What is Hunter’s Syndrome?
Hunter’s Syndrome is a rare severe genetic disorder that affects only the male. It is also known as MPS II. Mucopolysaccharide refers to long chains of sugar and carbohydrates that occur within the cells and help build bone, cartilage, tendons, corneas, skin and other connective tissue. Under this condition, the nutrients are not broken down completely. Consequently, this causes harmful accumulation of GAGS thereby causing a chronic progressive disease.
Hunter’s syndrome affects 1 in 100,000 to 1 in 150,000 males, on an average.
Signs and symptoms
Not everyone is affected in the same way. Symptoms of this condition are not very clear or obvious at the time of birth, until eighteen months.
A child suffering from Hunter’s Syndrome will usually have:
-Thick tough skin
-Short or broad hands with curled fingers
In the beginning, the condition can be severe with the symptoms like Aggressive behaviour, Hyperactivity, intellectual disability, jerky body movements and degrading mental functions. However, in numerous cases, there may be mild or no mental deficiency.
Children with MPS II grow steadily until about age 5, and then their growth slows and they develop short stature.
The joint problem, numbness, weakness, hearing problem, increased hair growth, having a frequent cold, bowel problems, weak muscles, bone thinking, trouble talking or thinking are also few problems with a child suffering from Hunter’s Syndrome faces.
Enzyme replacement therapy can help slow down the symptoms for a male child if the condition is not severe. It helps by replacing the protein their body does not produce. It also helps improve hair, facial features, muscle movement, breathing and walking.
Bone marrow or umbilical cord transplant can also be done if suggested by the doctor.
Medication and surgery can help with some of the complications and physical therapy can solve movement and joint problems.
Edited by Preetika Dubey