Boston in the Fall

Well, me and R finally decided to go to Boston for a long weekend to celebrate our tenth wedding anniversary. The in-laws offered to look after Abby and Oscar for the weekend so we were all set. We took the afternoon flight to Boston via Heathrow, during the main flight there was a choice of films to watch, and because we don't usually get the chance to watch any films during the flight (because we're usually trying to keep the kids entertained) we both crammed as much as we could into the flight!) I watched Moon, The Last King Of Scotland and Che part 2. Of the 3 I'd recommend Moon if you like intelligent sci-fi, and The Last King of Scotland if you like to be terrified...Che part 2 was a bit boring for me; well acted but devoid of tension because I knew the ending. I was also left not really knowing who Che Guevara really was...
We arrived in Boston late on the Friday and caught a cab straight to the hotel where some champagne was waiting for us in the room, we then stumbled around looking for a restaurant (I wanted to try an Afghan restaurant around the corner but R fancied an Italian so we eventually found one inside another hotel after going round in circles for what seemed like a long time ;-)) and then we were truly knackered so we went straight to bed.

In the morning the view from the hotel looked a bit too much like Manchester; cloudy, grey and raining but we decided to make the most of it, went to the shopping centre next door to buy a couple of rain coats for the baseball and then set off on the freedom trail. The freedom trail is a redbrick trail that starts off in Boston common and takes in loads of historic sites relating to the American revolution and the historic break from Britain, and the shaping of the American government and the constitution and bill of rights. It was interesting but I felt a bit funny as a British tourist reading about the British army being vilified and the British parliament hated for their taxes! (I think the paper tax, a tax on all forms of paper was the last straw!) In the guide book the walk was supposed to take 90 minutes, but when we did it it took us most of the day, we did stop for lunch and go into some of the state museums though. Beware when ordering lunch in diners/restaurants; sometimes the portions are huge! Me and R ordered a couple of basic pasta dishes from an Italian diner and the plates that came with the meal were enough to feed a family of four; we would have been better off ordering one meal between the the two of us! It is true what they say about America, if you are there for a long period you would easily put on at least a stone...
When we got back to the hotel it was time for a quick shower before going to see the Boston Red Sox on the Metro; R had always wanted to go to an American baseball match since I can remember. The match was good, The away side (The Cleveland Indians) started off well but the Red Sox seemed to have a bit more quality and after a massive score with a home run with 3 other players on the bases in the 3rd period they always looked the likelier winner (if that makes sense to anyone let me know, I'm not sure I understood it ;-)) Baseball is much more of a social affair than the football we have here, there's lots of chatting through the match and a constant procession of boys carrying food above there heads shouting "Clam Chowder! I got Clam Chowder Right Here" and "Hot Dogs! Get your hot dogs here" So you can scoff your face through the match without having to get up! I've got to say the local clam chowder is worth trying especially if you need warming up on a cold night!
On the Sunday thankfully the weather was brightening up. We decided to book the whale watching at Boston harbour; it's a three hour round trip and apparently it can get a bit hairy if the sea is rough. Thankfully for us it was a beautiful day and there were plenty of humpback whales around. They are quite breathtaking to see close up. When they surface they do a really loud exhale creating loads of steam -they exhale something like 90% of the air from their lungs so they can take a deep breath before diving back down- humans exhale 25% to give you an idea of the super-FEV1 that a humpback whale must have ;-)
On the way back we had a wander though the City, I was going to buy R something nice from Tiffany's but we didn't find anything she really liked so we decided to console ourselves in the Cheers bar. As you might expect it is a bit touristy in there. On the last (half) day we got a quick coffee and bagel (or cwoffee and baygel as they say in Boston) and had a wander around Harvard trying to look intelligent (!) before a last meal in the Cheesecake factory (desert is literally 100s of different flavours of cheesecake) and then home. I've got to say I really like Boston, there's less to do than in New York, but the people seem friendlier, and there's more of an old colonial/British-Irish feel that made me feel really at home. We missed the kids terribly though!

Everyone's poorly except me and my monkey

Everyone's poorly except me :-). Abby's got a stonker of a cold and has forgotten/is refusing to poo, this is giving her really bad stomach ache so we've put her on high fibre and Lactulose. We went to Blackpool to show her the illuminations last weekend but had to drive home early because she was in so much pain from needing to poo but refusing. I think it may be psychological as she's just started school and maybe it's all a bit intense for her; it's not like nursery where if she doesn't feel great/is tired she can take herself off for a snooze/some quiet time on the bean bags. Also, she's gone from being a big fish in a small pond to being a tiddler in a massive shouty lake! Oscar has just vomited the entire contents of his morning bottle of milk all over R, and he's had to take the day off nursery because he's just not himself. R went to the MRI to find out about her protein C deficiancy, they didn't really tell her anything she didn't know, except she should have a dose of heparin as well as wear the surgical stockings before long hall flights (oops! We just got back from Boston the other week!) As for me; well, I've discovered I'm allergic to Septrin (nasty rash on my ankles which seemed to be spreading up my legs, thankfully it stopped when I stopped taking the septrin.)

