Lucas Camilo

Despite the sweltering heat (which Lucas is NOT a big fan of), we’ve managed to get out for a few short trips recently.  On Sunday morning – with the heat index foretasted to be over 110 degrees in the afternoon – we packed our gear up early and attempted to beat the crowds to Washington DC’s tiny “National Aquarium.”  It’s not much more than a big basement with some fish tanks, but Lucas was totally captivated.  He did the “fish” and “turtle” signs about a thousand times, and then we tried to guess what the sign was for “alligator.”

This morning we took him to his first ever “story time” at the Mt. Pleasant library up the street.  Victor and Krista took Lucas, and they rolled his stroller right up to the circle of about 30 toddlers.  The kids (and especially their parents and caregivers) were taken aback by Lucas’s equipment at first, but we got to make a special announcement showing off our suction machine, and that seemed to be enough to calm most people’s curiosity.  Lucas doesn’t exactly love to have a crowd around him singing and clapping, so he cried through the first half of story time and pleaded to go.  But when we backed up a little and positioned ourselves where he could see the ceiling fans better, he calmed down and even made small “cat” and “duck” signs along with the story the librarian was reading.  By the time it was over a number of the kids came over to say goodbye.  Lucas’s tears had dried by then, and he even raised his hand to wave small goodbyes.

The arrival of late summer means transitions around our house.  Florence left with her family for a month in Uganda, so today Lucas is getting to know a new nurse.  His therapists are also taking vacations, so he’s had less visitors to the house recently – meaning he has more time in his calendar for people to come play or read books.  Meanwhile, tomorrow is Burke’s last day at Center for Community Change, the (day) job he’s been at for the past year and a half.  Finally, Krista is off this weekend to Minneapolis to attend the MTM-CNM Family Conference.  We’ll be sure to report on that adventure next week.

One of the highlights of our road trip north a few weeks ago was the visit to the Beggs Lab in Boston.  In a way, going to the lab was the ultimate destination of our journey up the eastern seaboard.  Though we knew that we weren’t going to come away with any major revelations, we also figured this would be the one time to take Lucas to the lab and meet some of the researches there before moving west.

So despite limited expectations, we were excited to walk into the lab on June 27 and meet the famous Alan Beggs.  In fact, Dr. Beggs is a very gentle, thoughtful man (who’s really only famous in the world of children with severe muscle disease), and he invited us into his office for a long chat, ready to answer any and all questions we had for him.  Dr. Beggs and the genetics counselor Elizabeth (who we had communicated with prior to the visit) were excited to meet Lucas, and it occurred to us that these researches probably didn’t have many opportunities to meet the wonderful children like Lucas who are affected by MTM.

The meeting started off with a bang: we laid Lucas down on Dr. Beggs nice couch and within minutes he had filled his shorts (after a 3-day hiatus from so doing!) and was gurgling with pleasure at the feat.   Meanwhile, Dr. Beggs had launched into an overview of the different techniques being researched as possible treatments of myotubular myopathy.

First, he explained that they had already come along way in figuring out that the particular gene mutation seen in people with x-linked MTM affects the production of an enzyme called myotubularin.  Lucas’s low muscle tone (and that of other people with MTM) results from the lack of myotubularin, which in turn prevents the proper development of muscle fibers.  One thing we learned from Dr. Beggs and Elizabeth is that, based on the reading of Lucas’s DNA test for MTM, his body is probably not producing any myotubularin, whereas some kids with less severe forms of x-linked MTM do produce some of the enzyme.  They were able to determine this because the test shows that the defective MTM gene is missing a pair of amino-acids.  Since genetic information comes in triplets, missing a pair throws the entire sequence out of wack and likely means no myotubularin is produced.

Dr. Beggs explained the different directions of research for possible treatments.  Their lab is looking into gene therapy, the technique we had heard most about, in which the mutated gene is replaced by a “good” gene mechanism.  Researchers are trying to figure out how to deliver the new genetic information to all areas of the body, perhaps attaching the new genetic material to a virus to help it spread.   The concept of gene therapy has been out there for a while but researchers are still far away from making it work – whether as a treatment for MTM or any other disease.

