Tuesday, March 29, 2016

Ask the Doctor: Repair the Soft Spot?

CCA posts a monthly blog series called, "Ask the Doctor." You can submit your questions to Annie Reeves ([email protected]) 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! March's question: "Should we surgically repair the soft spot?"

Ask the Doctor

Earl Gage, MD

Question: My son was diagnosed with sagittal craniosynostosis at 7 months. At 14 months he had sagittal strip craniectomy, with no helmet therapy afterward. He is now 5.5 years old and doing great. He had regular checkups every few months after his surgery, then every year. In 2014, at his annual checkup, they said he was doing so great he didn't have to come back for two years. His checkup will most likely be in May and I'm a little worried because his soft spot still hasn't closed up all the way. In the years past, his doctors have always reassured me that it's no big deal and that it will most likely close and that if it doesn't it's no big deal. They have said that if it never closes up, it's not a problem unless it bothers him aesthetically. I'm just scared that when we go this year for his checkup that they will say he needs surgery again. What are the odds? Is it really necessary to put in a plate if there is still not enough bone growth? I hear a lot of conflicting stories in all of the forums.

Answer: When craniosynostosis surgery is done in the first 12-18 months of life, babies have an amazing capacity to heal. Most of the time, soft spots will fill-in with bone within a year or two after skull surgery. Small residual soft spots are common and are often only visible by CT or MRI scans. Larger soft spots that you can see or feel are less common but still occur.

The primary reasons to consider patching a soft spot are for brain protection or for appearance concerns.  A soft spot, in and of its self, is not dangerous. It’s the theoretic risk of injury to the brain in the event of accidental trauma that worries surgeons. Whether or not to patch a soft spot surgically will depend on the age of a child, how large the soft spot is, how active a child is in sports or head contact activities, whether the soft spot causes an appearance concern and whether it is continuing to diminish in size on its own.  

Generally speaking, I expect improvement in a soft spot in the first few years following surgery. In the toddler years, therefore, it is not common to recommend patching a soft spot. As a child approaches school age, however, the chances are good that the soft spot may not improve further. This is because the skull’s ability to make new bone and fill in soft spots is best in infancy and diminishes over time. Your child’s surgeon should have a good idea whether the soft spot is getting smaller or not based on your yearly exams and imaging studies. It makes sense to me that if the soft spot is diminishing in size, it may be best to continue to wait on surgery, regardless of your child’s age.

If your child participates in contact sports, rough play or other activities where the head may get hit, sometimes patching a soft spot may be recommended to protect the brain. Opinions will vary from surgeon to surgeon regarding the size of defect that requires patching. It is my opinion that any defect larger than a quarter should be repaired if it is not getting smaller on its own.  Defects smaller than this are not likely to pose a significant risk for brain injury.

If the soft spot causes an appearance concern, then this may be another reason to consider repair. However, in this instance, the decision to patch a soft spot is highly individualized and may be influenced by the level of family concern, the child’s level of concern and the overall health of the child, among other factors. There is no one-size-fits-all approach. If a child is healthy, thriving and has no concerns about appearance, I often elect to wait on surgery as long the defect is not so large that I am concerned about brain protection.

I agree with you that deciding to sign your child up for another operation can be scary. If your child is doing well, appearance is not a concern and the soft spot is relatively small or is diminishing in size, surgery probably is not necessary. If the soft spot is large, your child is older and not likely to fill it in, or if appearance is a concern, then surgery may be a reasonable option. Hopefully, these general guidelines will be useful as you meet with your child’s surgeon and weigh the options with your team.

Thursday, March 24, 2016

Interview with Rob Williams, Look At Us

Today on the blog, we have Rob Williams, of Look at Us Alliance. Rob has been coordinating with CCA and we hope to work together more in the future helping kids get the surgeries they require. Learn more about his work and family in the interview below! 

Rob with son Robert, and several CCA Kids at the 2015 Family Retreat
CCA: Where are you from and tell us a little about you?

RW: I grew up in a large family in Dallas, TX.   My family was in the oil and ranching business.  I had numerous major reconstructive surgeries as a teenager, but did not know I had Treacher Collins until my son was born.  

CCA: When did you start Look at Us, and can you tell us how it came about?

RW: In January of 2014 we sent in our application to the IRS for our non-profit status the day before my family left for an eight month journey around the world.  We sold our home, put our belongings in storage, pulled the kids from school with only a one-way ticket to Bangcok carrying only backpacks.  The day we returned we received our approval notice.  Look At Us launched in November of 2014.  

