RIP Samuel Gibson

May 6 is Wishbone Day!

Top 10 Myths About Osteogenesis Imperfecta

Top 10 Myths About OI - 2016 (taken from an OI parent)

10) They don't have blue sclerae so it's not OI. (White, gray, gray-blue, or blue)

9) Kids with OI will "grow out of it". (Genetics)

8) OI is contagious. (Genetics, not infectious)

7) You think you can control a child's innate/biological instinct to move, crawl, stand, climb, explore. (You can't. Safe environment)

6) Every person with OI is affected the same way and presents the same way clinically, so should be treated the same way (spectrum condition, unique genetic information for each individual, individualized context based clinical care plans)

5) Toddlers are too young to have rodding surgeries (Fassier-Duval Rods - pulling to stand, weight bearing child)

4) Infants are too young to be started on medical therapies like IV Pamidronate. (Montreal Shriners - Glorieux, Plotkin, Rauch studies of late 90s/early 2000s)

3) The increased ambulation, activity levels, reduced pain levels, improved quality of life in children over the last 20 years are due to "nutrition". (See 4 & 5)

2) Infants, toddlers, children, adolescents, adults do not feel pain with fractures the older they get and more they have them. (Pain is real and affects recovery and long term outcomes)

1) Drinking more milk will cure OI. (Really?!)

Sitting Volleyball Men’s: Austin Hinchey – Captain and Veteran

Austin Hinchey, the Edmonton-based captain of his sitting volleyball team, overcame a major obstacle. Born with osteogenesis imperfecta (brittle bones) concentrated in his left leg, he decided to get it amputated. He made this life-altering decision at age 11. Now, at age 23, he represents Canada in para competitions throughout the world. After developing a passion for volleyball in high school, he began playing competitively. He has been the captain for Team Canada's Sitting Volleyball team since 2007.

  

In 2011, he and his sitting volleyball team won the bronze medal at the Guadalajara Parapan Am Games. So what does he think about playing on home soil in the 2015 Parapan Am Games?

"It's amazing to be playing in Canada. We (our current team) have only played one other event in Montreal every year, but we've never played something this big in Canada, and we have never had these kinds of fans in Canada. It's really exciting for us and we're really enjoy playing in front of the home crowd."

Hinchey is so proud of his current roster that going to the 2016 Olympics in Rio is "the goal." With an unbreakable attitude like that, his goal to go to Rio looks promising, especially with growing interest for sitting volleyball. Before I wrapped my interview with Hinchey, I asked him if he had any advice for young people with disabilities who long to get into sports:

"I think the biggest thing is just to try and find a sport that you love, and to enjoy playing it. We play ultimately to have fun and to enjoy the sport (of volleyball), and especially if you're an athlete that qualifies to play a Paralympic sport, just try to get involved. Most sports are inclusive and (organizers) are always looking for new people."

You can find an organization near you to try out sitting volleyball:

• Ontario Volleyball Association
• Pacific Volleyball Society
• Volleyball Canada

Happy Wishbone Day!

Osteogenesis Imperfecta Educational Film

This film from the UK's Brittle Bone Society is worth a look.

The Wheelmap app will take you places

Donald Barrie

Back in October, I (Donald) got to interview Raul Krauthausen, creator and developer of the Wheelmap app. Krauthausen is a 34-year-old social activist and communications specialist from Germany, who, in 2010, released an app that maps out what public places provide accessibility to people who use wheelchairs.

Wheelmap, according to Krauthausen, “is based on the principle of a cooperative community – many people collect and share their information on the wheelchair-accessibility of public places. For this purpose, the map uses a simple traffic light system which is easy to understand for everyone. Wheelmap.org is based on the geo-data of OpenStreetMap.org and works in all parts of the world. It is now available in 22 languages.”

Here are some of the questions I asked him, with his responses.

DB: What led to the creation of this phone app? Has there been anything like this before?

Raul Krauthausen

RK: The idea developed from an actual everyday life situation: A friend complained about having to meet in the same café every time. We both didn’t know in which café it’d be possible for us to meet. In this situation I thought that a map which shows the wheelchair accessibility of places in the surrounding area would be helpful.

