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FDA Advisory Committee (AdCom) hears the voice of the DMD community

On September 28, 2017, the U.S. Food and Drug Administration (FDA) held an Advisory Committee (AdCom) meeting to discuss the new drug application for ataluren for oral suspension (PTC Therapeutics Inc.). This committee allows stakeholders to present additional data, information and views on treatment. At the end of this hearing, the AdCom voted that more data is needed to prove that ataluren works in children with Duchenne muscular dystrophy (DMD).

About the results of the vote

The FDA Peripheral and Central Nervous System Drugs Advisory Committee voted overwhelmingly that efficacy data, supporting the PTC Therapeutics Inc. application for marketing approval of ataluren, was inconclusive (10 of 11 votes). Only one vote supported the position that clinical research data showed that ataluren was effective in children with DMD due to a nonsense mutation in the dystrophin gene.

Overall, this vote means that the FDA will continue their conversation with PTC Therapeutics Inc. The information presented to the AdCom will be considered as part of the company’s total submission for marketing approval.

La Force meets the DMD community

The La Force team was present at the AdCom meeting. We interviewed many families whose children with DMD have been on ataluren. They were all very positive about the benefits, and they felt that the FDA heard them. This blog gives an overview of what they had to say about the event, the benefits of treatment, and their reactions to the decision.

The role of families at the AdCom

Being able to complete a Lego set. Having more global energy to study. These incalculable benefits rarely emerge from clinical research data. To go beyond the data, the AdCom heard patient and expert testimonials.

 

Betty Vertin, mother of three boys with DMD

“Basically, it is a meeting that gives us an opportunity to put a case together in front of the FDA. They looked at the data, for them, and the data did not make a convincing argument that ataluren worked. So, they pulled in this group of experts. Basically, they’re giving us our day in court and we can put a face to the data and show what benefit really looks like.”

 

About overall benefits

Many benefits cannot be calculated by clinical research data. For example, for a boy with DMD, having more global energy to study is life changing. This is the reason why testimonials from children and young adults are very important at AdCom meetings.

 
Darrell Knight, father of Jack Knight, a 17-year-old with DMD

“Like I said, it varies from boy to boy, but most boys will usually lose their ability to walk and be in a wheelchair by the time they’re 8 years of age. Roughly around that area. My son was 14, almost 15 years of age. Did that have to do with the drug? We believe so.”

 

Making more treatments available

Eteplirsen is only approved for a small fraction of patients with DMD. The only other FDA-approved therapy, the corticosteroid Emflaza™ (deflazacort), isn’t a cure. Every treatment that gets marketing approval helps to move towards a cure and encourages the pharmaceutical industry to invest in finding new DMD therapies.

Betty Vertin, mother of three boys with DMD

“Getting one more through the door, getting one more approved, opens up the door. More companies are going to invest in Duchenne and be willing to go to bat for us, and those boys deserve that opportunity. So, even though this is a win for a small population of boys who will get access to the drug, it would be an even bigger win for the community.”

Darrell Knight, father of Jack Knight, a 17-year-old with DMD

“I think the FDA is looking for that home run ball, that in-the-park, grand-slam home run. For us, it’s not that at all. For us, it’s base hits. If we get a base hit here, a base hit there, we’re going to be in the ball game. We have to have the base hits, where they’re looking for the home runs. I think, if we can get them convinced that a base hit is worth it, then I think, we’re going to be moving on and I think it’s going to be very encouraging.”

 

Voices from the DMD community

The AdCom listened to voices from the DMD community. What they heard was compelling. Even if the clinical data didn’t convince them of this drug’s efficacy, their words – and message – had a positive impact.

Angela Willette Knight, mother of Jack Knight, a 17-year-old with DMD

“They gave us the opportunity to go and get more information. And they confirmed that what we do have is compelling. So, I think the fact that it is compelling is very huge. And we were heard, and that was part of the totality of the data and part of what went into the decision process.”

 

Anything could happen

The FDA typically follows the advice of its advisory panels. But, last year, an exception occurred when the FDA approved, for the first time, Exondys 51 (eteplirsen; Sarepta Therapeutics), a drug for children with a specific cause of DMD.

Our team feels very good about the positive feedback that the FDA gave to the DMD community. The story will continue on October 24, 2017, when the FDA will make a final decision. At this point, anything could happen.

