the gray-haired man who gave his medal to Alex.
 
We also thought how the day showed that despite the ignorance of some who believe others only get in their way, simple acts of kindness win…ALWAYS!
 
Even in a city where pigs fly!

IF NOT US

"…the kids could walk if they put their mind to it."

                     - Dr. Ronald Farkas, 
                       Clinical Team Leader
                       U.S. Food and Drug Administration

  
So expressed earlier this week in Washington, D.C., at an Food and Drug Administration (FDA) advisory panel meeting to discuss a potential life-saving drug known as eteplirsen. You may or may not have heard about the meeting, eteplirsen, or the ill-advised statement above, but the Duchenne community sure has been abuzz.

The D.C. gathering drew a standing room only crowd in support of a favorable recommendation despite a skeptical FDA advisory panel. Many boys and men living with Duchenne attended to express their needs, as did their families, friends, and relatives. The FDA advisory panel listened to more than 50 people share their unique perspective with Duchenne and how eteplirsen could impact their life. The advisory panel patiently listened as speaker after speaker approached the microphone and voiced their individual and collective views of the fatal disease, which currently has no cure. In the end, however, it seemed only an exercise in free speech as the FDA advisory panel DID NOT recommend eteplirsen for accelerated approval by a vote of 7-6 (including three abstentions). Despite the disappointment, the Duchenne community’s thirst for something…anything…to tame the beast known as Duchenne has never been stronger.
 
Sertepa Therapeutics, Inc., manufacturer of the exon-skipping drug, sought FDA approval for treatment of patients with Duchenne Muscular Dystrophy. Although treatment would apply to only about 13% of all Duchenne patients, it is a positive step. The issue, however, is not eteplirsen or any other drug (note: FDA denied another potential Duchenne drug, drisapersen, in January 2016). The issue is that we never stop our search for a cure to this unforgiving disease.
 
Don’t think those with Duchenne are unaware. They are aware, desperately. Every year, my son, Alex, meets with his neurologist, Dr. Brenda Wong, at Cincinnati Children’s Hospital Medical Center as part of his Duchenne clinic visits. Every year, Alex asks the same question.
 
“Have you found a cure yet?”
 
Every year, Alex hears a similar response.
 
“No, but….”
 
Don’t get me wrong, Dr. Wong does her patient and enthusiastic best to explain the latest in Duchenne research, and does so with hopeful seriousness. She compassionately tells Alex they are working hard to find a cure, and getting closer every day. Alex respectfully nods and smiles his appreciation, but you can clearly see his frustration and waning hope. Alex’s letdown is enough to break your heart every time.
 
So, when a drug such as eteplirsen shows promise, ANY promise, even if remote, hearts within the Duchenne community swell with tempered enthusiasm. We understand a cure will not be found overnight. We understand our sons, like Alex, may not be eligible for its use (eteplirsen applies only to boys missing exon 51; Alex is missing exons 49-54). We understand the drug may not work, as only marginal successes were observed in the few eteplirsen trials. We also understand the irreversibility of Duchenne, let alone the complexity of fixing a genetic-scale issue throughout the human body. We understand the long and difficult road ahead.
 
But we need something.
 
So, when a clinical team leader of the FDA says “…boys could walk if they put their mind to it,” while not recommending Sarepta’s request for approval to treat Duchenne,…well, it steals the air from your lungs and hope from your heart as Duchenne so often does to our boys.
 
Yet, it also makes you think…our sons’ futures are in the hands of those who don’t understand Duchenne?? I hope Dr. Farkas’ words above were misspoken. I hope he wishes his words back (note: Dr. Farkas’ email was shut down due an overload of messages from astonished and angry Duchenne families). Everyone says the wrong thing now and then…right? But, we’re talking about human lives, not a dismissive wave of the hand accompanying an insatiable need for more data and “substantial evidence of effectiveness.”
 
Despite the setback, the louder issue is renewed Duchenne awareness and our spirit to never give up. For everyone, not just those in the Duchenne community, the issue should be to chase every angle and turn over every stone in the search for a cure. The issue should be to do anything we can to cure this relentless, lethal, disease. Our boys are counting on us.
 
