Timecapsule - spot the PWCF ;-)

There is a person with CF on this picture. He/she is 9. It's 1981 or 1982. I think (memory isn't what it used to be). He/She has just learnt how to add fractions. There is no creon, only pancrex. No ciprofloxacin. No nebulised antibiotics. He/she has just been diagnosed, and is trying to get his/her head around the fact that he has to take enzymes with low fat food, skimmed milk(!), no chocolate(!), flucloxacillin and do physio with postural drainage....those were the days :-)

Fear, Loathing, and Physio Time (!)

Went to the all-new outpatients to do the hypertonic saline trial with Leslie (the physio) yesterday. I went in my lunch hour hoping it would only take an hour to complete; unfortunately it turned into a bit of an epic visit and I wasn't back in work till 4! Oops. Anyway, since the last trial of Tobi I've been having some problems with my chest; nothing I can put my finger on but I've been producing more mucus on a daily basis, and I've had a growing wheeze which (handily) means the mucus is harder to bring up. This all started at Christmas when I asked on the CF Forum about what was good for bringing up mucus plugs and the majority answer came back as hypertonic saline. Hypertonic saline is a high salt (7%) solution that works (in theory) by drawing more water into your lungs by osmosis, so if you have hard dry plugs or sputum that is in general hard to bring up then it should moisten these and make them easier to bring up.

We did the FEV1 blows before the Hypertonic trial (FEV1 2.75, FVC 3.75) which was down but not too bad, considering. We then nebbed some (preventative) ventolin followed by the hypertonic saline. We then did some physio to clear out whatever the hypertonic brought up. I've got to say, I found this a bit awkward as I actually hadn't done physio with anyone at Manchester since my last inpatients stay (10+ years ago). This meant I had to get over my reluctance of spitting in front of strangers, but the physio techniques she did -all variations on the active breathing cycle that Mary taught me ages ago- were very effective in getting mucus out of the small airways and into the large airways where they can be huffed up. It was a good refresher course in physio techniques; breathing shallow and deep but holding back a forceful huff or cough -which closes the airways- as long as possible. Over the years I'd simplified the technique in my head into slow deep tummy breaths followed by an almighty forceful huff. What she did was more subtle with breathe and hold followed by gentle and then faster exhalations with vibes to try and ease out the mucus. All good stuff, and (hopefully) it's improved my technique quite a lot. Over the past few years I've had very few problems (touch wood) so I'd gotten a bit slack on the physio front. Now I'm determined, for the sake of my long term health, to be more regular with my physio. We'll see how long it lasts ;-)

After the trial my FEV1 blows came back 2.45 which was down 10%, this is classed as a significant drop so Leslie referred it to Jen/Roly, which turned the visit into a full on consultation and they decided to step back from the hypertonic trial until they could get to the bottom of what was causing the wheeze and the possible current infection. I also asked about trialling Spiriva (I'd never tried it), but Roly said it was throwing one too many ingredients into the mix, as they want to figure out what's going on first, so I'm on two weeks oral antibiotics (Cipro and Rifamplicin) before the next appointment, so fingers crossed it all calms down by then.

Washed out at the car wash

I haven't blogged much about Abby and Oscar recently so here goes. Abby is now halfway through her first year at infants school and is doing really well. She is going for swimming lessons on Wednesdays (doing great) and ballet on Saturdays and she's turning into a very confident young lady (chatterbox!) of 4. Last week she won a spelling competition at school and won a prize so we were well chuffed.


Oscar we've been a bit worried about. Ever since he started nursery he seemed to catch every bug and cold that was going, in particular one weekend when he seemed to come down with Croup (barking cough sound, and seemed to have difficulty breathing) but in the end it wasn't too bad. I've mentioned in the past that it did cross my mind that he might have CF; when we did IVF we discovered my wife is a DF508 carrier and this led us to suspect the worst, however we contacted Leeds LGI and they said all donors are checked for CF so that would seem to make it highly unlikely. He also came back negative with the heel prick test. In any case in the last few weeks he does seem to have had a run of good health and this seems to have helped him gain some weight and he seems loads better in himself. His talking is coming on leaps and bounds as well, he is getting very good at mimiking and picking up some of the things we are saying to him. He can still destroy a room in 45 seconds as well!