Interesting links

https://seekingalpha.com/news/3298268-fda-ad-com-says-data-ptcs-translarna-inclusive-needed-prove-efficacy

https://pharmaphorum.com/news/fda-reviewers-slam-ptc-dmd-drug-ahead-key-meeting/

http://www.xconomy.com/new-york/2017/09/28/fda-panel-pans-ptcs-audacious-plan-to-force-vote-on-duchenne-drug/

Emflaza

Exondys 51 (eteplirsen; Sarepta Therapeutics)

Translarna (ataluren; PTC Therapeutics Inc.)

TAKING ACTION – PORTRAIT OF DUCHENNE – WHEN LIFE CHANGES

Alex Smith

CEO, Harrison’s Fund, United Kingdom

In this eighth interview of our series “ Portrait of Duchenne ”, La Fondation La Force talks with Alex Smith, CEO of Harrison’s Fund, a British-based, leading charity for research into Duchenne muscular dystrophy (DMD). Alex’s life changed when his son Harrison, now 11, was diagnosed with DMD. He decided to do something to make a difference.

 

Before diagnosis

Alex worked in brand marketing in an area near London, England. Every night, he came home to a lovely family, his wife and two sons. As a father, he had many dreams for his children’s future. He hoped that they would grow into productive young men, who would wed and start families of their own.

Alex wasn’t a sporty guy. For at least 20 years, he hadn’t worked out or participated in any sports. In fact, he was physically inactive.

He knew nothing about charities and how they worked.

 

After diagnosis

Then, his son Harrison was diagnosed with DMD.

“When it first happened, it was a really tough moment. The doctor, like (for) many other families, told us our son was going to die and that we should take him home and give him a good life. And, in that moment, our world changed, because it just felt incredibly numb,’’ Alex told us. ”

But Alex realized,

“I’m one of those people who needs to pick himself up and do something about it.”

He started Harrison’s Fund and dedicated his life to the charity.

“We raise money, raise awareness, then give that money to some of the best researchers in the world to try to make a profound difference to all children with Duchenne everywhere.”

 

Ironman

Alex has learned that every action, every movement counts. In 2016, he signed up for an Ironman competition to raise funds for research, but that wasn’t all. “What I wanted to do was (to) have something for Harrison that he could do with me.”

The two trained hard and raced to the finish line together.

“Finishing it with him was probably the most emotional day of my life,” Alex told us. “I remember coming around… the final finish corner with him, going running down the finishing chute, crowds everywhere, Dimitri with us, and he turned to me just before we went down the chute and said, ‘Daddy, we’re going to be Ironmen together.’”

 

DMD changes your life in a split second

The progression of DMD is unforgiving: the body’s muscles gradually weaken, leaving children with DMD in a wheelchair by age 12. They have a life expectancy of 20 to 30 years. There is no cure and, in Canada, no treatment for the cause. Two treatments for DMD are approved in the USA and Europe, but they don’t benefit all children with DMD. Promising treatments, in development, are expected within 5 to 10 years.

 

What’s next for Alex?

Stay rooted in the present. Enjoy every moment with your child. Be part of the solution.  

“The most important aspect of trying to remain positive is choosing to be positive,” Alex says. “Nobody else is going to do it for you, are they? You can’t sit back on your hands and expect everybody else to get it done, because they all need help as well.

 

“That allows the community, the global Duchenne community, when they see positive people, to keep hold of hope and to grasp it. And if they do that, then we’ll get there. And I truly believe we will.”

 

 

Acknowledgements

We thank Action Duchenne, who received us with open arms to conduct a series of interviews.

  

Action Duchenne   –   Harrison’s Fund 

To receive the next interview in our series “Portrait of Duchenne”, please subscribe to our newsletter.

Watch Alex’s full story HERE

IT’S DONE! 704 KM IN HOPE TO CURE DUCHENNE

It’s done! After completing the SSQ-Marathon Québec-Lévis this Sunday, August 28. Jean-Philippe Morand and his son Victor have cycled and ran a total of 704 KM. The father and son are on a mission to raise awareness about Duchenne. Every effort to raise awareness about this rare and devastating disease will help us facilitate access to new treatments.