The issue should not be risk aversion until absolute certainty of benefit. ANYTHING other than Duchenne’s status quo provides benefit! Some said the FDA meeting felt like a trial where families needed to defend their child’s right for treatment. As many are concerned, at least Eteplirsen proves a glimmer of hope. Why not pursue it?
 
Nonetheless, Duchenne families are familiar with obstacles and roadblocks. They’re common themes in our lives. Just when you think things look promising, Duchenne smacks reality in your face. Just when you think you’ve made progress, Duchenne knocks you back down. Just when you think you can handle the beast, the leash snaps and you must work to corral it all over again and again and again. It’s discouraging, tiring and, at times, demoralizing.
 
But, Duchenne families are known to get back up and continue fighting for their son’s lives, even with odds stacked heavily against them. We encourage, lift, and support our sons every day. We keep on fighting this dreaded monster of a disease that steals time, ability, and dreams. We instill hope in our sons. We promote life and happiness whenever possible and make sure our boys know they are loved and not forgotten. We let them know they are no different from anyone else. We let them know we have their back.
 
In turn, our boys teach us about life. They remind us what is important, like a smile, a hand to hold, laughter, and love. They show us how to live despite any obstacle. They show us to forget about what we are missing and focus on what we have. They teach us that joy can be found in the simplest of things and during the simplest of times. They teach us patience. They teach us perseverance. Most of all, they teach us love.
 
So, when a potential life-saving drug like eteplersin is advanced and then later denied, despite enthusiastic support from those who would directly benefit, the Duchenne community collectively sighs and shakes its head in frustration. Then, we then pull up our bootstraps and continue marching, hoping, and loving. We also get louder.
 
It’s what we do.
 
The FDA may believe our sons simply need to just put their mind to it. But we know differently. Are sons are fighters…
 
Every day,
 
Every night,
 
Every breath, and
 
Every heartbeat.
 
It’s what THEY do.
 
Their only wish for ALL OF US…you, me, everyone…

...is to find a cure.
 
 
(Note: Encouraging and nuanced comments from Dr. Janet Woodcock, Director for Drug Evaluation and Research at the FDA, suggest the advisory panel’s recommendation to deny eteplirsen approval may be reconsidered when a final decision is released by May 26, 2016.)


Photo by John Boal for The Boston Globe

The World In a Whole New Light

There comes a time in everyone’s life when the world seems out of focus. Be it social, cultural, political, or theological, the world just doesn’t feel right and is different from the one you are accustomed. Perhaps this is one of those times, perhaps not. This perspective may occur through your current filters, but almost certainly when you set them aside. When you do, has the world really changed, or simply your view of it? You could adjust the lens until the image sharpens, or you can set aside your viewport and welcome a new perspective, perhaps one that has been with you all along, waiting.

The Cave (http://amzn.com/0692431241) examines our perspectives of life in many ways, but one in particular is through eyes of its protagonist, Maria Bongiovanni. Maria is one of my favorite characters because of her confidence, compassion, and overall thirst for understanding. She is contemplative, but not afraid to speak her mind. When she does, she speaks with respect and inclusion. Despite her traditional upbringing, she remains open to new ideas and perspectives.

When Maria accompanies her fiancée, Salvatore Giordano, on a trip to Jerusalem to meet his college friends, her world changes dramatically for reasons unexplained. She is stunned, but adjusts predictably and with confidence that life will return to normal. She admirably cares for others, but soon finds herself fighting for survival in the face of conflicting ideologies and beliefs. She does not shy from the conflict before her and the questions emerging from deep in her heart. Through it all, Maria questions truths she once considered sacred and discovers a new perspective important to her survival and that of the world.

I encourage you to consider priorities in your life and your world around you. I encourage your compassion for others, while remaining true to yourself. Most of all, I encourage you to seek answers to life’s greatest questions despite what you may have been taught. Whether in a cave or your corner of the world, I encourage you to never stop exploring.

Because, like Maria, you may see the world in a whole new light…and, possibly, much better focus.

New Video Trailer for The Cave

https://www.youtube.com/watch?v=tpQdt...

