On Film and Digital Media

On Film and Digital Media Rick J. Scheidt, PhD, and Jim Vanden Bosch, MA, Associate Editors, Helen Q. Kivnick, PhD, Editor: Humanities & Arts The Gerontologist Cite journal as: The Gerontologist Vol. 54, No. 2, 322–326

© The Author 2014. Published by Oxford University Press on behalf of The Gerontological Society of America. All rights reserved. For permissions, please e-mail: [email protected].

“PUSHING THE ENVELOPE”

Video: Life According to Sam (90 min)

Released: January, 2013 This multiple-award winning video provides a smooth flowing, intimate, 2-year portrait of life within a family dealing with Hutchinson–Gilford Progeria Syndrome (HGPS), a rare and fatal genetic disorder more commonly referred to as “premature aging syndrome.” Oscar-winning videographers Sean and Andrea Nix Fine sensitively document how this disorder impacted the personal and family life of three people—mother Leslie Gordon, father Scott Berns, and their progeriaaffected 14-year-old son, Sam Berns. When Sam was diagnosed with the syndrome at age 2, doctors told Leslie and Scott to “go home and enjoy with Sam the time that remains.” Most children with progeria die around age 13 from heart attacks and strokes. Leslie and Scott, both medical researchers, were stunned and frustrated by the lack of existing medical research on the syndrome. Armed with a steely determination springing from deep love, they refused to accept the fatalistic prognosis. Instead, they turned aggressively against the progeria itself and mounted a remarkable effort that raised doubts for the first time about its inexorability. The video recounts their success in building an infrastructure—the Progeria Research Foundation (www.progeriaresearch.org/)—to raise awareness, provide information, and fund medical research aimed at finding the cause, treatments, and cure for the syndrome. It documents the efforts of a research team led by Leslie to demonstrate the efficacy of a drug to delay and to reverse damaging physiological changes caused by the progeria. What is the relevance of this activity for the great

322

The Gerontologist

Downloaded from http://gerontologist.oxfordjournals.org/ by guest on November 8, 2016

Produced and Directed by Sean Fine & Andrea Nix Fine

majority of us unaffected by the syndrome? It has drawn the attention of bio-gerontologists targeting underlying causes of so-called “natural” aging and its associated diseases. More information is available on the Progeria Research Foundation Web site and via Kipling et al. (2004, Retrieved from http:// www.sciencemag.org/content/305/5689/1426) Life According to Sam is filled with natural drama that holds one’s attention throughout its 90-min length. It captures 2 years (2010–2012) in the lives of Leslie, Scott, and Sam, who is 14–16 years of age. The video offers at least three significant learning vectors. The first is increased understanding about HGPS itself, which Sam refers to as “the entity.” The second is an appreciation of the power of parental love to question the right of this disease to destroy the hopes and dreams of Sam and other children who share this malady. Third, the video documents the efforts and achievements of the Progeria Research Foundation in bringing greater attention to this devastating disorder. Progeria is unlike any other childhood affliction. The film documents the premature changes in the physical appearance and health of children that mimic those of natural or usual aging. Characteristics of accelerated aging begin to occur at around 18–24 months. The changes are striking and exotic. Most affected children become bald. They experience stiffness of joints. Their faces become gaunt; they develop a tiny beaked nose, prominent scalp veins, and prominent eyes (Coppedè, 2013). The universal phenotype resembles that of a “little old person.” Cardiovascular and cerebrovascular disease is common. It is estimated that at any one time, there are between 200 and 250 children living with progeria worldwide; the prevalence rate is estimated at one per four to

DNA . . . causes an abnormal protein called “progerin” to be created. It goes into the cell and sticks to places that normal protein would stick, only its toxic.” The discovery led to a test to diagnose children earlier and more definitively. Additional research found that a new drug (Lonafarnib) might possibly aid children with progeria. Funded by Progeria Research Foundation, the video documents the first ever Progeria Clinical Drug Trial designed to test the drug. The research team is led by Leslie. Twenty-eight children from 16 countries came to Boston “every 4 months for treatment and drug supply.” Leslie, Scott, and Sam form close relationships with these families who share the common urgency in a race against time. Sam has a book that logs photos of those children who are still alive and those who have died. The drug trial is completed in 17 months; it takes 8 months to prepare the data. The video shows Leslie dealing with the frustrations ensuing from the choice of the research design for the study—a “single-arm clinical trial.” Due to the 100% fatality rate in HGPS and promising preclinical studies, every child was administered the drug. No children were held out as comparative “controls.” The first journal sent the manuscript rejects it, indicating that the results coming out of the design “are just not enough for us.” Leslie resubmits the paper to a second journal. It is also rejected. She persists. We watch as she opens an e-mail file from the third journal to receive the manuscript. She shouts, “It’s in!” Results provide “preliminary evidence that lonafarnib may improve vascular stiffness, bone structure, and audiological status” (Gordon et al., 2012, p. 6666. Retrieved from www.pnas.org/content/109/41/16666. full.pdf+html?sid=36bfd91f-8f01-45f6-b01aed59818cb48a). Life According to Sam is a wonderful tribute to these “envelope pushers.” Thanks to their persistence, someday children with progeria may enjoy an extended life span, allowing more of their hopes and dreams to become reality. Their efforts may also produce eventual benefits for those who seek to experience healthier natural aging. [Sam Berns died on January 10, 2014 of complications associated with progeria. He was 17.] References Coppedè, F. (2013). The epidemiology of premature aging and associated comorbidities. Clinical Interventions in Aging, 8, 1023–1032.

