Akilah’s Nose: What is Frontonasal Dysplasia/Median Cleft Face?

Special thanks to Children’s Miracle Network Hospitals for sponsoring this post!

Akilah Skye, our long-awaited baby girl, came to us on the sixteenth day of August, after a very short labor of just three hours. Upon her descent, she was scooped up, wrapped in a blanket and placed into my arms. Finally, she was here and I could soon relax and be home to see Jayden off to school the following week.

Exhausted, I looked down to get a better glimpse of our HamburgHer (her nickname while in utero) and there it was, the what if that occupied a little too much space in my head from the moment I saw those two pink lines. There were no signs or warnings or anything like that. I just felt the need to prepare for whatever, become resilient and hustle for the next nine months.

“She has a big nose,” my husband commented from a place of shock and concern. Her nose, visibly flattened yet pronounced with a divot, was the start of a new journey neither of us, I felt, would have been prepared for now, but certainly not three kids ago. After spending about an hour cuddling with and nursing baby Akilah, she was off for an extended round of tests.

We would meet the neonatologist. She would come in and mention one thing, then another thing. They needed to put a scope down her nose to make sure things were clear, that her cry face was a little crooked (we all have that crooked left lip curl in my maternal family) so bells palsy was a concern, the ears, nose and throat specialist would be in to see Baby, as would a geneticist.

Geneticist.

See. It’s all fun and games when we’re all casually talking about good genes and general quirks passed down through genetics, but there’s nothing comforting about having a geneticist involved in the care of your newborn.

The geneticist explained to us that it appeared that Akilah had a minor form of Craniofrontonasal Dysplasia. It is a condition in which a cleft was formed in her face (similar to a cleft palate), causing a shift in the formation of her nose. Her palate wasn’t affected; neither was the function of her nose. The condition is very rare, but seems to affect more females than males. Wanting to believe that the cramped quarters of my uterus and carrying low was the cause of her nose malformation and any other asymmetry, we continued to smile and nod.

The geneticist pulled out a book and shared some photos of other children who had more severe cases of the condition. Can you say awkward? *sigh* This was our moment to celebrate our new baby girl, but instead we were entertaining the idea of being two already worn out parents to a special needs child. The point was to further explain that the cleft usually causes wide-spaced eyes… but whatever… Brandy Norwood rocks her wide-spaced eyes, honey.

After doing some research, I discovered that frontonasal dysplasia usually causes a widow’s peak hairline as well… but whatever… I thought it was the cutest ever, now shared with her paternal Gamma and Titi. After we had gotten enough of the photographs, the geneticist asked us a round of questions, then reassured us that we did nothing wrong. She told us not to even think about going down that path of backtracking, calculating and overthinking. Clearly, she doesn’t know who I am…

While on the topic of genetics, we went over the probability of certain cancers and the onset of mental illness in the both of us as parents as well as passing them on to our children. She answered pressing questions that I’ve had about myself and alleviated fears I’ve been carrying as I near thirtyhood. It’s funny how receiving my baby’s diagnosis turned into a lesson and relief regarding my cognitive health. The geneticist was a passionate one and knew her stuff. She gave us names of top child craniofacial experts in the region, fun facts, and most importantly hope.

Hope keeps us strong in times of need and keeps us brave in times of fear. – Jade Raber

After all was said and done, baby Akilah’s health report was great and we would continue to watch her as she developed. If we had any concerns, we’d be able to take advantage of the Early Intervention program available to children diagnosed with conditions that may affect their development. As for the physical appearance of her nose, we decided to consult with a plastic surgeon who can work on the symmetry a bit. I don’t want to get into the why, because it’s very obvious. In a perfect, non-hurting, non-beauty obsessed world, we’d leave her nose as-is. We just want to ensure that she thrives and lives a happy life.

In October, we had our first consultation at the children’s hospital. We saw a craniofacial plastic surgeon, as well as a neurosurgeon. They wanted to be sure that her nose was free of brain tissue and any other masses, so she had a CT scan and an MRI, which confirmed that her nose was only a cosmetic condition. We will continue our journey with a meeting next month. They would like to monitor her cranial sutures to make sure they aren’t closing prematurely. The results of the previous scans showed that they were normal. Our next consultation will be another step towards surgical planning. I will be documenting behind the scenes, and will share details when I feel that I can.

The support that we received in Akilah’s care wouldn’t be possible if it weren’t for Riley Children’s Hospital and the Children’s Miracle Network. There are 170 children’s hospitals in the network, and they are able to provide the very best care because people donate. Children’s Miracle Network Hospitals provide more than $3.5 billion in charitable care every single year in the U.S. Donations stay local and are used however the hospital needs them the most. These donations help purchase life-saving equipment, conduct research and pay for the special things that are crucial to a child’s healing from specialized therapy programs, equipment in multiple sizes, playrooms–the whole nine.

An exorbitant amount of money goes into running children’s hospitals, and they are always in great charitable need. Put your money where the miracles are. Support your local Children’s Miracle Network Hospital. We’re committed to doing what we can to support the network that supports us back.