Tuesday, June 28, 2016

Ask the Doctor: TMJ Joint Replacement

CCA posts a monthly blog series called, "Ask the Doctor." You can submit your questions to Annie Reeves (areeves@ccakids.com) and we will ask Dr. Earl Gage of Kids' Plastic Surgery in St. Louis to answer them. Thanks Dr. Gage for helping spread information and resources for our cranio families!

Ask the Doctor

Earl Gage, MD, Kids Plastic Surgery, MercyChildren’s Hospital
Ask The Doctor
Earl Gage, MD
Kids Plastic Surgery, Mercy Children’s Hospital – St Louis, MO

Question: We are considering a new procedure for Peter's upcoming jaw surgery.  It involves a TMJ joint replacements (vs a rib graft & jaw distraction).  The company making the joint is TMJ Concepts (www.TMJConcepts.com) in California.  I'd love to hear if Dr. Gage has any thoughts about this new procedure.

Answer: Thanks for your question. I do not have any personal experience with artificial TMJ replacement in children. At the most recent meeting of the American Cleft Palate Association in Atlanta, I asked a number of my colleagues and mentors if they had any experience or thoughts, as well. Nobody I spoke with had ever used an artificial joint in a child. None of my colleagues were aware of any surgeon who is doing a large volume of artificial joint replacements in children either.

I would be concerned about placing an artificial TMJ replacement in a child because no artificial joint is likely to last a lifetime, particularly when you consider the high load and stress placed on the TMJ. This makes future replacement surgery likely, in contrast to using bone and cartilage which is perhaps more likely to last. In addition, I worry about how the changing, growing facial skeleton will affect an artificial joint since the artificial material will not grow and change with your child. Finally, any time you place something artificial in the body, whether it is an artificial knee, an artificial heart valve or a synthetic facial implant, there is a risk of that artificial material becoming infected and needing to be removed. When biologic tissue (like bone or cartilage graft) is used, this risk is much, much lower.

I recognize that innovation and technologic advancements in joint replacement may produce an artificial joint that outperforms bone grafts. At this time, however, I am not sure there is enough data and experience to recommend it if bone grafting is also an option.

Friday, June 17, 2016

New Must-Read Book for Teens

Ride High Pineapple Beats the Bully and Faces Up

CCA is excited to announce a new book in our collection of recommended reading. 
Children’s author, Jenny Woolsey, announces the release of her debut middle grade novel, Ride High Pineapple.

This gutsy contemporary realism novel, is a must read for any child or teenager. Ride High Pineapple is written through the honest eyes of a teenager born with a rare craniofacial syndrome, Crouzon Syndrome. She also suffers from severe anxiety. Written as a journal, the story deals with critical childhood issues: bullying, difference, mental illness, friendship ups and downs, and young love. It also shows how one can find self-empowerment through sports. 

CCA loves this book because it goes along with our educational initiatives in that it is yet another text that is relatable, enjoyable, and features a young person with a facial difference. Offering an additional perspective to Wonder, Ride High Pineapple captures the raw emotions that teen girl, Issy Burgess, experiences. The book presents another lovable character who struggles with bullying at school. Plus, since it's from an Australian author, you'll pick up lots of cool new Aussie jargon! 

From the author:
Adolescence is a tough gig, but Issy Burgess has a few added challenges—facial difference, anxiety, and a relentless bully. Issy’s story of confronting her fears and working through her difficulties will inspire middle grade readers. Kudos to Jenny Woolsey for writing Ride High Pineapple. It ticks all the boxes: skateboarding, besties, and boys. And it’s chock-a-block full of hope, insight, and solid strategies for handling bullying and managing anxiety. A must for school libraries and school counsellors! -Alison Stegert, School Counsellor & Kidlit Author

About the Author
Jenny Woolsey is a Christian, teacher and blogger. She is an advocate for inclusion of disabled children, and for people living with facial differences and mental illnesses. She has a Diploma of Teaching (Primary), Bachelor of Education, Master of Education (Honours) and a Certificate of Creative Writing. Jenny lives in Queensland, Australia.  She writes from personal experience and aims to help children with their social and emotional wellbeing.

