Any parents with a G6PD deficient child?

My son is G6PD deficient, I would love to know more information about this. I am so paranoid what to feed and what NOT to feed him ! There isn't much information given to me from the pediatrician except a really skimmpy pamphlet.


Anyone has actual experience to share? Are soybeans ok ? (Like Soy sauce?) Some say it's strictly the FAVA beans, but some say ALL beans?

One of my son is G6PD deficient,doctor said that soy-beans are ok,only avoid to eating FAVA beans and their products.


Glucose-6-Phosphate Dehydrogenase (G6PD)is an enzyme that catalyzes the reaction to stabilize red blood cells.G6PD deficient subjects normally enjoy good health but they will develop acute haemolytic anaemia when exposed to exogenous stresses such as fava beans,certain drugs or severe infection.


During acute haemolysis,haemoglobin becomes denatured into bilirubin,which will cause jaundice.Neonatal jaundice can be a potentially serious problem because the excess bilirubin can cause permanent damage to the developing brain.In severe cases,it will lead to neurological complications including mental retardation,cerebral palsy and hearing deficit,or even death.


[Etiology of G6PD Deficiency]

G6PD Deficiency is a genetic condition that is inherited in an X-linked recessive manner.This means that males are more likely to be affected by this cindition than females.In Hong Kong,the incidence of G6PD Deficiency is around 4.5% in males and 0.5% in females.In affected males,who have only one X chromosome and thus one copy of the defective G6PD enzyme activity is very low.Therefore they are at high risk of developing acute haemolysis when exposed to fava beans,certain drugs or severe infection.Furthermore,their brothers and sisters have 50% chance to carry the defective G6PD gene.

Females have 2 copies of X chromosome and thus 2 copies of G6PD genes.The G6PD enzyme activity in females carriers is higher and therefore there is little chance for them to develop actue haemolysis.However,occasionally female carriers have low G6PD activity and therefore have high chance to develop haemolysis.Brothers and sisters of female G6PD subjects may also be G6PD deficient but further genetic tests are required to determine the risks.


[What precautions should affected subjects take?]

Since G6PD Deficiency is inherited,patients are affected lifelong and there is no treatment of cure.In order to prevent haemolysis,affected subjects should take the following precautions:


1. Avoid Chinese herbal medicines:Rhizoma Coptidis(huang lien黃蓮),Calculus Bovis(neu huang牛黃),Flos Chimonanthi Praecocis(leh mei hua臘梅花),Flos Lonicerae(kam ngan fa金銀花)and Margarita.

2.Avoid drugs such as certain Antipyretics and Antibiotics(Nitrofurantoin,Nalidixic Acid,Sulfamethoxazole),Antimalarials(Primaquine)and Antispasmodic(Phenazopyridine).Consult a registered doctor and alert the medical staff before being prescribed any medicine.

3.Avoid eating fava or broad beans and their products.

4.Avoid close contact with mothballs(Naphthalene)and Naphthalene-containing products.

5.Mothers who are breastfeeding their G6PD Deficient babies should consult a doctor before taking any Chinese or Western medicine.

6.During haemolytic crisis,affected subjects will develop yellowish discoloration of the skin and mucosal membranes including the whites of the eyes and they may pass dark coloured urine.Neonates may look pale,seem to be lacking of energy and become excessively sleepy.Older children or adults may complain of tiredness,having rapid heartbeats,difficulty in breathing,and lethargy.If the above symptoms and signs are observed,medical parctitioners should consulted without delay.

7.Pregnant ladies should inform the medical and nursing staff of any family history of G6PD Deficiency.


We emphasize that if the above precautions are taken,affected subjects will enjoy normal growth and health.


Hotline:2361 9979

Web-site:http://www.info.gov.hk/dh/main_ser/CGS



Department of Health

Clinical Genetic Service

Genetic Screening Unit