What Is Spinal Muscular Atrophy For Which Child Was Administered Injection Worth Crores?

Hridayansh suffers from Spinal Muscular Atrophy (SMA) which is a rare disease and hence, was administered an injection worth Rs 17.50 crore at the JK Lon Hospital in Jaipur on Tuesday. The child has been kept under observation for 24 hours. Read on to know more about Spinal Muscular Atrophy.
Spinal Muscular Atrophy

Here's What You Need To Know About Spinal Muscular Atrophy

A 23-month-old child, Hridayansh was administered an injection worth Rs 17.50 crore at the JK Lon Hospital in Jaipur on Tuesday. The Zol Genesma injection was imported from America and was administered by Dr. Priyanshu Mathur and a team of doctors at the Rare Disease Unit in the hospital.
Hridayansh suffers from Spinal Muscular Atrophy (SMA) which is a rare disease. His parents, Naresh Sharma and Shama, came to know of the rare disorder when Hridayansh turned six. The parents raised the funds for injection through crowdfunding. The child has been kept under observation for 24 hours after he was administered the injection.
Spinal Muscular Atrophy is a rare condition which causes muscles to become weak and waste away. This is a genetic condition wherein people lose a specific type of nerve cell in the spinal cord (called motor neurons) that control muscle movement. It is caused by a mutation in the survival motor neuron gene 1 (SMN1), which is important for the maintenance of motor neurons. Cleveland Clinic says that without these motor neurons, muscles don’t receive nerve signals that make muscles move. “The word atrophy is a medical term that means smaller. With SMA, certain muscles become smaller and weaker due to lack of use.”
It is important that a child suffering from SMA receives treatment for the condition. If they don’t, the muscles develop weakness which eventually restricts muscular movements including digestion, the beating of the heart muscle and lungs and even the movement of limbs.

Types Of Spinal Muscular Atrophy

There are different types of SMA and it depends on the age when the disease starts. Here, take a look at the types of SMA.

SMA type 1 (infantile-onset SMA or Werdnig-Hoffman disease)

This is considered to be the most severe type of the condition. It happens to infants within the first six months of their birth. Some children with this type of SMA die before their second birthday.

SMA type 2 (intermediate SMA)

This happens when a child develops the condition between six and 18 months. In this type, the child might be able to sit up but they tend to have respiratory issues which shortens their lives.

SMA type 3 (juvenile SMA, Kugelberg Welander syndrome)

In this type, the condition affects children who are 18 months old or older. However, it might become evident as late as in the teenage years,

SMA type 4 (adult SMA)

In this type of SMA, the condition develops in adulthood. At this stage, the condition is rarely severe and doesn’t affect the lifespan of the patient.

SMA not linked to chromosome 5

Johns Hopkins Medicine says that some forms of SMA are not due to SMN1 gene mutations and SMN protein deficiency. “These forms, including Kennedy’s disease, vary in severity, and some may involve muscles farther away from the center of the body than those associated with SMA types 1 through 4.”
(With inputs from IANS and PTI)
End of Article
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news
news