Progeria, also known as Hutchinson-Gilford progeria syndrome, is a very interesting disorder because not only is it progressive, but it gives the appearance of aging.  As you can see in the image below, you can see the individual in the center seems older and has distinct symptoms; however, the background will hint that the individual is a child on the inside interested in playing with legos and other childhood toys.Image result for progeria

The disease is most often characterized by the early onset of extreme aging, but they seem normal at birth.  The individuals with with Progeria do not grow like most other children do, which often leads to the more well known characteristics.  They often have protruding ears, eyes that are very close together, and comparatively small chins.  There are no intellectual difficulties when individuals have the disorder, nor any decrease in motor function of any kind.

One side effect of the disorder is early onset of atherosclerosis.  Atherosclerosis is the hardening of arteries when plaque builds up.  This plaque then attracts various aspects of the individual’s immune system, such as macrophages, which build up and cause the plaque to grow in side.  In severe cases, the plaque can grow to decrease blood flow to the extent of cutting flow altogether.  This is when a heart attack can occur.  If the clot is dislodged and moves throughout the body, the clot can get stuck anywhere causing other medical emergencies such as pulmonary vein thromboses or even pulmonary embolisms to name two.

This disorder is autosomal dominant, which means the individual will become affected if only one parent gives the allele.  Even though it only requires one parent’s allele, it is still extremely rare; only about 1 in every 4 million newborn children have the disorder.

Sadly, individuals with Progeria do not have a long life span.  Due to the early and severe development of atherosclerosis, individuals usually do not live past approximately 13 years.  One cause of death that you would assume from atherosclerosis is a heart attack.  Depending where the hardening occurs, a stroke can also occur causing death.  There are some cases, however, where individuals with the disorder prove the life expectancy wrong and live past 13, sometimes even past 20 years of age.  In these rare cases, the individuals are studied in ways with the hopes of making progress in the treatment and possibly a cure.

Currently, there is no cure for progeria.  The best thing for individuals with progeria is medicine to prevent atherosclerosis.  This works to help extend the lifespan by slowing down the buildup of plaque in the arteries and their hardening.  Such drugs often include statins to reduce cholesterol, or anticoagulants such as Coumadin or Warfarin to prevent the blood from clotting.

Other than specific medications, there are no specific restrictions of lifestyle required.  The only aspect close to a restriction is consuming smaller meals in higher frequencies.  This is typical for a young child, and since development is slow for individuals with the disorder, the same type of meals are best.