Ride High Pineapple is available on Amazon.com as a paperback and ebook.

Monday, June 13, 2016

Hospodka Family Spotlight

Bridget, our CCA Kid, is the second oldest of 4. She is 12 years old and keeps busy at Holy Name School, which she attends with her little brother and sister.  Moving up to 6th grade and middle school has presented its own sets of challenges and opportunities.  She has three main teachers and has to keep track of her FM amplifier when moving from class to class.  Her classwork has gotten more challenging, but she is more than capable of doing the work and excelling. After school, the fun doesn’t stop!  Bridget keeps busy with mass choir, chess, maker’s club, and played soccer this spring.  She loves texting and talking with friends, but hanging out is the best. In other exciting news, Bridget got braces!  She’s sporting pink and green bands and getting used to the new feeling and what she can and can’t eat.

Big brother Daniel can be seen at meals and for brief moments when he is not in his room reading everything he can get his hands on!  He is adored by his littlest sister for his ability to carry her everywhere and swing her around like a carnival ride.  After a tough freshman year, Daniel  transferred schools midyear, and the change has been beneficial for him academically!  He is looking  forward to taking more culinary arts and design classes.  This summer holds big changes on the horizon, as Dan got his driver's license and is looking forward to his first job.

Little brother Conor is busy riding bikes and racing BMX, when he isn’t bugging his sister.  I keep telling them they’ll probably end up being best friends. Since starting out at the track less than 2 years ago, he has earned a shelf full of trophies and medals, while making friends and inviting friends to join.  Conor just finished third grade, and loves math.  He is a Wolf Cub in Cub Scouts, and worked hard on his pinewood derby car—it was a submarine with room for a Lego captain to ride along!

Adeline is our tiny terrific not-quite-a-toddler anymore, but not quite a preschooler.  Daycare (aka “school at Miss Shannon’s house”), potty training, and getting into whatever looks interesting are at the top of her to do list.  She sings about anything that comes to mind, she babies any doll or stuffed animal that crosses her path, and she loves the heck out of all of us.

Although we won’t be able to make it to the Retreat in 2016, we hope to make it next year! Can’t wait to see pictures!

See you all soon!

Tuesday, May 31, 2016

Ask the Doctor: Midface in His 20s?

CCA posts a monthly blog series called, "Ask the Doctor." You can submit your questions to Annie Reeves (areeves@ccakids.com) and we will ask Dr. Earl Gage of Kids' Plastic Surgery in St. Louis to answer them. Thanks Dr. Gage for helping spread information and resources for our cranio families!

Ask the Doctor

Earl Gage, MD

Question: My 23 year old son has severe type 2 Pfeiffer syndrome. He had the midface surgery using his ribs at age 6 with good results, but with time, his midface has become more retracted and his orbits bulge. We were planning another midface at age 19, but due to many other medical problems, it didn't happen. What are the options for a midface surgery at his age? I was told in the past not a candidate for the RED device since he is done growing.

Answer:  The decision to use distraction or not has a lot more to do with the degree of movement that your son requires to get his midface where it needs to be rather than his age. Distraction can be done at any age. While it is perhaps true that adults may not make bone as readily or as quickly as children, a young man at age 23 should still be able to heal well using distraction.

Midface distraction, as you likely already know, involves making cuts in the bone to “free up” the midface so it is no longer attached to the skull. Then, the distractor is used to pull the midface bones forward slowly over time. The goal is to move the face forward slowly enough that bone healing “keeps up” with the moving midface but not so slowly that the bone heals solidly before you get the bones where they need to be. The pace of movement can be anywhwere from 1-3mm per day, depending on a variety of factors.

The advantage of midface distraction is that a much larger advancement can usually be achieved with this technique than with non-distraction techniques that require you to make the full advancement at the time of surgery. When someone has had previous surgery, such as your son, and where a large advancement may be required, scar tissue and the tightness of the soft tissue of the face may limit how much movement you can achieve with traditional surgery. Because distraction gradually stretches the soft tissue, the limitations of scar and soft tissue tightness can often be overcome with distraction.

