First Nebuliser

Following on from a bronchoscopy aged 20 months, Harriet came home from hospital with a new addition to her treatment regime, a nebuliser. Like with everything else, at first it looked terrifying and complicated and I was never going to be able to use it. Three months later I can safely say that I was, as usual with these things, worrying over nothing! D-Nase or Pulmozyme is an inhaled drug which thins the mucus in the lungs. At the time of its release it was seen as a major breakthrough treatment for cystic fibrosis patients so it can only be a good thing.

Generally people are a little older when they start it but because Harriet has been so plagued by virus after virus it was agreed she would start. There is some concern over whether children this young (Harriet is two) can inhale the full medication properly but I think, for the most part, she does now. Certainly in the beginning she didn’t. We had to start by just wafting it in front of her face then slowly built up to getting the mask right over her mouth. We did this gradually and it took approximately three or four days. At the time of her getting D-Nase she really loved bubbles and she would shout and ask for bubbles all the time. Because of this we decided to call the nebuliser her bubbles and four months later it has stuck!

We were told to do this treatment at least half an hour before her percussion physiotherapy so we decided to do it at tea time after she had eaten and was still in her high hair that way there was no escape! Four months down the line we still follow this routine unless we have evening plans and sometimes it suits better to do it before her morning physiotherapy session. We are not yet at the point where she will sit and hold the nebuliser herself. She has tried but won’t keep it still. It has to be held in a horizontal position so as not to spill the liquid contents. I know of a few little ones who, aged two, can do this so hopefully one day soon Harriet will too. Not that I mind doing it at all but she likes her independence and for a while anyway would see it as a novelty to administer it herself!

The cleaning process of the nebuliser is quite specific in that it all has to be washed immediately after use in warm soapy water. We are not able to touch the filter part so need to be very careful. The individual parts are then all laid out on kitchen roll to air dry over night. I know lots of CF parents do things their own way but we just do as our team have suggested. We also sterilise it in a steam steriliser which has been a godsend.

The vials of liquid come specially delivered to our house from Lloyds Healthcare which is great. We get a phonecall to arrange delivery when we are running low and a text message the day before with a time slot. We are so lucky as some people have to collect theirs from their hospital. Someone has to be in as the drugs need to go straight in the fridge too. The nebuliser we use is an E-Flow Rapid and takes two or three minutes to work, again we are so very lucky to have a really new machine as some parents have ones that take up to ten minutes to deliver the same drug. Certainly a challenge to get a two year old to sit with a mask over their face for ten minutes plus! It also comes with a mouthpiece but I really don’t think we are even close to Harriet being ready to use that. For now, she will happily have the mask and that is good enough for us.

In terms of its success, I can’t say she hasn’t caught anymore infections than normal but on the other hand I would hate to take it away and for her to be even more prone to infection. It is a very expensive drug and we are so grateful she is able to have it. If we had to buy the nebuliser (some CF parents have) it would cost approximately £600! The drugs themselves cost approximately £20 per day so not a cheap treatment at all. This really scares me as cystic fibrosis patients have to pay for prescriptions when they reach adulthood in the UK, a law decided when not many people with CF made it to adulthood. Another matter that needs to change.

Hopefuly this post will help some when they too are faced with another addition to an already complicated treatment regime, it’s really no big deal I promise!

Read how we became CF parents here.

To learn more about cystic fibrosis and ways in which you can help click here.


Harriet is Two!

I can’t believe that Harriet turned two last week, seems like she’s been here a lot longer as she really has packed a lot into two short years. She has had three major surgeries, a bronchoscopy and about a million viruses! Throughout all of this though she has remained a very happy and content little girl. In fact she was so content that she decided not to bother with walking until she was twenty-two months old. In typical Sod’s law fashion, a few days before she had to have bloods taken to test for all forms of muscular dystrophy she started to walk. Now, aged two she has just mastered jumping, much to her delight as she had been trying for a few weeks! Really good news for us now though as she can start on the trampoline which is great physiotherapy for her.

A few weeks ago Harriet had her second annual review. These appointments are in a normal CF clinic but take that little bit longer. A regular clinic appointment generally takes about an hour as we sit in our own rooms and staff come to us to prevent cross infection. After being weighed and measured we see a nurse, physiotherapist, dietician and a consultant but for annual review bloods have to be done and a chest X-ray. This time was the first time Harriet stood up for her x-ray and she was so good! In fact I couldn’t believe just how cheery she was all day with everyone who came to see her. She had been in a phase of being not so happy for members of the CF team to check her over so hopefully this is a thing of the past.