The catalyst for Look At Us dates back to the summer of 2002, when my son Robert was born with severe craniofacial differences.  What should’ve been the most joyous occasion for my wife Jennifer and me became shrouded by worry, confusion and – truth be told – fear the moment an obstetrics nurse placed our precious newborn into my hands early on a July morning.  When I saw for the first time Robert’s underdeveloped facial bones, down-slanting eyes and malformed ears – the telltale signs of Treacher Collins Syndrome – I turned to the doctor and said, “look at him,” desperate for reassurance that he was going to be okay.  She refused.  “LOOK AT HIM,” I repeated, an anguished father pleading for the doctor’s care – a diagnosis, perhaps some words of comfort.  But she wouldn't.  Instead the OB/GYN hurried out of the operating room, avoiding eye contact and offering a patronizing “his color is fine, he’s doing what normal babies do."

Never had I felt so alone.  Yet never had I felt so determined.  My child shouldn’t have been ignored, and I was resolute that no other child like Robert would be.  Twelve years later, “look at him” became Look At Us, a 501(c)(3) organization singularly focused on the physical and emotional well being of children and young adults with craniofacial disorders due to birth anomaly or injury.

Since Robert’s birth, Jennifer and I have been steadfast in our quest to provide him with the best medical care available and to raise our son as a completely normal child, which he is.  To date, Robert – who’s an otherwise healthy, happy and active 6th grader – has endured ten reconstructive surgeries, and our journey has led us to discover the world’s top medical professionals and ancillary providers, to learn all about hearing aids, and to understand the psychological impact that craniofacial issues invariably have on an affected kid and his or her family. 

No doubt, we’ve come to know first hand that having a son – or daughter – with a craniofacial anomaly is traumatic.  Expensive too.  The good news is that our collective experience – including travels to remote areas around the world volunteering for those less fortunate than us and sponsoring surgical missions – has shaped the purpose of Look At Us:  to provide education, support, counsel and care to families touched by craniofacial differences.  We’re advocates; we fund surgeries domestically and abroad; we supply medical devices – like hearing aids – to kids in need; and we help affected families navigate the seemingly uncertain roads ahead of them.

Best of all, Look At Us is a hub of inspiration, empowerment and hope.  We let boys and girls – just like Robert – know that they too will live normal and productive lives.  And for their families, Look At Us is a welcomed source of faith that – in most every case – everything will be alright.  That’s something I wish that OB/GYN had the courage to look up and explain to me so many years ago.

CCA: What was it like for you growing up? Did you encounter discrimination or bullying?

RW: I was very happy and confident growing up.  As crazy as it sounds, I never really thought much about my appearance.  I had a great childhood.  My parents never focused on it and treated me normally.  The only real bullying I ever encountered was when I was a sophomore in high school.  I was an extremely wild teenager.  Think of a cross between Fast Times at Ridgemont High and Animal House.  My parents sent me to the Marine Military Academy for a year to straighten me out.  Unfortunately for me it was a year of really cruel comments and bullying.  I remember several times where I overheard other kids ridiculing me about being an “alien” or “an abortion that lived.”  At the time, I really couldn’t believe they were talking about me.  Also, for the first time I found myself in fights to defend myself.   I had never experienced anything like it. Eventually, I surrounded myself with a group of friends and earned the respect of those around me.  The thing about it, as awful as it was, I never lost my self-confidence.  

CCA: How do you overcome feelings of self-doubt?

RW: Everyone has certain “movies” they play in their head about themselves or certain situations.  Self- doubt comes from within and each of us has the ability to manage it through our thought process.  The trick is to consciously change your thinking at the time feelings strike.  No one else has to change for you to be happy.  It is entirely up to you.  

CCA: Do you believe there’s a difference in acceptance and tolerance? Can you explain your thoughts on that?

RW: Acceptance is a state of mind, tolerance takes effort.  Acceptance becomes part of your character.  Acceptance of yourself and others.  It is the ability to view the world with a larger perspective than just focusing on yourself.  

CCA: What’s the toughest part of working in the nonprofit sector?

RW: The most difficult part of running a non-profit, besides the accounting and IRS regulations, is the fact that you can’t help every person that requests assistance.  I was unprepared to be the decision maker on who receives care and who might not.  

CCA: Why do you believe it’s important to take an international focus?

RW: Our program in Haiti and other developing countries has been my proudest accomplishment.  As much as I love helping families who look just like mine in America, the impact that we are able to have for a child that doesn’t have running water and dirt floors in their home is unimaginable.  If we didn’t step in, literally, no one would.  This is why we are implementing our +Plus Another fundraising program. Families are able to raise funds for their child and also participate in a surgery or provide a hearing aid for someone else using the non-profit status of Look At Us. It allows individual donors and corporate partners to make tax deductible donations. Unlike other crowd fundraising sites, which have the emphasis on ME, +Plus Another helps YOU pay it forward to another family.  Our motto is if there is always +Plus Another, paying it forward never ends.  

CCA: What does kindness mean to you?

