Sunday 31 July 2022

Friday 29 July 2022

Klepsydra

A water-thief κλεψύδρα klepsydra is an ancient tool for getting an accurate handle on passing time. It relies on the molecular uniformity of water, much as an egg-timer relies on well sieved sand, to pass through a narrow opening at a regular and reproducible rate.

One of the issues with a polytunnel is that it never rains inside. That's fantastic for drying laundry but does commit tunnelistas to transport sufficient water to any plants which they choose to grow inside the structure. In an ideal world, the 900 mm of rain which falls on our farm each year would come a) during the night b) every third day c) at the rate of 7.5 mm / day. But Irish weather isn't as biddable and we have been known to receive a destructive 75 mm in 24hrs. That excess washed down the hill taking much of the roadway with it. I have installed a number of nifty fixes [gutters, rain-barrels] to capture enough rainwater to see the beans and tomatoes through any dry spells.

The ultimate back-up is a 1m tonne IBC [(R)]set up next to the compost bins whose base is about 2m higher than the roots of the tomatoes.  It is high Summer; there hasn't been more than a spit of rain for at least two weeks; and tomatoes are thirsty . . . not to mention the parsley sage rosemary and thyme which also need their wet. I have accordingly been draining the top IBC into its lower sibling to swell the reservoir of useful irrigant. I've plumbed in a half-inch pipe to the bottom spigot of the back-up IBC. It's little faster than watch paint dry, but only a little. I needed to find out how long it took to drain a set amount of water. That was I would know whether I had time to knock up a batch of scones or only time to make a cup of tea before having to nip up the steps to switch the tap to Off.

But marking the water-level with a permanent marker and returning 30 minutes later, I was able to determine that the drainage rate turns out to be 300 lt / hour. My IBC is not really a water-clock because my phone is much better at telling the time. Calibrating the rate is a sort of reverse klepsydra or as it might be Ardyspelk.

Thursday 28 July 2022

Lovelock gorn, dang!

Guardian reports that James "Gaia" Lovelock finally pegged out 2 days ago at the age of 103 . . . exactly. He was born on 26th July 1919. He made a modest fortune early on by inventing devices to measure minute quantities of specific chemicals in the atmosphere. With the money set himself up as an independent scientist and researcher (and prolific author) with his own lab on his own farmlet in the English West Country. Because you could, in those days, before the possession of property was polarized so fiercely between haves and have-nots. We made that cut also by purchasing, at public auction, a ruined farmstead and 7 hectares in 1996. Because Lovelock was a bigger person than I will ever be, he planted 10 hectares of his gaff with native English trees. We have contributed the twentieth part of that as our own arboreal carbon sink. He beat himself up about doing his planting wrong. But we should forgive the error as the logical nicety of a passionate but critical-thinking advocate. 

I met him twice, for a fleeting minute or two each time. Both times Lynn Margulis was in the room, actually or metaphorically, and it is appropriate to show these two maverick iconoclasts in the same frame:

There will be obits all over the place because, although regular scientists scoffed at the idea that our blue dot planet was a coherent homeostatic super-organism, nobody hated on him for questioning the status quo. I don't think he will have gotten any pleasure from dipping out before the most frighty of his predictions [agricultural failure and mass migration]came true. Although, at that talk I attended, he did implicitly compliment us for living in a place [Ireland is an island surrounded by water] that was likely to become an Ark for The Saved against the coming armageddon.

Chapeaux!

Wednesday 27 July 2022

What a piece of work is hand

I guess, as survivors, we can remember doing something bat-shit crazy aged 17. What could possibly go wrong? I asked as I gunned my mother's Vauxhall Viva up to 70 mph on a crappy rural road.  The thing is, when you're young and foolish, it seems you'll live forever. It's only later that you see your place in the Universe as small and vulnerable and get to wonder how ant-like people have launched such strong, ambitious, huge projects. To fit humanity's largest constructions into the picture frame, the people responsible become mere pixel-sized footnotes [R - Belfast 1912: that's just the propellors]. For big projects, the tools [diggers] are made big also: but they are still operated by human hands.

I have been tidying up in Crowe's Wood after Kiwi Sean's thinning. The 1 acre woodland is now dappled with sunlight and speckled with small piles of logs. In most cases, adjacent to the log-piles is a neat lay of brash. My task, with some help from Dau.II has been three-fold

  • clear paths with solid footing through the wood
  • consolidate the logs into bigger piles not in contact with the ground
    • we defo have gate-posts in there if not planks
  • salvage shorter, thinner branches for the woodstove 2 winter's hence 
    • my definition of firewood is more inclusive than a that of a professional forester

clear paths with solid footing through the wood is infrastructural: I need to be able to reach all the felled timber and extract it from the wood . . .

. . . but also aspirational. We want to be able to wonder-wander through the trees without losing an eye or falling headlong tripping over a log.
Notes to self:
a) Gdau.II is only 6, her eyes are not at my eye-level;
b) a brash-end as thick as my pinkie is a tripping hazard;
c) sapling stumps can stop a wheel-barrow. 

First half of July was dry and sunny, so I spent many hours in the woods pruning eye-pokers off the conifers and creating a new N25 southern cross motorway at the bottom of the wood, so we can easily get to the furthest corner with a wheelbarrow. The brambles were like trees in places down there but now an occasional snip will keep the paths clear. Elsewhere the paths are more rustic & meandering. Gdau.II identified Lords and Ladies (Arum maculatum) while preparing for the Easter Egg hunt earlier this year. We can probably expect more typically woodland forbs as the light works its magic.