Victor was diagnosed with Duchenne

DMD is an incurable and 100% fatal disease. Duchenne muscular dystrophy is a rare genetic disorder that affects mainly boys. The progression of DMD is unforgiving: the body’s muscles gradually weaken, leaving the boys in a wheelchair at the age of 12, with a life expectancy of 20 to 30 years. Today there is hope for DMD, and several new treatments are on the horizon.

Raising funds and awareness to access new treatments

Within the next 5 to 10 years (or even before then), some new treatment options should be on their way to completion.  Now, two treatments are approved in US and Europe, but not yet available in Canada.  Therefore, educating people about what Duchenne is and about access to new treatments is extremely important.  It will help children like Victor access the new treatments rapidly before the disease has evolved to much.

Pioneers in the Canadian DMD community

La Force has been associated with “Dystrospin” since 2016.  a fundraising event organized by the Morand family for the past 4 years.  A portion of the money raised was invested in the purchase of a suitable wheelchair. It’s because of this kind of generosity that Jean-Philippe can participate in sports events to raise awareness about Duchenne muscular dystrophy and access to new treatments. This year the “Dystrospin” will give the total profit of his event to two Quebec based researchers. Will give to Dr. Jacques Tremblay research on CRISPR-Cas9 and will give to Dr. Jérôme Frenette to his research on

Support our cause

There’s nothing better than a father’s courage to raise awareness about Duchenne muscular dystrophy. Today there is hope for DMD, and several new treatments are on the horizon. This hope motivates Jean-Philippe to go beyond his limits for his son; but what he does also benefit all children with DMD.

Every effort to raise awareness about this rare and devastating disease will help us facilitate access to new treatments.

We at La Force are proud to support Jean-Philippe Morand and his family.

To learn more:

·         Dystrospin

·         La Force & Dystrospin association 

·         Running chair

·         Research Dr. Jacques Tremblay – CRISPR-CAS9

·         Research on Osteoprotegerin

ANOTHER MARATHON FOR JEAN-PHILIPPE AND VICTOR!

How far would you go for your son? For Jean-Philippe Morand and his son Victor, the answer is 704 KM. The duo completed the Ottawa Marathon in May and “La Randonnée Jimmy Pelletier” in June (620 KM of cycling). This Sunday, August 27, they’re up for another marathon, this time in Québec… The father and son are on a mission to raise awareness about Duchenne Muscular dystrophy.

Victor was diagnosed with Duchenne

DMD is an incurable and 100% fatal disease. Duchenne muscular dystrophy is a rare genetic disorder that affects mainly boys. The progression of DMD is unforgiving: the body’s muscles gradually weaken, leaving the boys in a wheelchair at the age of 12, with a life expectancy of 20 to 30 years. Today there is hope for DMD, and several new treatments are on the horizon.

Raising funds and awareness  to access new treatments

Within the next 5 to 10 years (or even before then), some new treatment options should be on their way to completion. At this time, two treatments are approved in US and Europe, but not yet available in Canada. Therefore, educating people about what Duchenne is and about access to new treatments is extremely important. It will help children like Victor access the new treatments rapidly before the disease has evolved to much.

Leading the way for the DMD community

La Fondation La Force has been associated with “Dystrospin” since 2016, a fundraising event organized by the Morand family for the past 4 years. A portion of the money raised was invested in the purchase of a suitable wheelchair. It’s because of this kind of generosity that Jean-Philippe can participate in sports events to raise awareness about Duchenne muscular dystrophy and access to new treatments.

Support them

There’s nothing better than a father’s courage to raise awareness about Duchenne muscular dystrophy. Today there is hope for DMD, and several new treatments are on the horizon. This hope motivates Jean-Philippe to go beyond his limits for his son; but what he does also benefit all children with DMD.

If you’re attending the marathon, please encourage them. A group of friends will help the father and son go on the last stretch of the marathon, a very steep hill.

Every effort to raise awareness about this rare and devastating disease will help us facilitate access to new treatments.

We at La Force are proud to support Jean-Philippe Morand and his family.

 

To learn more:

Photo Credit:

PORTRAIT OF DUCHENNE – Ravi’s good life

Ravi Metha

Corporate Development Intern at Muscular Dystrophy UK

In this seventh interview in our “Portrait of Duchenne” series, La Force talks with Ravi Metha. Ravi has a business degree and Corporate Development Intern at Muscular Dystrophy UK. As a 27-year-old with Duchenne, he has a good life. This interview is his word of wisdom for all of us.

