By Kelly Koch

In September 2017, my husband and I excitedly became parents. The blessing of a child suddenly became our reality, and we gladly accepted our new titles of “Mom and Dad.” As I began to see life through the tiny eyes of our new baby girl, Clara Gene, the way I interpreted the world changed.

Clara as a newborn with a medical bracelet and a head covering.
Clara Gene, only a few minutes old
Photo courtesy of Irene Hannestad

It was no longer about me and my desires, but about Clara’s. I found myself continuously reflecting how my responses and reaction to any situation would affect her. Although becoming parents was brand new and filled with uncertainty, we were excited to live this life that God had blessed us with. 

At delivery, Clara was strong, and she met the standards one would expect of a healthy baby. Her gross motor skills, fine motor skills, and intellect progressed normally. We generally had no concerns about her health.

In fact, she developed far more rapidly than we expected she would. She rolled over at three months, crawled at six, and walked at nine months! It wasn’t until her teeth began to erupt that we had any reason to be concerned or even question her health.

Something Was Different

As her baby teeth began to grow, we noticed they were all conical in shape and quite pointed. Her teeth looked much like a baby kitten’s or shark’s teeth. I wept so much upon this development, feeling sad and alone. 

No one I knew had experienced the same thing, and my initial Google searches only led me to articles about syphilis.

When I posed questions on parent chat groups, no one had seen such a thing. When I asked my personal dentist about it, he also had no answers.  It was at this point we sought out help from our family doctor.

Within a year of working with a pediatrician, a team of dental specialists, and a geneticist we received Clara’s diagnosis: odonto-onycho-dermal dysplasia (OODD).

Clara received two mutated copies of the WNT10A gene, one from Eric and one from myself. As my husband and I possess little to no symptoms ourselves, we were completely unaware of this being a possibility for Clara. 

Now almost three years old, Clara still hasn’t developed her lateral incisors and only grew three out of her four second premolars. Overall, her teeth are quite gapped and small. Each tooth took six months to fully emerge which made the teething process prolonged and agonizing for her.  

Kelly and Eric Koch sit next to each other. Clara sits on her dad's lap.
Clara, at 23 months old, with myself and her dad, Eric

According to our pediatric dentist, we won’t know what this means for her adult teeth until the age of six or seven. At that age, they will be developed enough to detect properly in an x-ray. Generally speaking, children with Clara’s condition are missing most, if not all of their adult teeth. If they are lucky enough to get some adult teeth, these are often malformed too.

What This Means For Clara

We learned that if a child does not have adult teeth, their jaws are unable to develop properly without physical manipulation and intervention from a dental team. Clara might need dentures for most of her childhood until her jaw is fully developed, and she is also capable of receiving implants. 

Clara is standing in a field of tall grass wearing a pink shirt and a blue sun hat.
OODD syndrome causes Clara’s teeth to be shaped differently.

Thankfully, at this point in time, the only predominant symptom Clara has affects her teeth. Some of the other minor symptoms affect her hair, skin, and nails. Her hair is thin, but it is growing slowly.

She has eyebrows and eyelashes. She sweats profusely, and thus, her sweat glands have developed normally. The skin on her feet peels very easily. Her nails are brittle and concave, and we have yet to cut her toe nails. 

I understand there are more difficult conditions in the world and Clara’s seem mostly aesthetic, but we know this path for our little girl isn’t going to be an easy one. It certainly hasn’t been an easy one to defend or process, even as her mom.

Will she have kids now? How will others treat her? Will she be bullied because of this? If she has dentures as a little child, what does this look like? Adults ask me if I’m part vampire. Certainly she will hear worse in school, right?

Clara is smiling and looking up.
She has such a radiant smile!

There was a lot of panic and worry at first. A lack of peace and a state of constant concern. I had to mourn the loss of “normal.”

I questioned for nearly a year if we should have more kids or not. I felt conflicted knowing I couldn’t look at Clara one day and tell her, “I didn’t want any more kids like you.”

Clara Is A Beautiful Rarity

Clara’s smile may be different, but it is still as beautiful as anyone else’s.
Photo Credit: Olive Bresciani

Oh, have I been humbled. Apologies were written. Forgiveness was asked.

My empathy for others has grown tenfold, and I’ve found peace knowing that Clara, like me and everyone else, is a child of God.

She is fearfully and wonderfully made. God gave her the character to overcome and persevere. Wherever she goes, she goes with a force and she is strong. Her story is unique and special.