I recently started a training blog It's completely no frills, it's just for me to provide a record of the exercise I've done and hopefully when I step it up and do more, I might see some improvement in my lung function. My misses bought me a mountain bike for my birthday, and I've recently been joining my mates on off-road courses. They all started early last year so their fitness level is a lot better than mine, then again I do wonder if my fitness level is where it is because of my CF related lung function (78%) . Uphill biking is very hard work. On my first ride my heart was beating so fast I thought I would collapse and I could see spots in front of my eyes. I was sweating like a stuffed pig as well (attractive ;-)) But I am starting to feel a tiny little bit fitter now (on my fourth ride) so hopefully there will be some long term fitness benefits. It's good fun going downhill as well ;-)

Some pics of me and the kids washing the car. Abby wanted to do most of the hosing but Oscar was interested too. When Abby wasn't looking he managed to grab the hosepipe and soak Abby from head to toe leading to a change of clothing halfway through the car wash. Very funny but it meant the car wash took ages as Abby insisted on eveything stopping until she got changed. A true diva in the making. :-)

Utopia

How fucking good is this? What a singer, sends shivers down my spine.

The real Lady G...

One of my favourite bands Goldfrapp is bringing out a new album very soon! I'm very excited! I want to post the video for the song "Rocket" (like everything at the moment it's a bit of an 80s pastiche, but a fanastic tune) but it's not on YouTube yet, so I'm going to have to paste this instead...

The Sundays

I had such a crush on the Sundays' singer when I was a student, I loved her voice. Further reading (and an excellent article) here. A very british band.

The New Outpatients

First visit to the all new outpatients yesterday. This was for a Tobi trial with the Ineb. They have put the outpatients in the old Pearce Ward, so the outpatient rooms are the old in patient rooms, which is a bit odd, kind of brings the memories flooding back. The last time I was in there there was a communal kitchen / social room where you could hang out, watch videos and fix yourself breakfast (this was about 10 years ago before the cross-infection rules kicked in for everybody, not just Cepecia patients)

The Tobi trial went OK, I got a new lilac coloured filter with a bigger container for doing the Tobi nebs. Nice. Unfortunately, the Tobi takes about 4 minutes to dispense, and you have to do 2 lots of it, which is a bit rubbish because I can neb Colymycin and Ventolin in 50 seconds flat. DNase takes about 2 minutes. Apparently it's because the Tobi is a lot thicker.

The blows before the Tobi test were a disappointing FEV1 2.7 FVC 3.6. The blows are in that area where they're not that bad but not that good; so it leaves me in a bit of a no man's land. If they were down a lot (say 2.3-2.4) I would go straight onto IVs, and if they were up high (3.0-3.1) then I would be happy as a butchers dog. Meh. When I do the spirometry test I get a high pitched wheeze so I think the 10% loss of FEV1 over the last few months may be down to that, because my sputum seems to be completely under control with nebbed Colymycin and now Tobi. In fact the last time I had IVs I didn't *really* need them I just had them to see if they would improve my FEV1, which they didn't have a massive effect on.

Anyway, I asked the physio about trialing Hypertonic Saline, as I want to see if this can reach the parts that other nebulised solutions can't ;-) Roll on next month...

4, 8, 15, 16, 23, 42...

The final season of Lost is just around the corner. I'm so excited! Well almost...I must be the only person still watching this! Hopefully this time we'll get some real answers as to what the hell's going on as well ;-)

Questions:

• Who is Locke?
• Who is Jacob?
• Who is Richard Alpert and why does he never age?
• What is the black smoke?
• What happened to Claire?
• Why is Jack's dead father on the Island and is he really dead?
• Why spend 4 seasons trying to get off the island and then spend the fifth season getting back on?
• Could the people in the past (70s!) change the future or has it already happened?
• Does free will exist?
• If I can't exercise my free will not to watch the final season does that prove free will doesn't exist?
• Is Juliet hotter than Kate?

Last night I went for my first jog of the year. Just went for a run around the estate for 10 minutes after R had done her run. Didn't feel too bad, although the cold night air made my lungs burn. I made sure I took my seretide, ventolin, uniphyllin so that my lungs didn't seize up. Oscar has come down with yet another cold, he's not really full of it in his nose and throat, he's quite hot and he doesn't seem quite himself.