I also find that I have hauled just about 2 cords or 7 (steres = cu.m.) off loggy firewood into two adjacent stacks [L 4x5x5 ft; R 3x7x8 ft] in the breezy paddock just north of the gate into the woodland. 


I covered them over against the rain and they can hang there until next spring when they should be lighter but still cut-to-length-and-splittable for winter 2023-24. I went to some trouble to strip the bark off the chestnut and split some of the larger rounds before adding them to the stack. Bark is designed to be impervious to water and that cuts both ways. In spite of PPE, I have acquired the usual nicks and dents from the interaction my hands, steel tools and recalcitrant wood. 

One stere of stacked hardwood weighs at least 500kg, so I have apparently shifted 3.5 tonnes of fuel up a 10% slope over the last month. I had intended to make stacks in the wood and only haul them up to the yard when they were 20% lighter. But protestants will tidy and suddenly there they are. I regret stacking the fatter logs unsplit and 1.5m long. But it's mostly ash Fraxinus excelsior and I am confident that it will cut-and-split handy enough next year. For now it is stacked and rain-proofed. It's like a Javi Problem: start at the bottom and work steadily and, even with plentiful tea-breaks, you'll see progress.

I tell ya, I was way better at splitting wood aged 12 than I am now 50+ years later.  I split a lot of wood in the house my folks rented that year. Practice let my hand and eye eventually do it with elegance, efficiency and effectiveness. Of course, because 1966, there was no PPE. Of course I got a few dings. But mastering that skill helped me stand straight in the gale of life's buffets. Now, I'm stepping gingerly and trying to keep digits clear when swinging steel.

Monday 25 July 2022

Permission to chip? Sir!

We were gathering at the bottom of the lane prior to a session on the hill thrashing the bracken Pteridium aquilinum, in a (probably vain) attempt to create more fodder for sheep. And a more diverse flora to delight the eye and the various bugs and insects who eat those plants. Nothing eats bracken, that is why it grows so abundantly across the uplands. I had, accordingly equipped myself with scythe and lunch and a bottle of water. Everyone was at a loss when our leader asked "Before we go up, does anyone have a club hammer and a bolster chisel in their vehicle?". It's not a crazy request - the collective trunks of half a dozen farm-jeeps is likely to hold: antibiotic vial + syringe; baler-twine; calving-jack; dog; empty pop bottles; fork; ratchet-straps: screw-driver: tarpaulin jacket: vice-grips; wire-fence roll . . .

I had the required tools at home - somewhere - and said I'd pick 'em up when we went past our gates. And it was so! I was late to the hill because although I had a clear image of the chisel on a shelf, but not the shelf in one of three widely separated places where I store tools. 

The problem was that, when we up-graded the Mountain Gate at the corner of the commonage, we installed a previously owned wrought-iron pedestrian gate. This nice bit of history was hinged on a wooded stake but closed against a rough granite pier which in turn hinged the much heavier iron vehicle&flock gate. The pedestrian gate was meant to be secured by an integral bolt slotted into a hole drilled into the pier. But it was a awkward fit and far too many visitors left the gate improperly secured and stock has escaped both ways many many times over the tuthree years since the gateway was upgraded.


I'm not stupid, and I can bodge things up but nobody would call me handy. I therefore went up to the nearest group of fern-cutting neighbours proffering the hammer and chisel and inviting them to swap tools for a few minutes. Nope! There was a collective shaking of heads "You'd be at nothing there, Bob; that granite is quare hard; it would be easier to give the wooden stake a nudge". How hard is quare hard, I asked myself, and trounced back to the gate to find out. It was quite clear where the small gate was binding because there was a rusty smear on a granite wart about the size of an egg.

Much less than 5 minutes later, the rusty smear had been <tok> <tok> <tok>  replaced by white patch of unweathered granite [zoom L] which was ever so slightly concave . . . and the gate easily swung so that the bolt opposed its bolthole. I was quite chuffed. I live in my head rather too much for comfort; and a lot of that time is spent trying to imagine the perfick solution to a problem. My farming neighbours have taught me that good enough works too: often coupled with let's get this done. In fairness to the work-on-the-wooden-stake school, I notice that although the bolt is opposite its bolthole, it would slide better if the gate was raised by 5mm. I think that can be achieved by tricking about with where the upper hinge passes through the wooden stake and is secured with two nuts. Next time I go up the hill I'll bring me a spanner and test that hypothesis.


Sunday 24 July 2022

SantiaGoGo 2022

Tomorrow is the Feast of Santiago Matamoros [multibloboprevs]

Friday 22 July 2022

A hot mess

Phew wot a scorcher! used to be the red-top headline in England when 1960s temperatures inched over 70°F = 21°C; with pictures of young wans in their undies during their lunch-break in a London park. On Monday, Ireland recorded its hottest ever temperature of 33°C in Phoenix Park, Dublin. This national record was widely reported by newspapers with absolutely no skin in the game - it must have been a slow news day in Peebles - York - Surrey. If you follow any of those links, Monday's temp will be billed as Second highest temperature on record in Ireland rather than hottest ever; adding that it was 0.3°C below the record of 33.3°C observed at Kilkenny Castle on Sun 26 June 1887

I hope you are with me in calling doubt on this KK Castle record if only for the extra .3°C spurious accuracy [multiprevs] alert. The Marquess of Ormond's gardener meteorologist was not recording temperature in Centigrade in the 1880s, that's for sure. Indeed for many years that KK temperature was widely transcribed and copied as 92°F because everyone likes a record. The original ledger is no longer available for comment.  But it probably looked like this entry from a weather ledger from Markree Castle Co.Sligo for 1-18 July 1922:

Even then, the default was Fahrenheit. The cited link to the Royal Irish Academy has a really interesting discussion of quality control for handwritten records: is that a 7 or a 9? I had a similar experience scoring blighted potatoes near Wageningen in the [scorching hot] Summer of 1976. For the next several days after experimentally infecting a field  of potatoes with a controlled dose of Phytophthora infestans [blight] we went to each 4x5 plot of spuds and gave each plant a score out of ten . . . on a dictaphone, for transcription back in the office.  The Dutch for 7 "seven" and 9 "negen" can sound close enough on a scratchy recording so the convention was to enunciate "survə" vs "nayChen".

