This morning on my blackberry I had two Google Alerts. Not that it is unusual for sure, but today, these alerts pushed all of my buttons simply because of the language. They used words like "sufferers of Duchenne muscular dystrophy" and “genetic killer of kids”. We have to stop saying this. Our sons are not 'sufferers', in fact, they smile and their smile radiates and touches everyone in range. They are not 'suffering' as in pain and suffering. And, for the most part, I don’t see them suffering or hating their life. Rather, I see and hear from families that they love their lives.

Are we all projecting our 'suffering' on them? The fact that the word Duchenne causes our heart to break, that watching your son on the sidelines hurts, that the worry about what the future holds is painful, that as parents we often feel overwhelmed? Is it OUR suffering and we toss it over to our sons because, somehow saying we are suffering feels wrong? Adds to the guilt we often feel?

And “genetic killer of kids”…Our sons are living longer. Our expert panel members were not ‘kids.’ Duchenne is not a genetic killer of kids. Rather, it is a chronic disease that requires management. Our 'kids' become young men and adults. Let's not suggest they are dying as 'kids.' Let's be accurate. Duchenne is tough and we all want it treated and cured. But let's not frame this as if sons are suffering though life or that there is a 'genetic killer' lurking in the room ready to take his life at any given moment. Tell it like it is - Our sons need care, treatment, opportunities, and a cure. Without these things lined up, we all suffer.

Views: 174

Comment

You need to be a member of PPMD Community to add comments!

Join PPMD Community

Comment by Tammy on May 26, 2009 at 6:02pm
DMD is a genetic killer!!!! It Does take the lives of young boys. Sugar coating this disease is just absurd. I know in our own situation, my son fights harder because he knows what can happen. The thought of giving up because of a life limited diagnoses is not a reason to give up....it is a reason to fight harder. Life is what you make it. I do agree that a message is being sent as far as "suffering" goes...We all have hard times in our lifes and yes what our boys deal with is beyond anything we can grasp. But a picture is being painted, that our boys are "suffering" to the point that others my not find value in their lives. Our family has personally delt with this and quite frankly it is scary that someone thinks they can determine the value of ones live because of struggles or obsticules. We all know there are several factors that determine the life span of DMD...my son and I view this disease as a killer with no cure and until a cure is found it should be told that young boys are indeed facing death from DMD and we need all the help...not pitty or sympathy but help for a cure.
Comment by Perlita & Gordy Hains on May 4, 2009 at 10:02pm
God Bless you Michael for saying these things. Our beautiful son Levi is 2 & was very recently Dx w/ DMD - I smile brighter after reading your message - you've made an impact w/ me:)
Comment by Ian Anthony Griffiths on April 30, 2009 at 2:52pm
above comment in reply to michaels
Comment by Ian Anthony Griffiths on April 30, 2009 at 2:34pm
Well I have none of those, which then makes me feel like a lazy useless person. You may not suffer, but we are not the same, no two DMD's are the same. Again it doesnt detract from the point that children are still dying from this, and however old you are it is still a genetic killer, I always says its not just a childrens disease. If a hundred 21+ year olds die fighting for their country, theres national outcry, wheres that for DMD? Anything below 50 in a "normal" person would be a tradegy, yet people point at us supposedly "older" guys as wonderous events, yes its marvellous to get to 40+, but its still young.

What are we fighting here, each other about the symantecs of DMD, it is a killer and it is severely shortening lives. We shouldnt have to face "more than the usual number of challenges", all these things we have deal with, even before we get out of bed. I thought here we know exactly what its like to be living with it, not telling people their disease is just a "chronic" condition, like diabetes or something , when we get treatments then maybe we can call it that.

Sorry to go on

Staff
Comment by Pat Furlong on April 29, 2009 at 1:13pm
Everyone,
Thanks very much for the discussion. Accelerating the development of treatments and one day, a cure, is our mission. My original blog simply was to highlight that words do mean something and as a community we need to be careful about what words we use and in what context. Simply that.

