Gutwack

Talk about being born under a bad sign, I had an interview Monday afternoon and on the morning I started getting the tell tale signs (shooting gut pains) of the beginnings of a DIOS CF related gastric blockage. I only seem to get these about once a year but when I do they are horrible, painful things that I wouldn't wish on anyone (no, not even Thatcher ;-))  So far I've took 8 sachets of Movicol and 4 portions of gastro grafin, but still nothing!

It's my wedding anniversary today as well, CF has no sense of timing...

It's now Wednesday; I had been contemplating admitting myself into hospital to get this sorted but fortunately my bowels finally clicked into action this morning (and how, I've gone through nearly a full roll of toilet paper ;-) ) phew! It's completely ruined my anniversary though, son of a ....!

FEV1 was 2.3 have decided to go on IVs if I don't pick up next month...have got some orals to tide me over...

Itraconazole on the dole

So this last year I've been...

I have been having real problems shifting the mucus out from my narrow airways and what has been coming out has been hard and dry and causing me problems at night as my long suffering misses will attest. My LF has not responding to IVs either and I have lost 20% of my lung power over the last 4 years.

Anyway, I have been racking my brains trying to think of a way to treat this, and yesterday with my CF Team we came up with a plan that involved trying either Itraconazole, Hypertonic or nebulised Meropenem for a month to see which one improved my LF the most most. Itraconazole seemed a bit of a non starter because my IgE Aspergillus levels were fairly low. (there is no fungus in the mucus either, although there is Staph and Pseudo) When I got the email from my new boss the other week, one of the contributing factors to the decision was the thought of spending more time on my health trying to get to the bottom of just what the hell was going on in my lungs. Anyway, to cut a long story short, my gut instinct was to go with Itraconazole first for a month and, amazingly, within a couple of days; it's like flicking a switch, I feel loads better and the mucus is coming up much easier. Blows at the outpatients were FEV1 2.5 and FVC 3.5 so a marginal increase, but hopefully improving for my next visit in 6 weeks.

In other news I handed my notice in last Friday; it was nerve wracking - they wanted me to stay and think things over (but didn't offer me more money ;-)), but I'm still determined to change jobs - more for the excitement of a new challenge really. Having handed in my notice things became a bit clearer in my head; I realised I need to move to find a new challenge. As a developer, there is a real buzz to be had from solving problems and creating elegant solutions and in the last year at work there's been precious little of that happening. There *may* also be the option of working part time at my old place (20 hours a week) but we'll see - if it does happen it will only be a stop gap until I find something new. I realise my talk of taking some time off has now changed to finding a new challenge, but we'll see - in the short term it will be taking time off to concentrate on my health, but in the long term, hopefully there will be more intersting jobs out there, perhaps in the health sector, as I do have an interest in medical science and it would be great to help out in some small way.

For my next job I would like to have the working hours of a senior cat

Sods Law and the Roses

Sometimes CF can really kick you up the backside - like when you catch a cold on the last day of your IVs. When I went into the outpatients to do my scores I got no change at FEV1 2.3 /  FVC3.3 so it looks like the IVs were completely negated by the cold, gutted :-( So I'm back on 2 weeks of orals (Cipro and Doxycyline, again) to carry me over this chesty cold, and as much running as I can manage in this shitty weather.

Anyway, I did fit in a trip to see the Roses at Heaton park last week, which was brilliant. The Stone Roses are part of my misspent youth, they first broke through when I was about 16...pop music had become really bland at that point and when they first appeared on Top of The Pops (in the same week as The Happy Mondays) it was a seminal moment: finally here was something real after all that bland rubbish, and they were from Manchester as well, fantastic! We're the band any good? After a couple of glasses of cheap red wine in a comedy packed to the rafters metrolink who can tell? But the music and crowd were so happy and positive it seemed to carry everyone away on a nostalgic bubble of happiness that rarely actually happens at these type of concerts, and personally, I thought it was a amazing. :-)

The Politics of Kalydeco

Don't you just love politics? The first cure for cystic fibrosis, the first drug that targets the underlying cause of CF and I have no idea when and if it will be available in the UK. I am hopeful, but hope is all I have at the moment. The company that owns the drug (Vertex) wants to make as much profit as possible, the NHS wants to save money. The total CF budget for the UK is 100 Million. If Vertex charges what it does in America then it will cost 50 million to treat 5% of the people with CF who have the G551D gene and can benefit from Kalydeco. There is posturing on both sides and the months tick by. Meanwhile people on the transplant list are one bad cold away from intensive care. Please sign this if you can, if only to raise the profile of Kalydeco / vx770 :

http://epetitions.direct.gov.uk/petitions/34753

We're currently trying to gather together as many people as possible who have the G551D CF gene. Please leave a message if you know anyone in the UK who has G551D.