There were and still are various local applications of maps which have focussed on the accessibility of a city or a region. However, they are widely lacking the knowledge of the crowd, meaning the data had been collected and administrated by a central institution.

DB: How has your app enabled people with disabilities?

RK: Thanks to Wheelmap, millions of people with mobility impairments have a guide for wheelchair-accessible places now. With Wheelmap it is easier to discover new places in everyday life and to meet other people, impaired or not.

DB: How has Wheelmap impacted Germany?

RK: It would surely be overconfident to claim that Wheelmap.org has triggered a real change in society. However, I hope that people understand Wheelmap as a tool that contributes the perspective of innovation and self-determination to the topic of inclusion. More and more local initiatives in or outside of Germany rely on the possibility to become active themselves and take accessibility into their own hands.

DB: Would you describe Germany as an accessible country for people with disabilities?

RK: The level of accessibility still varies from city to city and region to region in Germany. In Berlin the public transport is quite good for people with disabilities considering the low-floor buses and the ramps that are available on the underground platforms. However, there are still elevators missing – or elevators that don’t work. The latter is also the topic of another project, called brokenlifts.org. (It launched on October 8.)

Last but not least, Berlin is also shaped by a lot of old buildings where sometimes only one step makes a location inaccessible. That’s why we’ve started the initiative “1001 ramps” and the shop wheelramp.de as an easy solution for the amount of only-one-step-places.

DB: Can you tell me how you’ve been able to navigate through the area of the country you live in, as a person with a physical disability?

RK: I use a wheelchair all day and every day. My daily route from my home to work and back is a quite navigable distance of 3 kilometers in each direction. There is only one bus line that I can take to work. But I only take the bus in winter because the bus takes longer than I do with my wheelchair. When I travel to another city I always check the website of the local public transportation provider to see if the train station is wheelchair accessible. If it isn't I have to rethink my route. I don't have a car or a driver's license.

DB: You obviously take an interest in technology. The name of the blogsite I contribute to is called Technology Enables Me. How has technology enabled you as a person with a disability?

RK: Starting with my electrical wheelchair, which affords me a level of independence and mobility that would otherwise not be possible, over my smartphone and my laptop which enable me to carry out my internet based work, technology is omnipresent in my life.

DB: Do you see the app catching on in other countries?

RK: Yes! There are ‘wheelmappers’ everywhere, from Japan to England to Switzerland. It’s great to see that so many people use the app and map places everywhere. And the app is particularly useful when abroad.

DB: What do you like most about Wheelmap?

RK: The app contributes to my personal freedom and happiness by making my everyday life simpler. Mobility is a major aspect of being able to lead a happy life, and the app provides information that allows me to be more mobile and participate in “normal” situations and events.

You can learn more about Wheelmap and download it at http://wheelmap.org/en/.

My trip to Indianapolis

This summer I took a road trip to the USA. The main reason was to attend a conference in Indianapolis for people living with osteogenesis imperfecta (OI), or brittle bones. It was my third conference with the Maryland-based Osteogenesis Imperfecta Foundation. 

My trip actually began in Kalamazoo, Michigan, where my parents and I spent the night before going to Chicago, Illinois. The most notable thing about Kalamazoo is that it is mentioned in a famous big band song by Glenn Miller, called “I’ve Got a Gal in Kalamazoo.” Needless to say, I did not get a gal in Kalamazoo. I did however have a good meal at Applebee’s and a restful night’s sleep at the hotel we stayed at.

The following day we drove to the north side of Chicago for a baseball game between the Cubs and the Colorado Rockies. The game was played at the historic Wrigley Field. At 100 years old this year, Wrigley is one of the last of the old ballparks still standing. I enjoyed the experience of being at Wrigley Field. It was a dream come true for me to watch a game there, and right at centre field. Wrigley is accessible as can be, despite the fact that it is old and not designed for accessibility. All visitors with disabilities require assistance getting on and off the lifts, because they cannot be operated easily.

What I liked most about Wrigley Field, aside from seeing a baseball game, was the friendly staff. They were eager to help anyone, and didn’t hesitate to greet people with a wave and a smile. The Cubs won the game 4-2.

