What Are The Treatment Options For Hunter Syndrome?

What is Hunter Syndrome?

Hunter Syndrome is a rare, genetic medical condition(1, 2). This condition is caused by a gene defect, which results in a missing or dysfunctional enzyme called iduronate 2-sulfatase(1, 2). This enzyme is essential for breakdown of molecular structures, which facilitate the metabolic processes and allows smooth functioning of the major organs of the body.

Brain is the primary organ that gets affected by Hunter Syndrome. The symptoms of Hunter Syndrome are not apparent right from the time of birth, but are seen by the time the child is about three years of age. Till that time the child remains perfectly healthy. The symptom onset begins by a significant change in facial expressions of the child followed by sudden aggressive behavior. The child then starts developing abnormal size and shape of the bones.

Once a child starts having symptoms of Hunter Syndrome, there is rapid decline in the overall health of the patient. Ultimately, due to cardiac and respiratory complications, the child succumbs by the time he is 10 years of age.

However, if the symptoms of Hunter Syndrome are mild, then the child may go on to live till he is 30-40 years of age, although the child will be constantly plagued by cardiovascular and respiratory illnesses. Hunter Syndrome is mostly seen in boys and not so much in girls. This article highlights some of the treatment options for Hunter Syndrome.

What Are The Treatment Options For Hunter Syndrome?

What are the Treatment Options for Hunter Syndrome?

As of now there is no cure for Hunter Syndrome. Treatment is basically aimed at preventing complications and controlling symptoms. Some of the treatment options for Hunter Syndrome are:

Enzyme Replacement Therapy(3): This treatment is aimed at replenishing the missing enzyme and aid in molecular breakdown, which slows down the progression of Hunter Syndrome. This treatment for Hunter Syndrome is mainly used for children with a mild form of Hunter Syndrome. With this form of Hunter Syndrome treatment, the patient will be able to ambulate without assistance, able to move extremely stiff joints, control respiratory distress, aid in growth of the child, and improve facial features. This treatment; however, is given for those children who have not incurred much damage to the brain as a result of Hunter Syndrome.

Bone Marrow Transplant: A bone marrow transplant may aid in inducting new healthy cells in the body and help in making the enzyme that is missing in Hunter Syndrome(3). Another way of generating new cells is by doing a stem cell transplant where the stem cell is taken from the blood of the umbilical cord of the baby, which is born with Hunter Syndrome(3). It is an extremely risky treatment and is done only if other forms of treatment are not deemed to be feasible for the patient.

Multiple Specialists for Treating Hunter Syndrome(3): For severe forms of Hunter Syndrome, there is no specific treatment to slow down the progression of the disease and only symptoms can be controlled. This is done by a team of specialists to include cardiologist who will monitor the status of the heart of the patient. A pulmonologist will also be required to check the respiratory status of the patient and monitor the functioning of the lungs.

Psychiatric Consultation in Hunter Syndrome: A psychologist and a psychiatrist will also be required to monitor the developmental delays seen with Hunter Syndrome and help the patient in coping up with the stress of the medical condition.

Physical Therapy & Medications: Physical therapy is also quite useful in taking care of the stiff joints associated with Hunter Syndrome(3). Medications will be given to aid in sleep, as this is also a common problem seen in children with Hunter Syndrome.

Conclusion

To conclude, a child with severe form of Hunter Syndrome is less likely to survive for more than 10 years(4). Thus, it is important for parents of such children to help them battle with Hunter Syndrome by bringing positive thoughts and including the child in various activities done around the home as long as it is safe for the patient.

The parents can also start stretching and range of motion exercises immediately after diagnosis of Hunter Syndrome to keep the joints flexible. Give the child lot of toys to play with ensuring that they are safe. It is also important for parents to handle such kids with lot of care and affection despite their behaviour, as some kids may be difficult to handle.

Eventually children with severe form of Hunter Syndrome will become bedridden, as their organs stop functioning and the parents are advised to consult with support groups for end of life issues and make the child as comfortable as possible.

References:  

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