I have been on holiday to Kefalonia for a week and have not posted about it. For shame. It was ridiculously lovely and probably my favourite holiday for a long time. I think staying in a villa suits me much more than the battery farm approach of all-inclusive resorts I have been to with the family recently which were fine enough but looking back I was perhaps missing something I only get when we can do what we want when we want. Here's some photos...

Physio: repeat after me...

I must do real physio, exercise is not enough on it's own...
I must do real physio, exercise is not enough on it's own...
I must do real physio, exercise is not enough on it's own...

I don't know how this happened but I figured out how to do this technique in early 2010 with the physio and I promised myself I'd keep doing it. But somehow I stopped doing it; don't get me wrong I still do huffing and clear outs but this (active breathing cycle / AD) technique seemed to fall off my radar, probably because I hate doing it in front of people, how sad am I? Now my FEV1 has slipped from 2.85 to to 2.45 in the last two years and maybe this is the reason why; I think I'm going to have to have this tattooed on my chest:

"Sigh out.

Breathe in slowly - pause - breathe out all the way.
Continue like this until you feel the sputum moves or changes.

Breathe in slowly - pause - breathe out, but stop when it feels natural/comfortable to stop. Continue like this until sputum moves/changes.

Breathe in slowly and more deeply - pause - breathe out and stop when it feels comfortable to stop. You can probably hear/feel crackles as you breathe out.

When sputum is high enough - huff/cough to clear.

Each breath in should be slow.
Each breath out should be like a sigh, so air is moving fast but not forced.
Keep mouth open as you breathe out."

Rant over ;-). Anyway, I went to see the musculoskeletal physiotherapist the other day down on Pearce Ward, and had my neck vertebra popped / exercised by the physio; very painful but hopefully worth it in the long run to stop my neck aching and my spine curving. She's given me three exercises to do: 

• the pulling your neck back in one where you look like you're giving yourself a double chin 

• the arms together over your head back stretch, 

• the one on all fours stretching my arms out and trying to touch my breastbone on the floor, which I can't really do (but will keep trying anyway.)  

I have another appointment next week so we'll see how it goes.

At the main clinic I was disappointed that my FEV1 blow was up a bit but still fairly low (2.45) after the orals (despite me feeling better) and have decided to go on IVs next month. My team also suggested nebbed meropenem to me at my appointment - apparently it's just normal meropenem (like you have in IVs) which can be nebbed normally. My LF seems to have declined a bit in the last year (from 2.8 to 2.5), so I'm wracking my brains to think of something that will help, (see rant above ;-)). I currently take Colymycin and dNase, I tried TOBI but I couldn't tolerate it so fingers crossed for the Mero trial, will try it after the IVs. I also (whisper it) have slacked a bit on my nebs in the last few months, missing out on my Colymycin neb in the evening so I'm feeling a bit of a fool really, CF is a full time job and I've been a part timer of late ;-).

Keswick Lakes bike ride 2012

I think my age (hopefully not MD) may be catching up with me ;-), for the first hour of the bike I was fine, then on a down hill section my front wheel hit a rut and I went flying over the bars and landed quite heavily. After that I was quite shaky and after another hour my neck was in agony and I couldn't lift my head to ride my bike. We had a rest as the other 4 lads were all up for going back to the cottage with me. But I told them to carry on the ride because there was no point in them all missing out. Gutted but it was the right decision for me, my neck was in a bad way but taking the flat road back to Keswick gave me an easier time than the rocky hills would have. I'm going to have 2 weeks off the bike and then see how it goes. Other than that a good weekend, lungs were pretty clear (after the ABs last week) and we had some great weather as well...