Donald inside Wrigley Field in Chicago

Getting in and out of Chicago wasn’t as fun due to bridge congestion on the south side. It was worth seeing Chicago again for the second time in my life. The long waits in and out of the city were worth it.

Late that night we arrived in Indiana. The conference began the following day, a Friday. At the opening address, the guest speaker said Indianapolis is one of the most accessible and disability-friendly cities in America. I put that theory to the test throughout that weekend.

A few hours before the conference started, I went on one of the city’s buses operated by the IndyGo transportation service. I wanted to explore the city by venturing outside of the downtown area where our hotel was located. IndyGo requires all wheelchair users to be tied down when boarding a bus. That was the best part of going on one of their buses. The worst part of the ride was making frequent stops on a ride that should have only been 20 minutes long. I decided instead to go back to the hotel. What I discovered, however, was that some of the streets do not have sidewalks. This made crossing the road dangerous and scary. Fortunately some kind residents guided me along to the other side of the street. I arrived back to the hotel safely by bus.

I love going to OI Foundation conferences because it keeps me updated on the latest research on OI, and also gives me a chance to reconnect with other people who live with this condition. It also provides opportunities to meet new people with OI and their families. It is estimated that this year’s conference drew in 800 participants. Though most of them were from the U.S, there were other people from Canada who attended. I also learned there were visitors from England, Denmark and Norway, giving the conference some international representation.

One of the highlights for me was meeting Robby Novak, better known to the world as the Kid President. He’s a 10-year-old boy from Tennessee who posts inspirational pep talks in a series of videos on YouTube and Facebook. He is just as lively and energetic as he presents himself in his videos. When I met him, I let him know I am a fan. (Robby, along with his sister Lexi, has OI.)

Donald meets the Kid President

In-between conference events I explored some of downtown Indianapolis. This included a unique bike and pedestrian pathway at a park across from the hotel. The Indianapolis Cultural Trail is eight miles long, and promotes accessibility to people with disabilities by including the wheelchair symbol along its paths. The pathways opened to the public in May 2013. 

One of these paths leads into the White River State Park. This park includes several attractions, one of which is the National College Athletic Association (NCAA) Headquarters and Hall of Fame. We stopped in briefly to look around, but didn’t stay for any tours. It is an impressive-looking building I think most sports fanatics will enjoy.

We took in a baseball game between the Indianapolis Indians and the Toledo Mud Hens. The OI Foundation sponsored the game played at Victory Field. A bonus that evening was getting to see the visiting San Diego Chicken, the Padres’ mascot! This popular major league mascot visits several minor league ballparks during the baseball season, with Victory Field being one of his favourites. The game went into 10 innings. Fortunately the Indianapolis team won 4-3!

Does this former Indy ballplayer wear his sunglasses at night?

The conference concluded with an awards dinner and dance. The foundation also announced that the next conference will take place in Orlando in 2016. I hope I can make it to that one.

As for my informal assessment of Indianapolis as a disability-friendly city, I believe it is a welcoming and inclusive city. The people I met were all friendly and I sense that the city has applied the Americans with Disabilities Act effectively, with more improvements to come. Though it has some shortcomings, particularly with the absence of some much-needed sidewalks, Indianapolis is impressive overall. I’m glad the OI Foundation picked it as the site for its 2014 conference.

Kid President's 5 Things That Make Summer Awesome

The Unbreakable Wyse Family

The family who motivated and inspired me to write a blog about them at the end of last year has now received coverage in this week's issue of People magazine!

Keith and Cheryl Wyse, founders of Agape Family Life House, adopted four girls from China who were born with brittle bones (i.e. osteogenesis imperfecta). The People article shows how these four bright and happy children possess unbreakable spirits, and have also lifted the spirits of everyone around them.

I met them all in July 2012, and I have maintained some contact with Keith since then. 
The family's story moved me so much I go so far as to declare Keith and Cheryl living saints. They rescued these children and have provided them with unconditional love and a higher quality of life.

