How Does Adrenoleukodystrophy Affect Life Expectancy?

How Does Adrenoleukodystrophy Affect Life Expectancy?

Adrenoleukodystrophy (ALD) is a rare and progressive genetic disorder that affects the nervous system and adrenal glands.

Life expectancy in ALD can vary greatly among different people and depends on the extent of symptom progression and type of ALD.

What is the impact of ALD on life expectancy?

The three main types of ALD cause different severity in symptoms. In childhood cerebral ALD (CALD), symptoms start appearing from ages 4 to 10 and progress rapidly. Prognosis for these patients is poor, with death occurring within five to 10 years of diagnosis if left untreated. However, early diagnosis and stem cell therapy can help slow down disease progression.

Adrenomyeloneuropathy (AMN) is the adult-onset form of ALD that is seen in about 25% to 46% of ALD cases. Symptoms usually appear from ages 20 to 30, and are milder than those of CALD. Life expectancy depends on whether or not the brain is affected. Patients whose AMN has no brain involvement have a better prognosis and can lead normal lives with proper symptom management. Life expectancy is significantly lower in patients whose AMN has brain involvement due to their progressive brain damage.

Another form of ALD called Addison’s disease is seen in about 10% of ALD patients. Addison’s disease causes damage to the adrenal glands, which results in the production of lower quantities of adrenal hormones such as cortisol, aldosterone, or both (adrenal insufficiency). Addison’s disease can be fatal if left untreated. However, the impact of Addison’s disease on life expectancy can be minimal if symptoms are managed with corticosteroid replacement therapy.

What can be done to improve life expectancy?

The effects of adrenoleukodystrophy on nerve cells cannot be reversed. However, several treatment options are available that can slow down disease progression and improve quality of life if the disease is diagnosed early.

Dietary changes can help in dealing with symptoms such as dysphagia (difficulty swallowing). Consult a doctor or dietitian before incorporating changes into your diet.

Treatments such as Lorenzo’s oil can help in preventing the progression of CALD, but its efficacy is yet to be demonstrated in randomized clinical trials. Lorenzo’s oil cannot reverse ALD symptoms but can slow down disease progression if administered before symptoms start to appear.

Physiotherapy and occupational therapy can help improve muscle strength and body posture, and allow you to carry out daily activities with minimal discomfort. A physiotherapist can also recommend assistive aids to help with mobility.

 

Last updated: Nov. 4, 2019

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Adrenoleukodystrophy News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

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Özge has a MSc. in Molecular Genetics from the University of Leicester and a PhD in Developmental Biology from Queen Mary University of London. She worked as a Post-doctoral Research Associate at the University of Leicester for six years in the field of Behavioural Neurology before moving into science communication. She worked as the Research Communication Officer at a London based charity for almost two years.