Doxycycline and Cipro - my new best friends ;-)

Well touch wood I seem to be feeling loads better after a week on Ciprofloxacin and Doxy - at this rate I won't be needing IVs, hurrah! My usual oral of choice is Cipro and Rifampicin but no more ;-) From now on it's the cycline all the way.

Staying down south in Norwich with work for two days and ended up at a hotel in the sticks (a recently converted retirement home ;-)) ; but it did turn out to have a big gym with treadmills and I managed to do a 30 minute run with a sprint finish so not all bad really even though it is a pain being away from the family. Presentation tomorrow so hoping my nerves don't get the better of me...

Sticking my neck out

I lied, I couldn't resist the lure of the internet ;-). I've been checking out some of these neck and shoulder exercises on youtube, some of which seem to reference the Alexander technique (based on straightening the head position when sitting/standing to relieve the burden on your neck and shoulders) I've tried out a few exercises and I *think* they've helped. We'll see...

http://www.youtube.com/watch?v=4uzd_nFzj0Y&feature=related

Pain in the Neck and Outpatients ;-)

Yesterday I went to the CF outpatients as my lungs seem to have been taking a nose dive; particularly after my recent bank holiday family weekend in Centre Parcs Penrith, which was great but maybe a combination of the damp weather and the overheated bedrooms at night had the effect to dehydrating me/making me produce more and more mucus. Was quite bad when I got back on Easter Monday, I was coughing and coughing and the muck just kept coming up - I also felt wobbly on my legs, maybe I had a virus or maybe just the sheer amount of coughing and volume of mucus was knackering me. Blows were down 2.35 / 3.15 so it was definitely time to try something. Anyway I / we decided that the way forward was to try 2 weeks of oral antibiotics (Doxycycline and Ciprofloxacin) and see how I go - if I don't improve then it will be time to bring out the heavy artillery (or IVs as they are more commonly known)

I have had a sore neck for the last 3 months and it's been making my life a misery. I think it maybe "laptop neck"; neck and shoulder posture related; maybe after too many years of staring down at a laptop while sitting down, it's started to take it's toll on my neck as the pain seems to ease when I'm not working on the computer at home. I told one of my mate's about it this weekend and he recommended this for pain relief. I asked the doctors about this at outpatients and they have referred me to a musculoskeletal physiotherapist in a months time so I will see how that goes first before trying anything exotic like the Alexander technique, etc.

I did ask about my recent problems speaking in the morning - I have been struggling at times to speak properly due to dry, sticky mouth and I was wondering whether I had blocked saliva ducts - but the doctor recommended upping my dose of salt tablets to see whether that improved things.

I was also considering being referred to the psychologist as I have been a bit up and down in my moods of late, (possibly due to losing my dad last year), but I bottled out - I'll ask at the annual review in a month or two.

The big 4-0

It was my birthday last week. I am now officially very old ;-). It's slowly sinking in that to my kids I must seem really old - I can remember my mum and dad's 40th birthdays really clearly; I must have been about 8-9, I can remember at the time getting older seemed an almost infinite way off. It's now here :-/.

The weird thing is in many ways I feel healthier than I did in my twenties - I do more regular exercise, I drink much less and I get more sleep (when the kids allow ;-)) I've not been an inpatient at the CF Ward since I was 26. And for that I've got to thank my brilliant wife for getting me away from drink and excess and into fell walking and fitness when I met her in my twenties. God knows where I'd be if I was single and still trying to follow the Jim Morrison rock star model of excess; I'd probably weight about 8 stone and be falling asleep in my own urine in a pub somewhere. As it is that's at least another year off...;-)

Anyhow, I can't get too complacent because although my CF at the moment seems under control (touch wood about 10 times) I know myotonic (muscular) distrophy may raise it's ugly head at some point. I have two genetic diseases for the price of one ;-) Mytonic Dystrophy is a late onset disease but it varies considerably so there is no way of knowing just when and to what degree it will effect me, so far so good but I've recently noticed my neck seems to be aching a lot when I wear a bicycle helmet on long bike rides - and I'm also struggling to chew roast lamb and roast beef fast unless it's really tender - maybe I'm losing some muscle tone in my head / neck before anything else? Otherwise I seem to be OK (touches wood another 100 times) although I feel I should probably join a gym to maybe build up my muscles a bit more.