Researchers have also been working on a technique for infusing the blood with the missing myotubularin protein.  It has been tested on mice with positive results and in recent years there has been excitement about finding a Labrador dog that also carries the MTM mutation to begin testing on.   Still, the method is in the early stages of development and needs a lot more research before it could have potential with humans.

Another area of research involves helping the muscles grow in strength, despite the missing myotubularin.  One possibility they’re pursuing is a muscle transplant-like procedure.  A person would be given new muscle fibers, which would then reproduce using the stem cells of the non-MTM muscles.  And finally, there are researchers looking into the possibility of giving muscles the capacity to grow bigger by inhibiting the action of an enzyme called myostatin that regulates our muscle growth.  Apparently, animals who have this naturally occurring genetic disorder are able to grow enormous muscles. (Beggs showed us pictures of really enormous-chested dogs and cows.)  If it worked, this kind of treatment might be useful not just to kids with MTM, but to people with other muscle diseases as well.

We knew we wouldn’t get any promises of treatments being available soon, and when we asked Dr. Beggs he walked the line of not giving us too much hope, but not dashing it, either.  He said he didn’t think anything would be ready for trial on humans in less than 5-10 years.  And he added that researchers gave a similar timeline for duchenne muscular dystrophy in the late 1980s, and yet they still haven’t found a treatment today.

The experience of being at the lab and in Boston was an important one for us and we left with a much better understanding of what Lucas’s disease is all about.  But the conversations at the lab were scientific and “interesting,” not personal.  It was only later, as we drove away, that we started to feel the heaviness of what we were learning.

Our belief has been that Lucas’s steady progress in terms of movement and strength will continue to be an upward curve.  After the experience in Boston we’re now aware that might not be the case.  Some boys who are on the more severe end of the MTM spectrum (as Lucas appears to be) have reached a peak at a young age in terms of strength and movement.  As their bodies got bigger and heavier to lift they’ve encountered more challenges, like having a hard time interacting and communicating without augmentative devices.  It’s scary to hold the possibility that all the amazing steps Lucas taken – especially in terms of his ability to use his arms and express himself through sign language – may not continue to expand.  In fact, scary is an understatement.  It’s downright awful, at times, to think about the future and the fact that Lucas could face even more physical barriers than he does now.

Of course, by the time we got home we were back to our regular routine of enjoying hanging out with Lucas and all his brilliance, goofiness, fussiness, challenges and strengths.   We continue to get excited about all the progress he makes with sign language, physical development, and everything else. Celebrating Lucas for who he is in the present remains our guiding philosophy, even if new knowledge sometimes makes us nervous about the future.

On the home front, Lucas and Burke had a good weekend in Washington DC – Krista was missed, but support from nurse Florence on Thursday and Friday allowed Burke to make it to work and meetings, and left the entire weekend for some quality time.  Nice weather in the mornings allowed them to lay out on the front lawn — swinging in the tree while battling mosquitoes — and some visits from friends gave Burke the occasional respite from non-stop book reading.  Lucas was in good spirits throughout the weekend and he (mostly) enjoyed the cranked up selections from Burke’s vinyl record collection (the harder-core punk rock sometimes got the “all done” sign.)  Whenever Krista called he would do the sign for “mommy” (thumb touching chin with fingers extended) and smile broadly.  He also learned to kiss his favorite new mini-stuffed ducky on the beak, which is pretty funny to watch.

Lucas made a lot of noise over the weekend too.  He isn’t always in the mood to verbalize but his voice is getting louder when he decides to pull it out.  He often tries to verbalize words for animals when he makes a particular sign.  He can now speak emphatically the words “hi,” “in,” “on,” and “aaat” (for cat).  He also tries to say dog, which comes out as “nooooan” while he pats his thigh to make the sign for dog.  It is ridiculously cute.

