The Perils of Adrenoleukodystrophy or ALD | Adrenoleukodystrophy | Causes, Symptoms and Treatment

The Perils of Adrenoleukodystrophy

In 1993, the movie ‘Lorenzo’s Oil’ was released and gathered positive ratings. It depicted the story of a young boy who was normal until age 7 but thereafter started displaying signs of mental disorders (total blackouts and memory lapses). He was diagnosed with Adrenoleukodystrophy. The parents of the child were baffled with the failings related to this deadly disease affecting their child and decided to research it on their own. This movie was based on a true story, the story of Lorenzo Odone.

Perhaps the most recognized patient of ALD, Lorenzo Michael Murphy Odone, was diagnosed with ALD at the age of 6. The doctors estimated that he’ll live for a maximum of 2 more years but actually he lived longer than that, due to his parents’ constant efforts.

What is Adrenoleukodystrophy

Also known as ALD or Addison-Schilder's disease, Adrenoleukodystrophy is a disease that’s extremely rare and is inherited. A child suffering from ALD survives for 10 years on an Average. It mainly affects Males, although a small percentage of Females have displayed this disorder in its milder form.

The Causes of ALD

ALD attacks the Myelin Sheath in the Brain. This sheath covers the nerve fibers and is vital to the functioning of the nerves. The damage in the Myelin Sheath leads to a damage of the nerve cells which can’t be reversed and can claim the patient’s life. Mutation in the ALD gene is the main cause of ALD. This gene synthesizes the ALD protein. Proteins like this get together to form tissues like the Myelin Sheath. In case of ALD, Myelin Sheath is damaged hence the nerve signals are disrupted which causes a variety of symptoms as listed below.

The Symptoms of ALD

The symptoms observed in those suffering from this disease include:

  • Seizures and Urinary disorders
  • Difficulty in reading, writing, swallowing and understanding speech (in children)
  • Cognitive, Behavioral, Vision and Motor function Impairment
  • Adrenal insufficiency, Brain sclerosis and demyelization
  • Fatigue, Vomiting and Weakness, Crossed Eyes and Paralysis
  • Hypo tension and Adrenal Dystrophy

Treatment of ALD

No cure has yet been found for this disease and a specific treatment does not exist. So far treatment has only been used as a way to slow down the progress of ALD or as a form of relief to the sufferers. However, eating a diet that has a low content of very-long-chain saturated fatty acids has displayed limited success (preferably used before symptoms occur).

A Special Oil named ‘Lorenzo’s Oil’ (after the child of the family who discovered it) is supposed to bring down the blood levels of very-long-chain saturated fatty acids. Oleic Acid is present in the Oil which works against the fatty acids. However, whether this oil prevents the progression of the disease is debatable.

Bone Marrow transplant is being tried out as an experimental treatment for ALD. This treatment is not recommended for patients whose symptoms are already severe. However for very young and pre-symptomatic patients, the outcome of cord blood stem cell transplant appears to be promising.

Steroids (like Cortisol) are also used by doctors.

These treatments are to be given to the patient as soon as possible right after ALD is diagnosed. The Diagnosis of ALD has proved to be difficult due to similarities with other conditions.

Afterthought

Being diagnosed with such a deadly disease at a young age, can be a devastating experience for a family. This is magnified by the limitations of the treatments available and the rarity of the disease. In future more accurate treatments may be available but until then a ray of hope exists in the form of certain trusts like Adrenoleukodystrophy Family Support Trust that offer their services for free to the families of such patients.

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