Diabetic Shmiabetic?

Ok, this blog is getting soooo out of date that I really dont know where to start, so I'll update with some med news (from my annual) apparently my long term blood glucose (BG) levels, otherwise known as HbA1c levels, have been rising steadily since 2006; it has gone from 5.5% back then to 6.3% this year and so I was referred to the diabetic nurse for a four day strap on test. This is where you wear a tiny device strapped to your belly (it doesn't hurt but get's in the way a bit. Sometimes ;-) ) which measures your BG levels continuously to see how the BG peaks and troughs are and how high and low your blood sugars go. You also have to do finger prick test four times a day so they can cross reference the belly device results with the finger prick device results.

I hope I'm not diabetic, it would be a real pain in the bum. So far it looks like my blood sugar is relatively normal apart from a slight peak in the evening after dinner; 2 hours after a roast squash risotto tea I peaked at 8.5, otherwise I seemed to be between 4.5 and 6.5, but we'll see when I meet up with the doctors in a few weeks...

End of the Ivs shower

Well, I can't wait for this set of IVs to end and to have that lovely first proper shower without worrying about getting my cannula wet - I also haven't had the sense of smell for the last ten days possibly thanks to the Meropenem. And the runs. it's all good fun ;-) ...also; I've been thinking about this a while but I don't think the IVs are having as big a clearing effect on me as they used to, it's taken till the end of the 2 week course for the IVs to start to kick in on clearing my lungs...I swear it used to take 3 days before my lungs would go clear...why this is now I don't really know - I suspect it may be because my small airways are getting more knackered (losing their elasticity, otherwise known as COPD) so it is getting harder and harder for me to bring up the mucus from the small airways. I was also wondering whether it was down to the IV Tobramycin causing a problem in a similar way to the nebbed Tobi which caused my lungs to produce more sputum. Don't know. Maybe I need to chat to the doctors about trying something else...maybe a three week course instead of just two (shudder, hurl), or maybe with prednisolone?? anyway here's hoping the holiday air will clear my rubbish lungs just as much as the IVs...

Off to France tomorrow (Vendee) with the kids, via a car, ferry, and getting lost in France...hope the sense of smell returns in time to smell the sea, the sun and the French food ;-)

End of ABs and the real work begins

Sorry for this geek post ;-) Last week it was the end of oral antibiotics and my lungs felt better without feeling *deep down clean* the way they do with IVs, however if you compare these two races - then my all round fitness does seem to have improved since the start of the minging infection (March 8th was in the middle of the infection and I was coughing all through the ride, March 22nd is on a sunny day today 1 week after the ABs) so hopefully I'm on the road to recovery...

Back to Activities

Bramhall Loop Road Cycling Tue, Mar 22, 2011 12:09 PM

Bramhall Loop Road Cycling Tue, Mar 8, 2011 12:38 PM

Summary

Distance	7.52 km	7.21 km
Time	00:29:28	00:33:02
Avg Pace	03:55 min/km	04:34 min/km
Calories	225 C	310 C

Avg HR (bpm)	123 bpm	131 bpm
Max HR (bpm)	148 bpm	151 bpm
Avg HR (% of Max)	66 % of Max	71 % of Max

http://connect.garmin.com/activity/comparison?activityId=74473657&activityId2=71944384

Last week I visited the hospital outpatients and was seen by Proff Web, it was nice to have a catch up with the grand old duke of CF ;-) because I rarely see him in clinic these days (he's semi-retired I think, but certainly knows his stuff). In the end we didn't talk about the usual CF things like I would at a normal oupatients clinic, we just talked about the good old days when I first moved to the Adult CF Hospital and the progress of the VX-770 trial. I was a bit depressed about the potential cost of the drug, and whether NICE would be able to fund it, but I talked it out on the forum and now I feel a bit better about it. FEV was 2.65 and 3.2 which is a bit down; I'm hoping it's going to pick up as I pick up the exercise...

Rough Week

Well, after the good news of VX-770 I was brought crashing straight back down to earth with a virus that cut out the middle man (nose and throat) and went straight onto my chest. So for the last week boat loads of sputum has been bubbling out of my lungs at night like mount Vesuvius. I rang up the Hospital and got them to fax an antibiotic order through to my doctors with the minimum of fuss. They're very good. I'm on Rifampicin and Ciprofloxacin, so I'll need to watch the mood swings and nausea...