Thanks to everyone for the outpouring of love and support (this is Krista writing again) in response to the post about my dad.  I had an amazing trip to Jackpot, and it felt wonderful to travel with all your support — texts and emails and calls and letters and comments on the blog.  The place was stunning, with wide-open, huge-sky beauty.  I traveled there with my uncle Bill and aunt Becky, my cousins Garrett and Nolan, and family friends Rogelio and Gloria.  Rogelio worked closely with my dad for nearly 15 years, and he built a cross to put at the sight of the crash shortly after my dad died.  So part of the trip was about finally placing the cross (which is featured in my pictures from the trip).  Visiting the place of his crash brought me a sense of peace that I hadn’t expected.  The northern Nevada plateaus were so lovely that who knows, maybe we’ll take Lucas one day.

(Krista writing) Tomorrow I’m flying to Idaho, then driving to Jackpot, Nevada, to mark the 10 year anniversary of my dad’s death.  It’s strange for me to have so many people in my life who never got to know my dad – he’s such a big part of who I am today.  So I thought I’d write a little about him here.

Gary Craig Hanson was brilliant, warm, and incredibly hard working.  He taught me how to build a house (when I was five) and how to ride a horse (when I was ten) and how to save money and run a business (when I was still way too young to question capitalism).  Somewhere I still have a list he helped me to make with my short, medium, and long-term goals scrawled in pencil on a torn-out piece of notebook paper.  My dad was also deeply emotional, at times passionate and happy, at others angry or overwhelmed with despair.

My dad died on July 7, 2001 when he was flying his small plane to a “fly-in” with other pilots in Jackpot.  He’d had his license for about five years and had flown that plane dozens of times.  Weeks later, when we were going through his file cabinets, I found a folder with old pictures of airplanes.  There was a cover letter from TWA to Gary Hanson, saying they had enclosed the pictures of planes he had requested.  It was dated 1950 – he’d been dreaming about flying since he was five years old.

On the day of the fly-in, when he banked the plane to turn to land, the conditions were just wrong enough that the wing tipped and the plane spun out of control.  At the time I was living in Cholula, Mexico and had no phone or computer, so it took 24 hours for word to reach me.  My uncles drove to Nevada and brought his ashes home to Oregon.

It’s been a strange thing to become a parent without having my dad here to witness and support and love Lucas and me.  I know he would have absolutely loved Lucas, and I know it would have made him happy to see me grow into being a mom.  He used to tell me how much he loved learning from my sister and me, and we were still practically kids (20 and 24) when he died.   So I know if he were still here he would have been enthusiastic about learning everything Lucas has to teach.

I’m not exactly sure what I’ll get out of this trip, but I’m thankful to Burke for encouraging me to go.  I’m also glad my uncle, aunt, cousins, and family friends will be there with me.  I’m looking forward to knowing what the place looks like – to seeing the scenery he saw last.  I’m looking forward to telling stories and remembering him.  And I’m hoping to find a place in the desert to sit and begin to tell him everything I’ve learned in the last ten years.

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Prayer for My Father

May your beautiful, graceful spirit know how deeply you are loved.

May we carry on your legacy of courage and curiosity,
service and awe at the workings of the universe.

May we continue your work of learning
self-acceptance and forgiveness.

May we wake each morning with gratitude
for the tree or mountaintop or field or flower
we can see from our kitchen window.

May we find connection through humility,
admitting our weaknesses and
wearing our greasy work clothes in public,
even when it embarrasses our children.

May we have the courage to dream radical dreams,
to believe that we will one day fly,
and be fearlessly content regardless of the outcome.

May we know the tenderness of a listening parent
and the truth in stillness.

May we forgive ourselves, our families, our friends,
then strangers and enemies,
knowing they, too, dream secret dreams.

May we treat animals with kindness and respect
and a love that recognizes equality of all beings living on this planet.

And may our hearts find freedom
so we can fully behold the magnificence of this life.

Gary Craig Hanson, Sept. 10, 1945 - July 7, 2001