Progeria Essay

Hutchinson-Gilford Progeria Syndrome other wise known as “Progeria”, or “HGPS”, is a very rare, and fatal genetic disorder characterized by an appearance of accelerated aging in young children. The rate of aging is accelerated up to seven times that of a normal life span in first 13 years of life. Progeria comes from the Greek word (πρό), “pro” meaning premature and (γῆρας), “gerias” meaning old age. While there are different forms of Progeria, the most sever form of progeria is formally known as Hutchinson-Gilford Progeria Syndrome, which was named after the doctors in England: in 1886 by Dr. Jonathan Hutchinson who described the syndrome, and by Dr. Hastings Gilford who independently discovered it in 1904 (Jameson).

Kittel 2

It is estimated that 1 in 4 million newborn children are affected with this syndrome worldwide. It starts with the children suffering from scleroderma, which makes the skin appear scaly and thin. Within the first year there is a slowing in their growth rate and weight gain the physical development becomes stagnant, at two to three years of age their hair starts to fall out and they lose subcutaneous fat. The intelligence, and emotional development of children with progeria are on pare with children the same age but not affected by the syndrome (Hennekam, 2006 pp. 2603-2624).

Development of Classic Progeria

Progeria is hypothesized to be a gene mutation that occurs during the time of conception or shortly after. During pregnancy the mother doesn’t experience any abnormalities or complications indicating a possible problem with the pregnancy. Newborn babies appear normal and on average have a slightly lighter birth weight a...

... middle of paper ...

...r Progeria. Monitory for cardiovascular disease may help with managing the heart conditions that occur later in the child’s life. Some children may have coronary artery bypass surgery of dilation of cardiac arteries (angioplasty) to reduce the onset of cardiovascular disease. Physical and occupation therapy will ease stiff joints and allow more flexibility and allows children to maintain a healthy level of playtime with friends or even some sports. Regular dental visits are important and having the milk teeth extracted will help prevent problems associated with HGPS. Good nutrition for caloric building and promoting smaller meals frequently through out the day will help with energy and growth. Hydration is very important when dealing with a child suffering from Progeria; due to the thin dry skin children with progeria are more prone to become dehydrated.