PPMD President Pat Furlong and Colorado FACES Coordinator Ivy Scherbarth co-blog about the older patients in the Duchenne community, and what we can learn from the journeys they have been on.  They are the real Duchenne experts. We hope that after you read these entries, you'll post your own thoughts on the topic.


Listen & Learn

by Pat Furlong

 

From the day of the diagnosis of Duchenne, I started worrying about the quality of life for Chris and Patrick.  Internally, I would make the calculation – “if this happens, then Chris/Patrick will…,” planning for a future and trying to imagine life at a certain age, and what their world might look like.   And I worried constantly.   If the ‘if- then’ did not work out (my heart set on a certain strategy, drug or approach), what then?  What would happy to the quality of their lives?

 

Quality of life (QOL) is a measure used to evaluate the general wellbeing of individuals and societies.  There are a number of research instruments to measure QOL, and there is a unique methodology that links the results of subjective life-satisfaction surveys to the objective determinants of quality of life across countries.  The term is used by politicians and economists to measure livability within a given place, but perhaps the most commonly used international measure of development is the Human Development Index (HDI), which combines measures of life expectancy, education, and standard of living, in an attempt to quantify the options available to individuals within a given society.    Parents and individuals participating in Duchenne clinical trials are probably familiar with the Pediatric QOL or the NeuroQOL.  But no matter the instrument, QOL may be defined as the individual’s own perspective of his or her life.  It reflects the difference, or the gap, between what is hoped for and what is expected and the present experience. 

 

As I so often found myself predicting my sons’ QOL with the ‘if-then’ instrument, I learned that Chris and Patrick  had no such instrument.  They lived their life.   They loved their life.   We all wanted treatments and a cure, but in the meantime they lived.  They were happy.

 

During a recent parent panel, one of the parents shared her experience:

 

“ In the beginning I worried constantly  about what would happen when my son lost the ability to walk and my other son was still ambulatory or vice versa.  I heard a father with two sons with Duchenne tell a story that brought me great comfort.  After his older son was in a wheelchair his other son asked whether he would need a wheelchair too.  The father felt like he was punched in the stomach, but responded calmly, explaining that if his son needed a wheelchair one day, they would get one for him.  His younger son’s concern was simple, ‘I just want to make sure that mine goes faster than my brother’s.’”

 

I’ve also recently participated in panels with young men living with Duchenne. When the individuals on the panel were asked about barriers to good QOL, one individual answered:

 

“For me barriers are when society fails to accept me as an equal person. When people fail to see me as a person who can study, have a job, have a family, have children, and contribute to society.  No, I can´t walk on stairs – but I couldn´t care less. Walking on stairs can be practical, but it is not important to have a good and interesting life. Most barriers are mental. If people see me as a ‘normal’ person and not a disabled person, I don´t have any barriers.”

 

When asked about dreams for the future, one young man answered:   

 

“So far, I have been able to accomplish all of my dreams and have absolutely no regrets.  I can follow my dreams through to completion as I have a strong support system with many family members and friends, who are always willing to lend a hand, encourage me, and enable me to realize my dreams.  One example is when I wanted to go snow skiing this past winter and my cousins helped me accomplish this dream by making arrangements for an adaptive skiing instructor, going on the trip with me, and making it a life-changing experience.”

 

Another answered:

“While my dream is to be a professor in the field of Religious Studies, I know that wherever life takes me I will find a way to serve others and give glory to God.  Nothing stands in the way of my vocational goal, but it is important for me to stay open to the many different ways I can give back.”

And finally:

“Sometimes my life has a plan. Sometimes it doesn’t. My plan right now is to travel around the world next year. Still I haven´t been to New Zealand and Australia - and I want to go there. After that my plan is to begin a Master’s degree in communication and German language.  I finished my Bachelor degree in business communication in 2009. These are my dreams.  And yes, I am able to follow them.”

Our sons are becoming adults. They have dreams and expectations for their lives, to live fully, to love, to have careers, to have partners and friends, and to live independently.   We need to listen and learn.

"Fact of the matter is, there is no hip world, there is no straight world. There is only a world, you see, which has people in it who believe in a variety of different things." – Frank  Zappa


Pat Furlong is the Founder and President of Parent Project Muscular Dystrophy. Follow Pat at her blog.

Respecting our Elders

by Ivy Scherbarth

I think the message that we keep hearing from the adult men with DBMD is starting to sink in for me. When we ask them what they most wish parents and caregivers could understand about how to raise a kid with Duchenne, they always mention a few key things. Ok fellas, here goes. Is this what you're talking about?