Thing is that 92°F is an extraordinary datum - the only European national temperature record from 19thC - but pretty much everyone, including Met Éireann, trots it out with neither sanity-check nor health-warning. We should follow my pal Tony's advice about extraordinary claims requiring extraordinary levels of proof. Last year a team from Irish Climate Analysis and Research UnitS (ICARUS) gave the claim some welcome side-eye

  • The KK Castle record is not the only >33°C temperature recorded from 19thC Ireland
    • 33.5°C Phoenix Park 16 Jul 1876
    • 33.3°C Dunmore East 29 Jun 1851
    • 33.3°C Markee 28 Jun 1851
    • 33.1°C Markee 27 Jun 1852
    • But whoa?! how likely is it that record temperatures at opposite ends of the country happened on consecutive days in 1851 rather than on the same day? could this be a typo? Because the Table 2 reporting these data has two other checkable typographical errurs: Kilrish, Clare and Boora, Offlay. [Note to self: this article is a pre-print, word is that they have resubmitted the draft . . . on Scorch Monday].
  • The KK Castle record is the out-on-a-limb outlier when compared to the 6 nearest weather stations with continuous max daily temp records [pink arrow indicates KK 26 Jun 1887] through the month of June:

Other clever data-smoothing and crap-detecting methods were mobilized by the Maynooth team to cast more cloud on the reported temperature on that Kilkenny day in 1887 and I invite you to read the PDF with care and attention to detail. One really nice touch is the record number of authors on the paper: the entire class for the 2019-2020 cohort of the MSc in Climate Change were tasked collectively to chase down and crunch a lot of historical met data. This must have been as galvanizing and inspirational [real science coal face alert etc.] as me helping isolate yeast mutants with my peer-group in 1974.

My pal Denis [prev] was visiting the night after the Phoenix Park scorcher and started the mind-hare running that skeptical metadata analysis existed about the 1887 heatwave. His sardonic assessment of lonely dot in the graph for June 1887 temperatures is that it is not real science. In his experience egregious outliers in any scientific dataset are ignored, airbrushed or explained away with articulate arm-waving.

Wednesday 20 July 2022

Built by association

At heart we are stick-shaking primitives under a veneer of rational thought. Thousands turned out for the relics of Saint Thérèse of Lisieux when they arrived here on tour in 2001. Is a book which was owned by {insert big cheese here} or signed by the author really more interesting / valuable than an almost identical book without these inside-cover scribblings?

I've mentioned before that I've been following Leo "Shipwright" Goolden's quixotic quest to rebuild an Edwardian sailing yacht called Tally Ho. Quixotic because everyone agrees that it would be cheaper and simpler and much quicker to build a new yacht than to try to replace pretty much every artifact which makes up the boat and/or try to salvage more than the anchor from the original build. All this is going down in Port Townsend, Washington - just across Puget Sound from Seattle. And the reason for that is because Port T. has one of the highest concentrations of grizzled and experienced shipwrights in the USA, maybe the World. Every couple of weeks Leo pushes out a ~30m video about the project and about the Effectives in his team. The first vid for July made no mention of Tally Ho. It was rather to celebrate the launch of another quixotic Port Townsend project: to rebuild a purse seiner called Western Flyer - this:

It's not [even?] a sailing boat! And it was launched only 85 years ago. And it's been sunk three times. It is not obvious why, in 2015, the wreck of the Western Flyer should have been worth $1,000,000 dollars to a marine engineer and entrepreneur called John Gregg. That's just the hulk, a king's ransom has also been splashed on the material and highly skilled labour for the rebuild. Part of skill is appreciating where your work fits into the greater project and making life easier, and more efficient, for those who come after you in the process. It is autonomy with interaction: boat-building, the the best science, isn't boss driven it is internal-standards and deep knowledge driven.

The answer to the million dollar riddle is that the Western Flyer was chartered by John Steinbeck in March 1940 for a collecting trip in the Gulf of California with his pal, marine biologist and fount of compassion, Ed Ricketts [whom prev].

That six week trip was written up as The Log from the Sea of Cortez [1951] a rollicking, inspiring tale of salt, fish, invertebrates and drink. Nobody died, anyway. The same cannot be said for the inter-tidal invertebrates, however, 500 different species, 50 of them new to science, of which were collected and bottled and brought back to Monterey for scrutiny and analysis. An international expert of Phylum Cnidaria, Oskar Carlgren, named three of the novel species of anemone Palythoa rickettsii, Isometridium rickettsi, and Phialoba steinbecki [L], after the principal investigators.  Too bad that current taxonomy reckons that Isometridium rickettsi is a synonym for Metridium farcimen which was described more than 100 years before the crew of the Western Flyer lifted their specimen from the fore-shore.

