Glowing Report!

I'm really happy to say that yesterday my lung function was 49%, my weight 55.8kg and my oxygen saturation (sats) levels were 99%! I can't remember the last time I saw figures like that, for months and months now my sats have sat at 93-95% and that was just normal for me, sometimes getting even lower when I felt unwell. So to see them at a normal number is great and for my lung function to be almost hitting 50% is amazing. My weight is back to pre pneumonia 2011 weight, it just shows how long it can take to put that weight back on once you lose it. I have started a 10 day course of IVs which sounds stupid given the numbers, but I have felt a bit iffy the past week or so with my energy levels and I started to develop a tickly cough and chest pains so decided I wanted to have some before we start fertility treatment again, rather than possibly end up needing them half way through.

So what I have been doing differently? Well I believe the main contributor is my new nebulised antibiotic Azli, also known as Cayston, also known as nebulised Aztreonam. I had high hopes for this nebuliser as lots of people have said how amazing it is and I believe them now! At first it made me really wheezy however that went after about 10 days, it does re appear every so often though. Then I started to be able to exercise more than usual and before I knew it I was going to the gym 3 times a week and doing 40 minute sessions involving about 25 minutes cardio and the rest doing weights. I'm feeling I can really push myself at the gym at the moment and I've noticed my heart rate has decreased too, my pulse at rest is in the 80s at the moment, I pretty sure it used to be about 100. I am still very breathless when exercising however I do have less mucus which is what the physio believes has helped bring my sats up and why I think I a finding the gym less hard work.

Then I have also started having insulin with lunch and tea and although I still need to learn how many units I need and not getting it right all the time, I'm getting there slowly. I'm having 2 units with lunch and 3-5 units with tea. I'm having lots of hypos (low sugars) which is not nice, basically every time I exercise and if I have breakfast early or tea late. It's easy to say, well eat your tea earlier or have breakfast later, but that isn't always possible!

So health wise I am doing well at the moment which makes me realise how important it is to be compliant with treatment and to be involved in your CF care. I know for a fact if I worked this would not be possible, I haven't worked for about 5 years now and I'm finally starting to feel I understand my CF and know what my body needs and I'm getting the balance right of rest and treatment. Some days I am so bored and fed up, I feel so useless and pathetic that all my day consists of is CF related activities and attempting to do household chores which mostly Pete ends up finishing off anyway!

I look back to when I first joined the CF community, my health was worsening and I was facing giving up work. I made lots of friends on-line who I felt understood me better than people around me, it was also when I started to take an interest in my health and ways to improve/stabilise it, can you believe I didn't even used to wash my nebuliser equipment?!

Lots of my friends have now either had lung transplants, need lung transplants, have passed away or their health has deteriorated. People that had the same lung function and health as me are now needing lung transplants which scares me but also makes me feel proud that I have managed to avoid this so far. CF is unforgiving, I work really hard to stay stable. I'm not admired or called brave, nobody calls me an inspiration, because in order to be those things you have to push yourself to work a full time job or go above and beyond what your body is capable of and I'm not willing to do that in order to end up dead or dying like lots of people with CF do. Lots of people probably think I'm lazy or one of those scrounges you read about in paper, on benefits, didn't you know the whole country hates people like me at the moment? Sometimes I feel guilty if I go out for a meal out as the papers make me feel like I shouldn't be able to afford my electricity and gas, never mind a meal out, because I am in receipt of benefits. However then I remember my husband does work, so we are not complete scrounges...!

Having CF at my level of CF is a job in itself, I have to do a hell of a lot to stay alive, some people with CF don't, they manage to get on OK with minimal extra effort. I'm not implying that people with CF who did push themselves are in the wrong, or that everyone who needs a lung transplant brought it on themselves. It's such a fine balance between having a life and looking after yourself, nobody gets it right and even if they do sometimes there is nothing anyone can do to prevent that downward spiral, I'm sure it will happen to me eventually. I just feel lucky that so far I'm doing OKish, I have a supportive husband to help me and I'm in a situation for the time being where I can concentrate on my health and not have to run myself in to the ground with work. This might all change through if I don't qualify for ESA though and that is why I am really scared of what may happen in the next few weeks. I really wish the government and society as a whole understood long term conditions more accurately.

Happy Festive Season!

I'm still here! 

I have recently discovered the joy of the mobility scooter! If you know me you will know I hate shopping of any kind, it makes me tired, I get out of breathe, I feel lightheaded, get headaches and I avoid it all costs. We get our food shopping delivered (when Pete is home to help unpack) and I just avoid doing any form of shopping in general. This is OK for most of the year as I don't have any money to buy things anyway, however at Christmas time it can become more difficult. There is only so much online shopping you can do, sometimes you just need to see the item in the flesh or try it on etc.

Say hello to the mobility scooter! Most shopping centres have them to rent for free, you book them in advance for however long you like and off you go! Now obviously there are downsides to these scooters, for a start they are not exactly very cool and I think it took a lot of nerve for me to admit that I needed to use one as it's just another way my CF makes me feel useless, feel different and acknowledge I have a life threatening illness that is worsening slowly. Secondly, there is a high demand for these scooters at this time of year and it can be difficult to book one. So you have to plan way ahead when you are going to go shopping and make sure you call as early as possible to book one and you have to turn up at a certain time and leave by the end of your session. You can't just think, ohh I'll go shopping tomorrow when I'm up and ready. It requires planning. Thirdly, these scooters are pretty big and won't fit in shops, I ended up taking out a box a mannequin was on the other week at the gap wasn't big enough! So you spend a lot of time getting off the scooter and going in the store or to look at a certain item as you can't get to it on the scooter. Lastly, they beep when you reverse, it's so embarrassing! As if it isn't bad enough being in your 20's on a scooter, without the damn thing beeping loudly when you need to reverse, and by the way people do not move for you!  