On Sunday I went for a bike ride to try and clear some of the gunk. It wasn't pretty ;-) My mate was going to come with me but he had to cancel at the last minute. Just as well really, he would have needed counseling if he'd seen what came out of my mouth ;-). I felt really ill during but it was worth it afterwards because I did feel a lot clearer. My heart felt a bit fluttery for a few hours afterwards though, maybe it was a side effect of the antibiotics...

http://connect.garmin.com/activity/71646577

Good, no, Great news Day!

Hey, look I know this is only really good news for us G551D's so far; but Vertex are working on a correction for you Df508's as well, so fingers crossed. I'm especially hopeful that this will be of benefit for Rob from the CF forum who is fighting to get on the transplant list at the moment.

"Phase 3 Study of VX-770 Showed Profound and Sustained Improvements in Lung Function (FEV1) and Other Measures of Disease Among People With a Specific Type of Cystic Fibrosis

- Relative mean improvement in lung function of approximately 17% from baseline compared to placebo achieved by people treated with VX-770; mean absolute improvement from baseline of approximately 10.5% compared to placebo; both measures through 24 and 48 weeks -

- Significant improvements in all key secondary endpoints for VX-770; patients were 55% less likely to experience a pulmonary exacerbation, had significant reductions in sweat chloride and, on average, gained nearly 7 pounds -

- Discontinuations due to adverse events were less frequent among people treated with VX-770 -

- Data support Vertex plan to submit U.S. and European regulatory applications for approval in the second half of 2011 -"

http://investors.vrtx.com/releasedetail.cfm?ReleaseID=551869

Current Eating/Treatment Regime

PWCF need to eat a lot to stay at their current weight. I think I'm lucky in that I don't have to eat as much as some, but still, in the last 6 months, I've somehow lost about half a stone without my appetite dropping or without eating any less so I have had to add a scandishake to the mix. I had a meeting with the dietician the other week at my annual and she suggested adding another scandishake at the end of tbe day.

Food / Med Diary (Week)

• 6.30AM Get up
• 6.45 Azithromycin and Flucloxacillin
• 6.50 do Dnase on INeb
• 7.00 Breakfast Scandishake with full-fat milk and cereal
• 7.00 Vitamins, uniphyllin, calcium chew
• 7.45 Drive to work, drop off kid(s), do some physio in car, spit into empty creon pot in car. (Timing is everything in this ;-))
  
• 8.30 Arrive at work.
• 8.30 Do Colymycin Neb in car at work
• 10.30 Apple, banana
• 11.00 2 Pork Pies
• 1.00PM Lunch (Sandwich, Mayonaise, Meat Filling, Crisps, Tango or Tea)
• 3.00 Chocolate bar (note: Boost is 300 calories!)
• 5PM Leave work, pick up kids.
  
• 6PM Dinner with kids (have to play it safe so kids have something to eat so we normally have chicken, brocolli, peas and mash, or bangers brocolli and mash, or Lloyd Grossman's Tomatoe Chicken Balti and Rice, or Spagetti Bolognaise or Pasta and Roast vegetables which the kids will actually eat.
• 8PM Kids in bed. And relax. Either go for a run/bike ride or get the wine out, depending on how healthy/lazy I'm feeling.
  
• 9-10PM Colymycin Neb

What an exciting life I lead. Need to try and squeeze in another Scandishake at the end of the day but at the moment it's a struggle, my tum needs a rest ;-)

Gene Therapy Trials

Been thinking a lot about Jess today, and the truth is, I can't really comprehend what CF is like for someone like Jess because CF is a completely different beast for me; it doesn't really impact on me that much. Sure, I take the same tablets, nebulisers, IVs, and do a small amount of physio and exercise... but CF doesn't really restrict me in the way that it does someone like Jess. While I was getting drunk, popping god knows what, and generally having a carefree time at university, Jess was on the transplant list and her life was very much on hold. It's taken me a long time to actually connect with the CF community, I think a lot of this is down to fear of what happens to others happening to me, I've been dimly aware of the more severe aspects of CF though my two stays in hospital when I was in my twenties; I met other people with CF who were having a much worse time of it than me and to be honest it scared the hell out of me; maybe because of that I never really looked at it in focus, I always kind of kept it at arms length. It's only in the last couple of years that I've begun to find some kind of acceptance and begun to seek more knowledge about CF and what it is, and how it affects others as well as myself. And what have I discovered? Well, it's tough, and the people that have CF are bloody tough. And an inspiration. RIP Jess, you are braver than I will ever be.

