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.
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.
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.
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?
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
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.
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!!
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
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://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
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!
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!!
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
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.
Hi Kelly!
I found Clara’s story very uplifting. My son is 11 months and we just found out he has ectodermal dysplasia. He also is a sweaty little guy and we wouldn’t have known if it wasn’t for his teeth coming in. It was very scary and shocking when we first found out and we spent a good week crying off and on adjusting to the news. Honestly our son is a bright, curious, happy guy and we are blessed! They are just so innocent and the mom guilt can be overwhelming. We are also located in British Columbia and I was wondering if you have any other information/ advice. Thank you so much for sharing!
Hello, Natalie. Thank you for sharing. We’re happy that you have found comfort in Kelly’s blog. We are here for you and your family. Please reach out at kelley@nfed.org if I can be of any assistance.
All my best,
Kelley Atchison
NFED, Director, Family and Community Programs
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.
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
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.
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.
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
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
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!
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
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
Tim, thank you for your kind words! She is such a happy little one and like you said spreads joy wherever she goes.
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.
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.
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.
Hi Keith,
Emailed you. Look forward to connecting.
Kelly
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!
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
Hi, I just came across your daughter’s story. I am a 26 year old woman with the exact same symptoms as your daughter. I’ve never met anyone or heard a story about anyone with the same condition as me. For a majority of my life, my family didn’t talk about the fact that I was clearly different…I mean we still don’t. Just seeing her story is so important to me and I feel so happy to know I’m not alone. I have been to many dentists who have never diagnosed me with anything, just told me they’d never seen anything like it. I was born missing 10 of my teeth, they’re very spaced out and sharp. Most of my teeth are baby teeth and I wear dentures everyday. While my condition is mostly aesthetic (as far as I know), it has affected me mentally my whole life as kids were not the nicest in school. I’m so grateful to have come across her story today. It feels good to have a name for my condition, so thank you so much for sharing
Hi, Kylie. We’re glad that you have found Clara’s story and our website. We’re sorry to hear that other children were cruel to you. That must have been very difficult for you. Getting a diagnosis is often difficult for our families and it’s incredibly frustrating. We hope that you perused our website as we have lots of information that could answer many of your questions. We also encourage you to complete this form at https://nfed.org/join-us/ so that you can receive updates, information about ectodermal dysplasias and other useful information. Also, know that our staff is always here for you if you would like to talk. We offer one-on-one support over the phone or through email. You can reach our team at 618-566-2020 or info@nfed.org. You are not alone! We are here for you. Just let us know how we can help. ~ Jodi, NFED, Director, Marketing and Communication
Kylie,
I am pleased to know you connected with Clara’s story. So sorry to hear about your struggles surrounding your differences. It brings tears to my eyes thinking about this potential for Clara also. I certainly encourage you to reach out and use the resources available through NFED. Please know though that you are beautiful! You have something unique so many others don’t! But you also share something similar with a select few! When I look at my little Clara I see more than just her teeth and hair. I see a smart individual with drive and passion. I also see a kind and empathetic person. I see her character! I’ve been able to connect with one lovely family already and welcome a conversation with you also. Contact the NFED and they can help us to do so if you feel led to.
With care,
Kelly
Wow Kelly. My heart just sank. This is my 33-month old daughters story. She hit all benchmarks way earlier than expected, she was also walking at 9 months, then the teeth started erupting. So very slowly. As soon as the first tooth appeared, I knew something was not normal about the pointed appearance. Then the 2 front teeth erupted and both are cone-shaped. The rest of the teeth we have so far are all abnormally shaped. We are missing the primary lateral incisors and her pediatric dentist has confirmed with x-rays that she is also missing the permanent lateral incisors. Her nails are horrible, with constant “hang nails” as she says. Her hair is lighter than the rest of the family and is very fine on top but wirey and unruly in the back. It is growing but at the slowest rate. I can’t do a thing with her hair. Her pediatrician will not entertain this conversation with me, although I have brought it up at EVERY appointment since 8 months old. Her pediatric dentist, however, has mentioned HED but said I need to address with the pediatrician.
Mentally and intellectually, she is beyond her age. Her verbal and receptive communication amazes everyone and she is extremely bright, observant, and sassy! So glad I found your story. I will be showing this to her pediatrician at our next visit. Hopefully I can get some sort of response or reaction. THANK YOU for sharing!
Taylor, we are so glad you found the NFED and this story! If you ever want help finding a team of medical providers to help you create a treatment plan for your daughter, please let us know. We can make referrals to those that are familiar with ectodermal dysplasias.
It also may be of benefit to confirm a diagnosis with a genetic test, so that you can prepare and create a long-term treatment plan. You can read more about getting a diagnosis, including genetic testing, here: https://nfed.org/learn/diagnosis/
Let us know what else we can do to help!
Best,
Veronica
Taylor,
Reading your comment brought me to tears. I know the journey you’re walking. It feels so isolating at times. I have moved from a place of doubt and worry into one of confidence about the condition Clara, and now my son Alexander have. With support and the correct medical team you can get there too! I am so sorry for the struggle you have had to get the correct help. Please feel free to connect with me. I’d love to chat and spend time helping you through this. You can get my contact information through the NFED.
Thank you for sharing. She is a beautiful child. I just found out that I am POSITIVE for OODD and it is on both of my chromosomes. The mind blowing thing is that I haven’t ever developed any symptoms, but I am in the process of trying to have a child and am considering the odds that my child ends up having OODD. My donor does not carry it so I am hopeful, but really unsure what will happen since they will definitely inherit a chromosome with it from me.
Hi, Jackie. We encourage you to seek a genetic counselor who can explain the results of your genetic testing as well as the chances of passing it on to a child. They should also be able to explain if the child did receive the gene whether or not they would have symptoms. Good luck! – Jodi, NFED