Struck by Adrenoleukodystrophy

While under normal circumstances an person might only be aware of brain disorders like Alzheimer’s primarily due to various articles and stories published in the reputed Readers digest issues and still people might have got a better view of a brain disorder with the latest Hollywood blockbuster “First Fifty Dates”, there still seem to be lot more discovered by the general masses. Though human beings are considered as the most intelligent species on the planet earth owing to their much complex superior brains and the scientific and technological advancements innovated or channelled through it, still there are instances when nature takes on the powers granted to this 560 cc mass of knowledge. Adrenoleukodystrophy is one such rare mental disorder which failed both scientists and human brain itself. Though perpetual research is being conducted for determining its cure and treatment but majority of the outcomes remain biased till date for this ambiguous disease.

Adrenoleukodystrophy is a rare genetic disease caused by a defective autosomal recessive gene located on the X chromosomes and scientifically speaking commences with degeneration of myelin sheath surrounding the brain and gradual loss of adrenal glands consequent into lethargic body self defense and loss of memory.

The Myelin sheath is an insulating protein layer surrounding the nerve cells or neurons which are responsible for conducting impulses for our physical reflex actions and motor responses, surrounded by two fatty layers it represents the simple but complex wiring of the brain and can be compared to the insulated wires used in the wiring of our home.

Adrenoleukodystrophy is associated with very long chains of fatty acids which are the prime reasons for the degeneration of myelin sheath once the myelin sheath is degenerated by constant acumination of fatty acids there is absolutely no question of impulse conduction this critical situation consequences into the poor performance of an individual socially and professionally. Like Alzheimer the patient suffering from Adrenoleukodystrophy also suffers with retention disabilities and gradually looses his past memories. The process generates much poignancy and is gradual, eventually leading the patient from death bed to grave yard.

Adrenal glands is one other organ affected by the dreaded Adrenoleukodystrophy like myelin sheath the disease leads to gradual paralysis of the functions executed by the Adrenal glands. Adrenal glands is solely responsible for metabolic activities in the body, suppression of inflammatory reactions in the body, it also facilitates the excretion of salts in the urine, maintaining blood pressure in the body and facilitating blood supply to our muscles and brain.

Hence with its gradual degeneration the body of the patient suffers from every possible ailment of the metabolic disorders these may include ailments related to hypertension, excessive perspiration, paralysis, stiffness of body parts, Mayo cardinum infraction or simply heart attack, loss of appetite and in many cases stones in the kidney and uterus. Since the body defense slowly and steadily degrades the chances of catching a viral bacterial or protozoan infection gravely increase leading to further deterioration of the patients conditions. Remyelination is an on going project initiated for finding a cure for the disease. The trick applied is simple, stop the human body from making any more long fatty acid as the myelin sheath gets destroyed due to surplus accumulation of fatty acids. Various findings and experiments state that stem cells extracted from human embryo which are prone to rapid cell division are useful and the key answer to this disease.

Lorenzo oil a mixture of a mixture of oleic acid and erucic acid is said to cure Adrenoleukodystrophy or at least is effective in containing the disease for sometime. Lorenzo Odone was the first patent who was treated with. Lorenzo oil and is still alive at the age of 28. Low fatty acid diets is also suggested. Bone marrow transplant is yet another way by which the earlier effects of Adrenoleukodystrophy can be checked efficiently.

Unfortunately there is no cure for this disease and what ever methods that have been suggested are temporary hypothetical treatments and do not guarantee stable and unbiased results. The lost myelin sheath has yet not be regenerated by any treatment undertaken thus so far. Hence the cause and treatment still remains anonymous till date.

The age group affected is between 5 to 10, yet the disease can occur at any stage to any individual at any possible life time as the gene is in recessive state and remains dormant in majority of the cases, only when the genetic aberrations are dominantly expressed then only the onset of the disease starts.

Brain disorders are not common but when they do occur patient in suffering needs proper medical and physiciatric help along with the most valuable emotional support of the family. Initial symptoms of the disease should not be misinterpreted for some lousy disease, if Adrenoleukodystrophy is taken into account and detected at its initial stages then the suffering and pain undergone through the disease can be minimized but as ill luck would have it can never be cured.


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