No Spoonfuls of Sugar Here

Having a positive attitude to taking her cystic fibrosis medicines helps my 23 month old take her morning medicines with ease. Recently she’s decided she wants to do them herself. Took a long time for her to get to this stage. Many of these, especially flucloxacillin, taste not so great. Next steps will be her own Creon and much more. Normalising this with positive language. No vile, yucky stuff here. We love CF meds to keep her healthy!

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Raising a Little Lioness

‘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.

Dolly has a PICC line too.

Dolly has a PICC line too.

Smiling with family!

Smiling with family!

Lioness bed hair!

Lioness bed hair!

Play in hospital with my brother. He misses me.

Play in hospital with my brother. He misses me.

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.

First Hospital Admission

Sudden turn of events. Life has now been refocused on what’s important. Minty has been fighting off a nasty cold for weeks. She became quite unwell yesterday afternoon, worse this morning. I took her to the children’s hospital today, by the sound of her breathing I knew this wasn’t like her usual infections. So tomorrow she goes in for a general anaesthetic, bronchoscopy and long line (central or picc) for 2 weeks IV antibiotics. It’s the day we’ve been trying to avoid. She’s at home now running around like mad after prednisolone and salbutamol. You’d never know she was unwell!

Going to be staying in with her. We are allowed out for home visits etc. Lovely Minty, always smiling.

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Baking Cakes, Being Grateful, Community & Fundraising

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There are 25 eggs sitting on the side, three packs of butter, two punnets of strawberries and fresh cream in the fridge. Tomorrow I’m looking forward to hosting our wonderful community weekly coffee morning. It’s my last before I return to work next week. Every week the mums and kids in our neighbouring streets get together to chat, eat and support each other. I’ve been very privileged to live in this community, where I can walk down the road and say hello to many people. I can tell you the names of most of our neighbours. There’s one woman in particular who organises this, Hannah H, she deserves a nomination for a honours for community work if you ask me.

Anyhow, tomorrow I’ve asked if they wouldn’t mind me hosting a fundraiser for the CF Trust, as part of the Big Cake Bake.  As you may know my daughter was diagnosed with CF a few months ago, my blog tells our story. A researcher recently sent me this message.

“You and your family should be very positive about your daughter’s future.  She was identified by newborn screening, the multidisciplinary teams that care for individuals with CF do an amazing job and new therapies that tackle the root cause are now being tested in the clinic.”

For our Cake Bake and coffee morning I’ve started collecting virtual donations from family and friends who cannot be there. I’ve been totally blown away by the generosity of people, especially during these austere times. I feel proud to raise money for such a worthwhile cause and proud to have family and friends who support our activities. I am extremely grateful.

If you feel, please visit our Just Giving page and make a donation. Doesn’t matter how small or large, every little helps.

There are so many fundraisers that it’s hard to pick which ones to donate too. It’s likely to be one that touches your heart or one that you feel I hope that never happens to me.

Please bear with me while I mention two other fundraisers.

My cousin has very recently experienced a family tragedy and I was humbled by the generosity seen on his fundraising page for First Touch, exceeding his target by 181%. I’m sure if you read his story you will feel touched by his efforts.  I’ve been reflecting on this giving and expression of support during difficult times. Seeing family pull together for a cause is inspiring. My lovely sister-in-law and 13 year old niece ran Race for Life to raise money for their charity. Wonderful!

At times of need some people step out into the forefront to provide support, often people who you least expect. If you are one of those people, stand proud as you are wonderful! Thank you and enjoy a slice of virtual cake above.

Play & Exercise as Physiotherapy for Kids with Cystic Fibrosis

Jumping for Joy & Health

Jumping for Joy & Health

As a mum of a baby with cystic fibrosis, toys and fun play can form an important part of daily physiotherapy.  Exercise is also integral to encouraging healthy lung function and enjoying life. Blowing and bouncing, what’s not to love?

Our CF nurse suggested starting a collection of blowing toys, which surprisingly aren’t as commonly available as I initially thought. So I’ve started a board on Pinterest with such toys after finding that searching for blowing toys often brings up results including toys of a sexual nature! You will not find such toys on this board.

Other Useful Links

Here is another excellent link on breathing games for children with Cystic Fibrosis and another on trampoline jumping. Physical activity is encourage and many people with CF live extremely active lives, running marathons, playing professional sport and climbing mountains. Some true inspirations. This link shows people in the public eye diagnosed with cystic fibrosis.

Here is a wonderful blog, Run Sick Boy Run,  that states ‘Blessed with Cystic Fibrosis and a positive attitude, it is my duty and passion to show the cystic fibrosis community that anything is possible through this blog that discusses the daily trials and triumphs that accompany not only life, but a CF life.’

Please feel free to add comments and suggest links that you think may also be useful. This is a work in progress.

We weren’t expecting you, but you are here now. Cystic Fibrosis – The Diagnosis

In medical school, the cystic fibrosis lecture was memorable. The lecturer, I do wish I could remember his name because I want to thank you for the hope he instilled, was passionate. He conveyed his excitement about the new drugs in development for CF, raved about the possibilities of gene therapy and said ‘there will be a cure one day’. It was like a performance, an uplifting speech, much of which went over my head as he talked about delta F 508, CFTR and so on. Interesting, I thought, but that will never apply to me as we don’t have the CF gene in our family. For sure we don’t; I’d have bet £1 million that we didn’t. Especially as I’m half Ghanaian. No way. That’s the thing, you imagine that these things always happen to other people. That’s what my doctor husband thought too when he sat through the same lecture one year earlier.

