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PRADER-WILLI SYNDROME

Name: PRADER-WILLI SYNDROME
Author: John Hernandez-Storr

HOW PARENTS AND PROFESSIONALS STRUGGLED AND COPED AND MADE GENETIC HISTORY

by John Hernandez-Storr ‧ RELEASE DATE: N/A

“I felt less alone after researching and writing this book,” the author asserts, and the same will certainly be true for...

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Hernandez-Storr’s debut deftly chronicles the advancement of scientific knowledge about Prader-Willi syndrome and its effects on families.

The author’s daughter was diagnosed with Prader-Willi syndrome at Cedars-Sinai Hospital in Los Angeles in 2002. Hernandez-Storr delves into the history of the condition and wonders whether it’s “a special hell for families.” Obesity and behavioral problems are two main challenges associated with PWS. Children with the condition are short, “floppy,” and notably overweight from age 2; boys also have underdeveloped testes. Most eventually have to be institutionalized. In this dynamic, journalistic account, Hernandez-Storr surveys the major events in the discovery of PWS, which was named after the two Zurich children’s hospital doctors, Andrea Prader and Heinrich Willi, who published a paper about it in 1956. Research eventually revealed either a microdeletion on chromosome 15 or two copies of chromosome 15 from the mother (an example of uniparental disomy) as the ultimate cause. The book handily alternates this layman’s history of the science of PWS with some case studies of families heavily involved in the Prader-Willi Syndrome Association. For instance, Shirley Neason, whose son Daniel had PWS and died at 14, was a founding member of PWSA and edited its newsletter, The Gathered View, from the mid-1970s. PWS patient Curtis Deterling’s progress gives an intimate view of the condition’s typical course. He struggled to follow directions at traditional schools and was later moved to various live-in facilities for people with developmental disabilities. The young man, however, was able to temporarily hold down a job and have a girlfriend. Although the book might seem to hold only niche appeal, the sense of genetic mystery is relevant to any disease’s evolution. In places, Hernandez-Storr gets too bogged down in PWSA conferences and leadership changes; better to avoid this more parochial material and maintain a focus on the universally applicable aspects of the syndrome—how it affects patients and families.

“I felt less alone after researching and writing this book,” the author asserts, and the same will certainly be true for readers affected by PWS.

Pub Date: N/A

ISBN: N/A

Page Count: -

Publisher: Dog Ear Publisher

Review Posted Online: Sept. 17, 2018

Review Program: Kirkus Indie

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THE RIGHT STUFF

by Tom Wolfe ‧ RELEASE DATE: Sept. 24, 1979

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Yes: it's high time for a de-romanticized, de-mythified, close-up retelling of the U.S. Space Program's launching—the inside story of those first seven astronauts.

But no: jazzy, jivey, exclamation-pointed, italicized Tom Wolfe "Mr. Overkill" hasn't really got the fight stuff for the job. Admittedly, he covers all the ground. He begins with the competitive, macho world of test pilots from which the astronauts came (thus being grossly overqualified to just sit in a controlled capsule); he follows the choosing of the Seven, the preparations for space flight, the flights themselves, the feelings of the wives; and he presents the breathless press coverage, the sudden celebrity, the glorification. He even throws in some of the technology. But instead of replacing the heroic standard version with the ring of truth, Wolfe merely offers an alternative myth: a surreal, satiric, often cartoony Wolfe-arama that, especially since there isn't a bit of documentation along the way, has one constantly wondering if anything really happened the way Wolfe tells it. His astronauts (referred to as "the brethren" or "The True Brothers") are obsessed with having the "right stuff" that certain blend of guts and smarts that spells pilot success. The Press is a ravenous fool, always referred to as "the eternal Victorian Gent": when Walter Cronkite's voice breaks while reporting a possible astronaut death, "There was the Press the Genteel Gent, coming up with the appropriate emotion. . . live. . . with no prompting whatsoever!" And, most off-puttingly, Wolfe presumes to enter the minds of one and all: he's with near-drowing Gus Grissom ("Cox. . . That face up there!—it's Cox. . . Cox knew how to get people out of here! . . . Cox! . . ."); he's with Betty Grissom angry about not staying at Holiday Inn ("Now. . . they truly owed her"); and, in a crude hatchet-job, he's with John Glenn furious at Al Shepard's being chosen for the first flight, pontificating to the others about their licentious behavior, or holding onto his self-image during his flight ("Oh, yes! I've been here before! And I am immune! I don't get into corners I can't get out of! . . . The Presbyterian Pilot was not about to foul up. His pipeline to dear Lord could not be clearer"). Certainly there's much here that Wolfe is quite right about, much that people will be interested in hearing: the P-R whitewash of Grissom's foul-up, the Life magazine excesses, the inter-astronaut tensions. And, for those who want to give Wolfe the benefit of the doubt throughout, there are emotional reconstructions that are juicily shrill.

But most readers outside the slick urban Wolfe orbit will find credibility fatally undermined by the self-indulgent digressions, the stylistic excesses, and the broadly satiric, anti-All-American stance; and, though The Right Stuff has enough energy, sass, and dirt to attract an audience, it mostly suggests that until Wolfe can put his subject first and his preening writing-persona second, he probably won't be a convincing chronicler of anything much weightier than radical chic.

Pub Date: Sept. 24, 1979

ISBN: 0312427565

Page Count: 370

Publisher: Farrar, Straus and Giroux

Review Posted Online: Oct. 13, 2011

Kirkus Reviews Issue: Sept. 1, 1979

Categories:

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AN INDIGENOUS PEOPLES' HISTORY OF THE UNITED STATES

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AN INDIGENOUS PEOPLES' HISTORY OF THE UNITED STATES

by Roxanne Dunbar-Ortiz ‧ RELEASE DATE: Sept. 16, 2014

A Churchill-ian view of native history—Ward, that is, not Winston—its facts filtered through a dense screen of ideology.

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Custer died for your sins. And so, this book would seem to suggest, did every other native victim of colonialism.

Inducing guilt in non-native readers would seem to be the guiding idea behind Dunbar-Ortiz’s (Emerita, Ethnic Studies/California State Univ., Hayward; Blood on the Border: A Memoir of the Contra War, 2005, etc.) survey, which is hardly a new strategy. Indeed, the author says little that hasn’t been said before, but she packs a trove of ideological assumptions into nearly every page. For one thing, while “Indian” isn’t bad, since “[i]ndigenous individuals and peoples in North America on the whole do not consider ‘Indian’ a slur,” “American” is due to the fact that it’s “blatantly imperialistic.” Just so, indigenous peoples were overwhelmed by a “colonialist settler-state” (the very language broadly applied to Israelis vis-à-vis the Palestinians today) and then “displaced to fragmented reservations and economically decimated”—after, that is, having been forced to live in “concentration camps.” Were he around today, Vine Deloria Jr., the always-indignant champion of bias-puncturing in defense of native history, would disavow such tidily packaged, ready-made, reflexive language. As it is, the readers who are likely to come to this book—undergraduates, mostly, in survey courses—probably won’t question Dunbar-Ortiz’s inaccurate assertion that the military phrase “in country” derives from the military phrase “Indian country” or her insistence that all Spanish people in the New World were “gold-obsessed.” Furthermore, most readers won’t likely know that some Ancestral Pueblo (for whom Dunbar-Ortiz uses the long-abandoned term “Anasazi”) sites show evidence of cannibalism and torture, which in turn points to the inconvenient fact that North America wasn’t entirely an Eden before the arrival of Europe.

A Churchill-ian view of native history—Ward, that is, not Winston—its facts filtered through a dense screen of ideology.