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Suggest Treatment For G6PD Deficiency In Children

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Posted on Wed, 4 Feb 2015
Question: Hi pediatricians,

My 2.5 year old daughter is a G6PD carrier with 1-2 out of the 12 genes responisble for coding G6PD drfected, else are normal. I want to apply one drop of pure lavender oil (diluted with a few drops of vegetable oils) on her feet once a day to make her sleep better as she almost never sleeps through the night. (I have almost tried everything and this is one of the two remaining methods i can think of, and a method recommended by many mums snd aromatherapists).

When medical research is done on what people with G6PD should avoid, were essential oils ingredients tested? I only have a common list of things to avoid like fava beans, some chinese herbs and some western medicine. I wonder if any essential oil ingredients are tested and whether lavender is safe for her to use.

Pls advise. Thanks!
XXXXXXX
doctor
Answered by Dr. Diptanshu Das (2 hours later)
Brief Answer:
Your child is not likely to be G6PD deficient.

Detailed Answer:
Hi

Thanks for asking on HealthcareMagic.

GPD deficiency is a X linked disease. A girl has two X chromosomes and hence if one of the chromosomes is defective, it still has a normally functioning X and so it does not really matter if the other one is defective. Our body has various enzymes (made of protein) which are required for it to function properly. G6PD is one such enzyme. There are a number of genes coding for this enzyme. If all of the copies of the genes are defective, the enzyme does not get produced and deficiency symptoms occur. But in her case only 1 or 2 of 12 genes are defective. Think of it as 10 or 11 of the 12 factories are functional. It is not a problem for the 10 or 11 factories to produce what the 12 factories would have produced. So, I do not expect any deficiency symptoms to result, ever, unless 8 or 10 of the 11 functional factories shut down ever in life, which is very unlikely, even at the age of 50 years, unless the normal X gets suppressed. And, even if the normal X gets suppressed, not all the genes on the other X are defective.

To my knowledge there have been no studies proving the safety of essential oils in G6PD deficient people. But logically speaking, there should not be a problem, at least with the small amount of oil that goes in with inhalation. Moreover, even if I assume that the amount of G6PD enzyme is lower than normal, it would still be ok.

Although I understand that you are worried about the sleeplessness of your child, I would suggest you not to worry and to let it go. Just let it be as it is and the child would get the sleep it requires.

Hope it helps.

Regards
Dr. Diptanshu Das
Above answer was peer-reviewed by : Dr. Raju A.T
doctor
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Follow up: Dr. Diptanshu Das (8 hours later)
Thanks Dr XXXXXXX

What are the chances that the normal genes for coding G6PD of a G6PD carrier get affected, or the normal X get suppressed, later into her life? Shall i assume this would happen later into her life snd at what age does it usually happen if at all? Or is it just something which haopens by chance and cannot be predicted? What are the statistics and science behind this?

Thanks!
XXXXXXX
doctor
Answered by Dr. Diptanshu Das (4 hours later)
Brief Answer:
Barr Body formation is a normal phenomenon of random X suppression

Detailed Answer:
Thanks for asking back.

There is a phenomenon called Barr Body formation where one of the two X chromosomes (randomly) gets inactivated. It is a normal phenomenon that is frequently seen in many of our cells. Naturally, not all the normal X chromosomes in all the cells of the body are expected to get 'suppressed' in the form of Barr Body. So, I would not fear about this. It is just a matter of chance which X will get suppressed in which cell but the probability of each is 0.5 of 1 (or 50% times). It is unlikely to ever reach 100%. Assuming it does, still not all the genes coding for G6PD are defective on the 'defective' chromosome. So, she is unlikely to suffer from complete G6PD deficiency although the amount can be lower than normal.

Hope that helps.

In case you are satisfied with my answer, I would be glad if you leave a positive review and a 5 star rating.
Regards
Dr. Diptanshu Das
Above answer was peer-reviewed by : Dr. Bhagyalaxmi Nalaparaju
doctor
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Follow up: Dr. Diptanshu Das (2 hours later)
Thanks so much for your detailed explanation Dr Das!

- Is there an age range when X chromosomes usually start to be inactivated randomly and naturally?

- And would the normal genes on the defected X chromosomes become defected randomly too? If so is there a usual age range when this can happen?

- Are the above more likely to happen when she ages than when she's still young? Or it's completely random?

Thanks a lot! XXXXXXX

doctor
Answered by Dr. Diptanshu Das (13 minutes later)
Brief Answer:
X inactivation is a normal

Detailed Answer:
Thanks for writing back.

1. The Barr Body formation or X inactivation happens right at or even before birth. But since it is random, the probability is 50% for either of the Xs and cannot rise to 100% which means that specifically the normal one gets inactivated in all the cells.

2. The normal X would not mutate to a defective one and you need not worry about that.

3. I would not consider age as an affecting factor. It is just that with advancement in age, the damage to cells accumulate but I do not think that you need to worry about this.

Reading the following may help:
http://en.wikipedia.org/wiki/X-inactivation
http://en.wikipedia.org/wiki/Barr_body

Hope that helps.
Regards
Dr. Diptanshu Das
Above answer was peer-reviewed by : Dr. Bhagyalaxmi Nalaparaju
doctor
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Follow up: Dr. Diptanshu Das (3 hours later)
Thanks Dr XXXXXXX

1. So you are saying that:

- either of the X chromosomes responsible for coding G6PD has 50 pct chance to be inactive

- but there's also a 25 pct chance that both of them active?