So yes, the mobility scooter, a life saver for shopping but not exactly something a woman in her 20's wants to be familiar with. Here is a picture of me stuck trying to get out of a lift, naturally Pete took a photo rather than help me...!

I'm rather excited to tell you I have a new inhaler that replaces my tobi nebuliser! I nebulise tobi which is the antibiotic tobraymcin twice a day, I nebulise tobramycin to try help control the infections on my chest. Through the old type nebuliser called a porta-neb this would take 30-40 minutes for each dose and that just one thing I nebulise every day. Then the I-neb was introduced and this cut nebbing time for tobi to about 15-20 minutes for each dose. Now I have a tobi podhaler which takes about 6-8 minutes to do each dose! But the greatness doesn't stop there. The capsules do not need to be refrigerated as they are a powder not a liquid, everything is delivered to my house (no GP's or chemist required!) and nothing has to be washed afterwards. I'm really impressed with some of the advances in CF happening at the moment, its improving not only quality of life as it means less time doing treatments but also it will improve compliance with treatments as well. I'm going to do a video of me doing my podhaler for my next blog but here is a picture in the meantime. The white tube is not actually the podhaler but the case, the podhaler is inside and much smaller. It does make me cough quite a lot, particularly the first breath and I find I have to do three inhalations per capsule rather than two as they suggest to breathe it all in. Also I am having to try it one month on - one month off rather than being on it constantly (I assume due to cost) which I'm nervous about. However so far I am impressed!

Following on from my last post I'd like to share the latest comment I've had that has upset me. A few weeks ago my friend and I went to see the new Twilight film (judge me all you want...!). The car park for the cinema is lower than the cinema so you have to walk up lots of steps. I tried to park in disabled but it was raining and cold so of course the spaces were all full of people that are not disabled. Therefore we had to walk up loads of steps in the cold and hence I was coughing away. A couple in front of me turned around and asked if I was going to see the James Bond film? I shook my head (coughing too much to speak) to which the man replied, 'good!'. His partner then quite nastily told me I should be in bed not at the cinema! My friend said she was so mad she had to bite her tongue! I was concentrating on trying to breath too much to have any kind of thought at the time. Don't you just love people, more concerned about their film viewing than if the girl behind them can breathe or not!

Merry Christmas to everyone! xx

VX-770

There has been some news surrounding CF and a tablet that can possibly help people with CF

Taken from the Daily Mail online (click here for the link)

The first drug to tackle the root cause of cystic fibrosis, rather than just the symptoms, could go on sale next year.
In trials, the twice-a-day pill dramatically improved the lung health of men and women with the debilitating condition.
They also put on weight and needed fewer antibiotics.
If trials on children are as successful, manufacturers Vertex Pharmaceuticals could apply later this year for permission to market the drug in Europe. Clearance is likely in 2012.
Britain's 8,000 adults and children with cystic fibrosis include Gordon Brown's four and a half-year-old son Fraser.
Cystic fibrosis is the UK’s most common life-threatening inherited condition and occurs when a genetic flaw produces a defective version of a protein key to the health of the lungs and digestive system.
Thick, sticky mucus clogs these organs, leading to recurrent chest infections and poor growth. Other symptoms include diabetes and infertility.
Although treatments have improved greatly in recent years, average life expectancy is under 40 and there is no cure.
In the trial, 161 people with cystic fibrosis were given either the new drug, which is known only as VX-770, or a dummy drug two times a day for a year.
At the outset, most of those taking part had just 60 per cent of the lung function of a healthy person - a figure that improved by almost 20 per cent by giving VX-770.
No other drug has produced such dramatic improvements, this week’s New Scientist reports, probably because they have tackled symptoms rather than the underlying cause, a defective protein in lung cells.
Patients also put on an average of half a stone in weight, suggesting the disease’s effects on digestion were eased, and were half as likely to need antibiotics for flare-ups.
Peter Mueller, Vertex’s chief scientific officer, said: ‘Treating the underlying cause of cystic fibrosis with VX-770 led to clinical improvements that were far beyond our expectations, providing support for an entirely new approach to the treatment of this disease.’

The Cystic Fibrosis Trust described the results as ‘very big news’ but cautioned that VX-770 would only be suitable for about 5 per cent of sufferers.
But a second drug, that is at an earlier stage in development, offers hope to another 75 per cent of patients.
Vertex is already testing a combination of the two drugs on patients and the first results are expected within months.


Now I'm not going to get excited, I'm not in that 5% and it's not a cure. I don't believe they will find a cure in my lifetime but I do believe treatments are improving all the time to increase average life expectancy. I have the common mutations of DF508 which I assume is the 75% they talk about, which they are running tests on next. But it's nice to know that in my lifetime there may be some people with CF who can take a tablet that deals with the cause of CF rather than the effects, there just isn't anything like that at the moment.
When I read that they have achieved a medication like this for my mutation and its available to take, that will be the day I have tears of joy in my eyes. Either way its great news for that 5%, it's not a cure but it's start!

A fellow blogger has a daughter with CF and she has done a great blog explaining more about the drug and how it works, click here to view