In summary, it is my opinion that your son’s age should not automatically disqualify him from midface distraction surgery. In fact, depending on how much advancement he needs, distraction may be the best option to achieve the advancement he needs and provide a stable, long term result. I encourage you to discuss this option again with your surgeon so that your son’s overall health and his specific needs may be taken into account as you make a final decision on how to proceed. Best of luck!

Tuesday, May 10, 2016

From the Bench with Francis

Today we have an entry from one of our longtime CCA members, Dr. Francis Smith. He recently returned from a research trip and shares this update with us. We are looking forward to the culmination of these interviews he is conducting. Thanks, Francis!

From the Bench with Francis
by Dr. Francis Smith

In the middle of March this year, I attended an international craniofacial research conference in Ventura, CA, called the Gordon Research Conference on Craniofacial Morphogenesis and Tissue Regeneration. Part of a larger series of Gordon Research Conferences, this one is held biannually, alternating between Ventura, CA, and Il Ciocco, Italy (hence, the next one, in 2018, will be in Italy). As a researcher, since I was in graduate (PhD) school years ago, I have gone to many scientific conferences in the US and abroad to present my research. Often I get to share not only my research, but also my unique connection to my research.

In advance of the conference, I made a poster about my ongoing public engagement initiatives and strategies for public outreach and fostering collaboration between researchers and craniofacial doctors.

Our conference was held at a resort hotel (designed by the late Frank Lloyd Wright’s architectural firm) with plenty of conference space and we took all our meals together in the spacious restaurant overlooking the Ventura Harbor.

On the day and a half before the actual conference, there was a Gordon Research Seminar for postdoctoral fellows (like me) and graduate students in which we presented our research to our peers, both oral and poster. I presented my poster on public engagement strategies and reconnected with old colleagues (and met new ones) from round the world.

On the day the conference itself began, I was happy to see many of my old mentors from London, San Francisco, and Calgary come in, as well as others I had met in the past through connections I made at previous conferences and other events. Throughout the rest of the week, I met many new colleagues (it is vitally important in my field to make as many connections as possible—it’s called networking) from across the nation as well as round the world.

Our days were filled with talks from eminent researchers about the latest advances in research concerning the genetics and molecular biology of embryonic development of the craniofacial complex, as well as craniofacial malformations (including some talks on Treacher Collins syndrome; they’re always finding some new culprit gene for it or something new about the etiology of it). 

Midday and afternoon were lunch and free time. I went to downtown Ventura a couple of times just to browse the bookstores and antique malls. I picked up an excellent condition 1946 textbook on oral surgery and walked around the entire harbor and marina, and once waded into the ocean. Later each afternoon there was a poster session (with posters all set up and people discussing them), then dinner, and a full slate of more talks into the evening, followed by social time with a bar on hand.

I presented about the need for us – craniofacial scientists – to reach out and educate the public (as well as government, craniofacial charities, and scientific organizations) about craniofacial anomalies and research. I also recruited more craniofacial scientists and professors in attendance to contact me about being interviewed in person (on video) in future; once I have collected enough interviews, I will make them available to the public and families of children with craniofacial disorders in order to educate them on the potential benefits of our research. 

I also stressed the need for ecumenical cross-communication between researchers and craniofacial doctors, so we could learn from each other and gain perspective from each other, through possible strategies such as attending each other’s conferences and having a comprehensive meeting one day for both sides of the field of craniofacial medicine, as a sort of Vatican II for the craniofacial field. Craniofacial doctors could learn a bit of the science underlying the craniofacial defects they treat, and we researchers could gain the medical perspective on craniofacial disorders from the doctors. I also mentioned the need to lobby the government about craniofacial issues and reach out to scientific societies and craniofacial organizations in order to disseminate our research findings. I also discussed my past and ongoing strategies whereby I have been a mentor to the craniofacial community, as well as reaching out to the public through my past talks around the world and my news media interviews.

All in all, this conference was a great way to reconnect with old colleagues, meet new ones, and spread the word about craniofacial public engagement.