Yesterday, I learned something I did not know about the Marine Corps – not all that surprising, since it happened after my time – way, way after my time. But it makes a lot of sense. I just hope some of those mementoes are representative of other faiths. The manger Christans need to respect and in a way emulate is emphatically not the one that Aesop’s dog was in. And, yes, I did watch the National Memorial Day Concert (a day late) – it’s still available to stream if anyone else missed it the first time.
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John Pavlovitz – Dangerous Christian Drag Show
Quote – They shamelessly don their cheap, glittery regalia, meticulously transforming themselves into a sickening inversion of who they actually are, putting on an unnatural false persona designed to indoctrinate young minds by passing as something they’re not. It is a vile bait and switch that decent human beings should be sickened by.
The political Right is the worst kind of drag show: that of hateful, heartless bigots pretending to be Christian. Click through for essay. What can I say? He nails it.
Crooks & Liars – Dem Senator Schools Chuck Todd On Why Congress Can Regulate Supreme Court
Quote – “The Chief Justice has to make this decision, though, right?” Todd asked. “Separation of powers, whether, I mean, it’s pretty established, Congress can’t make a law that does that, right?” Whitehouse informed the host he was mistaken about how the Constitution works. “No, it absolutely can,” Whitehouse laughed. “Well, it doesn’t mean it’s constitutional,” Todd quipped. “Yes, it does,” the senator explained. “It means it’s constitutional because the laws that we’re talking about right now are actually laws passed by Congress. The ethics reporting law that is at the heart of the Clarence Thomas ethics reporting scandal is a law passed by Congress.” Click through for story. Chuck Todd is far from alone in (apparently} believing the “SC” in “SCOTUS” stands for “Sacrd Cow.” It doesn’t. Whitehouse is a gem.
Experts in autocracies have pointed out that it is, unfortunately, easy to slip into normalizing the tyrant, hence it is important to hang on to outrage. These incidents which seem to call for the efforts of the Greek Furies (Erinyes) to come and deal with them will, I hope, help with that. As a reminder, though no one really knows how many there were supposed to be, the three names we have are Alecto, Megaera, and Tisiphone. These roughly translate as “unceasing,” “grudging,” and “vengeful destruction.”
Between an effective antibody for all the CoViDs (SARS-2) – mentioned in a short take today – and the promises of gene therapy, it’s been an impressive week. Yes, I realize I’ve put up a lot of good news/potentially good news today – but hey, it’s Mother’s Day.
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Gene therapy helps combat some forms of blindness – and ongoing clinical trials are looking to extend these treatments to other diseases
New gene therapies are helping to treat certain forms of inherited blindness. GettyImages
An estimated 295 million people suffer from visual impairment globally. Around 43 million of those people are living with blindness. While not every form of blindness can be cured, recent scientific breakthroughs have uncovered new ways to treat some forms of inherited blindness through gene therapy.
Jean Bennett is a gene therapy expert and a professor emeritus of ophthalmology at the University of Pennsylvania. She and her laboratory developed the first gene therapy drug for a genetic disease to be approved in the U.S. The drug, Luxturna, treats patients with biallelic RPE65 mutation-associated retinal dystrophy, a rare genetic disorder that causes visual impairments and blindness in patients early in life.
In March, Bennett spoke at the 2023 Imagine Solutions Conference in Naples, Florida, about what gene therapy is, why it matters and the success she and her team have had helping the blind to see. The Conversation caught up with Bennett after the conference. Her edited answers are below.
Jean Bennett speaks at the 2023 Imagine Solutions Conference.
What is gene therapy and how does it work?
Gene therapy is a set of techniques that harness DNA or RNA to treat or prevent disease. Gene therapy treats disease in three primary ways: by substituting a disease-causing gene with a healthy new or modified copy of that gene; turning genes on or off; and injecting a new or modified gene into the body.
How has gene therapy changed how doctors treat genetic eye diseases and blindness?
In the past, many doctors did not think it necessary to identify the genetic basis of eye disease because treatment was not yet available. However, a few specialists, including me and my collaborators, identified these defects in our research, convinced that someday treatment would be made possible. Over time, we were able to create a treatment designed for individuals with particular gene defects that lead to congenital blindness.
Gene therapy treatments are now available in pharmacies and operating rooms all over the world.