Review on Barnes & Noble Website

As posted on Barnes & Noble website - 5 out of 5 Stars

"The Cave was one of the best reads I have had in a while. A great first novel that leaves the reader deep in thought and ready for a sequel. A must read for those searching for meaning in life and trying to make sense out of the world, The Cave places old friends and their extended acquaintances in a situation where they must get along for survival. However, their prejudices and narrow life views hinder their chances. Alarmingly, the plight of the group is very similar to what our world finds itself in today. If you want to be challenged and expand your understanding you will enjoy The Cave. Other similar books that I have enjoyed included the Celestine Prophecy, The Shack and Jonathan Livingston Seagull. I hope all seekers take the time to read this book."

Ben Iden

What inspired The Cave?

What inspired you to write The Cave?

I am often asked this question. Friends have wondered what inspired a geologist, sports fanatic, and huge Dave Matthews fan to write a book with strong religious themes and based in Jerusalem. A fair question, and I believe the answer lies in who I am and what I have observed over the years.

Like many geologists, I am inquisitive and have a strong desire for understanding our natural world. I also love history, and learning about our world and its people. I am a voracious newspaper reader and read (more like devour) the paper every day, front to cover. I cannot get enough information sometimes, and often supplement what I read through online news channels.

What I read of our world troubles me. I read of conflict happening everywhere, every day, and I am befuddled by how this phenomenon has not changed throughout human history. It also seems that, more times than not, world conflict is rooted in religious perspective. This is ironic because religion, the ideal that should bring peace and unite us, often brings conflict and divides us. I wanted to address this issue somehow.

Being a newspaper reader, it could be said that my views have been shaped by a liberally-biased media, a media that focuses too often on negative aspects of our world. Some have said that if I focus instead on the positive, if I turn away from the ugly, I would view the things differently. Perhaps. I read and enjoy every bit of positive news. But, to ignore the problems of our world, especially human conflict, does not make it go away. Instead, I believe conflict and its root causes should be examined and brought to light, so we can become a better world, so we can survive.

Hence, idea of The Cave was born. I started the journey by writing various thoughts about life and the world, thoughts on how our world can improve. A theme of human survival soon emerged from my writing, so I contemplated a scenario of survival. Because the idea of human survival and associated conflict was deeply rooted in religion, I looked towards perhaps the one of the most religious cities in the world – Jerusalem. I researched the city extensively and found a cave – Zedekiah’s Cave – located directly beneath the Old City of Jerusalem. An ideal setting. The pieces of the puzzle were now before me. I started to organize and outline my thoughts and soon The Cave took on a life of its own. The journey was exhilarating. I hope you enjoy the story and the message it conveys --- Live with Love.

Publisher’s note: If you have questions about The Cave, the issues it confronts, or general questions for the author, David Click, please post a request on this blog. David will address each question received.

Recent Customer Reviews of The Cave

Read what others are saying about The Cave:

“Excellent book. Thought provoking story line and characters. I thoroughly enjoyed The Cave and could very much relate to the many questions that press on Maria's heart.”

“I really enjoyed this novel…. Compelling characters in a desperate situation made for a great adventure. Interspersed throughout the action were thought-provoking discussions and introspection about religion, faith, culture, and our duty to each other as human beings.”

“A must read for those looking to challenge and further their faith, spiritual understanding and their understanding of the world as a whole.”

“I enjoy books that challenge and inspire thought. For me this was that book.”

“I really enjoyed this book. It was thought provoking but it also had all the elements of a good read. My emotions were engaged throughout and I couldn't wait to see what would happen next.”

Order your copy today at http://amzn.com/0615832768

The Cave

The Cave was an absolute joy to write, and began by simply jotting down many of my longtime thoughts. Upon review, I thought "how cool would it be to somehow put these thoughts into a story." I had always dreamed of writing a novel, and now had the time while caring for my son. So, I started the process literally one word at a time. I clearly remember writing the first sentence, first paragraph, and first chapter and thinking "I can do this!" Before I knew it, a story emerged - a story that captures many of my longtime thoughts and questions about life that, I believe, are shared by many but rarely discussed.

I hope you like it.

David