Primordial Dwarfism

What is Primordial Dwarfism?

Primordial dwarfism is a very rarely occurring form of dwarfism in which the body is properly and proportionately formed, but remains quite tiny. This form of dwarfism begins in the womb itself, and can be diagnosed during pregnancy as a small fetus. These individuals are born with low weight and height, and doctors might even predict that these infants would not survive 24 hours. One popular example is that of The Real-Life Thumbelina Kenadie Jourdin-Bromley, whom doctors thought would not make the night (she was just a little above 2 pounds at birth). However, she survived, and today she is the most famous person with primordial dwarfism in the world.

Primordial dwarfism occurs in five different ways. These are the Russell-Silver Syndrome, Seckel Syndrome, Meier-Gorlin Syndrome, Majewski Osteodysplastic Primordial Dwarfism Types I/III (MOPD Type I/III) and Majewski Osteodysplastic Primordial Dwarfism Type II (MOPD II). These five sub-types of primordial dwarfism are considered to be the severest of the 200 forms of dwarfism that are known. The most severe is the MOPD Type II in which there might be complications of vascular problems, which shortens the longevity of the person. People with primordial dwarfism usually do not survive beyond thirty years of age.

What are the Causes of Primordial Dwarfism?

The exact causes of primordial dwarfism are not known. It is not due to any nutritional deficiency, and not even due to any deficiency of growth hormone. However, there are clear indications that this kind of dwarfism is due to genetic factors. When the embryo is formed by receiving one responsible gene for primordial dwarfism from each parent, then the child gets this condition. The parent themselves might not show this condition, but they might have the gene in a recessive manner. If during the reproduction, this recessive genes combine with each other, then a primordial dwarf will be produced.

There are clear indications of this genetic trait. That is because, though this is a very rare condition, there are families that have more than one children with it. Such sibling dwarfism indicates autosomal recessive inheritance. However, why some people have the genes in the first place is not yet known.

What are the Symptoms of Primordial Dwarfism?

The symptoms make their appearance felt at the fetus stage itself. The following is a list of pregnancy and birth related symptoms:-

• The belly of the pregnant woman does not grow much as the months pass.
• A small fetus is reported during ultrasound and other tests, but the proportion of growth is normal.
• The labor pains begin early and there is premature birth, at about 35 weeks of the gestation.
• After birth, every organ of the newborn is developed and differentiated properly, but there is very low height and weight. Typically, a height of less than 16 inches and a weight of less than 3 pounds is indicated.

As the child grows, more symptoms begin making their appearance. Here is a list:-

• Bone problems will be rampant, especially at the joints of the elbows, hips and knees. Scoliosis of the spine (curved spine) may occur.
• Voice might be of a high pitch and sound squeaky.
• Despite having taken birth with all organs in proportion, the head might appear to shrink, because it grows slower than the rest of the body. This condition is known as microcephaly.

How is Primordial Dwarfism Treated?

Sadly, there is no method yet to reverse the condition of primordial dwarfism. Since this is neither a nutritional nor a hormonal deficiency, administering supplements and injections of the two do not work. The main reason why there is no treatment yet is because the cause of the disease itself is unknown. Main treatment approaches currently include treating the peripheral problems that occur due to the condition, such as difficulties in breastfeeding when in the infant stage, problems of the joints and the spine and problems with vision.

What is the Society's Approach towards Primordial Dwarfs?

Different regions of the world have different kinds of approaches towards primordial dwarfs, but on the whole, the treatment is quite warm. In advanced countries like the US and in European countries, people with this condition get equal rights and are part of the mainstream society. However, some of these primordial dwarfs choose to distance themselves from society. They might also work in circuses and freak shows, thus creating an opening for self-ridicule, which is actually a very sorry situation in itself.

However, there are also organizations like the Little People of America which provide all dwarfs, primordial dwarfs included, with an opportunity to meet each other. These are the times when the little people can mingle with other people with similar conditions and really feel happy. Little people from all over the world throng to such events.