HOLT-ORAM-SYNDROME SERIES

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A visual conversation of hand and heart conditions:
 

This self-portrait series is an exaggerated, symbolic expression about the psychological and physical effects of living with Holt-Oram-Syndrome, a rare birth defect that I was born with. Affecting 1 in every 100,000 births, Holt-Oram-Syndrome is a genetic mutation that disturbs the development of the heart, hands, and upper limbs often resulting in malformations in bone and muscle development of the hands and upper extremities, as well as cardiac conduction abnormalities. It is an autosomal dominant hereditary condition and my father and brother were also born with this syndrome. 

Some of my physical characteristics caused by the birth defect include: missing and fused carpal bones, shortened clavicles, missing bicep tendons, underdeveloped thumbs and muscles in the hands and arms. In addition, I was also born with a ventricular septal defect, which is a hole in the wall that separates the heart’s lower chambers. I have an irregular and very slow heart beat (Bradyacardia) yet nonetheless, I have a fairly healthy heart; in fact, the hole I was born with naturally healed on its own over time. It's true for everyone that our hands and heart develop simultaneously while inside the womb and often heart defects result in upper limb defects, which is the primary characteristic of Holt-Oram-Syndrome. The mutation affects the TXB5 transcription factors which carry out critically important roles in maintaining limb outgrowth through the regulation of genes that are essential for limb development.  People with Holt-Oram-Syndrome will often display a limited range of motion in their shoulders, elbows and wrists, lack of opposable thumbs, extra or missing fingers, and in extreme cases some people affected by the condition are born without hands/arms or have major heart defects that require surgical intervention.

In this painting series, I am striving to express the beauty of my unique form as well as its awkwardness. I have chosen to utilize wings and birds to symbolize the irony of feeling restricted yet free.  The bird wings represent a sense of flight, freedom, rising above the challenges of physical limitations, and transforming weakness into strength.  The metamorphosis of human hands into bird wings speak metaphorically to the physical and psychological attributes of living with Holt-Oram-Syndrome, as well as the stages of human embryonic evolution.  The exploration of “self” within the conceptual content of these paintings is not to be misconstrued as narcissistic or vain.  Although this is not my primary agenda, in some regards the examination of the “self-portrait” inherently reflects the current idiosyncratic social phenomenon of a “selfie” obsessed culture. These paintings are specific to “myself” but only in hope to transcend internal reflection by publicly sharing my story as a means to inspire others.

© 2019 Angie Poynter Rehnberg

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