Frequently Asked Questions
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- What is progeria?
- What are the symptoms of this disease?
- I’ve heard it called the premature ageing disease. Do progeroid syndromes actually cause patients to get older more quickly than normal people?
- Is it contagious? Can I get progeria by being near someone who has the disease?
- If these diseases are genetic, are they passed from parent to child?
- How common are the progeroid syndromes?
- So if these diseases are so rare, why do they matter?
- Is it curable?
- I saw a movie with a kid who aged more quickly than normal. Was that progeria?
What is progeria?
Progeria is a term applied to a group of diseases whose symptoms mimic the signs of ageing. Most of these diseases manifest in early childhood, and cause patients to appear far older than their actual chronological age. They are also collectively known as progeroid syndromes. Of these, Hutchinson-Gilford Syndrome in particular is often known as simply ‘progeria’, although this designation is informal and may be confusing if other progeroid syndromes are also being discussed. The term ‘progeria’ comes from two greek words, which mean "early ageing". What are the symptoms of this disease?
Progeria is actually an umbrella term for a group of diseases, so which particular symptoms a patient will develop depends heavily which progeroid syndrome the patient has. The one common feature of the diseases is the appearance of rapid ageing. I’ve heard it called the premature ageing disease. Do progeroid syndromes actually cause patients to get older more quickly than normal people?
The short answer is no, but there’s a lot more to it than that. Progeroid syndromes mimic some of the effects of natural ageing, but none of these diseases precisely duplicates the natural ageing process. Symptoms of these syndromes may look the same as natural ageing, for example, many progeroid syndromes lead to hair graying and loss. However, all progeroid syndromes also lack symptoms of the natural ageing process, such as increased incidence of Alzheimer’s, or senility. These diseases also have features not associated with ageing, like skin ulcers in several syndromes. So while children with progeria may look as though they are simply getting very old very quickly, in reality the cell processes that cause these syndromes are very complex and do not, so far as we can tell, accurately model the process by which normal people age. Is it contagious? Can I get progeria by being near someone who has the disease?
No. Progeroid syndromes are genetic, and cannot be passed on to another person by proximity, touching, sharing fluids, etc. The only way to get progeria is to be born with one of the genetic mutations that cause the disease. If these diseases are genetic, are they passed from parent to child?
The answer to this questions varies from syndrome to syndrome. Some appear to be caused by a rare recessive mutation that can be inherited from parents. Other appear to be caused by a de novo, or brand new, mutation for every patient. How common are the progeroid syndromes?
Precise figures depend on the individual syndromes, but all progeroid diseases are very rare. Classic Hutchinson-Gilford syndrome has had only about 100 cases reported in the past century. Werner’s Syndrome is far more frequent, but still has affected only a reported 1300 cases in the past hundred years. All of the progeroid syndromes combined affect only a few thousand people in a century. By comparison, other genetic diseases, such as congenital predisposition to breast cancer, are downright commonplace. Progeroid diseases are not restricted to any ethnic group, although some syndromes are more common in certain ethnicities, but appear all over the world in many different people groups. So if these diseases are so rare, why do they matter?
Progeroid syndromes are interesting because they may provide clues to the normal human ageing process. While no scientist will argue that progeria exactly mimics that process, by determining which cellular interactions cause certain symptoms in progeria, scientists may be better able to predict how actual ageing affects those interactions. Progeroid diseases also often involve tremendously increased susceptibility to cancer, and scientists studying cancer prevention and cures are interested in how the genetics of progeria may be linked to the process by which the body prevents cancers from forming. Is it curable?
At this time, none of the progeroid diseases are curable. Progeroid diseases are genetic, and at this time no treatment exists that can cure a person whose genes are mutated to cause progeria. Some of the symptoms of the diseases can be treated, but the underlying cause will remain. I saw a movie with a kid who aged more quickly than normal. Was that progeria?
The movie Jack featured Robin Williams playing a boy who aged at four times the normal rate. While this is the general idea of progeroid diseases, Jack lacked all the other symptoms which characterize most progeria, such as short stature, bird-like facial features, etc. The disease that Jack had is fictional, and resembles true progeroid syndromes only superficially.
Additionally, the movie Blade Runner featured a child who was prematurely aged, with a disease the movie called “Methuselah’s Syndrome.” This child appears tentatively to be a case of progeria.