Here is a sample of their People profile. I bought the magazine today (it has Katie Couric on the cover). The expanded version has more details about the family and extra photos of the kids. It's a very inspiring read. I recommend picking up a copy before they're all gone.

A Tiny Poem to the World from Kid President

My first Wishbone Day

Today, May 6, is Wishbone Day. Wishbone Day is a relatively new occasion to spread awareness of osteogenesis imperfecta, or brittle bones. I am part of that rare breed of people who lives with this condition.

Wishbone Day involves wearing yellow and hanging out (if possible) with other people with OI. This past Saturday (May 4) I took part in my first Wishbone Day picnic and walk event. It was a fun day, and it was good to meet with other people with OI and their families (and, in some cases, their spouses).

The Wishbone Day concept developed of the Australian OI Conference in 2008. The first international Wishbone Day was observed in 2010, and it came to Canada in 2012.

Last July I met with some OI folk from Australia during the American OI Foundation conference held near Washington, DC. I even got to pose with Wishy, the Wishbone Day mascot.

I'm glad this awareness day exists since OI continues to be a condition that is often misunderstood or misinterpreted, even amongst medical professionals.

The Wishbone Day picnic took place in Cobourg, a town east of Toronto.

The True Story of Kid President

A Love Song from Kid President

From the Archives: "Story on Bisphosphonate therapy" (1999)

This is a feature story assignment I wrote while I was a journalism student at Ryerson University 14 years ago. My Newspaper/Print professor liked it and gave it a high grade. We even talked about getting it published in one of the major newspapers or magazines. Unfortunately this didn't happen because I ended up losing the work, or so I thought at the time. This weekend I found it again while going through old items in my apartment. Now I want to share this story with everybody. Though some of the participants may not be involved with this treatment today, the content remains relevant and timely. - DB

Story on Bisphosphonate therapy (1999)

Donald Barrie

Carlos Medina is a happy six-year-old, but unlike most children his age, his bones break easily. Carlos was born with osteogenesis imperfecta, or brittle bone disease, which affects about one in nearly 20,000 children born each year. In addition to frequent fractures, it causes progressive deformities of the arms and legs, and chronic bone pain. Lately, however, Carlos has been feeling stronger physically, thanks to bisphosphonate therapy.

Bisphosphonates are a group of synthetic compounds that are most widely used in the treatment of post-menopausal osteoporosis. They reduce bone turnover and suppresses bone resorption (a normal breakdown process that is excessive in some diseases including osteoporosis and OI). The most common bisphosphonates being administered to these children with OI is pamidronate, which is closely related to alendronate (Fosamax), used to treat osteoporosis in adults.

At Sunny View Public School in Toronto, physiotherapist Lynn Mueller takes Carlos out of his electric wheelchair and puts him on the floor. Mueller, skeptical of his mother’s claims about his increased mobility, asks him to crawl on the floor. Lying on his back, Carlos turns over on his stomach, and crawls gently on his hands and knees. Carlos then pushes himself off the ground, and sits in a kneeling position.

Mueller’s eyes light up, and so do those of the kids looking on. Another therapist watching him from a distance rushes to get her camera. “I can’t believe it!” the two therapists exclaim. “This is the first time I have seen him do that!” Mueller adds ecstatically.

“When Carlos arrived at Sunny View, he was very weak,” Mueller says. “Since the start of this school year, he has gotten stronger, and his mobility has improved. However, he has only been doing the treatment since August, and sometimes he gets too brave.” She notes that Carlos recently suffered a skull fracture when he tried to get off the toilet on his own.

“I feel less pain now since I started the therapy,” he says, as he gently touches the small bump on the left side of his head where he had the skull fracture. “It still hurts a little though. If I was not doing the drug treatment, it would probably hurt more.”

Prior to taking this drug, Carlos was unable to crawl or sit up on his own, and would get fractures at least every one or two months. Now he can use a walker in therapy, and he can grip a pencil more securely when writing.

Bisphosphonate therapy is still at the experimental stage in the treatment of OI. Carlos is receiving treatment as part of a research project being conducted by Dr. Francis Glorieux and colleagues at the Shriners Hospital in Montreal.

