‘What’s your name?’, we ask her. ‘Lion’, she replies and adds a lion roar to confirm this. Ask her again two hours later. ‘Lion’, she will reply, roar and laugh with glee. She knows her real name well. But on day 13 of 14 of our dear friends tobramycin, ceftazidime, flucloxacillin and colistin visiting if she wants to be a lion then so be it. You roar and you laugh as loud as you can. For you are our brave lioness.
Tonight after her last dose of IVs at 10pm, her PICC line will be removed and she shall come home again. This is her second hospital admission, her second GA and second course of strong IV antibiotics. A not welcome beast has arrived – pseudomonas. A pseudomonas complete with antibiotic resistance and a mucoid layer. Scum. Learning she had pseudomonas was in some ways similar to receiving the initial cystic fibrosis diagnosis. Shock, anger, upset, guilt, hopelessness and powerlessness. One of the few bugs we absolutely did not want her to get. Where were you lurking you ba**ard? Why her?
She fell asleep to Peppa Pig as the anaesthetic took control for her PICC line. She blinked only when the toe pricks were taken for her tobramycin levels. She carries on as normal. Her wee smells (ceftazidime), she’s gone off her food and has been separated from her siblings for two weeks. Yet, she has the energy and self-determination to throw her very expensive nebuliser across the room. She’s refusing this necessary treatment that she will take for the next three months. Play therapists have been deployed to make ‘it fun’. (Tips welcome.) Whilst not ideal I encourage her determined spirit, for it is that spirit that she needs to live with this awful condition.
It’s been hard at times to imagine the future. We are filled with fear. For the moment our hope has been dampened and we are full of worry. Worry is the reason I am awake now when I should be asleep. Worry is the reason I am cleaning every nook and cranny of the house with medical grade sterilisers, even though we cannot eradicate the bug from our home. I cannot wipe every page of each book, every lego brick and cuddly toy. But I feel at least I am doing something. Worry is the reason I follow orphan drug blogs, medical journals, CF research sites avidly. Worry is the reason that I believe her ongoing cough that remains despite these antibiotics means she has not cleared pseudomonas. Worry is the reason I am finding it hard to imagine the future. I am waiting you see. Waiting for the moment I read an article or piece of information that will change her future. A drug, a treatment, a cure even, that her life and future can be returned to her. For Pseudomonas makes her future bleaker. Time is precious. A cure or treatment cannot come fast enough. Around 50% of those with cystic fibrosis die before aged 40.
I was a person who really never did any fundraising before this, didn’t often donate either if I am honest with you. Now, if someone is running, swimming, baking or who knows what else I know the passion they feel for their cause and want to give what I can. Fundraising makes me feel like I am doing something. It removes some of that sense of powerlessness over the condition. It is something for her future AND the future of all of those with cystic fibrosis. For their parents, grandparents, sisters, brothers, cousins, aunts, uncles, friends and family. By giving one person a better life you are affecting all of those people. I have no active fundraising at the moment but we have an ongoing page here.
But one thing you need to know about lions, they are considered a vulnerable species by the International Union for Conservation of Nature. They are endangered and we must protect them. Like my lioness her future lies in the wider global community of researchers and pharmaceutical companies (like Vertex). But we carry on for now at home. We love her, cherish her and live life ‘as normal’ supported by the excellent care provided by her CF multi-disciplinary team.
I shall leave with this video by OneRepublic. Please watch and be inspired, thank you for sharing the story of this amazing teen with CF.
P.S. Many thanks to our amazing family and friends who have supported us through the difficult weeks. Every message, meal and hug has been appreciated.