Gene therapy is even being used to restore vision to people whose photoreceptors – the cells in the retina that respond to light – have completely degenerated. This approach uses optogenetic therapy, which aims to revive those degenerated photoreceptors by adding light-sensing molecules to cells, thereby drastically improving a person’s vision.
You created one of the first gene therapies approved in the US. What is the current state of the clinical use of gene therapy?
There are now many approved gene therapies in the U.S., but the majority are combined with cell therapies in which a cell is modified in a dish and then injected back into the patient.
Many forms of gene therapy are helping to treat blindness. GettyImages
The majority of those therapies target different forms of cancer, although there are several for devastating inherited diseases. The drug Skysona is a new injectable gene therapy medication that treats boys ages 4 to 17 with cerebral adrenoleukodystrophy, a genetic disease in which a buildup of very-long-chain fatty acids in the brain can lead to death.
The gene therapy that my team and I developed was the first FDA-approved project involving injection of a gene therapy directly into a person – in this case, into the retina. Only one other FDA-approved gene therapy is directly administered to the body – one that targets spinal muscular atrophy, a disease that causes progressive muscle weakness and eventually death. The drug, Zolgensma, is injected intravenously into babies and children diagnosed with the disease, allowing them to live as healthy, active children.
There are now more than two dozen FDA-approved cell and gene therapies, including CAR T-cell therapies – in which T cells, a type of immune system cells, are modified in the laboratory to better attack cancer cells in the body – and therapies for various blood diseases.
What are you currently working on that you’re most excited about?
I am very excited about some upcoming clinical trials that my team will soon initiate to target some other devastating blinding diseases. We will incorporate a new test of functional vision – how your eyes, brain and the visual pathways between them work together to help a person move in the world. This test utilizes a virtual reality game that is not only fun for the user but promises to provide an objective measure of the person’s functional vision. I hope that our virtual reality test will inform us of any potential benefits from the treatments and also serve as a useful outcome measure for other gene and cell therapy clinical trials involving vision.
What are the biggest challenges gene therapy faces?
The biggest challenges involve systemic diseases, or diseases affecting the entire body rather than a single organ or body part. For those diseases, super-high doses of gene therapy reagents must be delivered. Such diseases involve not only technical challenges – such as how to manufacture enormous amounts of gene therapy compounds without contaminating them – but also difficulties ensuring that the treatment targets diseased tissues without causing toxic immune side effects. That level of a problem does not exist with the eye, where relatively small doses are used and exposure to the rest of the body is limited.
Another challenge is how to address diseases in which the target gene is very large. Current approaches to delivering treatments into cells lack the capacity to hold large genes.
Cost remains a key issue in this effort – gene therapy drugs are enormously expensive. As drug manufacturers are able to refine this technique, gene therapy drugs may become more commonplace, causing their price to drop as a result.
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AMT, this news carries a lot of potential. Some of it will probably not work as expected and hoped. But even if only a few of the possibilities pan out, the results could be amazing. I’m thinking today of the pain mothers of children with some kind of genetic problem have had to suffer ocer the millennia, and hoping that may someday be a thing of the past.
Yesterday, it being May Day, my mind filled up with associations, such as the fact that May 1 is the big labor holiday internationally (and once was here as well.) That led me to think of Odgen Nash’s poem about the man who hated spring – a psychological illness which had begum when someone told him to “Come down to Union Square, it’s Mayday,” and he had misheard it as “Come down to Union Square, it’s payday,” and the disappointment was more than he could handle (it ends with his wife shooting him, and his last words are “Thank you, honey, it was thoughtful of you to use the autumn-atic.” Which leads right in to the second short take.) Then my mind turned to Walpurgis – actually, I think, Mayday Eve – one of the four great pagan festivals which Christans, of course, associated with witchcraft. And then they associted witchcraft with people, especially but not exclusively women, who wanted to harm children. And that seques naturally into the first short take and comes right back the the Labor Day thought I started with.. I swear I did not plan any of that. It just happened. Coincidence actually is a thing. Also – I’m sorry to report that Gordon Lightfoot has died.