 

We families living with Duchenne know that we no longer have the luxury of living unconsciously as soon as that diagnosis lands on our doorstep. It seems to me that once we get used to examining and questioning and evaluating our thoughts and motives, we can do even more to improve our perspective, and thus our parenting. We can envision a possible near future, one with better resources than we have today, but in which we are still working toward an emerging cure. This is a little bit like "fake it till you make it." It is a practice of behaving as though we expect success until we either believe it or forget about it once the habit of succeeding is established. It is possible to shape our thoughts toward the best vision of reality even as our emotions remind us of the worst possibilities. We can start expecting workable treatments before we try to imagine what they look like. We can expect our sons to live the best lives they can, as they are and as they will be, without trying to change them or magic away the facts of Duchenne. It sounds simplistic, but perhaps we can visualize our boys as the men we intended them to become from the very beginning, only with Duchenne. Perhaps if we never stop thinking of them as emerging adults, then they'll just go ahead and become themselves.

 

Suppose we were all to make a resolution together. Suppose we decide to stop looking at Duchenne as a killer of little boys. I'm not saying it isn't that too. I am certainly not trying to hide or disavow in any way the families who have lost their beloved sons much, much too soon. I'm just saying, suspend your disbelief for a minute and suppose we stop thinking of Duchenne this way. It's like looking at the glass as half empty or half full. There is the same amount of water in the cup. Duchenne is what it is. But we can choose our point of view. How would it be if we decided to see Duchenne as a chronic condition that we are living with instead of a disease that we are dying from? It's funny to think of it, just because we never do, but people are alive, right up until the moment that they die. All people, everywhere. We don't look at ourselves and think, yes, I am dying now, only 50 more years in my life expectancy. Of course not. We are alive! Well, couldn't we extend that thought to our own sons?

 

The face of Duchenne is changing. We parents think of our dear little boys, frozen at the moment of diagnosis. But that guy who just rolled up to the bar, yeah him, the one with the beard and kind of balding on top, the one with the fancy wheels and the tattoos? He is also the face of Duchenne. We can't live our lives trying to preserve some image of perfect toddler adorability in those magic presymptomatic days, letting that one moment color and blot out the reality of our changing children. We have to try to see them as they are, to see them as they see themselves. We have to let them grow up and grow old at their own rate and in their own way.

 

Even in the current absence of a cure, the absence today of any acceptable treatment, life expectancy for folks with Duchenne continues to rise. The oldest living man with Duchenne is over 45. Nowadays men with Duchenne often graduate from high school, college, and graduate school. They often pursue productive careers. Men with Duchenne have active lives. They go out, they date, they marry, they have children of their own.

 

Just look at the amazing power of modern wheelchairs, cough assist machines, sip and puff computer controls, the internet. Steroids are part of the standard of care and undeniably add years, but some of our elders have never used them. They are alive despite the inadequacy of our pharmaceuticals (though, of course, we are working on research for new drugs like never before). These men are alive not because we've come so much closer to the cure we all wish for, but because they've got better gear, better informed care providers, and better advocacy and support. Of course we still need to do everything we can to support new research and new pharmaceuticals. But even as we accomplish that huge task, we can also take on more quotidian problems, solving them piecemeal as they come up.

 

The men who are living with Duchenne today need their community to help them get some more resources established. They need more accessible housing. They need more professional caregivers. They need really good adult interdisciplinary medical care. They need financial planning and long term health maintenance planning. They need some help making the leap to independence, transitioning responsibility for their needs away from parents and onto themselves. They need us to trust them to be the men they were destined to be.

 

We have an entire generation of experts, men living with Duchenne, upon whose experience we can draw. They are writing books, making movies, telling their stories about how to live a good life as a man with DBMD. And it is a good life; the vast majority of men with Duchenne rate their quality of life very highly.

 

We have an obligation to listen to these guys. After all, they are the elders of this community and we should listen to our elders. Come to think of it, quite of few of these men with Duchenne are older than I am.

Ivy Scherbarth is a Colorado/Wyoming FACES Coordinator for PPMD. Follow Ivy at her blog, My Son, My Rain: A personal, biased account of one family living with Duchenne Muscular Dystrophy.

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Comment by Brian Denger on January 22, 2012 at 12:42pm

Even as a parent of two young men who have DMD I can only imagine what life is really like living with the disorder.  Sure, I have a fairly good idea, but I am not the person with the condition, it is my sons who actually go about their business, seemingly effortlessly, while they relegate DMD to a minor place of importance.  My sons are remarkable teachers and I stopped speaking for them some time ago learning to take their cue when I want to know what their lives are like or what their opinion is about a certain issue.  What I learned is remembering perspective is crucial when assessing what I observe. 

My opinion was much like many parents at the time of diagnosis.  I worried constantly about my sons' well being and ability to cope.  Things happened along the way allowing me to understand that if I continued being "dad", my sons would be fine.  Dads typically are responsible for encouraging their children to be the best they can along with the job of fixing things that are broken.  That doesn't need to change for a family affected by DMD.  Setting reasonable goals is still necessary and the "fixing" becomes a series of removing of obstacles and working through alternatives when our sons want to participate in an activity. 

