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Progeria Facts

Progeria Facts

Here's giving you facts on progeria or 'rapid aging disease', which is a condition that affects a very small percentage of newborns, and is a degenerative disease. Find out how this takes a hold on those who have it, and what are the symptoms and end results that come up, when the stages advance.
HealthHearty Staff
Last Updated: May 12, 2018
Progeria, which is also known as Hutchinson-Gilford Syndrome, is a rare disease that is genetic in nature, due to a problem in the chromosome network. When we age normally from one age to the next, those suffering from progeria age seven times faster than the normal aging rate. By this, it means, that a child that looks 11, will take on the physical appearance of someone in their 70s. The affected person would have similar problems that old people usually have.

Cause: This condition comes about because of an abnormal gene copy and a normal gene copy coinciding, thus labeled under as a genetic disorder. If he/she were born with two normal copies, there wouldn't have been such a problem in the gene structure.

Facts in Numbers
  • Progeria was virtually an unknown disease (till about 30 years before), until research on it began in the 1990s.
  • As of July 2012 the known cases of children with Progeria are 92 in 33 countries.
  • Statistics state that only 1 in 8 million get affected by this rare disease.
  • First signs of this disease begin to appear at 18-24 months of age.
  • Every one year of the child corresponds to 10 years of aging.
  • Average lifespan is 13 years.

The condition isn't subject to any one kind/type of person, and affects all races of people. It has a 1 in an 8 million chance roughly, of affecting newborns. There are ways to identify those who have this condition.

Changes in Physical Features and Health Problems

First Symptom
The very first symptom that is noticed is scleroderma which is an autoimmume skin disease that involves thickening of skin along with changes in blood vessels, and internal organs of the body.

Other Symptoms that Follow
Atherosclerosis, poor vision, hair loss, kidney failure, cardiovascular problems

First Sign
In the first year itself, growth failure will be observed.

Other Noticeable Signs
Hair loss begins early, balding, no eyebrows or eyelashes, wrinkles, teeth loss, large head, small jaw, and dislocated hip

There are ways that one can identify when the other is suffering from progeria, by noticing certain physical factors from the outside. There are also health related problems that arise as part of the progeria symptoms in patients, that are evident upon examination.
  • A pinched nose
  • Cardiovascular diseases
  • Tumor formation
  • Small jaw
  • Loss of hair (alopecia)
  • Scaly skin
  • Voice is high pitched
  • Wide eyed
  • Beak shaped nose
  • Enlarged head
  • Diabetic
  • Generalized atherosclerosis
  • Thin skin
  • Short in height
  • Wrinkled skin on face
  • Tooth formation is delayed
  • Motion is limited
  • Osteoarthritis problems
  • Stiff joints
  • Eyelashes and eyebrows are thinned out
  • Narrow face
  • Growth failure usually takes place during the first year of being born
  • Veins visible on scalp
  • Age spots
  • Ears stick out
  • Cataract

Researchers from the National Human Genome Research Institute established the fact that the cause of progeria was due to a defective single gene (occurrence of point mutation) called lamin A (LMNA). However, this condition is not hereditary due to point mutations taking place in a single gene.

What You Should Know

There are different facts that revolve around this condition. We look into them, and try to fathom what this disease is like, and how it takes a hold on young ones.
  • Lifespan can reach the age of about 20-21 years. It was recorded that a Japanese man with progeria, lived till he was 45.
  • This disease affects 4-8 million births.
  • Aging is seven times faster than normal.
  • Mutation takes place in the gene design that contains the protein lamin-A, wherein the problem lies.
  • Eighteen months is the earliest that a child shows signs of aging.
  • Children can suffer from problems like strokes, blood pressure and an enlarged heart.
  • It doesn't only affect a particular race, but all kinds.
  • No prevention or cure has been found, but treatment is available.
  • The facts indicate that those with this condition usually don't suffer from cancer.
  • Patients have a mind of a 10-year old, but a body of an old man/woman.
  • It also includes Werner's syndrome, or 'adult progeria'.
  • Unstable cells leads to premature progeria.
  • Patients can be a part of activities, both educational and recreational, since mental capacity is stable.
  • Supplements for those with progeria include - Ensure, Boost, Enlive, PediaSure and more.
  • There is occupational and physical therapy given twice/thrice a week to ensure maximum motion range and everyday functioning.

Testing for Progeria
: Although there aren't specific diagnostic tests, diagnosing of the condition is done based on the common symptoms such as growth failure and hair loss. As atherosclerosis is a characteristic symptom, HDL blood tests are done.

Treatment Objective: Treatment is based on enhancing quality of life, reducing the symptoms such as joint stiffness, and bettering mobility and activity. Weight loss is countered by encouraging tube feeding in infants. Low dose of aspirin is given to prevent chances of a heart attack/stroke (they being the main reason for death).

These facts are not easy to digest, knowing that there are kids who have to cope with this condition at barely 18 months of age. It isn't a condition that one can have a significant amount of control over, and it all takes place within the cells, and relies on a situation purely based on the odds. Help those out who have this condition, and don't treat them any differently than you would do to a normal kid.

Disclaimer: This article is purely for informative and educational purposes. Please seek the advice of a registered medical practitioner before consuming any of the above medicines mentioned here.