I also am not willing or able to judge the quality of another's life. The quality of our lives is determined by a number of factors for sure, but the evaluation of that quality belongs to the individual.

From where I sit, I would not want my sons to hear over and over 'genetic killer of kids' and live as if they had a gun pointing at their head. It is not how we lived. We had very happy days (in spite of Duchenne) and some sad days, but the happy/fun/smiling days outnumbered the sad. My boys had Duchenne. It was part of our lives, but it did not ‘own’ us. We loved every moment we had together.

Clearly children die from DMD and a host of other problems, but typically the 'kids' with DMD live to be adults. This is exactly why we need Standards of Care, why primary care physicians need to learn about Duchenne, why we are working with CDC on outreach and education.

Suffering... well, only the individual can confirm whether, in fact, this is the case. And our sons' need to speak for themselves on this issue. As parents, let’s not judge them from our perspective.

I am thankful we are all able and willing to have a discussion about issues that are near and dear to our hearts. Thanks to all of you for that!

Ever since Duchenne entered my home, I felt a certain sensitivity to words - the things people say in kindness or sometimes in ignorance. Often, a neighbor would make a comment and I would have to work very hard to control my desire to scream or worse. So I listen carefully to words.

So, the bottom line is that we need to think about what we are saying, what words we are using and why. My choices may be different than yours at times and the same in others.
And while our boys are not statistics, when speaking to Congress, NIH, Healthcare professionals, Industry, we need statistics/evidence. Otherwise we are simply expressing feelings and emotions which does not change clinical practice or drive investment.

Ophelia, I would love to have a discussion about interventions and we will never have interventions without some risk of side effects.. I think we have far more than steroids in terms of interventions. Steroids, PT, night splints, supplements, diet, potentially growth hormone, bisphosphonates, idebenone or CoQ10, ace inhibitors, beta blockers, cough assist and ventilation have changed the natural history of Duchenne. It is not unheard of to see boys walking as adolescents and ome boys still walking at 16. We have several learning to drive. Improved treatments and additional interventions will never be fast enough and we all want/need better therapies with less side effects. I think about this nonstop and have always held the view to 'look under every rock'. To that end, we are currently reviewing proposals on novel approaches, asking for really smart scientists to think outside the box. UPPMD is also in a review process of novel therapies. The goal is to insure the pipeline is full of potential opportunities at every stage of the process and work to accelerate in every way possible.

I am fully aware that we need something yesterday. Today Patrick is gone 13 years. There is never one minute when the fight to endDuchenne is not in my head or heart.
I am deeply grateful for your opinions.
Warmest regards,
Pat
Comment by Ofelia Marin on April 29, 2009 at 11:36am
No one doubts that there have been advances in the search for an adequate therapy for DMD and I am really grateful for that! I am a realist and I believe in science; I read all the research in the field and when I have questions I ask the scientists working on it. Based on all I read so far, I do not believe a cure will be found in time for my son. Do I hope that there will be a treatment improving his quality of life in the next 5-10 years? Sure I do. But at this point in time no one can say more, not even the scientists working on these treatments. They all HOPE it is going to work and they all caution us that it will take several more years until something can be approved IF proven successful.

As I mentioned, for me, the average age of death is meaningless when the quality of life after a certain age is as it is. Just looking at the short to medium term prognosis for my son who is very young (I am talking about the “average” prognosis not the outliers). Last year, when my son was diagnosed, I was told that the age to wheelchair is 10-12 when using steroids. I do not see a significant difference from what Gretchen was told in 2001! Also, besides the fact that the steroids come with many known side effects, just recently I looked at these articles:
http://www.ncbi.nlm.nih.gov/pubmed/19375446
http://www.ncbi.nlm.nih.gov/pubmed/19233868
I read statements like “Prednisolone caused acute sarcolemmal injury, increased expression of myocardial TNF alpha and fibrosis, resulting in left ventricular dilatation and diastolic dysfunction.” or “DFZ lead to a decrease in the total number of muscle fibers and an increase in interstitial fibrosis.” and I ask myself, is this the gold standard for treatment in 2009? Is this ALL the modern medicine has to offer to my son TODAY to “improve” the prognosis? I really hope the future will be different!

