Memories, Milestones and Marvel.
Our daughter was born with an incredibly rare facial anomaly. She has a condition known as Arhinia which means her nose didn’t develop. It simply isn’t there. There are very few babies like her in the whole world. As we come to the end of her first year and I reflect on how far we’ve come already I feel ready to write about what we’ve experienced over the past twelve months. We have cried… in despair and relief. Struggled… to keep it together and with exhaustion. Succeeded ….and watched our baby succeed. Laughed….. like only families can laugh at themselves. Panicked ….at the rational and irrational ……and everything in between! And we’re still just at the beginning!
I hope this blog, through sharing some of our story, will help, inspire or encourage others who may be faced with similar situations or challenges.
Her Arrival Earthside.
Tessa was born on Valentines Day. On the most love filled day of the year, my baby girl made her entrance. She was born naturally with no pain relief in a hospital birthing pool. Her birth itself was extraordinary since I had had my previous two babies via cesarean section and fought very hard to give her the best birth possible. We were expecting a perfectly healthy baby. An abnormally flat fetal profile had been detected during our 20 week anomaly scan and although we received counselling where we were told to prepare to make difficult decisions, our follow-up scans and amniocentesis were all normal. We put the trauma and heartbreak of those few days behind us and moved on with what we believed to be an ordinary pregnancy. …. But nothing in our lives has been ordinary since our beautiful Tessa choose us to be her family.
The NICU Weeks.
Tessa spent her first five weeks in a Neonatal Intensive Care Unit. These were undoubtedly some of the worst weeks of my life. Having to remain separated form my newborn and the uncertainly of our new and unexpected situation was painful and terrifying. Her first surgery at eight days old gave her a second airway by means of a tracheostomy tube to allow her to eat and sleep safely. Her consultant explained that when T was old enough to cope with eating and breathing through just her mouth, they would decannulate (remove tube) and to expect that to be somewhere between six months to a year. It was a daunting prospect but having an end in sight was encouraging. Sending her to theatre was every bit as terrifying as you can imagine but once her ‘trachy’ was in place and I completed the care training, it was our ticket home. Thankfully after thirty-three agonising days in hospital we were discharged. We were finally going to be together as a family albeit with a mountain of intimidating medical equipment and an exhausting list of follow-up and check-up appointments.
Our biggest challenge once home and my proudest achievement was establishing exclusive breastfeeding by eight weeks as Tessa had been previously tube fed. I have written our Breastfeeding story HERE and am humbled and grateful that it continues to inspire mothers around the world not to give up on their own dreams to breastfeed. Settling in to our new lives at home was made all the sweeter by the arrival of those first, incredible smiles and getting to see her personality begin to shine through…and the more she smiled and the brighter she shone, the more her ‘differences’ ‘issues’ and ‘diagnosis’ faded into the background.
Born with multiple associated eye anomalies, Tessa’s eyes kept us busy from the start. I had to quickly familiarise myself with her bewildering array of conditions including coloboma, ectopic pupilae, congenital cataract, persistent hyperplastic primary vitreous (PHPV), strabismus, microphthalmia and nystagmus! It was difficult to imagine her ever having normal sight but we knew she could see a little, so remained hopeful and concentrated on maximising the sight she had. We were told that with drops to help enlarge the small, irregular, off-centre pupil in her right eye that vision would improve. When tests showed that removing the dense cataract on her left eye might result in some vision there too, we were tentatively excited but it meant that at eleven weeks old, our tiny Tessa would face her second surgery. Unfortunately, when her two hour surgery lasted four, the news was not good. Blood vessels throughout her eye (the condition known as PHPV) caused bleeding that was not easily stopped, the result left her blind in her left eye. The after care was gruelling for many reasons, the hourly eye drops meant weeks of unrest, for weeks the surface stitches caused her pain that would only be relieved by short acting anaesthetic drops, she had to wear an eye-shield to protect the eye while it healed, we had after care ophthalmology appointments twice a week for months and the stress and hospital stay had resulted in Tessa coming down with her first nasty cold. The near-constant coughing and suctioning, day and night, that her tracheostomy required when she was unwell was truly, truly exhausting. It was a particularly difficult time for us. Our extraordinary baby, through it all, still found a way to smile and remind us that she was most definitely worth it! The Summer
While mostly restricted by heavy medical equipment, anxiety about public perception, Tessa’s light-sensitive eyes and lack of confidence in general about the whole situation, the summer passed by quietly. Our outings were almost exclusively hospital visits peppered with short but memorable excursions that made me yearn for the freedom that we would one day feel when Tessa ‘s trachy would be a distant memory and life wouldn’t be so ‘contained’. At home, in the meantime, Tessa’s relationships with her siblings was really beginning to blossom and their often hilarious interactions were some of my summer highlights.