RW: Kindness means love.  To be kind or to love a child who is part of the most vulnerable population on earth takes on a spiritual quality.  It’s humbling and leaves one forever changed.  

CCA: What’s your motto?

RW: Do it scared!  Basically it means don’t let fear stop you from accomplishing your goals.  A few years ago I could never of imagined speaking in front of hundreds of people, sharing my story or leading a global organization.  Stepping through the fear and doing something that scares you builds confidence.  Everything you want in life is just outside your comfort zone.  

CCA: What do you want CCA Families and readers to know about Look at Us?
RW: Look At Us is transforming lives all over the world and we would love them to join our alliance!  Please contact us to discuss ways in which we can work together to assist children who otherwise will go without care.  Unlike a large, bureaucratic organization we are nimble and able make decisions quickly to affect change.   

Check out our website at lookatus.org

Tuesday, March 22, 2016

Siblings in a Special Needs Family

Today's guest post is from Amy K. WilliamsAmy K. Williams is mother of two and a former social worker, specializing in teen behavioral issues. Parenting is her passion and she is especially involved in spreading the word about positive parenting techniques.

Siblings in a Special Needs Family

Relationships in families can be complicated, especially when siblings are involved. They are our first friends, confidants, partners in crime, and even our first enemies. Growing up in the same household allows siblings a unique chance to bond and forge relationships that are sometimes tricky to understand.

After all the fighting and backseat squabbles, siblings are bonded by shared life experiences that can unite people in very strong ways. Even though we might pick on our brothers and sisters, it’s nice knowing that they will always be there or have our back. Siblings relationships are precious and dicey, because occasionally real life interferes causing a few rough patches along the road. This is especially true in our families where one of the children has special needs.

The Needs Of Siblings of Kids With Special Needs

The reality of raising a child with special needs is that parents are often required to put in a little more one-on-one time or give attention towards meeting that child’s needs. Siblings often pick up on this and often develop feelings of resentment if they perceive a brother or sister is receiving all our attention. These very real emotions of sibling rivalry can be challenging for any parent, but can be especially taxing in a special needs family.

The brothers and sisters of a child who has special needs often are supportive and willing to help their sibling. However, there are times, just like in any other family, where jealousy might rear its ugly head. Siblings may surprise us and react in unpredictable ways if they are feeling one of the kids are receiving a lot of our attention and time.

“The siblings aren’t given as much attention, and they may not understand why,” said Dr. Jill Emanuele, a clinical psychologist at the Child Mind Institute. ‘Why are Mom and Dad always worrying about Peter? Why aren’t they paying attention to me? What did I do?’ ”

Underlying competition for our attention can easily cause a rift between siblings and even resentment to develop toward parents. This can occur even in normally understanding and protective siblings- after all, it is a common occurrence among brothers and sisters. In the same breath, it is important to realize that a side effect of these feelings is that children tend to act up, hide their emotions, and even deliberately disobey parents.

Support Siblings With The Following Ideas:
Remember that children are still children. Sibling conflicts are natural parts of growing up and the age of child can impact how they process the situation. Younger kids typically react in outbursts and have difficulty understanding why their sibling receives extra attention. As they age, new worries develop. Now a child might begin to notice bullying at school, worry about the future of their sibling with special needs, and if the condition could possibly be inherited by their own offspring.

Openly discuss a child’s special needs and the needs of other family members. Talking will empower family members and help kids understand the situation. Children often will seek any form of attention if they are feeling neglected- positive or negative. Allowing everyone a voice will keep the lines of communication open to benefit everyone. Remember, knowledge is powerful.

Find a support group for siblings in a special needs family. Whether it is online or at the local community center you are offering the youngest family members a safe place to share their feelings with peers who can relate to the situation and experience. Check out the Sibling Support Project or turn to the Internet for additional resources for siblings.

Help a sibling find an activity of their own to become involved in. Allow children to choose a cause or sport they are passionate about. If they decide to try soccer or 4-H, these organizations allow children to develop their own identity and give a boost to their self-esteem.

Allow siblings to tackle chores and duties around the home. By allowing siblings to help the family will ultimately create a solid unit and provide ample opportunities for positive feedback on a regular basis.

Schedule dates with all the children. Caring for a child with special needs does require extra work and taking the time to schedule quality time is necessary to show all children they are valued. Even if you need to find a sitter or hire help, taking the time to bond individually with our kids is definitely worth the extra hassle.

What ways do you try to keep sibling rivalry at bay?

Friday, March 18, 2016

Volunteer Invents a Onesie for Babies with Medical Needs

Recently the creator of Snapsie, Sue Wilson, reached out to CCA to tell us about her invention that really could make life easier for parents of children with medical needs.