 

Ravi is committed to living life to the fullest despite DMD!

He has deep brown eyes that are full of life and determination. He loves to grab a drink in pubs with friends in London… If the place isn’t accessible for wheelchair, it doesn’t matter, because he’ll find a way to get in there. You have to fight to live a normal life when you have Duchenne. He has a message for all of us: either you’re part of the DMD community… or you’re not! To people who are completely healthy but sometimes feel life sucks, o every single one of us with preconceptions about disabled people, o the parents, to the young child, to the young adult with DMD:  

 

“Don’t just give up, never give up, on living a normal life. I’m always saying don’t give up and don’t take no for an answer.”

 

In the video, Ravi Metha answers our questions about living with Duchenne

 

  • Overcoming the challenges of living with DMD

When I was younger there were challenges like getting help during the daytime, getting dressed, personal care, going to school, basically all walks of life. But as I got older I learned to adapt more, I learned, you know, compromise … kind of a big word that you have to get used to, you know, because, sometimes you’re gonna get your way but, you know, other times you have to fight quite a lot to get what you, kind of… need and what is rightfully yours to have. In terms of having… going to a public school, you have to fight for that, going to university you have to fight for that, having the care I need you have to fight for that. So it is a lot about fighting, and so you know, that’s kind of, you know, my daily life, to be honest. But as I said, I’ve got older now, I still have barriers, but, you know, it’s something I don’t think about; I always think about getting past it, you know. I don’t, I’m not gonna be like, seeing there’s no access getting into a pub or a bar doesn’t stop me, I still get in there. But you know I try to live life as full as I can.

                                                              

  • What would you like people without Duchenne to know that would make your life better?

Well, you know, I think what I come across is… they see physical disability so they see me having a mental disability as well. So, you know, there are people, you know, that are quite condescending, quite patronising, because they think: “oh, he’s in a wheelchair, so he must be disabled in every part of him”, so that’s a big barrier for everyone, not just in the UK but everywhere around the world. So I mean, yeah, having people know that your mental capability is still there, even though your physical capability is not the same as everyone else. And also, you know, we’re normal. We’re still normal human beings, I can be in a wheelchair but, you know that shouldn’t affect anything.

 

  • Your words of inspiration for other people with DMD

If there’s a barrier or if there is, like, an issue going somewhere you need to go, don’t just give up, never give up, ‘cause otherwise, you’re going to be the same all the time, you won’t live a normal life. I am always saying ‘don’t give up and don’t take no for an answer’. And, yeah, just, I always, am just in a wheelchair but that is nothing to me; I still feel like I have the same rights as everyone else and that what I fight for.

 

To receive the next interview in our series “Portrait of Duchenne”, please subscribe to our newsletter.

 

 Acknowledgements

We thank Action Duchenne, who received us with open arms to conduct a series of interviews.

 

LET’S JOIN FORCES TO HELP MICAEL!

The total cost of adapting a home is mind-boggling: costs can amount up to $300,000. In the case of Duchenne muscular dystrophy, these are huge amounts year after year. The support provided by governmental assistance programs is not sufficient to cover all of these costs. This is the case for Micael and his family.

Micael, 22, lives with his mother Pierrette. Afflicted with Duchenne muscular dystrophy, he has been restricted to his wheelchair for several years now. At this time, even the simplest everyday actions are becoming obstacles. For Micael, taking a bath has become extremely difficult and is causing his mother Pierrette serious back problems (facet syndrome). This affects her ability to work, and she is the financial pillar of her family. In fact, it’s become impossible for her to lift her son because he has become too heavy. It’s now inevitable for the family to install a rail-lift on the ceiling in order to facilitate access to the bath and the toilet for Micael. In the absence of adequate facilities, it will become almost impossible for the family to remain together.

Unfortunately, installation of this equipment represents an approximate amount of $10,000. There is a government program called the PAD (in Quebec) that provides $16,000 over a five-year period. This amount has already been paid to the family for other work. The cost of adapting a home for a young adult with DMD goes way beyond the amount granted within the framework of this program.

It is for this reason that we ask for your help to finance this initiative for this family. This is an essential need in their lives.