Young men with DMD are surviving longer and not because of one specific therapy, yet largely because of a combination of therapies and interventions.  Combine cardiac care, noninvasive ventilatory support (NIV) and a power wheelchair and you'll begin to understand some of the components that lead to an improvement in outcome for someone with DMD.  1900's physicians tried to take credit for improving life expectancy in the general population due to improved medical practices when in fact improved methods of sanitation, hygiene and reliable methods of transportation and storage of food actually did much more to add years to people's lives.  The same is true in DMD.  The cumulative effects of these interventions have helped young men's health and quality of life.  Yet, it doesn't end there. 

Success begets success.  My opinion is that providing and/or encouraging these young men to set and pursue goals is as important as any other intervention.  The human spirit is as mighty a force as an other known.  There are innumerable examples of people overcoming adversity by sheer determination both within and outside of the DMD community that should encourage our sons and us as parents and family.  This is where our roles as parents change.  Instead of fielding fly balls with our sons we can look at removing the obstacles that prevent them from achieving their goals.  It may be finding a way for them to participate in an activity they love.  I've seen some interesting all terrain wheelchairs innovative parents/relatives have built for their son.  Or it might be challenging an administrator who can't come up with a way for a young man to participate in a science project because there is no easy solution.  This becomes our vocation. 

Life with DMD isn't easy, fair or is perfect.  Come to think of it, no life has all those components.  A John Lennon quote I like is "Life is what happens when we're busy making other plans" is relevant to this topic.  While we are busy wondering what may happen with those living with DMD many young men are actually in the "doing mode" and showing us what is possible.

Many thanks to Pat and Ivy for initiating this conversation.

Brian Denger

Comment by amit gupta on January 12, 2012 at 7:50pm

nicely put, tulika.

Comment by Tulika on January 12, 2012 at 4:55pm

A few days back there was a small new piece in an Indian news paper. A poor farmer has 2 sons with Duchenne about 16 years old. The farmer raised a mercy petition to Govt for mercy euthanasia. The petition was declined.

It is great to find there is advancement and Duchenne is a bit more manageable. I am grateful for quiet a few who participated in trials and brought out the medicines that current day kids use. 

All that apart lets not forget the poor father who asked for euthanasia of his beloved kids. There are thousands who are praying for cure. They dont understand QOL, rating scales, or cough assist machines. They long for the simple joys of life and wish for the supreme being to make its presence felt.

While I enjoy each and every moment I can with my kid, I have learnt from the elders in the community that cure is at the finger tips. I would not have learnt this if I did not have the drive to seek CURE. 

My future will be bright based on what I do now. I will not rest till there is cure.

Comment by Liisa Underwood on January 12, 2012 at 4:29pm

Another QOL perspective is the mutation type of Duchenne.  Those with different deletions or the stop codon issue all present their physical decline at different capability levels which perpetuates an individual perspective of QOL. 


Staff
Comment by Pat Furlong on January 12, 2012 at 7:55am

Hi Betsy,  Quality of life is a very individual perspective.  We have interviewed a number of adults with DMD who believe their QOL is very good.  In the film, when asked to rank QOL from 1-10, with 10 as the highest value, the adults interviewed ranked their lives between 8 and 10.    There is no doubt this is a very complex issue.  The primary caregiver (typically the mom) has a 24/7 job of providing care which becomes increasingly difficult as the primary caregiver ages and tries to balance /manage his/her own life.   In so many families, mom spends every moment providing care, arranging medical care, navigating the system (medicaid, insurance, services), often ignoring her own mental and physical health.   These are incredibly painful and difficult issues for families and need to be addressed.


Staff
Comment by Pat Furlong on January 12, 2012 at 7:41am

Ofelia, there are several papers written on this topic and several in press. 

Moxley RT 3rd, Pandya S, Ciafaloni E, et al. Change in natural history of Duchenne muscular dystrophy with long-term corticosteroid treatment: implications for management. J Child Neurol. 2010 Sep;25(9):1116-29. Epub 2010 Jun 25.

Clearly steroids, physical therapy (afo, stretching)  cardiac interventions, improvements in respiratory care and ventilation have contributed significantly.  Other factors have contributed to include better understanding of the pathophysiology  as well a philosophical shift from therapeutic nihilism to proactive care and (at least in some areas) earlier diagnosis.

Comment by Betsy Fields on January 12, 2012 at 7:12am

I'm commenting as a frustrated parent of an elder DMD "kid."  When did "quantity" of life become preferable to "quality" of life?  Most of us give heart meds, which definitely improves the heart, but now something else loses function.  So, we treat that.  Once that is treated, then something else loses function.  And, on and on we go.  What are we actually accomplishing? 

Comment by Ofelia Marin on January 11, 2012 at 4:06pm

"Even in the current absence of a cure, the absence today of any acceptable treatment, life expectancy for folks with Duchenne continues to rise. "

 

What data do you base this on? How recent? Do you compare the life expectancy to the one 10, 5, 2, 1 year ago? Before and after steroids, ventilation, heart medication introduced in earlier stages?

I am sorry but it is just an inaccurate statement. Life expectancy increases due to more aggressive care and treatments and NOT in absence of treatments. The only way to extend life expectancy for Duchenne and other diseases is a better understanding of pathology and more aggressive, targeted, more appropriate treatments.

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