Nicely, neatly, John Gregg's plan for the Flyer is to abstract her six-weeks' experience as a marine biology station in 1940 and make that her 100% future. Being small and nimble, the hope is that the Flyer will go where larger government- and foundation-funded fear to tread: inshore and round the coast to document the status of this crucial ecosystem. Crucial because the shore is the interface between humans and the ocean so its health has a disproportionate impact on ordinary people - wild-swimmers; beach-combers; kids with shrimp nets; poets; surf fishers. But also because edges are always more diverse and interesting.

Monday 18 July 2022

The Fittest

 John Wyndham was a British science fiction author in the 1950s. His most famous book is The Day of the Triffids, which I've written about before. Triffids was triffic fro 12 y.o. me: it fuelled a fantasy of making do with what was available in a survivalist self-sufficiency way. I was too insensitive to balk at the casual way in which the book disposes of so many people, and not just walk-on characters. Re-reading the book with adult eyes, it is driven by a messianic sub-text about who shall be saved. Not to mention a cliché sexism about the roles of men and women. I liked that book and read most of the rest of Wyndham's books including a Penguin volume of short stories.

Borrowbox has been pushing earbooks of Wyndham's The Chrysalids [and his Midwich Cuckoos]. Eventually, resistance being useless, I caved and downloaded this other attempt at imagining a bleak post-apocalyptic future . . . where a small cohort of special folks are Saved. The conceit is that civilization has been burned to a frizzle and molten glass in The Tribulation a thermonuclear mutually assured destruction - a long long time ago. One of the fallouts of this nuclear war is a persisting drift of radioactive dust which mutates the inhabitants of Labrador; which escaped the immediate destruction. People are living in a post-industrial economy which looks like North America in, say, 1750. 

A religious leader, also long gone, has set out definitions of what it is to be a) human b) suitable for humans to eat. Case b) are destroyed by fire, so that whole fields of corn which, on  inspection, don't make the grade are conflagrated into the sky. Calves, chicks, foals, lambs are closely inspected for deformity and likewise summarily killed. Some backsliders, less able to take this loss, are liable to scarf down the offending animal to feed their families before they alert The Inspector. Some neighbours, in righteous indignation, will denounce this practice.

Case a) humans with the wrong toe count or too many freckles are treated differently. They are sterilized [Wyndham spares us the details of how this is achieved in a world without regular basic medicine, let alone state of the art surgical wards] and then exposed in The Fringes at the edge of their known world. Accordingly, Labrador is surrounded by Others, who have survived exposure and understandably bear a grudge against The Government which sent them forth into the wilderness.

The story centres on a small group of children who, although outwardly normal for fingers and freckles, are super different because they can communicate across distance by "thought shapes". They know that discovery will mean expulsion or worse. The narrator's sister is born and develops into a kind of super-telepath who among other things can message others a long long way away in Zealand.

In the climax of the story these latter-day Kiwi telepaths appear in a long-distance deus-ex-helicopter to air-lift our heroes to safety in a more tolerant civilisation on the far side of the World. As with a lot of Wyndham's bit-players, their attackers a casually choked to death by constricting filaments released by the rescue party; their bodies left to be eaten by the teratogenic monsters of the fringes.
Verdict: worth reading despite being the creation of a particular time when I was young and the existential threats were different. Probably not worth re-reading, though.

Sunday 17 July 2022

Sun 17t Jul Mix

Some news, not all good

Friday 15 July 2022

Coder DoBro

My latest Borrowbox 👂earbook is Coders: The Making of a New Tribe and the Remaking of the World [Penguin 2019] by Clive Thompson, who writes for the NYT and Wired. It's read rather well by Rene Ruiz and is an interesting dive into TechBro culture and its sense of manifest destiny: the delusion that Silicon Valley is a meritocracy. All those white frat-boys who retired on their money before they turned 30 and believe that the $$$s showered down because of their unique native genius. There is a shiver of truth in that someone had to have the idea (and someone had to implement it in code) which changed how millions of people interact with the world.  But lots of .com millionaires were just adequate coders who were available when required . . . and prepared to pull a few all-nighters to prepare product for launch day.

So now we have a FAANG culture driven by Harvard and Stanford math-geek graduates - and their wannabees - who are complacent in their bubble, intolerant of difference, and profoundly ignorant about the real world. Facial recognition software that is excellent at distinguishing one spotty white face from another but flags all black faces as "gorilla". That sort of thing is a failure of imagination as well as ethics. It all your bros did the vom in Harvard Yard back in the day because they couldn't hold their liquor, then there is nobody to pull you up on the narrowness of your worldview. Foosball in the Google workplace? That seems a peculiarly adolescent perk to have fitted as standard.

Thompson unpicks this sense of entitlement with several compelling counter-examples. The most obvious one is the up-and-down history of women who code. The first ever coder was Ada Lovelace [1815 - 1852] after all and most of the early "computers" were women. The boys thought that the interesting stuff was the well 'ardware - voltmeters, vacuum-tubes, screwdrivers and left all that typing and card-punching to girls which became software. Of course, when it became apparent that coding was mentally challenging, fulfilling, fast-moving and, well, the point of all the hardware then the boys stopped disparaging and started displacing. Even today front-end, user interface is considered soft while back-end, server-side is where the real men hang out. Serial misogyny since 1948. Grace Hopper - Margaret Hamilton - Margaret Dayhoff - etc. etc.