Last October, the New England Journal of Medicine (NEJM) published results of their initial findings, arising out of an uncontrolled observational study on bisphosphonate therapy. The study, one of the first to investigate the use of bisphosphonates in this patient group, involved 30 children who were three to 16 years old and had severe osteogenesis imperfecta. The children were administered the drug pamidronate intravenously. The results of the study found that chronic bone pain decreased, motor function improved, bone mineral density increased, and bone resorption decreased.

Dr. Glorieux has extended the study of the effect of pamindronate to other children with OI. “We are currently following more than 170 patients suffering different types of OI,” Dr. Glorieux says. “It includes all the departments involved in the care of patients with OI. [We] aim to respond to all their needs, not only the medical management of their condition.”

As director of research at Montreal’s Shriners Hospital and professor of surgery, human genetics and pediatrics at McGill University, Dr. Glorieux has treated people with OI for the past 25 years. He found other medical treatments for OI unsuccessful, so he turned to bisphosphonates after seeing their effects in animal studies and in adults with osteoporosis.

The main concern against the use of these drugs in children was that it could affect growth. So he started treating very severe patients for whom height was not an issue. He discovered that growth was not arrested.

When a person like Carlos goes to Montreal for the treatment, it is for a three-day period every four months. During his first visit, the doctors made a small cut inn his hip to get a sampling of his bone structure. For the next three days, Carlos had pamidronate taken intravenously for six to eight hours each day.

Dr. Horacio Plotkin, one of Dr. Glorieux’s colleagues and co-author of the report in the NEJM, said in a phone interview from Montreal that the only side effect to pamidronate found is an “acute phase reaction.” It is a flu-like syndrome patients often experience the first time they receive the drug. This reaction resolves in 24 hours, and usually does not reappear with new infusions.

There are no other similar treatment facilities in Canada. Dr. William Cole, chief of Orthopedic Surgery at Toronto’s Hospital for Sick Children, says he is supportive of Dr. Glorieux’s study, and has referred patients to him. At this time, however, there are no plans to open a similar treatment facility in Toronto, as the treatment is still experimental. The Shriners Hospitals in Los Angeles and in Springfield (Massachusetts) are providing the treatment to selected cases.

Since Dr. Glorieux began his bisphosphonate therapy clinic in 1992, OI children from the United States, France, Brazil and Kuwait have come to Montreal for the procedure once every four months.

“There is no plan to end the study. All patients are improving, so we do not want to discontinue the treatment,” Dr. Plotkin explains.

The long-term benefits of bisphosphonate therapy are not known, and the knowledge of side effects is limited to seven years at the Montreal clinic. “It is probably the only treatment so far that is able to produce significant and positive changes in the quality of life of the patients,” says Dr. Plotkin, currently co-chair of the Pediatric Committee of the American Society of Bone and Mineral Research. “It is not the ideal treatment, and active research is taking place to find better treatments. Currently, we are only accepting patients under four years of age in Montreal, due to the overwhelming demand.”

As a 25-year-old adult with OI, I expressed personal interest in bisphosphonate therapy to Dr. Plotkin. But he cautioned me that bisphosphonate therapy may be less effective for anyone whose bones have stopped growing. Alendronate would be most appropriate for adults with OI, according to Dr. Plotkin.

At present, there are no reports that confirm the benefits of bisphosphonate therapy for adults with OI. In the long term, Dr. Plotkin believes it could help strengthen bones and reduce the likelihood of recurrent fractures later in life, when bone loss occurs with age. One serious side effect of alendronate is that it may cause esophagitis (i.e. an inflammation of the esophagus).

Both Dr. Glorieux’s paper and the editorial commentary in the NEJM make clear that bisphosphonate therapy is not a cure for OI. It reduces the rate of fractures, and improves bone mineral density as seen on X-rays. According to the editorial commentator in the NEJM, this treatment provides cause for cautious optimism. It strengthens the bones, but may not enable the more severely affected patients to walk. For some patients, walking is not their primary concern.

