What Is The Best Treatment For Hunter Syndrome?

Hunter syndrome is a rare genetic disorder that is inherited from the mother because of a defective X – chromosome. Thus, it is an X -linked recessive disorder.

What Is The Best Treatment For Hunter Syndrome?

Hunter syndrome cannot be cured. However, the symptoms can be managed with treatment and the severity of the condition can be decreased, in an attempt to improve the quality of life. The treatment mainly focuses on symptom reduction and management of overall condition of the child having hunter syndrome.(1)

Treatment For Complications Of The Respiratory System-

  • Removing the adenoids and tonsils can broaden the narrowed airway to a certain extent. As a result. The problems like difficulty in breathing, sleep apnea etc. can be solved(2)
  • However, as the disease goes further, these problems may return as the airway continues to thicken
  • In order to keep the airway open, certain devices can be used, that can keep the airways open by exerting a pressure on them. This can provide some relief in sleep apnea and airway obstruction. This can also take care of the reduced oxygen supply due to thickening of airways

Treatment For Complications Of The Cardiovascular System-

  • The child may be needed to be monitored closely for high blood pressure, heart murmurs etc.
  • In case of increasing difficulty, the child may have to be operated and the heart valves may be changed

Treatment For Complications Of The Skeletal System-

  • Surgery is not a very good option for children suffering from hunter syndrome as they do not heal quick and well.
  • Hence, in case of joint problems, a physiotherapist may be of help
  • The physiotherapist can recommend exercises for reducing stiffness, maintaining function and improving the flexibility
  • However, physiotherapy cannot stop the degenerative process of the disorder. There may be a need for wheelchair as the stiffness and pain increases

Treatment Of Complications Of The Nervous System-

  • It is difficult to treat the spine and brain of the affected child, because of the sheer difficulty in accessing these areas due to the risks associated with it
  • Your doctor may recommend draining the excess fluid built up in the brain
  • He may also prescribe anticonvulsants, in case your child suffers from convulsions

Treatment For Sleep And Behavioral Issues-

  • The child with hunter syndrome usually develops disorganized sleep patterns. Your doctor may recommend sedatives for that
  • Maintaining a bedtime routine can help your child sleep better
  • Treating behavioral problems with medications might not be the best option as these medicines are known to cause other side effects and worsen the already existing complications(3)

There are certain other treatment methods which are yet in the emerging stage. More studies and research need to be conducted on these treatment methods. These include-

Enzyme Replacement Therapy-

  • In this method, man made or engineered enzymes are used to replace the child’s defective enzyme
  • It is seen that with the administration of this method quite early on in the disorder may help in delaying and even preventing certain complications. However, more studies are required to prove if these changes can significantly improve the child’s quality of life
  • Also, this therapy has not shown much result in improving thinking and intellectual abilities
  • There may also be other side effects to this method, like allergies, headaches etc. however, these effects can be managed with a lesser dose or over time

Stem Cell Transplant-

  • In this method, healthy stem cells are infused in your child’s body
  • It is expected or hoped that these healthy cells will make the defective or absent enzyme
  • However, there is still some more scope for research on this method

Genetic Replacement-

  • In this method, the defective chromosome is replaced with a healthy chromosome
  • However, much more research is needed before this therapy can be made available

Hunter syndrome cannot be cured. However, with proper treatment the symptoms and complications can be managed.

References-

  1. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3249184/
  2. https://www.mayoclinic.org/diseases-conditions/hunter-syndrome/diagnosis-treatment/drc-20350712
  3. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4312709/

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