Another nice story is about the foundation, by Rusty Justice and Lynn Parish of Bit Source in Pikeville, in the Kentucky rust belt. This gave the lie to the easy dismissal of [ex-]coal miners as burly, dusty, not-very-bright rednecks. If they were clever problem solvers, after all, they would surely be financial traders in NYC. Not so, coal miners do very little with a pick and shovel anymore. They operate, tune, fix, optimize really expensive and temperamental equipment in adverse circumstances where time is money - miners are problem solvers. Bit Source has been hiring and training them as programmers, architects, designers, managers, support technicians, and SCRUM masters and selling their skills and insights across the US including Wall Street. Once-upon-a-miners get to stay in Appalachia and earn good money solving other people's problems. "I'm the only one I know who speaks hillybilly and javascript" YT5mins.

A couple of times Thompson indicates the wrong-headed folly going to college to read computer science or math or physics in order to ease your path into retire-at-thirty territory. Upskilling in C, Java, Python, CSS, SQL will only give you tactical skills. And that market is systematically undercut by hardworking coders in Bangalore. You'd be better off, and have better fun if you go Arts Block for your undergrad: history; languages; exegesis of scripture or the world literature canon; philosophy [R who?] will teach you cogito ergo sum. It will also expose you to different ways of seeing and force you to acknowledge that there are several ways to skin a cat. You can learn Java later. Liberal arts encourages strategic rather than tactical. And heck, if you don't make a million from thinking big at least you'll have the perspective to accept that pursuit of money is deeply unsatisfying.

Coders: good stuff, recommended.

Wednesday 13 July 2022

Let the children walk

My rant on Monday about cherish the children [♬ ♫ ♩ ♪] was an aside for today's update on SMA Spinal Muscular Atrophy a suite of inherited diseases which devastate a couple of dozen residents of Ireland and their families. Last time we looked at SMA, it was to showcase a new therapy Spinraza which could ameliorate the symptoms if a child was diagnosed early enough andif somebody would stump up €500,000 a year to inject the drug into the cerebro-spinal fluid 4x a year forever. The watchdogs of the public purse aka NCPE felt that we-the-people couldn't countenance such an outlay for so few beneficiaries. What galled me up a bit was that 12,000 people were prepared to sign a petition to persuade The Minister to over-ride the advice of his own experts but probably not able to stump up the actual folding money to achieve their started aim. It's the dilemma we all face whenever we bother to think about it: we'd rather spend the money on a) ourselves or b) on whatever is floating our boat this week rather than paying more taxes and letting the government make those decisions on our behalf.

SMA is in the news again because there is another petition to add SMA to the tests which are carried out on heel-prick blood-samples gathered at birth onto Guthrie cards [prev] and processed to early-detect progressive genetic diseases [including PKU, CF] for which therapies are available. Nope point in diagnosing conditions for which medicine has nothing to offer? This is a classic example of utilitarian thought, where you can, in theory calculate (QALY benefit of the therapy) ÷ (€€€€ cost of the therapy) and use this to decide rationally / fairly where a limited tax-take should be allocated. in 2019 NCPE said no to Spinraza. The additional cost to adding a tenth test to the existing Guthrie infrastructure is only €5 per heel or €300,000 a year to catch perhaps 6 de novo cases of SMA. Some EU countries already do this test.

But there is a new therapy now which uses a once-off intra-venous delivery rather than a [short] life-time commitment to spinal <ouchy> injections. Spinraza works by geeing up the production of another gene SMN2 [for survival motor neuron] which is related to SMN1, defects in which trigger the adverse symptoms. SMN2 would rather have a quiet life in a mostly dormant condition, hence the need for repeated jabs to keep it delivering. The new drug Zolgensma is a complete copy of SMN1 which is packed up in an adenovirus and delivered adjacent to the cells which normally make the SMN protein. Adenovirus makes its living by being taken up by human cells and hijacking the replication machinery . . . to make more copies of adenovirus.

It has been the holy grail of gene therapy to insert "good" genes into cells with "defective" genes and switch them on to work in place. My former colleagues [Hi Jane, Hi Pete] in Trinity College Genetics have been pursuing this dream to cure retinitis pigmentosa RP for at least 20 years. So far no patent [but plenty patients!], let alone a working product.

SMN the protein [structure R] is part of a complex which helps assemble another complex (the spliceosome) which processes genes into a final RNA form which can be translated into proteins. That sounds like an over-complicated I danced with a man of danced with a girl who danced with the Prince of Wales way of doing things; but in normal circumstances it works adequately. Evolution is about good enough, that will do rather than some perfectly crafted machina designed by deus. A single change Q136E in a single codon in the gene SMN1 prevents SMN the protein binding to ELAVL4 and so the first complex fails to come together and so (for want of a nail the kingdom was lost) cells, particularly some key motor neurons can't get their stuff together. But that Q136E lesion is just one of several ways in which SMN can go wrong: after all the protein is composed of 294 amino acids, any one of which has the potential to change. A111G, D030N are known natural variants which cause a milder form of neuromuscular degeneration known as SMA2. P245L and other changes cause SMA3 which is also rather less destructive of normal function.

Mais revenons nous a nos Zolgensmas. Zolgensma was approved for use as an orphan (minority use for rare diseases) drug by the FDA on 24 May 2019 and  the EMA on 18 May 2020. The Novartis blurb includes this telling disclaimer This press release contains forward-looking statements within the meaning of the United States Private Securities Litigation Reform Act of 1995. Forward-looking statements can generally be identified by words such as "designed to," "to halt," "hope," "can," "could," "possibilities," "potential," "leading," "excited," "milestone," "committed," "will,". I'd give a cynical chuckle at this point, except that my current take is that science has pulled a rabbit out of its hat this time. The fly in the ointment is that this single IV delivery costs €2 million. That's right on the cusp of what NCPE and their European oppos consider "worth it": 1 QALY being equal to €80,000.