The rest of the year would trundle by in a haze of magical firsts and a sea of used suction catheters. Tessa continued to amaze both us and the medical world with her strength of spirit and contagious smile. Here are some of her marvelous moments :
Tessa’s First Sounds
A tracheostomy prevents any vocalisation since the air passes in and out below the vocal chords. After months of longing and watching the silent crying and noiseless babble, we finally got to hear her voice for the first time when she was nine months old, with a the aid of a special speaking valve for tracheostomies. Her silence, whether crying in pain or cooing in delight is one of the most heartbreaking aspects of her trachy for me. I’ll never take a baby’s cry for granted again. A speaking valve trial was our first chance to ‘hear’ her and while we were excited there was no guarantee she would tolerate the change in her breathing. We were not prepared for how emotional that appointment would turn out to be. I’ll never forget how it felt to hear my baby girl and see how obviously excited she was to hear herself. I cried, her dad cried and the therapists assistant cried! It was beautiful.
Tessa’s First Kisses
Having been showered in kisses (often against her will) from the day she came home, Tessa’s first attempts at reciprocation was one of the absolute cutest moments of my year! Her tiny lips puckered up for smooches is still as adorable as the first beautiful kisses she blew my way!
Tessa’s First Trip to GOSH
After some research (quite a lot actually) I learned about a children’s cranio-facial plastic surgeon in Great Ormond Street Hospital in London who I felt we should meet so in September, my husband, Tessa and I flew to London for 2 nights. I was anxious about travelling with Tessa, about having to use her huge noisy suction machine in public and scared of how I might handle unfriendly stares or comments. I had for months kept to my own little bubble of people who knew and loved her to protect her (and me) from any kind of negativity and now we were heading off to a major city, to traverse public air travel, subways, a busy hotel, stations and London crowds. I was extremely nervous. In preparation I emailed the Hotel management to let them know our situation and included a picture of Tessa as I felt more confident when I knew people weren’t seeing her for the first time. After some very stressful travel (involving the airline refusing to clear her suction machine for travel) I will be forever grateful to the amazing staff who left the most thoughtful surprise in our room. The note read; “Hi Tessa, I’ve been waiting for you, I want to be your friend….”. There are some truly kind people in the world.
Tessa’s First Steps
Tessa seemed keen to prove that medical issues aside she was more than capable of regular baby antics and her determination and drive were soon evident as she devoted herself to hitting each milestone. By seven and a half months she mastered the belly crawl and was up on her knees a few months later. She was able to pull herself up to standing by month ten and two weeks before her first birthday she took her first steps. By this point I had almost stopped worrying about how much only having sight in one eye might hold her back. Her achievements and obvious joy was our privilege to watch and she filled us with enough pride to float a thousand airships. I so desperately wish that someone could have looked into their crystal ball this time last year and whispered into my ear as I sat beside her incubator. They would have told me not to cry…. told me not worry ….told me that a year from now my tiny girl with the tubes and machines would be walking through the kitchen, flashing her gorgeous smile and pointing for a cracker! It still makes me well up to think about. Here are some of her first shaky steps.
Tessa’s First Birthday.
Valentines day marked Tessa’s very first birthday. It had been without question the longest year of our lives and yet none of us could believe that our sweet little Tessa could really be ready for a birthday! In so many ways and because of the huge impact she had had on our lives in that time, it felt like she must surely have been here longer … and then on the other hand she was still so small that I found it hard to believe what this milestone indicated ; that she was growing up and would soon need me less and less. It also marked the anniversary of the day my baby was taken from me and the memory of that felt quite raw again for a few days.
In celebrating our extraordinary daughter turning one, we were celebrating surviving a truly challenging year as a family. Celebrating everything we’ve learned and the amazing people we have met thanks to Tessa. Celebrating the beauty and the importance of difference and acceptance. Celebrating her still being breastfed at 12 months. Celebrating the love and laughter that this little girl has given us in bucketfuls and most of all we celebrated a funny, mischevious, cuddly, expressive, curious, playful one year old girl …… just like any other … but who we just happen to think is pretty extraordinary.
Although the plan had been to decannulate Tessa around this time, her surgeon in London is planning a first-of-its-kind procedure to place a small nose shaped implant behind the skin on her face where her nose should be to create the profile of a nose. Her trachy will stay in place until that surgery is completed this summer (we hope) as it is much safer for a baby this young to have two airways while under general anaesthetic. While not a working airway, her new ‘nose’ will give her definition and a more ‘normal’ profile. Once decanned she should cope fine breathing through only her mouth and we are still unsure whether any kind of airway construction might be worth the risks in the future. Her ‘nose’ will be up-sized every two years, to grow with her face, using the same incision at her hairline so any scarring will be invisible. We are cautiously excited about this innovative solution and believe that this has the chance to give us the best aesthetic and social outcomes for the least risk, as despite the idea being a world-first, the process itself is a fairly simple one.
Who knows what the next twelve months have in store or the years to come after that, but I am fairly certain that none could be as emotional, challenging, life changing or exhausting as the the past year. But where a year ago we were full of heartache, fear and apprehension, we face the year ahead with hope, love and much more faith in ourselves than I ever thought possible. It almost feels silly to have worried so much. If I had to pass on one piece of advice to any parents who find themselves in a similar situation, it’s “give yourselves a year” because you won’t believe the difference a year can make.
If you would like to help our family ease the financial strain that Tessa’s condition brings, please consider donating HERE.