Growth and Nutrition for Children with Ectodermal Dysplasias

(This is an excerpt from A Family Guide to the Ectodermal Dysplasias Syndromes, published by the NFED.)

Growth abnormalities are common in children with ectodermal dysplasias.  Weight deficits are present at an early age and persist throughout adolescence. Height deficits are seen primarily in children with ectodermal dysplasias other than hypohidrotic ectodermal dysplasia (HED). Parents should monitor their child’s height and weight gain each time they visit the pediatrician’s office.

The pediatrician generally plots the child’s height and weight measurements on standardized growth charts prepared by the National Center for Health Statistics. These growth charts serve as the barometer for assessing the health, growth patterns, and nutritional status of all children, including those with ectodermal dysplasias.

Good nutrition is essential to maintain normal growth in children with ectodermal dysplasias. Although chewing difficulties may impair their eating capabilities, cooking or blenderizing can modify food textures to facilitate better nutrition.

The use of dentures at an early age also promotes good nutritional health.  Adequate oral intake of dietary energy (carbohydrates and fats), protein, vitamins, and minerals is essential for normal growth.

The best way for children with ectodermal dysplasias to develop good eating habits is to follow the recommendations of the U.S. Department of Agriculture’s (USDA) food guidance system, “MyPyramid”, for children.

MyPyramid Food Groups

MyPyramid is divided into six food groups: grain products; vegetables; fruits; milk and dairy products; meat, fish, poultry, eggs, and beans; and discretionary fats and sweets (figure above). A well-balanced diet for children with ectodermal dysplasias includes daily servings from all the food groups in amounts sufficient to meet their energy and nutrient needs for normal growth.

Chart of Age and Energy

The best way to assess the adequacy of the child’s diet is to determine food portion sizes based on his or her energy needs.  A child’s daily energy need is estimated from his or her age and level of physical activity (figure above).

Once energy needs have been determined, the child’s pattern of food consumption and serving sizes can be derived using the USDA website. For example, a 10-year-old boy who is physically active for 30 to 60 minutes daily has an energy need of approximately 1800 kcal per day.

Based on this information, he should eat approximately 6 oz of grains, 2.5 cups of vegetables, 1.5 cups of fruits, 3 cups of milk, and 5 oz of meat or beans. Children with ectodermal dysplasias who have growth deficits need to consume larger food portion sizes and may use more discretionary foods to restore height and weight deficits.

If an ectodermal dysplasias child’s appetite or weight gain is persistently poor, parents should seek advice from a physician. Additional diagnostic studies and therapeutic interventions may be warranted.

(Editor’s Note:  This summer in Denver, researchers are investigating growth issues in Goltz syndrome. Learn more.)

Other NFED Blogs of Interest

My Smile – 23 Years in the Making – Part 3

Overwhelmed with Love and Belonging

The Battle of Hair

My Smile – 23 Years in the Making – Part 3

Jacob with his parents, Tina and Richard.

Jacob with his parents, Tina and Richard.

By Jacob Moss

This is part 3 of my dental implant story. Part two left off after I finished up my upper jaw bone graft.

As previously stated, following the bone graft there was a period of about six months before it could be determined if the graft was successful. During that time I returned to life as a full-time college student. Fortunately my graft went as planned and after six months I was given the green light to proceed with my treatment plan.

Next up were the actual implant surgeries. Compared to the bone graft, the implant surgeries were easy. I had two separate procedures that were spaced over the course of about a year. The first procedure involved six upper implants. I was surprised at how straightforward the surgery and recovery was. I was in and out of the doctor’s office in a few hours.

After about two weeks, it was as if I hadn’t had surgery at all. I was extremely diligent about following the doctor’s post-op instructions which is why I think my recovery was so quick. Following the surgery there was once again an extended period of recovery to give the implants time to heal. After about six months my implants were uncovered and tested for strength to determine if they were successful and ready to support an upper bridge.

The time eventually came for my final implant surgery. At this point I had a temporary upper bridge that was affixed to my implants as well as my natural teeth. My lower jaw had nothing but my natural teeth. This surgery was a little bit more ambitious than my first implant procedure. Instead of capping the bottom implants and letting them heal before attaching them to a bridge, we decided to affix a bridge to my new lower implants the very same day.

It was explained to me that loading the implants immediately slightly increased the risk of implant failure but after talking it through, it was clear that the it was worth the risk. This meant that rather than wait another six months, I would leave the doctor’s office with a full set of (temporary) teeth for the first time in my life.