The whole review went really well. She had gained weight and height. Her blood results showed that her vitamin levels were really high so we have to drop another supplement which is fantastic. We are, however, picking up another antibiotic for the winter called Septrin which will hopefully prevent her picking up as many coughs and colds as she has done in the last year. Fingers crossed! She is also doing really well with her nebuliser that she started in June. She has her ‘bubbles’ once a day, usually early evening before her night time physio. We are so lucky that she is so compliant, for now anyway!

In terms of Harriet’s development she is really coming on a treat. She can now put words together to form sentences, not so great when she’s shouting ‘mammy juice NOW!’ She is enjoying one day a week at nursery, especially this last week as she got to take a birthday cake in and everyone knows just how much she loves her puddings. She is now trying so hard to run, she will count herself down then try to speed off but her little legs just can’t quite get there yet. She looks hilarious trying though.

For her birthday we are having a little party with family and friends. We are also taking her to see Frozen on Ice with her big sister and I can’t wait to see her little face. She is so very excitable, she squeals with joy just spotting a cat in the street so I’m guessing she’s going to be off the scale excited seeing Elsa and Anna.
Over the last two years we have been through so much as a family. There is a lot to learn still about cystic fibrosis but we will keep going, keep on raising as much money and awareness as possible while bringing up two beautiful girls. We will do everything we can to achieve a life unlimited for our our daughter and everyone else with CF.

If you would like to read more about cystic fibrosis and find out how you could help click here.

Check out last years blog post from when Harriet was one here.

Never just a cold…

This is going to sound really awful but when my eldest daughter was smaller I would rarely bat eyelid if she had a cough, cold and snotty nose. As far as most parents are concerned it’s just par for the course when kids are building up their immunity. How totally and utterly rubbish to then fear the most common, pathetic of illnesses. To us they’re a chore, I just have no time for colds, so annoying. For someone with cystic fibrosis, however, a cold can be potentially very dangerous. Cough and colds can lead to hospital admissions for IV antibiotics and cause irreversible lung damage which we cannot have happen. We need to keep Harriet’s lungs in pristine condition.

Since Harriet was born I can honestly say she has caught every single cold and virus going and then some. Six weeks is the best run we have had in two years without having a cough which is a pretty depressing fact. Every time Harriet gets a cold or cough she has to go on extra antibiotics, more medicine twice a day on top of all of her other medicines. Since the start of the year Harriet has started to follow quite a regular pattern of illness which is both baffling and exhausting. Every two or three weeks for the past eight months Harriet will catch a virus of some sort. She will always start with a dry cough and anyone who knows me will have certainly heard about it! Once the dry cough starts it is incessant. For two or three days and nights we do not sleep, we sit up all night while she coughs without any respite. There are no gaps or breaks inbetween the coughs, it is literally NONE STOP.

I defy any loving parent to lie in bed all night and listen to their child going through this without going in to comfort them. I say comfort, in our case she sits in our bed and watches the iPad while I try to cuddle her! She doesn’t get distressed at all, unlike me, who has in the past taken her to hospital when this first started happening. It is clear that she cannot sleep for coughing so I am certainly not going to make her try. I have discussed, or rather moaned at great length about this to our CF Team but no one knows why this is happening. Our team are really only involved if Harriet gets a wet cough, those are the troublesome ones, so at first we thought she may have recurrent croup. After two or three days of the dreaded dry cough it always then turns wet. Again, sounds awful but as soon as it goes chesty it is so much more manageable. She will then barely cough and can actually sleep all night. We were given a blue inhaler to try with the dry cough but we have found it provides little, if no relief.  So puzzling, if anyone in the CF community has experience of this please get in touch!

We have to be thankful that throughout all of this Harriet has never been unwell with each virus. She is always full of life, happy and a joy to be around even when she’s not sleep for days- unlike me! Because we have followed this pattern for such a long time now, Harriet is going to have a winter antibiotic called septrin. She will take this daily throughout the winter to try and stave off any viruses. I have high hopes as I do not know how much longer we can keep up with these all nighters every few weeks! Fingers crossed for us it does the trick and breaks this awful cycle we are in.

If you’re reading this and have a cold maybe spare a thought for those with CF and be thankful that in a few days you will be back to full health. After seeing what my daughter has been through in her two short years and how she deals with it all so well I try to never moan or complain (much!) again. I read somewhere that one persons cold could be another persons hospital admission so please think of the vulnerable people around us and try to keep your germs to yourself! 

If you would like to learn more about cystic fibrosis and ways in which you could get involved please click here.