Her story has taught me to trust God even more which has tested my faith and made it stronger. I’m thankful for exactly the person Clara is and I wouldn’t change having her for anything. 

NFED Community Connections

Today, we are thankful for the community at the National Foundation for Ectodermal Dysplasias (NFED). They offer the support we need to help bring peace in our times of worry. The relationships we have begun to establish have been loving and kind.

My husband and I welcome connection with others going through anything similar. Understand and know that we are not alone in this. Together, we can support each other, educate, and bring awareness about ectodermal dysplasia. 

Kelly Koch is a guest blogger for the NFED. She lives in British Columbia with her husband, Eric, and their daughter, Clara.


Share Your Story

23 comments on “Wonderfully Rare”

  1. 1
    Tim Mickelson on June 10, 2020

    Kelly – Thank you for sharing your story. What a beautiful family. Clara is truly a radiant little girl. She is filled with joy and light!!

  2. 2
    Caitlin Gosling on June 10, 2020

    This is a beautiful story and hits home with me as our family has just discovered the same diagnosis in our four year old daughter- double mutations of the WNT10A gene causing the teeth issues and thin short hair as well as some sweating issues and brittle nails. It’s taken over a year and a half to get to this point and we still feel so alone and so lost. Thank you for sharing your story

    1. 3
      Jodi Edgar Reinhardt on June 11, 2020

      Hi, Caitlin. We are so glad to know you found your way to our website and can relate to Kelly’s story. Please, please know you are not alone. WE encourage you to fill out this form at https://www.nfed.org/join-us/ so we can send you information and support. If you would like to talk, call our office at 618-566-2020 and ask for Kelley. She can listen and answer your questions. She can also connect you to other families who have the WNT10A gene if you so wish. We’d really like to talk to you! We are here to help. ~ Jodi, NFED, Director, Marketing and Communications

    2. 4
      Kelly on June 11, 2020

      Caitlin,

      The initial feelings are so hard when discovering something is different with your child. Would love to connect with you on a deeper level. You can find me on Facebook as Kelly Jo. Please reach out via messenger. I also recommend what Jodi has suggested for connecting with the NFED. They have some great resources. You are not alone. Lots of care!

  3. 5
    Trisha Stoller on June 10, 2020

    Thank you for sharing your story! We to have experienced similar struggles with our daughter Kambree now 6 soon to be 7 with an unknown type of Ectodermal Dysplasia! You have a beautiful family!!

    1. 6
      Jodi Edgar Reinhardt on June 11, 2020

      Hi, Trisha. Getting that diagnosis can be such a long and frustrating journey. Know that we are here to help and listen in any way we can! ~ Jodi, NFED, Director, Marketing and Communications

    2. 7
      Kelly on June 11, 2020

      Trisha, thank you for your kind words. I’m so happy you were able to connect with our story. Please feel free to connect with me on Facebook. I go by Kelly Jo on there. I find so much comfort talking with other parents going through something similar.

  4. 8
    Selma on June 10, 2020

    Kelly,
    My daughter had a similar condition.
    We’ve been through all this.
    First, let me say you’ll get through this.
    Second, find an excellent dental specialist.
    We found one and he guided us through the process.
    While she was small, our dentist made “braces for her to fit on what teeth she had and filled in the missing teeth with fake ones.
    So she just looked like she was wearing braces.
    Unfortunately she wore that type of thing through high school.
    In college we were able to have implants put in.
    They look just like real teeth and wear just as well.
    Her smile looks wonderful!
    The problem you might face is other children, especially in middle school, making fun of her.
    Pay attention to this and give her lots of support and help.
    My daughter is a very loving and compassionate person and has some wonderful friends. True friends won’t care.
    My daughter now is in her 30s and is an accomplished professional with an excellent career.
    So you can get through this and come out great.
    Your little girl can have a great future.
    I just want to encourage you.

    1. 9
      Jodi Edgar Reinhardt on June 11, 2020

      Hi, Selma. Thank you for sharing your story and wisdom which is incredibly helpful. Sounds like you did great job parenting and that your daughter is doing so well! Love that. ~ Jodi, NFED, Director, Marketing and Communications

    2. 10
      Kelly on June 11, 2020

      Selma,

      You seem like a wealth of knowledge! I didn’t even know the braces with false teeth was an option. We are currently working with the pediatric dental team at our local children’s hospital. They have access to funding and support financially. As Clara gets older we will certainly be researching to find the best dentist possible. As it stands we have only had to monitor her progress this far. I’m curious to know at what point you had to begin with more invasive treatments.