I know I digressed from the original topic and I apologize for that!
Comment by Brian Denger on April 28, 2009 at 10:38pm
When working towards any long term goal it is necessary to stop and recognize one’s accomplishments from time to time, otherwise frustration, demoralization or disinterest takes over. Often factors change and there may be a need to reassess strategy after realizing the goal is further away or maybe closer. Even recognizing subtle changes or minor successes help in maintaining focus. Pat’s original topic and subsequent discussions can be seen as points along a path towards improving the quality of life and ending Duchenne.

Does it change what a family thinks about DMD when they read a child “suffers” from the disorder? I say yes. The word suffer is negative and we already deal with enough of that. Families don’t need the reminder. It is like referring to a person as “crippled”, an outdated and inappropriate term. Stating a child “suffers from DMD” implies there is constant pain and distress. I see more distress in parents of children who have DMD than with their children, so I think the term is misapplied. By no means am I saying that a child or adult who has DMD doesn’t have reason to feel sadness or is never in pain. My point is that this is not how all individuals who have DMD are living each and every day. Researchers have studied families of children with DMD and determined parents, particularly mothers, have higher rates of stress than parent who are not caring for a chronically ill child, while other tests indicate boys who have DMD cope as well as their unaffected peers. It is more suitable to say a person is “affected by DMD” and make the same point.

The other point to consider is with the progress made in the long term prognosis for DMD. My sons were diagnosed with DMD almost twelve years ago. The information available at that time was terribly bleak and while it still isn’t great, recognizing that progress has been made is important. I never expected my sons would survive beyond their mid-teens and yes, some boys still do not. With improved care and management these young men have a much better chance of survival well beyond those earlier standards. Much still must be done to improve the quality of life for those living with DMD. Educating the medical community about evidence based standards of care and providing families and individuals the support needed for independent living arrangements as longevity increases are among the new goals.

Looking ahead, our goals must shift. While working towards finding therapeutics that improves function, and extend and improve quality of life we also need the right people to care for this population. There is a need to expand the ranks of physicians caring for adults affected by DMD. The promotion of appropriate independent living arrangements and employment is necessary. Research progress is painfully slow as we watch so attentively, but changes are being made. As this happens we need to be mindful of these changes and plan the next steps. Staying optimistic may be difficult, yet focusing on the accomplishments that have been made will be crucial to that end.
Comment by Donna on April 28, 2009 at 10:24pm
Hello. My name is Michael and I am 41 years of age with DMD. I would agree with Pat Furlong. In my experience with DMD I do not suffer. Certain I feel I have more than the usual number of challenges. However-- I do live independently. I manage my nursing and medical care. I am college educated. I am employed. I travel frequently. I have hobbies. I especially enjoy adaptive digital photography and I have recently started sailing. They have sail boats equiped with sip and puff! I am in a loving relationship. I love my family I am active in my church and I have a strong faith in God. I have DMD but I live a joy filled life.
Comment by Ian Anthony Griffiths on April 28, 2009 at 7:50pm
I agree Ophelia
Comment by Ofelia Marin on April 28, 2009 at 2:59pm
My son is very young so I am not talking from experience. I did however read several blogs/postings from young men with DMD on the AD and other sites. I think that until any adequate treatment modifying the progression, improving their quality of life, allowing them not to struggle as much with basic things like breathing, feeding, bathing, toileting, dressing is found, we cannot consider the average age of death a meaningful measure of their life or anything for that matter.

Need help using this community site? Visit Ning's Help Page.

Members

Events

© 2019   Created by PPMD.   Powered by

Badges  |  Report an Issue  |  Privacy Policy  |  Terms of Service