Monday 11 July 2022

Cherish

In the 00s, I spent a lot of time poring over the the text of Bunreacht na hÉireann / The Irish Constitution; especially Article 40 - Personal rights; Article 41 - The family; Article 42 - Education and children. My interest was pushed by our desire to educate our children at home a right which is, for complex 1930s Realpolitik reasons to do with the protestant middle class, specifically allowed by Article 42.

In my head, at such times, is the aspirational phrase  "cherish all the children of the nation equally" even those who are, in the widest sense of the word, different. The Blob has been hot-and-bothered about unequal treatment in cases of medical misadventure; the vulnerable; orphan drugs for rare diseases; the imprisoned. I'm not the only one who recognises the power of this ideal. Cherish was founded in 1972 to advocate for lone parents and their, then "illegitimate", children.

In a recent episode of hot-and-bothered, I searched the constitution for cherish and came up almost empty. Only mention is Article 2 "Furthermore, the Irish nation cherishes its special affinity with people of Irish ancestry living abroad who share its cultural identity and heritage" which is about Chicago policemen and the diaspora rather than small citizens in diapers.

Turns out the cherish phrase is from a much shorter, more shouty, earlier document The 1916 Proclamation:

The Republic guarantees religious and civil liberty, equal rights and equal opportunities to all its citizens, and declares its resolve to pursue the happiness and prosperity of the whole nation and of all its parts, cherishing all the children of the nation equally . . .

These children, no more than De Valera's Article 2 children, are not small humans below the voting age; they are rather mythic children which have sprung from the loins of Cathleen ni Houlihan or even Caitlín Ní Uallacháin the personification of Ireland. A bit like Athena being abstracted, fully formed and in armour for the fray, from the head of Zeus. The Proclamation Children are grown-arsed adults being required to wave the flag and ready to die on the barricades. Ireland, through us, summons her children to her flag and strikes for her freedom . . . supported by her exiled children in America and by gallant allies in Europe . . . and by the readiness of its children to sacrifice themselves for the common good

Full disclosure: the picture [L] is not Caitlín but rather the Madonna by Margarito d'Arrezzo; the oldest painting in the care of the National Gallery in London and recently restored to something nearer the artist's original intention.

My point is that for human rights, it's a cop out to refer to precedent or defer to authority. We have to fight for these rights for ourselves against a penny-pinching and complacent bureaucracy. But more important we have to fight for the rights of the dispossessed because, by definition, the ability to advocate for themselves in curtailed. To the barricades! To the picket lines!

Sunday 10 July 2022

Friday 8 July 2022

Splút

We shuttle between our mountain home and the Costa na Déise a lot and the journey is almost always via New Ross [R] - arguably the most depressing town in Ireland. All through the 20thC, small town Ireland was, it seems, too poor to afford house-paint. But the grim grey grey facade of cement-rendered public buildings (often convents and banks) was most obvious when you could see the whole panorama from the other side of a river. Ross has a chance now that the Rose FitzKennedy bridge has started diverting belching Rosslare-bound HGV traffic from the quayside. In 2010, for example, the Royal Hotel went up in smoke just after plans were announced to re-furbish it. For more than a decade the steep awkward town-centre site was vacant except for a set of weeping steps running beside the weed-abundant lot. Just this year, however, the former hotel site has been re-purposed as a steep awkward public park - absolutely not wheel-chair accessible; but for the moment full of promise.

At the Northern extremity of town, where the ring-road meets the road to Enniscorthy and Dublin, Mannion's farm-shop and garden centre has opened right opposite Mannion's Bar (food and drink). For us, this emporium was both too close and too far from home, and we were always destination-bound, so we never stopped to buy honey, strawberries or carrots . . . until Saturday 25th June. For a couple weeks, I'd noticed a discrete ad for Scúp ice-cream on the fence but that Saturday arvo, having Dau.II in the car, I swung into the car-park to chekkitout. 

Mannion's stock a variety of exotic food-stuffs that you'd never find in a regular Irish grocery: noodles, tamari, Italian petit-fours and, of course, a Baskin-Robbins-like bank of scúpable ice-cream from . . . Scúp which creates Luxurious Gelato in an industrial shed on the edge of Wexford town. Like Sustainable Sausage, so long as the product is edible, you can generate more sales with a professional website than by making something distinctively different/interesting in a blind taste-off. They paid for 1 minute of  marketing video too.You can get a taste of Scúp's aspirations by knowing about their Individual Masterclass €1,950. You can expect Scúp, fadas and all, to follow Nobó [prev] into the international market. After some dithering, I plonked €3 down for one scúp of mango-and-passionfruit in an edible cone - so I didn't have it in a single use waxed paper tub. Dau.II was off her feed after a curry binge the previous night, so sensibly refused to join me. 

I was disappointed at myself, the scúp-technician and the structural integrity of the product when about 40c-worth of my scúp detached itself from the cone and went splút on my shoe as soon as I stepped out into the dusty car-park

Mannion's don't have all their eggs in one basket. They have just started selling "Handcrafted Gourmet" Popsicles - the arty exotic line of colourful sorbets on a stick which was launched in Cork in the middle of the pandemic winter. I knew all the principals in Popsicle before they were famous and have given them a puff before. I'm delighted that their reach has penetrated 150km along the N25 towards Rosslare and the big international mondo di gelato 

Wednesday 6 July 2022

Cake tomorrow

The rule is, jam tomorrow and jam yesterday—but never jam today.

