Thursday 22 March 2018

'She is just the smiliest, happiest child' - What it's like to have a child with a rare facial condition

New film 'Wonder' tackles the issue of a kid living with an irregular profile, something mum Grainne Evans (34) knows a little bit about

Tough decision: Grainne Evans says getting a nose implant for her daughter Tessa was a difficult choice to make. Photo: COLM LENAGHAN/PACEMAKER
Tough decision: Grainne Evans says getting a nose implant for her daughter Tessa was a difficult choice to make. Photo: COLM LENAGHAN/PACEMAKER

I read Wonder a few years ago and fell in love with the story and the characters. When I found out they were making a film, I was delighted! But I can't even watch the trailer without crying, so I'll be bringing a full packet of tissues to the cinema.

My four-year-old daughter, Tessa, was born without a nose. When I was 20 weeks pregnant with her, I had a scan that showed 'an abnormally slack foetal profile'. I remember those words so well because I was worried about what they meant. But when my husband Nathan and I came back for a 3D scan and amniocentesis, both came back looking normal.

When she came out, I knew there was something wrong. I said "baby's not okay" and when the midwife saw her face, they started hitting all the buttons and shouting for people up the corridor. Then they took her away from me.

It's horrible to put myself back in that moment because, at that point, we honestly didn't know if Tessa was going to live or develop normally. She was just 6lbs 6oz and spent the first week of her life in an incubator where I couldn't even hold her.

The doctors decided she needed a tracheostomy when she was eight days old. That tube, her 'trachy' is now such a part of her (it's basically a second airway, her 'nose' but in a different place) but back then, the thought of someone putting a tube in my tiny baby's neck was excruciating.

I spent most of her first year incredibly nervous in public. Wearing her in a carrier really helped grow my confidence because she was safely snuggled into to me and I could choose who saw her. It was helpful in other ways too. Babies with tracheostomies don't make any noise so you can't tell if they're crying - something that's really heart-breaking - so I always wore her and we co-slept, that way I knew if she was crying, I knew that she was happy and breathing.

Julia Roberts and Jacob Tremblay in Wonder
Julia Roberts and Jacob Tremblay in Wonder

Eventually my anxiety about being out and about went away and I wasn't just happy for people to see Tessa, but proud. She's always been so happy to meet people and just the smiliest, happiest child. People underestimate her - they have a tendency to assume that children with facial differences all have a learning disability or an intellectual disability, and that can be quite difficult because Tessa understands everything.

Happily, 99pc of people's reactions are positive but there have been a couple of instances in her life where I've been very upset with someone sniggering or pointing. It's not for me, but I don't want her thinking there's anything wrong with her that merits that reaction.

It's one of the reasons why a lot of soul searching went on before proceeding with the option of surgery. Also, until recently, the only option for children like Tessa was to create a nose with very invasive surgery using skin grafts from the forehead and cartilage or bones from the hips and ribs. I'd seen it done and didn't feel like the results were what I'd want for Tessa. A craniofacial surgeon, Jonathan Britto, at Great Ormond Street Hospital came up with a new option. Using 3D printing, he could print Tessa's skull, mould a little implant exactly to fit the contours of the bone in her face and insert it behind the skin.

When Tessa was two, she became the first person to have this type of surgery done and it was very, very scary - it was still scary when she had the second op a few months ago! But ultimately we believe this is the best decision for her and we're still very happy with it. She'll need a new nose every couple of years as she grows and then, with her last nose, she'll get shade and nostrils tattooed on. It won't be a working nose, but it'll be a much better aesthetic nose than has been made before.

But making that initial decision to proceed with surgery felt like someone ripping my heart out. My head said it was an excellent option and a chance for Tessa to have a much more normal profile, something I knew would help her socially as she grew up. But, at the same time, as a mummy, I thought she was already so perfect.

As soon as she came round from her last operation, she wanted to nurse. Breastfeeding is one of the things I'm most proud of. It was incredibly hard to establish, but it has brought her such comfort and I feel like, in those early weeks especially, it was so healing for me. It's something that's been there for us through every sickness, every hospital visit and every operation. I always know when she nurses, then she's okay.

There are silly little things that are hard - like try doing 'Head, Shoulders, Knees And Toes' when you've nothing to point to for a nose, and other important things we've to be mindful of like water, dust or fine sand getting near her trachy. It's also hard leaving our older children Cassie (6) and Cathal (8) to go to London for operations or, as happened recently, if complications occur and Tessa needs to be seen. I don't think I ever escape the guilt that comes with knowing that one child is getting more attention than the others.

I'd always wanted four children and one of the things that broke my heart after Tessa was born was that I would never have another baby. But when Tessa grew up and proved that, yes, she was a little bit different, a little more complicated, but happy, healthy, clever and able to do, not only all the things other people could do, but great things, it felt like a lovely healing thing to have Naoise 15 months ago and complete our family.

No one has ever been able to say 'why'. Tessa's condition is called BAM syndrome and it's officially listed as a rare disease. There was a small mutation in her genetic code that caused the development of her mid-face to stop in the womb before it should have, so her nose didn't develop and her eyes are underdeveloped.

When she was born, there was nothing in terms of information. No leaflet, no website, no support and it was an incredibly lonely and scary place to be. That's why I'm happy to share Tessa's story (I keep a public Facebook page 'Tessa; Born Extraordinary') because I want anyone else in the same position to be able to find us and to know that that dark place in the beginning gets so much better.

Wonder is coming out at such an important time in Tessa's life - it's her first year at school and there are obvious parallels between her and the film's main character, Auggie. I hope that as many people as possible see the film and take its message to heart because it's already done so much to raise awareness of the issues around facial differences. It gives me hope that Tessa will grow up in a society that is less quick to judge people on appearance.

In conversation with Chrissie Russell

Irish Independent

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