However small, every amount is important! This way to Micael’s fundraising campaign:

Micael’s fundraising campaign

Facet syndrome 

Rail-lift

Residential Adaptation Assistance Program

Jean-Philippe and Victor race for DMD

Learning that your son is suffering from Duchenne muscular dystrophy is watching your child lose his muscular ability month after month. This is Jean-Philippe Morand’s reality: his son Victor is stricken with Duchenne muscular dystrophy. In adversity, some men go beyond their limits, and this is what Jean-Philippe will do this Sunday, May 28, by participating in the Ottawa Marathon in the company of his son Victor.

Actually, for the past 4 years, the Morand family has been holding a fundraising event called the “Dystrospin”. A portion of the money raised was invested in the purchase of a suitable wheelchair. It’s because of this kind of generosity that Jean-Philippe is able to participate in the Ottawa Marathon with his son. There’s nothing better than a father’s courage to raise awareness about Duchenne muscular dystrophy.

 

Hope for treatment

Duchenne muscular dystrophy is an incurable and 100% fatal disease. Duchenne muscular dystrophy is a rare genetic disorder that affects mainly boys. The progression of DMD is unforgiving: the body’s muscles gradually weaken, leaving the boys in a wheelchair at the age of 12, with a life expectancy of 20 to 30 years. Today there is hope for DMD, and several new treatments are on the horizon. This hope motivates Jean-Philippe to go beyond his limits for his son; but what he does also benefit all children with DMD.

Every effort to raise awareness about this rare and devastating disease will help us to facilitate access to new treatments.

For this reason, we, from the bottom of our hearts, invite you to share this story of courage.

We at La Force are proud to support Jean-Philippe Morand and his family.

 

To learn more about:

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Disability doesn’t stop Shaan from driving!

You CAN drive with Duchenne Muscular Dystrophy (DMD)!

Being able to drive is a dream that means a lot to any teenager. Shaan Lail is living that dream. Even though he has Duchenne Muscular Dystrophy (DMD), Shaan will soon be driving his own modified SUV. Shaan is the first Canadian to drive with a state-of-the-art Swiss electronic driving system called Joysteer. This new technology has given more independence to people with disabilities.

Since Shaan was little, he’s always been really into cars, so being able to drive his own vehicle is a real dream come true for him.

“I didn’t wanted DMD to steal away the pleasure of driving,” his mother told me right away. “You see, this disease takes so much away from these boys and young men. Ever since he was very small, Shan’s always talked about cars, cars, cars and more cars…  I wanted to do everything in my power to make sure this dream wasn’t one of the things he was robbed of.

Shaan is doing great in his life. At 19 years old, he’s a student at the University of British Columbia (UBC). His long-term professional dream is to become a lawyer.If you’re familiar with DMD, you know that it is a degenerative and progressive life-limiting disease that relentlessly steals away muscle strength. But, somehow, Shaan is defying DMD.

Behind every child with  Duchenne Muscular Dystrophy (DMD), there is always a strong parent.

I asked Shaan’s mother, Vee, how she manages to keep her son motivated to pursue his dreams. What she said really touched me.

“Ever since Shaan was small whenever he was upset about something he couldn’t do, I tried to teach him to always focus on his abilities rather than his disabilities.  I used to demonstrate by write the word DISABILITY and draw a big X over the letters DIS to reveal the word ABILITY,” Vee told me. I believe and I taught Shaan that one cannot control what life throws at you – you just have to do the best you can.”

Even with all of Shaan’s positive energy, Vee tells me that life with DMD is never easy. It takes a lot of energy to get around campus and keep up with his courses.  But driving a car is a great milestone for Shaan.  How accessible is the state-of-the-art Swiss electronic driving system?

How accessible is the state-of-the-art Swiss electronic driving system?

Unfortunately, right now, it’s expensive. Vehicle conversion or new vehicles start at $40,000, and used vans may cost about $15,000 or more. The driving system is another at least $70,000 more.  Even though the technology is pricey, at least the possibility is there. Hopefully, prices will drop as more and more people with disabilities outfit their cars. And, families can always try crowdfunding as an option.

Congratulations to Shaan’s family for getting this technology to Canada!   It’s very encouraging for the DMD community.

 

Watch this video for more information.

More about Joysteer here.

Send us your inspirational story here.