On a more positive note -because hopefully Gene Therapy can one day prevent the severe lung damage like what Jess has suffered- this VX-770 trial that I've just missed out on has opened up something of a rabbit hole for me which is going to take a while for me to sift through and figure out. I've just had a quick scoot around to see what's happening with gene therapy trials. I still don't understand how the CFTR works, but when I do I'll let you know...

VX-770
VX-770 is a drug being tested by Vertex Pharmaceuticals in people with cystic fibrosis who have at least one copy of the G551D mutation. The drug may actually be able to target the defect in the CFTR gene and restore its ability to open up chloride channels, thus allowing salt to flow in and out of the cells properly. Unlike gene therapy, VX-770 would not replace the defective gene. Rather, if successful, VX-770 would repair the problem in the existing gene. Patients who took the drug showed significant improvement in several key CF measures, including lung function, nasal potential difference measurements, and sweat chloride levels. The findings suggest that VX-770 improves function of the faulty CFTR protein. This is the first time that any potential therapy has improved the abnormal sweat chloride (salt) levels in a person with CF. Excessive sweat chloride is a key clinical indicator of cystic fibrosis.

VX-809
VX-809 is another drug being tested by Vertex Pharmaceuticals in people who have two copies of the ∆F508-CFTR mutation. It is similar to VX-770 in that it may be able to get salt flowing through the cells properly, but it works a little differently. If it works as researchers hope it will, VX-809 would open chloride channels by moving the CFTR protein to its proper place on the airway cell membrane.

Miglustat
Miglustat is a drug manufactured by Actelion Pharmaceuticals that is already in use to treat other conditions, but it is currently being studied for use in people with cystic fibrosis who have two copies of the ∆F508-CFTR mutation. The study is small scale -- consisting of only 15 participants -- but so far the results have been promising as Migulstat has been able to reverse the CFTR defect and restore normal activity to cells.

Ataluren
Ataluren, which was once called PTC124, is being studied by PTC Therapeutics as a possible cure for people with CF who have nonsense mutations. In nonsense mutations, a piece of “gibberish” code appears amidst the normal code in the CFTR gene. The nonsense code acts like a stop sign, preventing the cells from reading any code that occurs after it. Ataluren may be able to correct that problem by helping the cells to ignore the stop sign and keep reading the code that occurs after it, thus restoring normal function to the cells.

http://cysticfibrosis.about.com/od/cysticfibrosis101/f/cure.htm

Doh!

Just missed out on the VX-770 trial. I asked my CF Team what my genotype was in September. Unfortunately it was buried in my notes somewhere so the team had to get a third party Gopher to find it from my notes. I got the letter back on Christmas Eve, probably missed out on the trial by a matter of days. C'est la vie...

From: J
Sent: 11 January 2010 15:00
To: DJC
Subject: VX-770 Trial

Hello ,

I am a 38 year old male with cystic fibrosis. I have recently learned that my CF Genotype’s are G551D / N1303K. I have been told that I may be eligible for the VX-770 medical trial. Would it be possible for you to give me any further details?

Kind Regards, J


From: DJC
Sent: 11 January 2010 17:22
To: J
Subject: RE: VX-770 Trial

Dear J

I am really sorry to disappoint you, but recruitment to the trial you mention closed just before Christmas as the drug company had exceded the numbers they needed.

On a brighter note, they are planning an interim analysis after everyone has completed 6 months of treatment, ie. early summer, and if things are looking good, they will be applying for a license to allow your clinical team to prescribe this agent. It's difficult to predict how long this will all take, but they will certainly be trying to get it through as fast as they can.

I'm sorry not to have more positive news for you personally

Best wishes, J

I'm not too disappointed by this really; in the long term this is good for the CF Community because we are actually getting to the stage where we may be able to take a gene-correcting drug that may stop things like pseudomonas getting established in our lungs, it may stop our pancreases becoming progressively more damaged, and it may prevent us developing diabetes. Fingers crossed!