- and it's very unlikely that both of them are inactive in which case she'd be a G6PD sufferer?

- can i tell from her comprehensive blood test at birth that which one of the Xs is active and which one is inactive if an?

2. Would the inactivation pattern change at any point in time? For example, if the defected one has been the only inactive one, could it become active at any point in time and the normal one become inactive? Or can the active one shut down also at any point in time in which case she would become a G6PD sufferer?

3. If the defected one is active, could more genes on the defected chromosome become defected at any point in time, in which case she would be more prone to sufferring G6PD?

All i am trying to figure out is whether she would be more prone to sufferring G6PD at any point in her life, and when i need to start watching closely and avoiding the forbidden things. Hence my above questions...... Sorry if i have missed your points due to lack of medical knowledge ... Pls bear with me...

Thanks!

XXXXXX




doctor
Answered by Dr. Diptanshu Das (25 minutes later)
Brief Answer:
X inactivation is a normal process that occurs in ALL cells of the body

Detailed Answer:
Thanks for writing back. I understand your concerns but let me assure you that you are worrying too much and that too unnecessarily.

Two copies of X are not required for a single cell to work. As a result of this, randomly one of the Xs in EACH cell of the body practically gets inactivated. If you randomly take up any cell of the body of a female, you will see a barr body which is basically an inactivated X. You cannot check all the cells of the body and it would be meaningless too. In some cells you will find the normal X inactivated while in others you will find the defective one to be inactivated. If you take the total number of cells, in 50% of the cells the normal one would be inactivated and in the other 50% the defective one would be inactivated. It cannot be that in ALL the cells of the body, the NORMAL one is SELECTIVELY inactivated. So, naturally there would always be some amount of G6PD activity present. And, hence I ask you not to bother.
Above answer was peer-reviewed by : Dr. Ashwin Bhandari
doctor
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Follow up: Dr. Diptanshu Das (1 hour later)
I see.

1. So all in all, there are ALWAYS more normal genes functioning than defected genes, because majority of the responisble genes (10 out of 12) are normal and only a minorty are defected. Is this correct?

2. Does this pattern stay forever, that if we take the total no of cells of her body at ANY point in her life, there will always be 50 pct of her cells with the normal X active and the other 50 pct the defected X active?

3. If we only talk about the defected Xs, now 1-2 out of the 6 genes responsible are defected. Would this defected ratio increase at any point in her life? If so, what is the probability and when would that happen? Some pediatrician did say to me that the patterns may change later into her life say 50 when she would be more prone to sufferring G6P. But I didn't ask for details at that point and now i wonder why she said that ...

Thanks a lot for your patience.
XXXXXXX

doctor
Answered by Dr. Diptanshu Das (18 minutes later)
Brief Answer:
The pattern is always likely to remain more or less like this.

Detailed Answer:
Thanks for writing back.

1. Point no. 1 is correctly mentioned by you. Nothing to add.

2. 50-50 can be 48-52 but not 75-25 or 100-0. So basically, the pattern is likely to remain more or less like this always.

3. The number of defective genes is not likely to increase later on in life. Since I do not think that this will ever happen, I am not going into the probability or when such a thing is to occur.

Hope that helps.

Regards
Dr. Diptanshu Das
Above answer was peer-reviewed by : Dr. Vaishalee Punj
doctor
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Follow up: Dr. Diptanshu Das (26 minutes later)
Thanks a lot for your patience and clear explanation Dr Das!
doctor
Answered by Dr. Diptanshu Das (6 minutes later)
Brief Answer:
Glad to be of help

Detailed Answer:
I am glad that my explanations helped.

In case you are satisfied with my answers, I would be glad if you leave a positive review and a 5 star rating.

Regards
Dr. Diptanshu Das
Note: For further queries related to your child health, Talk to a Pediatrician. Click here to Book a Consultation.

Above answer was peer-reviewed by : Dr. Vaishalee Punj
doctor
Answered by
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Dr. Diptanshu Das

Pediatrician

Practicing since :2005

Answered : 3875 Questions

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Suggest Treatment For G6PD Deficiency In Children

Brief Answer: Your child is not likely to be G6PD deficient. Detailed Answer: Hi Thanks for asking on HealthcareMagic. GPD deficiency is a X linked disease. A girl has two X chromosomes and hence if one of the chromosomes is defective, it still has a normally functioning X and so it does not really matter if the other one is defective. Our body has various enzymes (made of protein) which are required for it to function properly. G6PD is one such enzyme. There are a number of genes coding for this enzyme. If all of the copies of the genes are defective, the enzyme does not get produced and deficiency symptoms occur. But in her case only 1 or 2 of 12 genes are defective. Think of it as 10 or 11 of the 12 factories are functional. It is not a problem for the 10 or 11 factories to produce what the 12 factories would have produced. So, I do not expect any deficiency symptoms to result, ever, unless 8 or 10 of the 11 functional factories shut down ever in life, which is very unlikely, even at the age of 50 years, unless the normal X gets suppressed. And, even if the normal X gets suppressed, not all the genes on the other X are defective. To my knowledge there have been no studies proving the safety of essential oils in G6PD deficient people. But logically speaking, there should not be a problem, at least with the small amount of oil that goes in with inhalation. Moreover, even if I assume that the amount of G6PD enzyme is lower than normal, it would still be ok. Although I understand that you are worried about the sleeplessness of your child, I would suggest you not to worry and to let it go. Just let it be as it is and the child would get the sleep it requires. Hope it helps. Regards Dr. Diptanshu Das