This "tender way to dress a little one" features a cute and comfy onesies that snap at the top, so nothing has to be pulled over baby's head.

We think these onesies are a great! But even better is the story behind it.

Sue explains that in 2012, there was a study taking place at Mary Bridge Children’s Hospital in Tacoma, WA to reduce central IV line infections. The team was looking for a volunteer (who turned out to be... you guessed it, Sue!) to sew “infection control onesies” for the research project being conducted by the infection control department. The team felt that if they could keep the central IV lines on babies going up and away from the diaper area, they could eliminate one more source of potential infections.

The Solution
"I started by trying to remake the hospital onesies so that they would open from the neck through the shoulder and sleeve to allow the IV lines to thread up and away from the diaper. Well, those remakes turned out pretty ugly! After a great deal of thought I came up with the very simple design for Snapsie®. The hospital staff loved the design! I then went into production mode and made 48 Snapsies in 3 sizes on my home sewing machine for the team to use in their study."

The Response
The feedback from the Mary Bridge staff and families who used the Snapsies at the hospital was overwhelmingly positive and they wanted more. They started my journey to having Snapsies manufactured. To date, there have been no central line infections in babies who have been involved in the study that included Snapsies™ … and that is the best part of this story!

About Sue
"I have loved to sew from the time that I sewed my first project in my sophomore Home Economics class in high school. In fact, I went on to graduate with degrees in Home Economics and Elementary Education from Oregon State University. I taught Home Ec, but ultimately had a 20-year career as an elementary teacher.

Being a teacher meant learning to solve problems. It is those problem-solving abilities along with my knowledge of sewing that helped me design the Snapsie. I also credit my parents. They instilled in their seven children a strong work ethic and a can-do attitude. My dad usually concluded any counsel that he gave us with '… you’ll figure out what to do.'"

Check out Snapsie online for more information and to purchase. 

Wednesday, March 16, 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!

In January I traveled to the United Kingdom for work related to my craniofacial research. I was already familiar with London, having studied at King’s College London (for a degree in biomedical and craniofacial sciences) from 2004-2007. It was good to revisit my alma mater for the first time in 9 years—it was 9 years too long! After all, London was my first home away from home (and my first move away from home and family), and it was the cradle of my career in craniofacial research.

Shortly after I moved to Denver last summer to begin my current research position at the University of Colorado, my supervising professor, my former mentors from the past, and I came up with the idea for me to start interviewing my former professors and colleagues (in both the US and UK) about their craniofacial research and its potential impact and benefits for families of children with craniofacial anomalies as well as the public. These interviews would be videotaped, collected, edited, and ultimately compiled into a publicly accessible online video program as an educational resource.

So, with the help of my current supervising professor and our lab’s grant manager/travel arranger, I made plans to travel to the United Kingdom in mid-January 2016. I would spend a week in London, give some talks and do an interview of my former professors at King’s College London, then spend three days in Dundee, Scotland, to interview another colleague at the University of Dundee Dental School. I eagerly counted down the months, then the weeks, then the days, to my trip; I was excited to get back to London after so long.

After a nonstop overnight flight from Denver to London, I spent my first day reestablishing my bearings and exploring long-familiar parts of the city. The next day, I got right to work. I visited my alma mater (the 18th century Guy’s Hospital campus of King’s College London) and met up with a bunch of my former anatomy lecturers and reconnected with my craniofacial professors and colleagues (and met new ones) in the department’s laboratories on floor 27 overlooking the Thames. I gave a lecture to my craniofacial and anatomy colleagues recapping what research I have done over the years since I graduated from King’s in 2007, including my work on the effects of hypoxia (low oxygen) in early developing chick embryos. Over the next few days I sat in on other lectures in the craniofacial department. The most important thing I did, however, during that week, was that I interviewed three of my colleagues (while being videotaped) about their research, what advances they have made, and what impact these advances could make on the public and families with craniofacial conditions. We also discussed strategies for education and outreach to the public, as well as how researchers and craniofacial doctors could communicate and work with each other (and ways to promote integration between them) and how we could stimulate global cooperation amongst researchers.

The following week, I traveled north to Dundee, Scotland, to meet a newer friend/colleague who was a professor at the University of Dundee Dental School who also did craniofacial research. Over dinner one evening, we discussed at length our research and strategies for public outreach and advocacy for craniofacial research and awareness, as well as strategies for research-clinical interaction and global research collaboration. The following day, I interviewed him like I did my London colleagues.

All too soon, it was time to head back home to Denver. Revisiting London only strengthened my desire to return there one day long term if my career provides the opportunity. These interviews are only the first two of a series of interviews I intend to collect (from both US and UK colleagues) and ultimately compile into a publicly accessible educational video resource. I look forward to one day making this available as a valuable resource for families with craniofacial conditions as well as the public.