This procedure was a little bit more complicated than my upper implant surgery for a number of reasons. Not only was I getting four new lower implants but I was also having all of my natural teeth pulled. Up to this point my upper bridge was attached to my implants and cemented onto my natural upper teeth. The problem with this was that the natural teeth prevented the bridge from sitting flush with the implants.

Once my teeth were pulled and my lower implants installed, Dr. Simon (my prosthodontist), joined the party. He arrived to assist my oral surgeon with reinstalling my upper bridges and installing my lower bridges for the first time. This whole process took quite a while because he had to modify the temporary bridges to ensure a correct fit.

All in all the final procedure took almost 12 hours! It was a very long day but I was pretty out of it the entire time so I didn’t experience any pain. As far as I was concerned the whole thing took 15 minutes!

Following my final surgery I took it easy and once again followed my doctor’s instructions regarding the healing process. In a few days I felt pretty much back to normal except for one huge difference: I had bottom teeth for the first time. Dr. Simon warned me that it could take me a while to get used to my new teeth.

Much to my delight and surprise, I adjusted quickly and didn’t have difficulty chewing or speaking. Being the pessimist that I am, I had assumed I would have to relearn how to talk and how to eat. It did feel different chewing and talking but after a few days I was able to adapt to my new mouth set-up and within a few weeks it was like I had always had a full set of teeth.

I had all my implants but I still had temporary bridges.  Now came the most tedious part of the entire dental implant process: dialing in my bridges. Every few weeks I would go to Dr. Simon’s office and depending on the day he would sometimes remove my bridges and take bite impressions or leave the bridges in and take impressions.

He took many detailed photos of not only my teeth but also my face, smile and side profile to see how the teeth looked. Every few months he would install a new temporary bridge that had been slightly modified to fit and look better. This part of the process got a bit frustrating but I am very thankful for how detail oriented Dr. Simon was.

He was meticulous because he wanted to be absolutely sure that when he had the final bridges made that they would fit perfectly in my mouth both for aesthetic reasons and most importantly for health reasons. Finally the day came and I received my final bridges. The funny thing is that they didn’t really look any different from my last temporary bridge. The only difference was that the finals were made from a much stronger material.

I wish I could say I had some glorious moment where I broke down in tears of joy upon realizing I was done with the whole implant process but instead it just felt very normal which I figured was a good sign.

Some Concluding Thoughts

The entire process itself was a slow one. I could write chapters and chapters on each of my appointments and procedures. Luckily, I did not experience any significant complications. As far as the procedures and the healing process goes, everything went as planned.

The most challenging part of this entire adventure was the anxiety and fear I experienced before and during the process. I was anxious about the surgeries and what having a full set of teeth would be like. Would I look different in a bad way? Would I be able to talk normally? Would I be able to chew my food? What happens if something goes wrong with an implant? These were things that were constantly running through my head.

While those concerns were perfectly normal to have, I was channeling them in a not so helpful way which caused me to feel incredibly anxious about the overall process. Part of this was because of the experience I had with the first doctor and part of it was because I am an anxious person and always have been.

Before each of my surgeries I would work myself up to the point of exhaustion. When the surgery was over and I was fine, I would feel extreme relief. My rationale was that the pre-surgery anxiety would prepare me for the worst and if the worst didn’t come then I’d be relieved.

Thinking back now, I realized that was a silly way of going about it. My feelings were completely justified but instead of taking a constructive route to minimize my anxiety I was stubborn and caused myself much more mental anguish than I should have.

I started my dental implant journey as a 17-year-old senior in high school, I ended it as a 23-year-old college graduate with a full-time job. It was a slow process, at times an uncomfortable one. It still hasn’t fully sunk in that I’m done but the one thing I know for sure is that deciding to get implants was one of the best decisions I’ve ever made.
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Jacob and his finished smile.

Jacob Moss is a guest contributor to the NFED Blog. Read parts one and two of his story here:

Overwhelmed With Love and Belonging!

By Terri Andrews

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Currently, I am the new liaison for the NFED in Alabama!

I was born in a little rural area in Alabama in 1959. Not much was known about ectodermal dysplasia nor the type of ectodermal dysplasias I was born with called ectrodactyly-ectodermal dysplasia-clefting (EEC) syndrome. If I received any type of major treatment or surgery, I had to travel at least 100 miles, which was not always easy.

When I found the NFED in my 50s, I only knew a little about EEC from some papers that a surgeon gave me when I was in my 20s. The NFED has been my life saver, giving me valuable information and meeting others with ectodermal dysplasias, especially with EEC. They have become my family. I finally knew I was not alone. There were others like me.

I asked the NFED if they had educational awareness material to give out about ectodermal dysphasia and they did. When I received them, I passed them out to every doctor, surgeon, and dentist I went to and I also told them about the NFED. I wanted to get that information out there in Alabama for us and future children affected by ectodermal dysplasias.