Lewis through the Looking Glass

We shared a birthday in Allihies. There was cake [R]; it was a work of art; it was generously proportioned; the work of human hands. But it only took 3 days for four adults to consume the last crumb. And yes, my hand is indeed bigger than my sister's head

As An Old, I recognise that some things are slipping the cables of my memory. Like, in the rush to make a slab of flapjacks a few weeks ago, I forgot to add the sugar. The mix seemed a bit soupy, so I added oats until it was approximately the correct consistency. One of the issues with my recipe is that it includes 2 tbs of golden syrup - a quantity of some imprecision - being dependent on room temperature and the size of the tablespoon. I also had a figarey to add ginger as well as cinnamon. So those flapjacks were crumbly, hygroscopic with a bit of a gag after-taste.

When I returned home from Allihies and had started back to work around the farm - a never-ending task-list after all - I found myself drifting into the kitchen round about tea o'clock. I reckoned I deserved a wedge of fruitcake which Dau.II had made in the Spring with really cheap brandy-soaked Marks&Spencer vine-fruits. But it wasn't in the tin! I shrugged and supposed that I had done a Michael Rosen on myself and finished the cake in the middle of some night earlier in the month. Humans, and I daresay dogs and dolphins, are naturally predisposed to make sense of the world by telling ourselves a convincing story.

A couple days later, I mentioned the End of Fruitcake to Dau.II and she said that, of course, the tin had been required for transporting a certain birthday cake to Allihies and the fruit-cake had been transferred to this other cake-tin. That was a win! because I was now half a fruit-cake ahead of expectations; and this was like finding a €10 note on the street. I get such a rush from finding things (glasses, car-keys, a working pen, ) that it's almost worth losing them. Gawd, it must be easy to prank old folks. I'll take this as an opportunity to say that if we decluttered more then the essentials wouldn't find it so easy to hide in plain sight.

That Michael Rosen chocolate cake monologue was a much loved and  repeated part of the family repertoire when the girls were kids. But that was before I'd taught Human Physiology for 8 years on the trot. Through a HumPhys lens, I wondered if Rosen might have a case of Graves' Disease one of the symptoms of an over-active thyroid being buggy-out eyes like Marty Feldman. Indeed progressed Graves can require the insertion of a skin-graft gusset in the eye-lid so that the eyes can close properly to sleep. A little knowledge is a dangerous thing and I fully accept that my knowledge of Human Physiology is barely above pub-quiz level (catechism: there a 206 bones in the human body . . . maybe; sometimes; in most folk; depending on definition). It rather turns out that Rosen is a poster-boy for Hypothyroidosis   - picture - and has for a long time been on artificial thyroxine to keep his metabolism ticking over at something approaching normal.

Monday 4 July 2022

fuar fliuch imnioch ocrach anróch

 "cold wet anxious hungry miserable"

Apparently status normal for fisherfolk off the West coast of Ireland. That's according to Diarmaid Ferriter's On The Edge Ireland's off-shore islands: a modern history [2018] which I read through while sofa-bound in a cottage in Allihies, Beara, Cork for the long weekend of my birthday.

My twin sister has pals in England who gave her family rates on a well-equipped, dry, comfortable house looking out over a white-sand beach and towards end of the Beara peninsula at Garinish and Dursey. We have shared only a handful of bdays since we were kids. Dau.II was commissioned to make a half almond half chocolate ☯︎ cake with cherry meringue cream filling. Nothing better for thickening the arteries. I picked up the prayer-flags at the Dzogchen Beara café and Buddhist retreat which hangs out on a cliff facing Bantry Bay on the S side of the peninsula. That's defo worth a stop. Unfortunately the Dursey Island cable-car was closed for maintenance in March, so Tom and Izzy were almost the last folks to yomp to the end of the island this year.

Dursey features in Ferriter's book and the depopulation statistics are typical of the once densely populated off-shore communities of Ireland.

Year Dursey Heir
1911 210 294
1951 95 116
1969 Cablecar Nun
1975 35 32
2010 3

Coincidentally, while I was bucketing along the whale-road to Diarmaid Ferriter's islands, my audio book was Richard Nairn's [prev] Wild Shores: The Magic of Ireland’s Coastline. The conceit here is a complete circuit of Ireland - by yacht, ferry, car and foot - starting and finishing at the Antrim coast in a meta-reprise of Robert Lloyd Praeger's The Way That I Went. Walking in Praeger's footsteps is now a genre; what with By Cliff and Shore: walking the Waterford Coast by Michael Fewer [prev]. There is definite utility in going over the same ground after an interval of years - Praeger lived [1865 - 1953] in a world where corn-crakes Crex crex were abundant and molluscs and fish seemingly inexhaustible. Unpretty much anywhere on or off the coast, the view is despoiled, diminished and dusted with plastic crud. For a brief moment at the end of June 1922, I have been an expert on periphery of Ireland. But nobody invited me to a Coastwatch Ireland pub-quiz, so I was unable to monetize the knowledge before it all degraded and leaked out of my ears and onto the strand.