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Letters from an American – April 28, 2023
Quote – Those in favor of the new policies argue that fewer restrictions on child labor will protect parents’ rights, but in fact the new labor measures have been written by the Foundation for Government Accountability (FGA), a Florida-based right-wing think tank. FGA is working to dismantle the federal government to get rid of business regulations. It has focused on advancing its ideology through the states for a while now, but the argument that its legislation protects parental rights has recently enabled them to wedge open a door to attack regulations more broadly. FGA is part of a larger story about Republicans’ attempt to undermine federal power in order to enact a radical agenda through their control of the states. Click through for full article (you may have to click on “Keep Reading.”). This a few days old, but any time is the time to address this (so close to Mothers’ Day, too.)
Colorado Public Radio News – Here’s why voters are unlikely to have a direct say in an assault weapons ban
Quote – In theory, a group can work to place a ballot initiative on the statewide ballot. That process involves taking a proposed initiative to the Secretary of State, agreeing on the language that would be on the ballot and gathering valid signatures as a sign of support. Then, and only then, will voters have a chance to have a say on a possible assault weapons ban. Non-fiscal ballot measures like gun control rules can only be considered in even-year elections. Click through for details, including the difficulty of defining an “assault weapon,” which is a huge problem no matter who is pushng the legislation. Legislators and activist really need to drop the term and think in terms of characteristices which make some weapons so deadly and legislate those, without ever giving any weapon a name. Also , this is Colorado, but most if not all states have some kind of process which is analogous.
Yesterday, I woke up to find that there had been a power failure while I was asleep. I don’t mind resetting the clock and the microwave, but I also had to reset the radio in the computer room, and that is a real pain. Both speakers fell; they weren’t damaged, but the first one knocked a full glass of water off the desk, drenching the desktop, the floor, and one of my sleeves. The one positive consequence was that it stretched me just right so that I didn’t need to use the TENS. Nevertheless, it is NOT something I want to do every day. The luck of the Irish was not with me (not surprising, since I have no Irish DNA – I have one redneck grandparent, but she was English/Scottish.) I did figure out how to send everyone a greeting card directly through the blog, and since TomCat did a two-day Saint Patrick’s Day cartoon sequence, here is your card to go with day two.
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PolitiZoom – Jimmy Carter Asks President Biden to Deliver His Eulogy: Will Trump Show Up?
Quote – Some people go very quickly after pulling all the meds that had kept them alive. Others? Well, they’re tougher, for all we know, Carter could live another six months. But he is preparing now and he asked President Biden, two of the finest men to ever live, to give his eulogy…. These two go back a long time. “When Jimmy Carter ran for president the first US Senator to endorse him was Joe Biden.” Click through for details and speculation. You may have seen the headline, but, even if you’re as old as I am, you may not realize quite how far back the relarionsship goes. As for TFG, can we hope that some court will take that out of his tiny hands?
Robert Reich – How to stop playing whack-a-mole with the banks [Spoiler: Bring back Glass-Steagall]
Quote – It’s time to admit that banks that take in deposits are public institutions that shouldn’t gamble with those deposits. Regardless of size, no bank has the capital on hand to manage a sudden full-blown bank run. This is why, ever since the bank bailouts of 2008, the biggest banks have been “too big to fail.” And it’s why last weekend the government decided to backstop smaller banks, too…. The Glass-Steagall Act was the law of the land until 1999. It prohibited banks from making profits off of the deposits entrusted to them. I say, bring it back! Click through for article – He doesn’t mention something I read elsewhere this week – that Glass-Steagall was repealed because “It worked so well, we don’t need it any more.” I wanted something to beat my head against.
Yesterday, I got an email from Move On, who have an insidious plot. They did not post the contents of the letter so I can’t provide a link, and will summarize. They want to put up three billboards with quotes on the route which Trump** must use to get to the airport, since his Mar-a-Lago heliport is gone. They want each one to highlight a different Tucker Carson text from the trove in which he admittied he does not care for Trump** This, they believe, will cause Trump** to post – let’s say shade – at Carlson every time he takes that road. This will cause Tucker’s viewers to leave him. That’s far more than they say even at the donation site. I think they don’t want the right to get even a hint of it unless and until it’s a done deal. That’s a pity, because it’s quite amusing, but it’s a good strategy. If and when they get the billboards up, we can share the details. Also, I got The New Yorker’s Name Drop answer from the first clue – and actually, any of the clues alone would have given me the correct answer. In this case it was just someone I know so much about that it would have been difficult to wrtie a clue whivh wouldn’t have given me the answer immediately.