ANTOINE, A 7-YEAR OLD IRONKIDS, GOES ALL OUT FOR LA FORCE

IRONKIDS Mont-Tremblant Fun Run is mission accomplished for Antoine. He was so touched by Anakin’s story and La Force Foundation, he decided on his own to take action. Sometimes we tend to think that individual action is not effective for a cause. Well, this boy has proven that this isn’t true!

IRONKIDS SEE Y’ALL AT THE FINISH LINE!

In 2015, a team of three Tremblant athletes completed a full Ironman. Team 1 – Vie-Tesse, added an additional difficulty to the ordeal by using single speed bikes for the cycling portion. On this occasion, the Foundation team had produced a video to raise awareness about the challenges associated with access to new treatments.

 

SHAKEN BY THE REALITY OF DUCHENNE MUSCULAR DYSTROPHY (DMD)

When I spoke to Anthony’s mother, Annie Bergeron, she recounted the story in these words: “I participated in a La Force event, and one evening I was watching the 1 – Vie-Tesse Ironman video to improve my knowledge of the cause … It was then that Antoine came and asked me some questions … ”

Annie then taught him that life for Anakin and other children with Duchenne muscular dystrophy is not at all easy… The kids gradually lose their strength, instead of growing like other children. Antoine was upset by this revelation. He said: “Why him, Mom? It makes no sense that things like this happen … He’s little … What can I do to help? “That’s when they got the idea to participate in the IRONKIDS event. It was his project: he was going to train, drive his own business with the Foundation in order to become a La Force Athlete and go all the way…

 

EVERY ACTION HAS ITS POSITIVE EFFECTS

It’s sometimes difficult to motivate children. That wasn’t the case with Antoine: he was on fire during the four weeks of training. Each evening on his return from school, he would drop his pack and leave to enthusiastically run his daily kilometer. We at Team La Force were deeply touched by this boy’s initiative.

 

Moreover, and this is not just anything, Antoine is the athlete having completed an individual event who has raised the most money so far this year! That’s not all: Antony decided to form a La Force team with friends next year.

Thank you, Anthony. Team La Force is really proud of you and we look forward to supporting you in your other sporting challenges.

Special thanks to Nicholas Latreille for his amazing support for Antoine.

 

Would you like to attend an event and raise money for La Force? Just email us at info@laforcedmd.com

 

 

 

Duchenne muscular dystrophy won’t stop Samuel…

“When you focus on your dreams and objectives, the disease disappears!”

At 22 years old, Samuel Lachaine recently completed his Bachelor’s degree in journalism at UQAM. He also got his first job in the field as a sports journalist for RDS. But that’s not all. Samuel has Duchenne muscular dystrophy (DMD).

DMD is a degenerative muscle disease, for which no treatment exists. It causes a progressive loss of mobility and premature death. However, DMD hasn’t stopped Samuel! Despite the extreme competitiveness in the field of sports journalism, Samuel hasn’t been treated any differently than other students or colleagues. He’s paved his own way.

When I spoke with Samuel, I wanted to know where he finds his inner strength. He told me, “When you focus on your dreams and objectives, the disease disappears. I’ve been dreaming of becoming a sports journalist since the age of 10. I remember meeting a career counselor, who told me that it wasn’t a good idea, that it was very difficult to make a career in that field. To this, I said: ‘Just watch me!’”

Life after the shock of receiving a DMD diagnosis

Samuel’s parents played a very important role in his success. A note: Samuel’s brother also has the disease.Samuel explains: “When my parents first got the diagnosis, their life stopped, but they managed to remain positive. They told us: “DMD might cause you some difficulties, but you need to carry on with your dreams and keep your drive alive. We will be there to support you.”  This support and positive attitude proved to be a major factor in his life. Today, Samuel lives his dream; he has become a journalist. His success has had an impact on his brother, who is a business owner.

“DMD might cause you some difficulties, but you need to carry on with your dreams and keep your drive alive. We will be there to support you.”

Samuel is a true inspiration! He’s proving to each of us that we can make our dreams come true. DMD could have squashed his dreams, but it didn’t.

In future, Samuel’s ambition is to host his own sports show. We’re all behind you, Samuel! When we look at you, we are truly inspired by your determination!   

Thank you for sharing Samuel’s story!

We invite you as well to share your story here! #myduchennelaforcestory

For more inspiration from Samuel, you can follow his blog, here. Enjoy!