In 1841 there were at least 60 inhabited islands with a combined total of 34,000 people living out there making their own livelihoods and entertainment. Getting the cattle to market had a whole extra level of faff that mainlanders didn't experience. Many of these islands have been completely depopulated, some with government help like happened in St Kilda in 1930. The common story about island life is how hard it was: without running water, no flush toilet, a long way from a shop or a post-office. But in 1995 our home had no running water, was further from a bus-stop than many of the islands and it was either shit in a bucket or in the lee of a hedge. When our neighbour Mary White was canvassing to become a Green TD in 2007, she witnessed shockin' levels of deprivation behind the gaily white-washed facade of cottages in our CW-KK constituency. 100 km from the centre of Dublin, citizens and voters were simply abandoned to a squalid poverty because they had nobody to shout their corner. Islanders otoh were poster-children for De Valera's pantomime Ireland - speaking Irish, dancing at the cross-roads and wearing, like, Aran sweaters.

Sunday 3 July 2022

Knuckle-walking? not yesterday


Friday 1 July 2022

Brash-hammock

In 2007, my pal Rene made me an offer I couldn't refuse: N=800 10 year old trees at €1 a go. They were mostly oak Quercus robur, but included chestnut both sweet- Castanea sativa and horse- Aesculus hippocastanum. Ten year old trees are the divil to transplant because the windage is enormous but the root-ball is small. Our consultant forester Rissoles Hayes advised splitting the largest trees into a) poles and firewood b) root-ball for coppicing. But we took a chance on the 50 runtiest oaks and planted them "entire" and staked them up in the most sheltered part of the site. The rest were settled each in its mini-digger hole and firmed down in hope. That all filled a week of hard graft and about a third of the (1 acre = 0.4 hectare) site - between us and the loud silver barns of our downhill neighbour.

The following year, 2008, we bought whips of Scot's Pinus sylvestris, larch Larix europaeus, ash Fraxinus excelsior. An EU forestry grant may have been involved but the spade work was all in the family. And the year after that, 2009, we filled in the final third of the site with more, smaller, tractable, oaks. And did I mention that we fenced off the acre and planted a hedge up against it - hawthorn sceagh Crataegus monogyna, blackthorn draighean Prunus spinosa and hazel coll Corylus avellana. Over the next few years, whenever I came across a sapling in the wrong place, I transplanted it into the hedgerow:  ash and wild cherry gean Prunus avium; three more Scot's pines; holly Ilex aquifolium, and with no effort at all a couple of sycamores Acer pseudoplatanus have self-seeded.

Fast-forward 13-15 years, the trees have grown according to their propensity and the local competitive conditions. Just take the oaks which were planted on 2m centres in early 2009. Some of these are as tall as the house and 15cm across at the base while next door will be a runt 2m high and as thick as a broom handle. There are also plenty of standing dead of various sizes scattered through the wood. These 2009 oaks came from the same supplier and were quite possibly acorns from the same tree but natural selection has culled out the weak. "Natural selection" including a lot of luck: local variation in the soil; access to better sunlight; a beetle choosing this bark to chew rather than that.

At the tail end of Winter we called in another forestry consultant for an appraisal with a view to selective felling / thinning of this mixed mostly native woodland. Kiwi Sean arrived a few days later, complimented us for the woods ["oh La, I'm sure you say that to all your clients" taps with fan] and said he'd come back the following week as he expected a window in his day-job as a commercial estate forester. A banjaxed back (occupational hazard in the profession) and 3 months later, Sean rocked up on a Saturday in June. The gestalt of the woodland was quite different as everything was now in leaf, which made species identification easier and everything danker . . . and the midges were out. Some of the ash were leafless, these poor babies are standing dead from the ash die-back and almost all are visibly affected by the consequences of fungal infection.

Thinning is a minimax problem: if you leave everything in place the trees crowd each other out and fast growing ash or sycamore will shade out the slower growing oak. If you let too much sun reach the ground, you'll have a riot of bramble, nettle and struggling seedlings of all sorts and varieties.  Thinning is also a technical challenge because in a dense wood it is damaging and potentially dangerous to fell out trees in the normal notch and back-cut way. Sean does a neat line in chunking - making an oblique cut in the trunk at shoulder-height, so that the top of the tree falls down beside its own roots - rinse, repeat.

Sean found us two days work and said to call him back in two years time. 13 years is a little early to thin for pure oak stands, but the shading from the ash - which is doomed before maturity - and the fact that we asked made an early pass possible and no insane. Foresters play a long game: in 100 years time the several hundred oak trees now will be reduced to maybe 1 dozen mighty champions with long straight trunks [if we prune off the side branches as high as we can reach] too girthy to get both arms around. Our dark woodland is now attractively dappled in the sunlight where shading side branches and ugly trees next to handsome ones have been laid low and clearings are filled with logs and brash.

Kiwi has left the tidy up to us. I have the rest of the Summer to get "fire-wood" off the ground and into some variety of airy shelter so that the wood can shed about a third of its weight as evaporated water. It is much breezier out of the wood than in its own mossy shelter but I'd rather move light logs than heavy logs. I also have a more inclusive definition of what a log is. Foresters tend to treat anything smaller than their arm as brash and either push it through a chipper or leave it in the wood to recycle its nutrients. I, otoh, can get flaming utility out of something much thinner especially if it is conveniently straight [looking at you, ash] for stacking. Logs, especially small logs left in contact with the ground will soon enough be food for microbes rather than fuel for macrobes.  At the edge of one of the new clearings Kiwi had left a square of four ash "gonna be dead soon anyway, not worth my time and your money cutting down". I thought "pallet" or "bed-frame" and suspended four chunky straight logs horizontally from these trees. We then criss-crossed this base-line [about 50cm above the ground] with small branches for eventual burning. Really true long branches have been set aside for bean-poles or laundry props.