Anyway, I need to blog more but I'm too busy at work! Here's some 70s psychedelia from the Flaming Lips, haven't got any of their albums but I may buy this new one. I love the bassline!

Don't be concerned...

If this song were a car it would be a convertible 1957 Thunderbird driving the ocean drive along the West Coast of America from Los Angeles to San Francisco. Ah, the optimism of the sixties. I love it!

Four Sleeps 'Til Boston...

Only four sleeps till me and the misses go for a long weekend in Boston for our tenth anniversary! Boston in the fall. I'm torn; I'm excited, but it's the first time we've been away from the kids for longer than a night, so we'll miss them big time. It'll be strange being husband and wife rather that mum and dad for a few days. What the hell will we talk about? ;-)

We've been "mum" and "dad" for so long I've forgotten what we used to talk about before the rugrats arrived. Mealtimes will be strange as well without having to figure out what Abby will eat on the menu and quickly then wolf down our food before Oscar gets bored and attempts to swallow dive off the top of his high chair...:-)

Demolition Man

Oscar is at that age (14 months) where he can trash a room in 1 minute if he's left alone. In the kitchen he pulls out all the pots and pans, all the cereal boxes (including once, when he covered himself in Ready Brek) and all the washing up powder. In Abby's room he pulls out all her clothes and all her toys. All good fun ;-)

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 ;-)

history

I've been meaning to post something like this for a while...Kudos to Jo for reminding me.

I was diagnosed when I was 9 thanks mainly to the persistence of my mum and to the doctor who finally decided not to fob us off and referred me for an x-ray. From the x-ray they thought I had TB so they put me in an isolation ward for a month, when the TB test came back negative they did the allergy scratch test. Finally they did the sweat test, and on the second sweat test I was diagnosed. Looking back all the signs were there; every winter I would pick up a cold and it would stay on my chest for weeks or months, I would produce sputum by the bucketload. From the age of 7 I started to lose weight noticeably. When I was a toddler my mum frequently complained to the docs about my farts smelling wrong and I used to get the runs from time to time, presumably because of the amount of undigested fat in my system.

This was back in the 70s, and in those days no one had heard of CF, The yellow pancease tablets (Pancreatin? I honestly can't remember the product name) were rubbish, and the wisdom then was to go on a low fat diet (skimmed milk and no chocolate, is it any wonder I rebelled? ;-) I was also put on regular Flucloxacillin, which I more or less take to this day.

I think back in those days I had mainly Staphloccocus on my lungs. It wasn't until about 12-13 that I remember the word Pseudomonas being used regularly by the docs. I generally went into hospital for IVs about 3-4 times a year. The doctor would first give me oral antibiotics but when they invariably didn't work I was put on IVs through a cannula (the cause of many a trauma, although thankfully they always did the job in clearing my lungs) As a kid I can remember thinking/feeling that my health was sliding, but halfway through secondary school my health seemed to stabilise, I think this was down to a combination of Creon becoming available -which helped my weight gain, coupled with teenage testosterone kicking in which helped me with exercise (I should probably give the hospital credit here for the IV courses as well, although they weren't appreciated at the time as you can probably imagine). I seemed to get better at sports, took part in the 10K a couple of years running. When I was 16 the doctor let my mum do IVs at home. I have always done IVs at home since then. When I was 18 I was referred to the fledgling Manchester CF Unit, then in Monsall Hospital.

When I was 25 I got hospitalised a couple of times with major DIOS gastric blockages. The second time was quite scary as I was in for a month and it didn't seem to clear and surgery was mentioned. Thankfully it eventually cleared. The DIOS was caused partly because my pancreatic function was declining and had finally reached the point where Creon 10 was no longer working properly, I switched to Creon 25 and things gradually got better. I also started taking salt tablets, especially in the hot weather. Over the years I've become quite good at spotting the early signs (stomach pains) of DIOS and hit it with the Kleanprep early doors.

And that's it really. I'm now 37. I still have IVs about 3 times a year. I got married when I was 27 to a wonderful woman. We tried IVF for 5 years but it wasn't to be, so we decided to go through the donor insemination route and now have two beautiful kids, 1 and 4. Luckily my LF has stayed pretty stable throughout (it's about 80% atm). DNase made a big difference to me day to day in reducing the sheer volume of mucus produced, I also do Colymycin twice daily. I have no idea how I would do nebs without the Ineb, as it allows me to run after toddlers while inhaling. I think my health has been more stable when I have more weight on; I look back at some of the photos when I was a student and I look scarily thin, I feel like going up to my skinny-ass younger self and saying "eat some pork pies."

Cork and Kerry Mountains

My mum and my sister have travelled the length and breadth of Ireland to build a picture of our family tree (On my mum's side, both grandparents were from Ireland). Apparently they've found quite a few relatives, I can't wait to hear the tales. Anyway, this gives me an excuse to post this song, which I love, I don't know why :-)