Ten months ago my life turned upside down. After a fairly straightforward pregnancy and normal scans, my daughter Freya was born with a very rare genetic condition called cerebrocostomandibular syndrome (CCMS). She has a small jaw and a deformed ribcage leading to her having smaller than usual lungs. Overall a bad combination when it comes to breathing.
After diagnosis, we were shocked to find out that CCMS has a high mortality rate, particularly in infancy. As for Freya, CCMS has meant that she has spent more time in a respiratory ward than anywhere else, and I as a mother have become an unofficial respiratory therapist, suctioning master, and connoisseur of all things snot.
Freya has tried a fair few things during her hospital adventures: oxygen through nasal cannulas, Optiflow, tracheostomy plus oxygen, and now tracheostomy plus part time ventilator on BiPAP settings. We have tried countless Heat Moisture Exchangers (HMEs), and done the cuffed and uncuffed tracheostomy tubes. We have been blue-lighted to hospitals more times than I would like to remember. We’ve had little bugs land us in the PICU, and big bugs clearing on their own.
When I now look back over the last 10 months, I wonder what I would have told myself if I could get transported back in time. What would a newbie in the respiratory world want to know?
1. Knowledge is power
All of us know the dangers of Google. You type in a couple of symptoms and suddenly you can diagnose yourself with the plague. New to the world of parenting a child who is medically complex, we realized early on how dangerous it can be to go on a Google rampage (or to obsessively read your child’s medical records as I first did). I recall some sleepless nights and crying inconsolably in the hospital car park after a consultant made some comments that I thought meant a terminal chromosome deletion based on urgent Googling. Freya did not have this, but we did have some bleak days as a family waiting for blood results.
Once we found out that Freya had a rarer-than-rare genetic condition, the internet did become our friend again. We read about children with CCMS who had survived infancy and were thriving, and that we could learn from the few studies that existed. We became aware very soon that a tracheostomy and a ventilator might be an option. We could come to grips with what these terms actually meant, and could speak to other parents, if not about CCMS, then about trachs and vents and so on.
It is us, the parents, who know our children the best. All doctors really do is make educated guesses based on books and past experience. Parents of children who are medically complex can play a very important role in conveying what is normal and what is not for their child. For me, becoming an expert in all things Freya has given me the feeling of having some control and purpose in circumstances where I initially felt completely powerless.
My advice would be to ask questions and write notes. Speak to other parents. Learn what is normal for your child and what isn’t. And get all the training you can. We would all like to think emergencies don’t happen, but if they do, you want to be prepared.
2. Pick your team
Freya’s got five specialist teams looking after her. We have been in four different hospitals. I can’t even guess how many doctors have been part of Freya’s care to date.
As with any profession, you have good doctors and you have bad doctors. Some you immediately get along with; others just don’t feel like a good match. We met our “bad match” doctor when Freya was three weeks old. A well-respected respiratory consultant told us that as “a last resort” Freya could have a tracheostomy, but that we would have to consider her quality of life were we to go down that road.
Fortunately, we met other doctors who thought differently about tracheostomies, and now, seven months after we opted for an elective tracheostomy, I am disgusted at the first consultant’s comments. Tracheostomy has been the one thing that has significantly increased quality of Freya’s life—and saved it several times. Countless children—and adults—live long term with tracheostomies and do absolutely normal stuff. I have met a little boy who goes swimming every week and know kids who have travelled the world.
A mother of a child who is chronically ill told me early on in this journey that if I wanted to give Freya the best fighting chance, I should find a doctor who wants to champion her. Someone who is willing to push for treatment and who is willing to honestly discuss all options with you. You need someone you can communicate with and who listens to you.
Don’t feel bad about moving on from one doctor to another. These people are supposed to walk beside you and your child for big parts of your journey, and I can tell you that this is one trip you want to choose your travel companions well.
3 .Be prepared for a fight
Being a special needs parent often feels like you are a personal assistant, chauffeur, nurse, physiotherapist, and an advocate all rolled into one. I have spent countless hours making sure Freya’s needs are met, and more often than I would have liked, I’ve had to push hard to get what we needed.
I hope the journey will be gentler for you, but be prepared to fight—fight to get the right treatment, the right operations, the right equipment, sufficient nursing hours at home, suitable accommodation, and the financial support you are entitled to. You need to fight to get physical, occupational, speech, and respiratory therapists visiting often enough to get your child the therapies he or she needs. The list is long.
Don’t let anyone make you feel like a failure because you are asking for help. Kids with respiratory issues are often fragile and need constant supervision. No mum or dad can do it 24/7. Find out what your rights are. Don’t take what people tell you at face value; ask to see it in writing. If need be, put in complaints. Challenge decisions. Go to your local press. Threaten to sue. It might not work every time, but I have found that becoming a massive pain in the backside often gets you the help you need.
4. It’s ok to feel like you feel
My life after Freya was born has been an emotional rollercoaster—sadness, joy, despair, feeling numb, jealousy, anger, hope. I have felt it all. You will have people telling you all the time what you should do, how you should feel. You will meet other families with children just as special as yours, and you will feel in awe at how well they are coping. You’ll meet others who are barely managing.
This path is for you to walk, and only you can do it. Feel the feelings that come your way, and yes, it’s absolutely fine to feel weak at times. My gentle reminder under this heading is to try not to dwell too long—there is much beauty and joy in this journey you do not want to miss. I try to avoid dwelling in the negatives in fear that whilst I do so I will miss out on the positives.
5. The importance of peer support
Having a child who is medically complex is isolating. There will be times when you will very acutely feel that your friends and family simply do not understand what you are going through (and how would they?). You will find out which friends remain with you for the long haul, and which drop out.
You will, however, soon find your new community. This may be the other “long-termers” you meet in the hospital café or at the parents’ accommodation, or it may be through online communities. There are many Facebook groups dedicated to tracheostomies, all kinds of specific conditions, and things like blended diets and tube feeding. There are online magazines and resources to help you research, reflect, and connect with those traveling similar journeys. It may feel that you are the only one in the world going through this, but there are others! This revelation has made the challenges of our journey manageable, and I can say with my hand on my heart that the special needs community truly has some of the finest people on this earth.
And what else would I say to me 10 months ago? I’d say you’ve got this one, Mama! It will be hard, and you will never come out on the other side the same person you were before your child came. But that’s not necessarily a bad thing.
And whenever you feel sad and tired and like you cannot go on, you have to remind yourself who is the true superhero of the story—it’s your little warrior princess—my Freya—who gets up every morning to beat the odds and live her life to the fullest.