Updated: Aug 22, 2022
November 9th is a day that passed me by just last year. This year it means a lot. It is Microtia Awareness Day
Those who know me will know that my 2nd Daughter was born on 20th August. Being our second, we initially thought it would be like our first, plain sailing with no concerns. We weren't quite right with that. Unfortunately, our first scan showed she was small, needed additional scans, and had a potential issue with her kidney. She caused many sleepless nights even before she was born worrying about what may or may not happen.
Coming up to Ada-Marie's due date, she was breached; now, we have two weeks remaining, and we are told she has less than a 3% chance of turning unaided. She turns with ten days to go...
At this point, we had decided to have a Caesarean section, long gone were our dreams of calm relaxing home birth. Ada-Marie's turning hadn't changed that plan, only complicated it somewhat. We were called in at the last minute due to having some space at Stoke Mandeville hospital, and my wife gave birth the next day.
Those who have had a caesarean or been in the room will know just how strange it is to speak about one thing to the anaesthetist about the zoo, and the next, you are told you have a baby coming out. Now other than the surrealism that is a baby just appearing from my wife, I was delighted to see that she was not the tiny baby we feared she would be.
I straightway walked over to see her in her gory glory and noticed that she had some skin tags and an issue with her ear in addition to a port-wine stain (3 in 1,000 babies or 0.3%) above her right eye. At this point, I haven't thought much about it as she is minutes old and I am more interested in getting to hold her and show my incredible wife the daughter she has spent the last months caring for. We were then told that Ada-Marie had a 'True Knot' in her cord, meaning there were some very relieved midwives in the room that we elected for the c-section.
This happens in just 1-2,000 pregnancies (0.05%).
Once we returned to the ward, I realised there was more to the ear, and my wife noticed she had no ear canal. We mentioned this to the doctor, who then mentioned 'Microtia and Atresia' for the first time.
If, like me, you read these words and aren't sure what they are, you aren't alone. My wife and I didn't know what it was despite seeing people first-hand with this condition. For those that don't know, Microtia (Greek for ‘little ear’) is a malformation or misshape of the external part of the ear (pinna) and can vary in severity from the ear being smaller than expected to various degrees of absence of the outer ear. There is no conclusive cause of Microtia.
The odds of a child having Unilateral Microtia are 1 in 6,000 (0.016667%)
Ada's Microtia is not too severe, especially in comparison to some, and her ear is mainly formed, as you can see. She does have a couple of skin tags, and other than its cosmetics, it doesn't cause her discomfort.
The more troublesome issue is that she has Aural Atresia as well, which has caused her lack of ear canal. This means she has conductive deafness in her right ear. Atresia is essentially the blocking of an orifice, it could be almost any in the body, but in Ada-Marie, it has presented on her ear. This ultimately means she will and already does struggle with sound direction; she finds it difficult with multiple sounds. So we must remember not to play the Hamilton Soundtrack in the background when we are playing with her, and we have to make sure she can see us to communicate with her.
Aural Atresia occurs 1 in 10,000 babies (0.01%)
Both of these conditions happen in the first stage of pregnancy (around nine weeks), and little is known as to why this happens, and there is rarely any genetic link. All of this occurs during the same stage of pregnancy as kidney development, so very often, the ears will be at least checked if any kidney issues are present in pregnancy. Honestly, I hadn't thought about it before, but the link between kidneys and ears is definitely something new for me. Here is a link if you're interested in finding out more.
During our research, we have found many helpful resources, from the Microtia UK website to Facebook groups and free video sessions on bone conducting hearing aids. I want to thank them all on behalf of Ada-Marie, my wife and me. They have been fantastic, and the community is very supportive and kind. Microtia scared us at first, and tears were shed when we were told officially she was deaf. With the support and education these sites, communities, consultants, and families have given us and seeing just how incredible Ada is. We are now comfortable with what it is. I want to thank you and encourage you to help support these charities wherever possible.
Regarding Ada's Ongoing care, we have been referred to Great Ormond Street for any potential scans, and we are embarking on a long road of tests and scans. We have also been referred to genetics to look for any possible syndromes that have caused the collection of issues Ada presents. We are on a journey of discovery, but Ada has been and is very happy throughout. She is developing perfectly and has already given our family an incredible amount of joy. She has shown strength and has allowed my wife and me the ability to grow and learn as well.
Thank you once again for reading what is a long post. Thank you for spending the time on a cause close to my family and me. And finally, thank you to Ada for teaching me to be stronger.
P.S. The bayesian maths behind finding the probability is slightly beyond my current Python skills. I am not able to tell you the exact % of all these things happening together, but either way, she is an impossible (improbable) child.