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CPR News – As gun deaths rise, Colorado is trying something new — a public health approach to gun violence prevention
Quote – In a first-of-its-kind partnership, the Office of Gun Violence Prevention within the Colorado Department of Public Health and Environment is teaming up with researchers from the Injury and Violence Prevention Center in the Colorado School of Public Health. They’ll create and maintain a resource bank of regularly updated and accurate materials regarding gun violence in Colorado. In addition to the resource bank, officials at the new office also developed a grant program to fund evidence-and-community-based gun violence prevention initiatives. Click through for details. I really thought it was more widely known than apparently it is that guns are a public health problem. It’s too early to say what this iniriative will accomplish, but we can hope
The Conversation – The retention problem: Women are going into tech but are also being driven out
Quote – So, questions arise: Why does the technology industry have a retention problem? Why are women who are employed by the technology industry quitting in such high volumes? What factors contribute to this low retention of women in the technology industry, and what kind of support do women need to stay and succeed in it?… Mainstream media often reports on open-source software’s toxic “tech bro” culture. In recent years, high-profile leaders in open-source software have been exposed for their abusive behavior. Click through for full article. Back in the day, women who went into male dominated professions knew we had to be tough and we prepared for it. Is it a drawback of greater societal acceptance in general that we don’t sufficiently prepare kids to cross invisible lines?
Food For Thought
Now, this is what I call trolling!
Russian state media: Finns will die of cold in winter due to cutting ties with Russia
Yesterday, looking at the weather forecast (which I automatically do on my way to get the times of sunrise and sunset, but normally don’t pay a lot of attention), I had the thought that this could be am – intersting – week weatherwise. Fristly, they are taking about snow for tomorrow, starting tonight (we could possibly have a couple of inches by 7 pm.) It’s a bit clumsy to describe the graph, but it’s a broad strip covering ten days from left to right, with temperatiure aand sunlight at the top and precipitation at the bottom and everything else you can think of in between – pressure, wind speed, even wind direction, and more. You can move your cursor across it, and wherever you put it, you’ll see a top-to-bottom line of everything. If you want to know what the wind direction will be next Tuesday at noon, you can set a line at that day and time and it will tell you. In fact, it tells me way more than I want to know. The reason I bother with it is that it has thousands, mabe tens of thousands, of weaher stations, and you can select, not just the zip code, but the exact weather station you want to track. And in Colorado Springs, where uou can go through three seasons just driving from 80911 in the south to 80921 in the north, that is important to me. The weather station I use is maybe four blocks from my house. And while even with that kind of pin point data, though still not perfect, it’s amazing how close to reality it can be. But I digress – I was going to say that, after tomorow, we an expect several days of sun, and a weekend with highs in the 50’s, before getting hammered again a week from today. Not unusual. But it does feel unusual to have a good idea what to expect.
I also learned that at least soe classical musicians and commentatore, probably beginning at Juilliard, are no longer using the perk “accompanist.” Instead, they are saying “collaborativepianist.” Ilikethat. I’ve often thought accompanists do not get enough credit. I was fortunate enough to meet one once, Anne Epperson, who told me that her teachers all wanted her to become a concert pianist, but that she preferred making music in collaboration. If you are ever thinking of investing in a CD (or whatever format comes along) of a piece with a soloist and a pianist, and you have a choice, buy the one with Epperson. Also, apparently the State Department just issued a warning to any Americans in Russia to leave, and any not in Russia NOT to go there. While this is just common sense, I feel that bringing it up now probably means they know something we don’t.
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Capitol Hill Seattle BLog – Video shows East Precinct officers back down after bystanders step in over heavy response to Capitol Hill ‘shots fired’ 911 calls
Quote – The quickly formed and instantly tense standoff is an example of how fast a police response to a 911 report involving a gun can escalate and also shows how perceptions of police in a standoff situation have shifted after repeated incidents like the killing of Tyre Nichols…. According to East Precinct radio updates, police had been dispatched to the area after a 911 caller reported a gunshot and a second caller reported two shots along with somebody yelling, “Everybody is going to die.” But people at the scene told police there was no shooting and no gun — only a young person in a yellow sweater upset and suffering a crisis. Click through for details. This story is going on two weeks old now, but re-reading it, I am still struck at how new it is. And that it couldn’t have happened just anywhere. It needs more visibility – a LOT more visibility.
