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PRADER-WILLI SYNDROME

HOW PARENTS AND PROFESSIONALS STRUGGLED AND COPED AND MADE GENETIC HISTORY

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

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

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NIGHT

The author's youthfulness helps to assure the inevitable comparison with the Anne Frank diary although over and above the...

Elie Wiesel spent his early years in a small Transylvanian town as one of four children. 

He was the only one of the family to survive what Francois Maurois, in his introduction, calls the "human holocaust" of the persecution of the Jews, which began with the restrictions, the singularization of the yellow star, the enclosure within the ghetto, and went on to the mass deportations to the ovens of Auschwitz and Buchenwald. There are unforgettable and horrifying scenes here in this spare and sombre memoir of this experience of the hanging of a child, of his first farewell with his father who leaves him an inheritance of a knife and a spoon, and of his last goodbye at Buchenwald his father's corpse is already cold let alone the long months of survival under unconscionable conditions. 

The author's youthfulness helps to assure the inevitable comparison with the Anne Frank diary although over and above the sphere of suffering shared, and in this case extended to the death march itself, there is no spiritual or emotional legacy here to offset any reader reluctance.

Pub Date: Jan. 16, 2006

ISBN: 0374500010

Page Count: 120

Publisher: Hill & Wang

Review Posted Online: Oct. 7, 2011

Kirkus Reviews Issue: Jan. 15, 2006

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THE PURSUIT OF HAPPYNESS

FROM MEAN STREETS TO WALL STREET

Well-told and admonitory.

Young-rags-to-mature-riches memoir by broker and motivational speaker Gardner.

Born and raised in the Milwaukee ghetto, the author pulled himself up from considerable disadvantage. He was fatherless, and his adored mother wasn’t always around; once, as a child, he spied her at a family funeral accompanied by a prison guard. When beautiful, evanescent Moms was there, Chris also had to deal with Freddie “I ain’t your goddamn daddy!” Triplett, one of the meanest stepfathers in recent literature. Chris did “the dozens” with the homies, boosted a bit and in the course of youthful adventure was raped. His heroes were Miles Davis, James Brown and Muhammad Ali. Meanwhile, at the behest of Moms, he developed a fondness for reading. He joined the Navy and became a medic (preparing badass Marines for proctology), and a proficient lab technician. Moving up in San Francisco, married and then divorced, he sold medical supplies. He was recruited as a trainee at Dean Witter just around the time he became a homeless single father. All his belongings in a shopping cart, Gardner sometimes slept with his young son at the office (apparently undiscovered by the night cleaning crew). The two also frequently bedded down in a public restroom. After Gardner’s talents were finally appreciated by the firm of Bear Stearns, his American Dream became real. He got the cool duds, hot car and fine ladies so coveted from afar back in the day. He even had a meeting with Nelson Mandela. Through it all, he remained a prideful parent. His own no-daddy blues are gone now.

Well-told and admonitory.

Pub Date: June 1, 2006

ISBN: 0-06-074486-3

Page Count: 320

Publisher: Amistad/HarperCollins

Review Posted Online: May 19, 2010

Kirkus Reviews Issue: March 15, 2006

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