Terri Andrews 2015

Myself and my wonderful husband Joe at the Tuscaloosa, Alabama Combined Federal Campaign Kick Off creating awareness for the NFED.

CFC II

When Lea at the NFED asked me if I would help with the Combined Federal Campaign (CFC) in Alabama, I was excited. I wanted to tell as many people as I could about ectodermal dysplasia and about the NFED.

I wanted to get the information about ectodermal dysplasias and the NFED out there! There were lots of people who came by my table and seemed really interested, even doctors and nurses. I don’t know if anyone signed up, but my main purpose was to get the information out there – create awareness about this wonderful foundation and all it does for people affected by ectodermal dysplasias as well as what ectodermal dysplasias are.

Terri Blog II

Myself and my very supportive daughter who organized/helped with the Global Huxman Run 2 Sweat in Southern Alabama!

Last year, my daughter and I headed up the Global Huxman Run 2 Sweat in my area to raise money and awareness for the NFED. It was a lot of work but fun at the same time. What made me happier than anything was the passion my daughter had in getting this all together. It made my heart swell with love.♥

This year, I became an NFED family liaison for Alabama. I want others in Alabama born with ectodermal dysplasias to know that they are not alone…they have someone to talk to, ask questions, or whatever.

I remember when I thought I was the only one out there and I don’t want anyone to feel that way. I know since I have met others with ectodermal dysplasias, it has made me more confident, because I know I’m not the only one.

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Part of the EEC Chicks who try to meet up with each other at the Family Conference each year!!!

That is why I became a volunteer through the NFED’s Helping Hands Volunteer Program. I was so overwhelmed with love and belonging from the people who work at the NFED that I wanted to share that love with others along with the information that they have given me.

Check out these other NFED blogs about families who choose to give back:

The True Meaning in Life is to Sit Under Trees From Whose Shade You Never Expect to Sit

A Little Step May Be the Beginning of a Great Journey

Support Each Other: We Are Family

The True Meaning In Life Is To Plant Trees, Under Whose Shade You Never Expect To Sit!

 By Ashli Matus-George

Allyson Kelso’s Aunt

Founder, Rally for Ally

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Ashli Matus-George and her beautiful family, the founders of “Rally for Ally” with the help of family and dear friends.

When Ally was born on December 22, 2004 in Oxnard, CA, it was an exciting time.  A new baby, Christmas for her three-year-old big sister, Morgan.

My then fiancé, Tom, and I were recently engaged and planning a wedding, Kristin had a thriving career, and Mark was anticipating some family time before deploying with the US Navy in early 2005.

We were all there to celebrate the arrival of Allyson Glenn Kelso and Christmas.  Life was GOOD. Our family is always there and present for each other, that’s the constant.

It is also the prevailing theme to Rally for Ally year after year:  To show up.  Be present.  Lend a hand.  Those were my thoughts when I found the quote to support what would become our annual fundraiser for the NFED:  “The true meaning in life is to plant trees, under whose shade you never expect to sit.”

Matus I       Ally IIIII      Ally sister and Ally

To help and support my sister and her family in any way possible, I searched for some key words the doctors had shared with us and found the National Foundation for Ectodermal Dysplasias (NFED) website.   I emailed. They emailed.  I called. They called.

They immediately became invested in Ally and our lives.  We instantly became a part of their world, too.  It was then I knew I needed to do something for the NFED to help support Ally, others affected by an ectodermal dysplasias syndrome, and all the families who care for these children.

My family and I simply thought of what we could do best. We called some friends, asked my dad to barbeque his famous brisket and ribs, put the drinks on ice and hosted a backyard alley party behind our home in Washington, DC more than 10 years ago.

I asked everyone to bring $5-$10 to toss in the bucket to raise funds and awareness for the NFED.  Another friend suggested a raffle and silent auction.  Imagine my surprise when that little backyard gathering that Saturday afternoon raised $6,500 for the NFED!

Rally for Ally Matus I

Ally with her Grandma Terri & Grandpa Geep, sister Morgan, and her cousins.

I could have never anticipated the roller coaster and incredible journey our family was about to embark upon.  I also had no idea that the backyard cookout and fundraiser we hosted that first year in 2005 would eventually expand in to a local park and raise over $15,000 each year for the NFED.

Hosting a fundraiser, creating an event to support the NFED and coming up with a way to introduce the NFED and those living with an ectodermal dysplasias syndrome should be something that works for you and your family.

What business connections do you have?  What small part can you and your neighbors, school, community or place of business do to lend a hand and show support?