Further reading here on the cystic fibrosis transmembrane conductance regulator (CFTR), and on G551D and N1303K. If anyone can explain to me in more detail how the CFTR works then please let me know ;-)

Tobi, Tobi, Tobi, Tobi!

Went to the clinic the other day for my nebulised Tobi trial with the physiotherapist. The trial is to take nebulised tobramycin for a month and see how things go, and hopefully see some LF improvement over the next month as my blows have been a bit rubbish recently. We did the test and she also rejigged my iNeb with her laptop from tidal flow to TIM (Target inhalation mode) this is better because it quarters the time needed to do the inhalation, and it turns out I will need this extra time with all the extra stuff I'll be inhaling next month. She asked me about my iNeb compliance before she brought up a flow chart telling her exactly how compliant I had been with my nebs over the last 12 months! It turns out my compliance was only 70%, I was well embarrassed, I felt like a naughty schoolboy!

I had heard that Tobi was inhaled once a day, but for the monthly trial I will be nebbing twice daily with an old compressor neb (like I used back in the 80s! Doh!) You take it twice a day and also you need to nebulise Ventolin before it to stop the possible bronchiectasis. that'll be fine in the evening when the kids are in bed, but I will struggle to fit that into my morning schedule, which consists of getting showered, doing a bit of physio (in the shower), getting breakfast, getting Oscar and Abby dressed and fed, and dropping either Oscar or Abby off at nursery / school before work. I think I'm going to have to use the iNeb for Dnase in the car on the way to work, which I don't really like doing as it's not safe and I could get pulled over. The physio also said I should neb Tobi in a ventilated room away from the kids. Hum. Looks like I'm getting up even earlier in the morning...

Anyway enough moaning! I actually felt quite loose in the chest after the trial tobi neb so hopefully I will see some real benefit when the month is over.

Outpatients and the sun

Hospital appointment yesterday; and for the first time in many weeks the sun came out! On my first spirometry I blew so hard that everything went black for a second and I got a massive headache ;-). On the second blow I hit FEV1 2.82 FVC 3.8. Hum. Blows were again a bit disappointing but I'm not producing much, I just feel slightly wheezy. It's now been a while since I broke the magic 3.0 level. To be fair Doctor J.H. noticed it was down for me and actually looked up the percentage (a first as they don't normally do that in Manchester CFU, except for the annual) and I had it confirmed as 78% -my score for last years annual was 83% so some way down really. In mitigation I have just come off the back of a cold, but my chest does seem to have cleared so I don't know why really. I asked the Doc about whether my pseudomonas had any resistance to Colymycin, but my last lab tests had all came back with no resistance (to Colymycin or Tobramycin) She did mention Tobi nebs and how they could be more effective (standard practise is to do one month of Tobi, one month of Colymycin; the downside is that there is a slight risk of hearing loss and there is more of a chance of pseudomonas developing resistance to Tobi than to Colymycin; although she said it was quite rare and she could "count on one hand" the number of patients whose bugs had developed resistance to Tobi. Anyway, I'm down for a Tobi trial with the physio when I go back in a months time, probably using the INeb with the new tidal flow (she said it can take 30 minutes to dispense if you use the old way! Nooooo!). I've also been put on two weeks of oral Septrin (Co-Trixomazole) and Rifampicin to see if it can clear anything left on my chest from the cold. I was warned that Septrin can cause a skin rash, but so far so good. (Last night I had a dream that I developed a rash all over my arms, but when I woke up there was nothing there. I hate having "obvious" dreams ;-))

I also asked what my two CF gene's were (they couldn't find it in my notes; they'll get it for next time) and what my CRP level was, apparently the last one was 5, and it was 6 when I had my last set of IVs in summer, although it can get as high as 40 when I have a really bad cold and chest infection. Otherwise I'm going to make an effort to get out and get some exercise on the unused mountain bike that's been in my shed/garage for the last ten years; this summer some of my mates have turned into mountain bike bores; seriously, the last time we went out for a drink it was *all* they talked about, I was not impressed! But I guess if you can't beat em you may as well join them, I'm not going to talk about bike magazines in the pub though ;-)