Progeria is a condition wherein children tend to look old. Confused about this rare condition? Let’s have a look at some of the facts in the following article.
Robin Williams, a well-known American actor, once played a role of Jack in a movie with the same name. He was diagnosed with Werner syndrome, a disease similar to Progeria. Jack tends to age four times faster than a normal child. As a result by the time he reaches graduation, he is already looking as old as an eighty year old men. You must have also seen children documented on the Discovery Channel or similar shows, that look way older than their actual age. The kid may be just 3 years old, but looks thrice his age. The child is said to have Progeria.
Overview
Progeria is a rare disease that makes a person age faster than normal. This is caused by a genetic glitch that causes gray hair, wrinkled skin and stooping posture in affected children. The word is derived from a Greek word. The word pro means before and geras means ‘old age’. This extremely rare genetic condition always proves to be fatal. It is also called Hutchinson-Gilford Progeria syndrome or Hutchinson-Gilford syndrome. It is said to affect 1 in 4 to 8 million children. A child with Progeria will live up to 13 years of age at an average. A few have even survived their teenage years and one individual is said to have lived up to 30 years. Till date there is no cure for the condition.
Causes
It was first identified by Dr. Jonathan Hutchinson in 1886 and then by Dr. Hastings Gilford in 1897. Both Hutchinson and Gilford identified the cases in England. This brings us to the question what causes Progeria disease. It is found to be a mutation that occurs on a gene called LMNA. This gene produces Lamin A protein that helps in holding the nucleus of a cell together. If there is any abnormality in Lamin A, it causes the cell nucleus to become unstable. When the cell is unstable, it causes accelerated growth and therefore premature aging. It is not thought to be a hereditary condition. It is a sporadic autosomal dominant mutation. It is therefore an extremely rare case of genetic glitch.
Symptoms
Symptoms are not observed until the child is about 10 to 24 months old. These children are born just like any other normal baby. As the toddle begins to enter the infancy stage, the symptoms observed are as follows:
- Loss of body fat
- Loss of hair (alopecia) on scalp, skin, eyebrows and eyelashes
- Skin begins to show signs of aging
- Failure to thrive
- Limited growth
- Distinctive appearance, like pinched nose, small face and jaw (micrognathia)
- Small, fragile body like an old person
- Atherosclerosis
- Diabetes
- Cardiovascular diseases
- Prominent scalp veins and eyes
- Delay in tooth formation
- Joint stiffness
- Hip dislocation
Facts
It can be diagnosed by observing the symptoms and carrying out genetic test. Children are given medication and treatment to cure the symptoms of arthritis, heart diseases as well as respiratory problems. One of the major facts is that most children do not live beyond the age of 13 years. Most of them die due to heart attacks, stokes or other cardiovascular problems. They are also called in for occupational therapy and physical therapy as their range of motion is limited. There is no cure for the condition till date. However, scientists are carrying out research related to gene therapy and drugs that helps slow down aging in children with this condition.
Hayley Okines, is an English girl who was diagnosed with Progeria in 1999. She tends to age 8 times faster than a normal person. This means she may live up to the age of 13 years. She has been documented by Discovery Health, and seeks treatment from a hospital in America. Till date, there have been 64 children in 30 different countries known to be living with Progeria. In the state of Bihar, India, there is one unique case of Bisul Khan and his wife Razia Khatoon. This couple has had 7 children in total of which 5 were diagnosed with Progeria. Of these 5 children, 3 are already dead. This is said to be the only family in the world with 5 cases of Progeria occurring within the same family.
Progeria is a rare genetic disorder, where a child ages faster than normal. 90% of these children die due to complications related to the heart. Hope the above information has helped you understand more about Progeria disease.
