- Adrenoleukodystrophy (ALD) / Adrenomyeloneuropathy (AMN)
- 2009 DeKalb, Illinois, USA - the ULF meeting on ALD/AMN
Discovering what others had long known: Female x-ald carriers do often get neurological symptoms. An expert group leading the female session at this meeting in DeKalb in 2009, focused on the symptoms females often get. My motivation behind attending
this meeting was to find treatment for my mother. Details on how the symptoms may affect female x-ald carriers in their every day life where extensively explored at this session attended by many female x-ald carriers. The understatement was clear: If you do
not have any symptoms yet, they will probably come. A female with this gene is at high risk of developing neurological symptoms. This is a fact. Women with this gene may have been told that they need not worry or their symptoms may not have been considered
as more than typical female problems. This expert group was clear on the opposite. Having seen and heard
all the stories told by women in all age-groups made me convinced this expert group was sharing important information from their clinical experience. Now there are many published articles on this issue that verifies the same.
Some facts: The x-ald gene leads to a defect in the metabolism of very long chain saturated fatty acids (VLCFA). The result of this may
be elevated blood-levels of these fatty acids. Having an overload of VLCFA will cause or may cause damage to nerve cells /myelin. More details about ALD / AMN / x-ald female carriers can be
read at www.ulf.org
Women have two x-genes compared to men with only one.
Very long chain fatty acids are found in normal foods as well as being produced in the body by an endogen production. They can be lowered through dietary means including special
fatty acids, which you can get through Lorenzo´s oil. This treatment has been available for 30 years now. This oil was developed to reduce the levels/concentrations of VLCFA. It is supposed to be taken with a fat reduced and fat modified diet. The active
ingredients in this oil uses the body’s own competitive enzyme system as a "tool". The result is lower blood values of saturated VLCFA.
Lorenzo´s oil with the
special diet is used for boys with x-ALD in the attempt to prevent further progression of the disease. But the treatment has to start very early to get the best results. Family members of boys with ALD or men with AMN may if found early before their symptoms
start, also try this. An early onset of dietary/Lorenzo´s oil or other treatment may be preventive.
In the future we may have an effective and safe treatment for everyone affected
by this gene even when diagnosed after the disease has manifested with clinical symptoms. A lot of studies are being done in this field. The results that may come sometime in the future, might be promising.
I do? I try to keep my blood values of VLCFA within the normal range by dietary means. Why? : Female x-ald carriers may get neurological symptoms. The x-ald gene may lead to a metabolic defect in the metabolism of fats. There are ways to get
the blood values of VLCFA normalized. Getting the blood values normalized or lowered may be preventive. It may stop, slow or delay neurological symptoms.
- -It has been stated that between 20 % and 50% of women who are carriers may manifest some symptoms (Jangouk P, Zackowski KM, Naidu S, Raymond GV, Mol Genet Metab. 2012 Feb;105(2):180-5. Adrenoleukodystrophy in female heterozygotes:
underrecognized and undertreated.)