Our daughter’s life had a brief 3 week period of no known CF. We received cards and gifts, fell in love with her and celebrated her welcome into our life. She will be my last baby and she took quite a journey to get here. We were thrilled to meet her.

Here she is!

Here she is!

It’s always on the power of hindsight you look back and see hints of things to come. The newborn heel prick was memorable as she was reluctant to give her blood. The midwife came another day to try again. I nearly said ‘don’t bother’ as those things won’t apply to her. The test took longer than usual to come back. Odd I thought, I think they are repeating it. Still, I thought nothing of it. Some three weeks later while feeding in the middle of the night I thought, ‘it’s still not back I better just give a call’.

Didn’t need to because at 9am one Tuesday morning a worried sounding health visitor called to say we needed to go to the children’s hospital at 2pm that day. She sounded like she didn’t want me to ask her any questions. Panic ensued and I Googled the name of the person we were to see. Palpitations set in as I saw that she was the CF specialist nurse. My husband tried to reassure me saying it was probably nothing and that the blood tests were going to be repeated.

Now, I often tell patients to use the Internet with caution as there is a lot of misinformation out there. Googling positive CF newborn screening I found such misinformation. Confident that this was a big mistake and that the test would be repeated we went down, I nearly told my husband not to come. As a doctor, I had no idea that the heelprick test could be diagnostic. Merely a screening tool for other tests I believed. There she was a lovely healthy baby with nothing wrong with her.

As we arrived at the hospital I knew something wasn’t right. We were ushered into a room quickly, taken out of the waiting room. I felt this to be a bad news room. When the specialist nurse and consultant walked in carrying and large folder with our baby’s name on I thought, this isn’t good. This isn’t good at all. Why was the consultant there, what did he want?

They began by asking how the pregnancy went and how she was doing. “Fine, OK, she was just breech that’s all,” I said. From then on he explained how the test had shown the 99.5th percentile for the enzyme produced in CF patients. The blood sample had already been sent all over to labs here and there. Long story short, two genes were found. “That means that she has a positive diagnosis of cystic fibrosis,” he said.

Stunned.

Terrified.

Devastated.

Shocked.

I looked down at my baby. It was like I had been told she was dying. That’s what it felt like, looking back on it now. I was shaking so badly I thought I would drop her. My dear 3 week old baby, what life does she has ahead of her I thought. Tears ran down my face as I looked at hers, so innocent and so new. How was this tiny baby going to live her life like this I grieved. “Is there any chance that its a mistake?” I asked. I knew it wasn’t but just had to be sure. I was simply unprepared, not that you could be, for the diagnosis. I would pull myself together for a few minutes then burst into tears again. My two year old tore road the waiting room as if nothing had happened. We asked questions, we absorbed answers, though probably only a fraction of what was said. It seemed completely surreal. This couldn’t possibly be my child. My husband looked pale, in shock and spoke quietly. He felt the need to be strong, matter-of-fact, optimistic to support my postpartum collapse.

I felt that her future had been re-written. I felt that she was facing an unknown world. I wondered if we were up for the job for looking after her. I remembered patients I had cared for on a respiratory ward and I remembered the battles these patients had faced, the antibiotics, their life in hospital, their lines, their life. I remembered the lady I helped prepare the papers for her lung transplant. All of this flashed before me. In a moment, I imagined quitting my job, my child being ill, my family and a trillion other worries all at once. Our family’s future was also re-written. It was too much to take in.

This is where the consultant said, “you must not change any of your hopes and dreams for her or for you all.” “You must live a normal life.” Lots more was said about her going to university, school and travelling the world. I realised by the end of the two hours we spent there that we did need to carry on as normal. She needed us to be strong for her, to be her rocks of support, her advocates and love her unconditionally.

Coming to terms with the diagnosis and quest for information I found it hard as words like, life threatening, fatal, lung transplant and so on stood out. I chose not to read information that told me her life expectancy is 41. I know now that those diagnosed through screening can do very well because we catch it early.  We are now around 3 months into prophylactic antibiotics, Creon, daily physiotherapy and the amazing multi-disciplinary care team support we have for her. This is where I truly understand and appreciate how each individual contributes synergistically to brilliant care. I shall not go into details as every patient has their own care plan and experience of this journey. What I will say is that now, more than ever, I know we must be grateful for the NHS and the service it provides.

Reactions from family and friends were unpredictable. That was very hard. All I can say is call people, communicate even if you don’t know what to say. We experienced a sad sense of abandonment which will stay with us forever. If you don’t know what to say, say just that “I don’t know what to say”. Cards and gifts stopped and her birth stopped being celebrated. Some even said “I bought a present for her but I feel that I shouldn’t send it now”. Please do. It has taught me that no matter what every child’s life must be celebrated and welcomed. Each child has taken a difficult journey to get here. That said, support has been great. It’s brought many family members closer together. Our family unit of six seems stronger than ever. I’m extremely grateful for my rekindled relationship with my youngest brother, who has been outstanding. Thank you to him and to you all.

If you would like to know more about what cystic fibrosis is please look at the CF Trust website. If you have a few spare pennies or pounds (not that many of us do these days) please consider a donation to the charity. In America? Try the Cystic Fibrosis Foundation.

I am hoping that in her lifetime we shall find a cure. I believe her future, though uncertain, looks bright. But there’s much to be done.

My daughter and I

My daughter and I