Another way we raise funds each year is to ask a local business owner to help us host “NFED night” or “Rally for Ally day” at their establishment, where a portion of sales during a designated time will be donated to the NFEDIt’s a simple ASK – we never know what others are willing to do and give until we try.

Rally for Ally Family Photo Major Donor Letter

Ally with Mom Kristin, Dad Mark & Sister Morgan at Rally for Ally’s 10th Anniversary in 2014

When it comes to family and supporting the NFED, let’s all do our part and just ask so that we can create opportunities to share a loved one’s story, introduce them to the world, and let them know about the NFED and all it does to give back to our families.

Rally for Ally is on Saturday, June 27 from 2 p.m. – 6 p.m. in Nottoway Park – Pavilion & Picnic Area (reserved), 9610 Courthouse Rd, Vienna, VA 22181. Click on Evite for more Info.

Other NFED Blogs

The Battle of Hair

A Little Step May be the Beginning of a Great Journey

NFED Class of 2015 – We Are So Proud of You

The Battle of Hair

When I was a little girl, my hair was slow to come in and grow. Once it did grow, it was so blonde it looked white and stuck up everywhere. It was hard to manage. My momma would put Vaseline on my hair just to try to lay it down.

When I got older and my hair was a little longer, momma would put pin curls in it and the curls would stay all day long. My hair was very coarse and had lots of body. It was not really thick but it looked thick because of all the body.

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My sister and me (pin-curls)

My hair remained blonde and very slow to grow as I got older. All my other friends would get their hair cut and before you knew it their hair would be long again. My hair grew so slow I didn’t even want to get a trim.

One day I got a wild idea and I thought I would get my hair cut short. My cousin got her hair cut and it looked so cute. Well, I hated it on me! I cried for days… no, months! It took FOREVER for it to grow back out! I never got my hair cut again, except for little trims.

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I was always afraid to go to hairdressers. Some would get so scissor happy and before you knew it they had cut too much. Every time I went to the hairdresser, I would have to stress, do not cut much of my hair!

When I got older, my hair got a little darker. It kind of had that mousey color. I was so used to my hair being white-blonde, I started getting highlights. It probably was not good for my hair being as fragile as it was, but it seemed to be ok. I used lots of conditioning treatments.

In my 30’s I started noticing that my hair was getting thinner. I tried all sorts of products for thinning hair, but nothing worked. Then I had a little bald patch. We have heard it said that a woman’s hair is her crowning glory, so it can be very depressing for a woman to lose her hair.

I went to the dermatologist and he did scrapings, biopsies and more treatments for the scalp, but nothing worked. My hair kept getting thinner and thinner. I tried hiding it by fixing my hair differently. But by my early 40’s, I had to try something else.

I started wearing clip-in hair pieces. Soon those started breaking my hair off, which made things worse. I tried a wig. I hated it! I was so active in playing sports, going in the pool or ocean and riding in the boat on the river. I was always worried the wig would come off. In fact, on two occasions it did. Once while playing volleyball and the other time in the ocean when a wave hit me! I was devastated!

That’s when I went another direction. I went to a hair place where they measured my head and made a hair piece that could be glued to my head. It could be treated like my own hair. I loved it. No worries of the wind blowing my hair off.

Well, that didn’t last long. I started having a reaction to the glue or something. My scalp broke out and began to smell bad. They tried different glues, but nothing worked. They finally sewed clips in the hair piece, but then I was back to square one.

I finally broke down and tried wigs again. It took a lot of trial and error to find one that didn’t look like a wig and didn’t make me look like I had too much hair. Finding the right color was also a challenge.

Some wigs I tried I had to keep because they had no return policies or you could only exchange one time. So, now I always read the wig companies return policies. I have a small head, so petites fit the best. The only problem with that is that they don’t make many styles in petites.

You still have to watch when people or kids want to touch your hair or if someone is hugging you. You have to be careful when you scratch your head or you might readjust your wig. Watch out if the wind is really blowing! If I know I’m going to do anything active, I use the wig tape and it does hold it pretty well.

Wigs can be hot and itchy at times. It is something that you have to get used to. At times, I think of going bald and forgetting it. Some people can pull that off. I just can’t bring myself to try that yet.

Maybe one day they will invent something better than wigs, or better yet, something that will grow hair. I know that ectodermal dysplasias researchers are now able to grow new skin and hair on mice, so maybe someday they will be able to do that on people!

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Terri Andrews is an NFED Family Liaison. She lives in McShan Alabama with her husband Joe. Terri is affected by Ectrodactyly Ectodermal Dysplasias Clefting Syndrome (EEC).