  5. 11
    Kimberly Brokaw on June 10, 2020

    I understand your feelings. I too have a 15
    year old daughter with the same issues.
    She is beautiful and strong, but only sees her Teeth and thin hair.

    She now has 2 false teeth inserted into her braces until she is old enough for implants.
    There have been many tears.

    Stay strong.

    1. 12
      Jodi Edgar Reinhardt on June 11, 2020

      Hi, Kimberly. The teenage years can be tough, right? So heartbreaking for both your daughter and for you as her mom. Sounds like you are doing what you can to help her through this time. Keep it up! If you ever want to talk or need support, know that the NFED team is here to listen! ~ Jodi, NFED, Director, Marketing and Communications

    2. 13
      Kelly on June 17, 2020

      Kimberly,

      Thank you for your kind words. The battle girls continually face about their image is so hard. Praying for this continually over little ones affected by ectodermal dysplasia.

      Kelly

  6. 14
    Jenna on June 11, 2020

    Hi Kelly!

    My daughter Harper (also born in 2017) displays all the same symptoms, and it sounds like my journey as a mother and parent has been so similar to yours. From the tears, the unknown, a tentative diagnosis and the hope and the fears, it has made me so aware and compassionate of others, and also grateful too. From the moment her initial teeth popped through to now, I worry so much every day in what she is yet to face. But hot dang these kids are amazing little forces, and there’s no doubt they will teach us so much more than we will teach them in many ways. Clara is so perfect and I adore her little smile. Best of luck in your journey, you guys are not alone!

    1. 15
      Jodi Edgar Reinhardt on June 11, 2020

      Hi, Jenna. You hit the nail on the head. The journey to a diagnosis is both unique to each person but also similar to many others who have a rare disorder. In that, our families do bond. And you are so right in saying no one is alone! That is truly why the NFED exists…to assure all families that 1) they are not alone; 2) we can help with information and support and 3) your little person is going to be just fine. Yes, there are likely going to be hard times and challenges, but with information, support, a positive attitude, you can expect great things for your child and family! We’re here to help. ~ Jodi, NFED, Director, Marketing and Communications

    2. 16
      Kelly on June 17, 2020

      Jenna,

      The initial feelings of the unknown are daunting. Having answers, I’ve learned, are a blessing. With the answers we can be proactive to support our little ones. Please feel free to connect with me further on FB. My name there is Kelly Jo. Would love to chat more personally.

      Kelly

  7. 17
    Kelly on June 11, 2020

    Tim, thank you for your kind words! She is such a happy little one and like you said spreads joy wherever she goes.

  8. 18
    Keith on July 1, 2020

    Hi Kelly – thank you for your story. I have a daughter about six months younger than Clara who is going through some of the same issues. I myself have a mutated copy of the WNT10A gene and have a full set of implants and dentures attached to them.

    Prior to having children, my wife and I went through genetic testing. My wife does not have any WNT10A mutations so we were hopeful that our children would not be as affected as me. So far our daughter looks like she will have to have extensive dental work in her future. We have another younger daughter and it’s too early to tell for her.

    We would welcome connecting with you. I know my wife would appreciate some mother-to-mother connection in this. I can provide my email if that helps.

    1. 19
      Kelly on September 3, 2020

      Hi Keith,

      I am so sorry I am just reading this now. Please reach out. I would love to connect with your family. I am truly sorry you are going through a similar experience as ours. It’s so sad to think of our little ones differences. I imagine you know this far better than the rest of us having lived it yourself. Your wife can seek me out on FB under Kelly Jo. I look forward to connecting.

  9. 20
    Keith on September 5, 2020

    Kelly, I’m told that NFED provided my contact info to you. My family would welcome connecting with yours – please reach out at your convenience.

    1. 21
      Kelly on September 9, 2020

      Hi Keith,

      Emailed you. Look forward to connecting.

      Kelly

  10. 22
    Brandy on October 24, 2020

    Seeing sweet Clara reminds me so much of myself growing up as I have the same condition. One of my sons is a carrier and doesn’t sweat, my other son is homozygous for a different variant on the wnt10a gene. He has a couple very pointed teeth, he always bites his tongue and cheeks, it breaks my heart. His feet constantly peel, and we all have issues with our nails and toenails. For my permanent teeth, I only had my two top front teeth and three molars come in. If you ever want to chat I’d love to talk with you!

    1. 23
      Kelly Koch on October 26, 2020

      Hi Brandy,

      Thank you so much for sharing with me. I’d love to connect and speak further. Let me know the best way to do this.

      Kelly

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