The Conversation – Diversity and moderation over tradition – why Democrats moved South Carolina to the start of the 2024 presidential campaign
Quote – As political scientists in South Carolina, we understand how important the state’s primary is to the Democratic Party. Working at the College of Charleston for over a decade, we have seen dozens of campaign visits and events by presidential hopefuls of both parties to our city and campus. Given our front-row seats, we wrote “First in South: Why South Carolina’s Presidential Primary Matters,” a book about South Carolina’s primary process. Published in 2020, it examines South Carolina’s demographic makeup, the state’s primary electorate and how it compares with each party’s typical national primary and caucus voter. What we learned was, on several key metrics, South Carolina voters are a better reflection of the demographic diversity and moderate stance on issues the party prioritizes than voters in Iowa and New Hampshire. Click through for rationale. Some are questioning spending money here, in a state we are never going to win (as if we arent already doing that in Iowa.) But remember, this is a primary we are talking about. Democrats in South Carolina are going to be good, strong Democrats with a good sense of what the nation actually needs. If we learn those lessons, and don’t forget them, I’m thinking our money will not be ill spent.
Food For Thought
I put this into a comment on Nameless’s recent MTG srticle, but I thought it too good for anyone to miss:
Yesterday, the radio opera was L’Elisir d’Amore by Donizetti. If I remember correctly, that was included in the summer lineup, I think from China, and I think I remember it was an American Old West setting – cowboys and a snake oil salesman and recruiting for the Civil War. So there’s not much to add. I can mention that the characters are straight out of the Italian Commedia dell’Arte and that their names are part of the humor – “Nemorino” means “little nobody,” Sergeant “Belcore” means “beautiful heart” (that would be only on his own mind, as his character is the “braggart soldier,”) and Dr. “Dulcamara” means “bittersweet.” Commedia dell’Arte heroines were spared from this, so Adina is just Adina. The one thing that has always bugged me about this ooera, although it’s necessary to the plot – wouldn’t you think of a love potion as something you’d give to the object of your affections to change his or her feelings? That’s certainly how it works in “Tristan und Isolde,” and in Shakespeare’s “A Midsummer Night’s Dream,” and just about everywhere else. But in this, Nemorino drinks the potion himself to make Adina fall in love with him. Somehow it works. Also (via Heather Cox Richardson), Representative Bill Foster (D-IL), an award-winning physicist with real credentials, tweeted a welcome to the Science Committee to George Santos as a “Nobel prize winner” who also received a math prize “for his groundbreaking work with “imaginary numbers.” I’m not sure that “poorly educated” MAGAts know that “imaginary numbers” are an actual thing – but even those of us who do can be sure Foster is not talking about that kind of “imaginary numbers.” And I hesitate to say the DOJ trolled Gym Jordan when all they did was explain that they cannot reveal any information on ongoing investigations, but they’ll be happy to help with crafting legislation when it’s just pure fact, but it’s going to sound like “Keep your nose out of grownup matters, little boy, and do your own job. We’ll help – you obviously need help.”
The New York Times (no paywall – The U.S. will name the Wagner mercenary group a transnational criminal organization.
Quote – The United States has decided to designate the Russian private military group Wagner as a significant transnational criminal organization, the White House said on Friday, a move that will expand the number of nations and institutions that can be prevented from doing business with the company “Our message to any company that is considering providing support to Wagner is simply this: Wagner is a criminal organization that is committing widespread human rights abuses,” John Kirby, the White House national security spokesman, told reporters at a news briefing.
Click through to one or both. The first link will demonstrate how they operate on deceit (and throw their own under the bus). The second is more about what we are trying to do about it.
Crooks & Liars – GOP Congressman Greg Steube Falls Off His Roof
Quote – You know, I’ll bet Kevin McCarthy just might start to worry about getting rid of proxy voting. Yesterday Florida congress critter Greg Steube fell off his roof and is in the hospital, which leaves Kevin with a two-vote margin. His caucus might start to get Manchin-and-Sinema-type ideas! Click through for story. From Susie Madrak’s keyboard to God’s iPhone. We’d only need to flip two. Or, I won’t wish them injuries or death, but if we could only flip one, and one could be arrested and detained long ehough, we’d have the votes to raise the debt ceiling right there. Yeah, I know, McCarthy won’t bring it to the floor. Except he might. He has already shown willingness to bring a vote to the floor without having the votes – what was it, 15 times?