Vol. 54, No. 2, 2014 323


one in eight million births. Despite its rarity, it is an “equal opportunity” disorder; it affects both sexes and all races. It has been discovered in over 40 different countries, with 20 cases in North America (Progeria Research Foundation, Retrieved from www.progeriaresearch.org/). The children have normal mental and motor development and like to do the same things that other kids do. Sam attends school and parties and concerts. He participates in sports and is an important member of a school drum line. He is an A student and mentors other students in his classes. He is compassionate, mature, and wise. Sam is a recognized leader among his classmates. The film succeeds beautifully in showing that he is not defined by the “facts” of HGPS. An insight into the determination and character of Leslie and Scott and their parental love for Sam is revealed in a small moment during their meeting with Sam’s drumline coach. Sam is 15 at the time. He had long dreamed about being a member of this percussion ensemble. The activity requires balance, energy, and strength—enough to carry the weight of a metal drum frame and the drum itself across his shoulders. His coach seems more than dubious and tells them it is dangerous for Sam to be on the field—“that could really be pushing the envelope.” However, he doesn’t know fully whom he is dealing with. Leslie’s response to this and similar challenges for Sam is, “Can we make this work?” Leslie explains her position in a voice-over: “There isn’t a God anywhere that would have done that to a child on purpose, that would subject a child to that kind of pain, to that disease. I feel impassioned by it. I feel guided by it. I don’t get angry about this. I don’t wallow in, ‘This isn’t fair’. What is driving this is love for Sam and for other children. There is no time to dwell on the other stuff.” A trial period is agreed upon. Sam carries a newly designed drum frame that weights less than a pound. He becomes “one of the most important members of the team.” The Progeria Research Foundation (www. progeriaresearch.org/) was formed by Leslie and Scott 6 months after they received Sam’s diagnosis in 1998. Scott explains: “There was nothing we could do. It just wasn’t part of my psyche or the way I think to accept that, certainly not as a person, certainly not as a clinician. I was working as a pediatric emergency physician. I was fixing things; that was my job.” It ignited efforts to find the gene for HGPS. The video shows the 2002 National Institute of Health Press Conference announcing its discovery. Leslie, who contributed to its discovery, describes its effects: “this one tiny change in

Gordon, L. B., Kleinman, M. E., Miller, D. T., Neuberg, D. S., GiobbeHurder, A., Gerhard-Herman, M., … Kieran, M. W. (2012). Clinical trial of a farnesyltransferase inhibitor in children with HutchinsonGilford progeria syndrome. Proceedings of the National Academy of Sciences of the United States of America, 109, 16666–16671. Kipling, D., Davis, T., Ostler, E. L., & Faragher, R. G. (2004). What can progeroid syndromes tell us about human aging? Science, 305, 1426–1431. Progeria Research Foundation. Retrieved from www.progeriaresearch.org/

Rick J. Scheidt, PhD School of Family Studies and Human Services Kansas State University Manhattan, KS 66502 E-mail: [email protected] doi:10.1093/geront/gnu008

“QUESTING FOR CONNECTION”

Film: Philomena (95 min) Produced by Gabrielle Tan, Steve Coogan, & Tracey Seaward Directed by Stephen Frears Released: November, 2013 (USA) Film: Nebraska (110 min) Distributed by Paramount Vantage Produced by Albert Berger & Ron Yerxa Directed by Alexander Payne Released: November, 2013 (USA) Back in the mid 50s in Ireland, when she was a teenaged unwed mother to be, Philomena Lee was banished from her family and sent to a convent to have her child. She and the other young women sent there for the same reason were allowed contact with their babies for 1 hr a day. The rest of the time they worked long hours at menial jobs to “compensate” the sisters for taking them in. They also had to sign a consent form giving up all parental rights to their babies. When Philomena’s child was 3 years, he was abruptly taken from her and adopted by an unknown family. Philomena eventually left the convent and became a nurse. In the film, Philomena, we see Philomena Lee (Judi Dench) some 50 years later renewing her search for information about her son. Her search comes to the attention of a former BBC journalist, Martin Sixsmith (Steve Coogan). With his contacts in the United States, he finds out that her son was adopted by a wealthy American family and that he became a high-ranking official in both the Reagan and Bush administrations. The rest of the story I will leave for you to see unfold for itself in this very engaging film. The film uses flashbacks to let us in on Philomena’s vivid memories of the birth,

324

The Gerontologist


Distributed by The Weinstein Company

and then the loss, of her son, Anthony. It also uses an intriguing device to let us see pieces of the life of her son after he was taken from her. We are given occasional glimpses of what appear to be home movie footage of Anthony (renamed Michael by his adoptive parents) at various stages of his life as a boy, as a teen, and then as an adult. These short flashback clips actually serve as “flash forward” hints for us about the life of Michael that Philomena, at those points in the film, does not yet know. This, combined with the marvelously expressive face of Judi Dench as she portrays the conflicted Philomena during her journey of discovery, makes for a very moving and memorable film experience. What fuels Philomena’s search is her deep need to tell her now grown son that she did not abandon him. And she wants to know if he ever thought of her, or at least wondered about his birth mother. The longing for restoration of this basic parent/ child connection is part of what makes Philomena such a moving and powerful film. As we watch the story of Philomena’s search, we also begin to see the character of this woman. The convent had done her and thousands of other young women and their children a grave injustice, and then years later, burned all the records of the adoptions. In spite of this, Philomena does not express outrage at the church. She expresses forbearance for the current sisters of the convent when they tell her they cannot help her with information about her son. She maintains her strong religious faith, and in the end, actually conveys forgiveness for the nuns who brutally wronged her. Is this sense of acceptance and forgiveness an expression of the core person she always was or had she come to this after a long life of struggle and maturation? Her character, as developed in the film, triggers reflection on these kinds of questions about personal and emotional