SRK’s Zero and Achondroplasia

iieHealth Lifestyle Health Blog

The latest movie of Shah Rukh Khan – Zero – celebrates the journey of a man who suffers from dwarfism and goes through many trials and tribulations in life in order to win the trust of his lady love and make history by being part of a manned space mission to Mars. It is a remarkable story indeed. Lets find out what Dwarfism is all about?

What is Achondroplasia?

In the movie Zero, Shah Rukh Khan suffers from Achondroplasia. It is basically a disorder of bone growth that causes someone to be a dwarf and that too in a disproportionate way. Dwarfism can be defined as a condition where you are short even as an adult.

People who have this disease are short in height – their torsos are of normal size but their limbs are shorter than normal. In these cases, it has often been seen that the head is a large one and it looks abnormally so at times. As far as different kinds of disproportionate dwarfism are concerned it happens to be the commonest one of them. It is estimated by CUMC (Columbia University Medical Center) that among every 1 of 25,000 live births this disease occurs. It is similarly common as far as both the genders are concerned.

However, it has also been estimated that it can happen in 1 case out of any 15,000 to 40,000 live births. On an average, men who suffer from Achondroplasia have a height of 4 feet 4 inches and for the women this height has been measured at 4 feet 1 inch.

Why does it happen?

At the early stages of fetal development, it is cartilage that makes up most of your skeleton. Under normal circumstances, most of this cartilage gets changed to the bone.

However, in the case of Achondroplasia this conversion does not happen. This happens because of mutations in the FGFR3 gene. It is this particular gene that asks your body to make a protein that is needed for growing bones and maintaining them as well. If the gene is mutated the protein becomes hyperactive. This, in turn, hampers the normal development of your skeleton.

Achondroplasia has also been described as an autosomal dominant disease.

Is it inherited?

Some experts also say that this is a genetic disease, albeit a rare one. It is said that one gene from a parent is enough to develop this condition. As per data provided by the NHGRI (National Human Genome Research Institute) in 80 percent of the cases it has been seen that Achondroplasia is not inherited. As has been stated already, these cases happen because of a spontaneous or new mutation in the FGFR3 gene.

What are its signs & symptoms?

It needs to be mentioned that there are several symptoms of Achondroplasia. However, each child that suffers from this disease may experience them in her or his own unique way. Normally, their arms, fingers, and legs are shorter. In these cases, the thighs and upper arms are shorter in comparison to the lower legs and forearms. As has been stated already, the heads in these cases are large with a flattened nasal bridge and a prominent forehead.

The teeth of such individuals are either misaligned or crowded. Their lower spines are curved. This particular condition is also referred to as lordosis or sway back. This, in turn, can lead to a situation named kyphosis.

What are the treatments ?

Since most cases result from unexpected new mutations, there is no treatment to prevent Achondroplasia currently. Some cases in children  can be treated with growth hormone, but this does not significantly affect the height of the child with achondroplasia. In some rare cases, surgeries can be an option to lengthen legs.

Kyphosis or “hunch-back” needs surgery if it does not correct itself when your child starts to walk.

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