“[Dr. Glorieux] seemed to be so determined that I would walk and that’s not a big priority for me. It took a while for them to understand that,” insists Kristin Hayes, a 15-year-old who has been having bisphosphonate therapy since 1997. “I wanna get stronger but walking [to me] is not that important… After my first two treatments, my swimming coach noticed my endurance ad speed had suddenly improved, and, of course, bone density improved.”

As she hadn’t had bone pain or a fracture for four years before starting the treatment, the effects were less dramatic than those seen in younger, more severely affected patients.

In Dr. Plotkin’s view, bisphosphonate therapy has opened the door for new treatments, has increased the interest of the medical community in the disease, and he hopes it will lead to a change in the lives of patients with OI.

It has already given hope and strength to Carlos and his mother Eusemia, who feels more confident knowing her son is benefiting from the treatment. “The doctors told us that we could stop the treatment at any time, but we will keep going with it, because it is making his bones stronger,” she says as Carlos sits in his chair watching television at home after a long school day. “Carlos has become so confident since starting the therapy that he thinks he can do anything. He is not strong enough yet to walk on his own, but the doctors said someday that may happen.”

-30-

'Kid President' Robby has a message for us grownups

My favourite moment of 2012

Me with Rebeka, Lydia and Rachelle Wyse
(Esther not pictured)

Before 2012 draws to a close, I want to share my favourite moment of the year with you. It happened in Washington last July, during the American conference for people with my physical disability, osteogenesis imperfecta (brittle bones).

While I was enjoying a Saturday night party at the hotel, I came upon these young pretty girls with OI enjoying cake with a middle-aged couple. When I asked the couple how they all knew each other, they said they were with four of their children adopted from China.

Originally from Illinois, Keith and Cheryl Wyse have lived more than 10 years of their lives in China. They became passionate about establishing a home for orphaned children with OI after adopting twins Rebekah and Rachelle in 2002. I met the twins, and also two of their other adopted daughters, Lydia and Esther. The Wyses have eight children, and of the six adoptees, four of them have OI.

When I met with them again during breakfast the next morning, they talked about a centre they co-founded in China called the Agape Family Life House. The Agape Family Life House is also a Christian ministry that reflects the deep faith shared by the Wyse family. The Wyses want to shine a light to the world by showing that people with OI can be brought up to live long and healthy lives.

The OI Foundation honoured Keith that evening with an award recognizing his efforts to help improve the quality of life for children and youth with OI. After the conference ended, I read more about Agape and the work it has done and continues to do. Though I live in Canada, I want to help the ministry grow any way possible.

The Wyses’ story moved me so much that I seized every opportunity I could to spend time with them and get to know them. Meeting Keith and Cheryl and their four children, and learning about the Agape Family Life House, ranks as my favourite moment of 2012.

From the Archives: "Things I Learned and Discovered at the OI Foundation Conference" (8/8/2008)

I had an awesome time at the OI Foundation Conference this year! It was actually my first one... I thought I'd share what I took away from this conference.

Here are some of the things I learned:

* Most cases of OI (85-90%) are caused by a dominant genetic defect.

* While there are no concrete stats available, it is estimated there are 20-50,000 people living with OI in the US. The number of people with OI in Canada may be similar to US estimates.

* Exercise is crucial for people with OI. One good workout regimen is stretching.

* Bones can atrophy just like muscles if they are not used.

* Vitamin D is a hormone, not a vitamin.

* Vitamin D plays an important role in calcium absorption and bone health.

* About 50% of people with OI also have dentinogenesis (i.e. brittle teeth). There are also some people who have DI but not OI.

* Pulmonary infection is the leading cause of death for people with OI. This is why ALL people with OI are told to exercise daily and get a flu shot annually.

* Canada needs its own OI Foundation. There used to be an active group, but it dissolved several years ago.

* China has its own OI Foundation, as does the former Soviet state of Georgia.

Here are some things I discovered at the conference:

* Many of us OIers are fun, friendly and talkative.

* Many of us OIers are positive, intelligent and forward-thinking.

* We possess an 'unbreakable spirit.'

* Over 800 people attended this year's conference (#16), the largest one ever!

* Many attendees came from the US and around the world.

And last, but not least...

* There are a lot of cute girls - and pretty ladies - with OI! ;)

Wishbone Day coming May 6