Adrenoleukodystrophy is a rare genetic disease linked to the X chromosome that affects the adrenal glands, nervous system and testicles. The disease particularly affects men and can manifest at any age.
Adrenoleukodystrophy changes myelin, a protein present in the white part of the nervous system. The nervous system works as a kind of electrical circuit and myelin serves to isolate the nerve cells in this circuit. When there is a change in myelin, the conduction stops being done correctly and the nervous system loses its functions.
Symptoms of Adrenoleukodystrophy
The symptoms of adrenoleukodystrophy appear gradually. The individual loses adrenal functions, the ability to speak and interact, has to wear glasses due to strabismus, has difficulty walking, starts to eat through a tube, has many seizures and in a short time the individual is as if in Coma state. Everything happens because your body is unable to maintain itself, needing the help of devices.
Life expectancy
Adrenoleukodystrophy can manifest itself in the first months of the baby's life and in this case, life expectancy is 5 years. If it manifests between 4 and 10 years of life, the child has a life expectancy of approximately 10 years and when the disease manifests itself in adulthood the individual can live for many decades, although the deterioration of the nervous system equally progressive.
How the treatment is done
The treatment of adrenoleukodystrophy is done with the use of hormones from the adrenal glands and with Lorenzo's oil, to delay the evolution of the disease. Physiotherapy and psychotherapy are also indicated.
Bone marrow transplantation may be indicated in specific cases. Docosahexaenoic acid is indicated for the treatment of neonatal adrenoleukodystrophy, but currently adrenoleukodystrophy has no cure yet.
The film "The oil of Lorenzo" portrays the story of a boy who has Adrenoleukodystrophy and is based on real events.