Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD Deficiency) – English

Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD Deficiency) – English

November 17, 2019 8 By Bertrand Dibbert


Hello everybody have you ever heard of an inherited condition called Glucose-6-Phosphate
Dehydrogenase Deficiency? It is also known as G6PD Deficiency in short or Favism When taking certain drugs or foods such as fava beans or in contact with mothballs people with G6PD Deficiency will develop acute haemolysis that is sudden damage of the red blood cells In view of this the Department of Health provides a newborn screening programme for G6PD Deficiency In order to find it out
early and prevent health problems caused by
damaged of the red blood cells The Genetic Screening Unit of the Clinical
Genetic Service under the Department of Health performs screening tests for babies born
in hospitals under the hospital authority by testing the umbilical cord blood For those babies born in private hospitals parents can consult their private hospitals Obstetricians or Paediatricians for further information Let us learn more about this condition by
watching how a nurse counsels a couple with a G6PD Deficient baby Hello this is nurse Tong calling from the Genetic Screening Unit of the Department of Health May I speak to Mrs. Au please? Speaking, what’s up? Mrs. Au, congratulations on your new baby Do you remember the hospital has
taken some umbilical cord blood from your baby for testing? Now we’ve got the results Your baby is confirmed to have
Glucose-6-Phosphate Dehydrogenase Deficiency a condition commonly called
G6PD Deficiency or Favism Oh, never what’s the problem with my baby? Is it serious? Oh please don’t get too worried Nurse Tong could you explain a bit more about it? Sure G6PD Deficiency is the most common inherited metabolic disease in Hong Kong It is also very common in Southern China People with this disease do not have enough amount of an enzyme called
G6PD in their red blood cells This enzyme helps to keep red blood cells stable Most affected people do not have any symptoms When they are exposed to some
Chinese or Western medicine moth balls or have serious infection It sounds serious What can I do for my baby? Try not to be too frightened In fact such a rapid breakdown of red blood cells can be prevented People with G6PD Deficiency
usually enjoy good health if they avoid exposing to certain triggers So, Nurse Tong, what should I do? Starting from today you have to observe for any jaundice that is yellowing of skin of your baby New born babies usually develops
some degree of jaundice on 3 to 5 days after birth This will gradually go away
without affecting the health of the baby On the other hand if a baby with G6PD Deficiency is exposed to certain triggers that caused
the breakdown of the red blood cells the jaundice may appear on the first
day after birth or at a later time Moreover the jaundice may persist or even get worse What will happen to babies if
jaundice persist and get worse? Just as I’ve mentioned breaking down of the red blood cells produce a lot of bilirubin This pigment can enter the
brain of the new born baby causing long term brain damage like hearing loss limp movement disorder and intellectual impairment etc However with early detection and treatment the jaundice can be controlled effectively So please do arrange an appointment with the Maternal and Child Health Centre as soon as possible to follow
up your baby’s jaundice So apart from jaundice are there other symptoms I have to take note of? The major symptoms include: yellowing of the white of the eyes and dark coloured urine Besides the baby may appear overly tired and sleepy Older children may look
pale and extremely tired They may also complain of difficulty in
breathing and heart beating rapidly If the above symptoms are observed you should consult the doctor immediately Just now you’ve mentioned that our
baby should avoid exposing to certain triggers So what are these triggers? It’s a very good question People with G6PD Deficiency
should remember to avoid the following things
for their whole life: Firstly avoid the following Chinese herbal medicines such as Rhizoma Coptidis Calculus Bovis Flos Chimonanthi Praecocis Flos Lonicerae and Margaritas Secondly Western medicine such as certain antibiotics and Antimalarials Aspirin group Antipyretics If used in high dose should be avoided as well To make it simple all medicines should be prescribed by doctors In order to avoid taking those
drugs which can trigger red blood cell damage Thirdly for food stuff don’t let your baby eat fava beans that is broad beans or their products such as fava pastry Lastly for household products avoid your baby in contact with moth balls or naphthalene containing products The above information is
listed on the G6PD Deficiency card also known as green card and the leaflet on G6PD
Deficiency produced by The Department of Health Please keep this handy green
card with you all the time Nurse Tong we haven’t got the green card yet How can I get it? Don’t worry if you haven’t received it from your hospital we will post it to you in a few days Finally remember to show this green card to your doctor and, inform him your baby
has G6PD Deficiency Do you have any other questions? Hmm, not now, thanks. Should you have any other questions you can call our hotline
2361 9979 or, ask the nursing staff or medical staff
in the Maternal and Child Health Centres Mummy I’ve followed nurse Tong’s advice and removed all the food and
products our baby should avoid Like that Flos Lonicerae and
Margaritas we’ve bought before The moth balls inside our
wardrobes have also been removed I’ve also reminded grandma grandpa and the maid not
to use moth balls anymore Oh how about that Bo Ying Tan, an insecticide? Should we dispose of them as well? Hmm let’s ask the nurse in the Maternal and Child Health Centre next time Good morning Mr. and Mrs. Au I’m Nurse Ho I’ve just checked your baby’s jaundice It’s not very high so you don’t need to worry Today, it’s your first visit to our centre. Do you have any questions about
taking care of your baby? Nurse Ho my baby has G6PD Deficiency can I continue breast feeding him? In general you can continue breast feeding your baby However if you need to take those medicines or Chinese herbal medicines stated on the green card you have to stop breast feeding temporarily to prevent passing those medicine
to the baby via the milk Meanwhile it’s better that you contine to express breast milk to maintain milk supply In that case you can continue breast feeding after stopping those medicines Nurse Tong of the Genetics Screening Unit told me that people with G6PD Deficiency should avoid certain Chinese herbal
medicines and Western drugs Can my baby take those Chinese folk
medicine like Bo Ying Tan and traditional meal supplement
for babies indigestion? Indeed Parents should not give
medicine to their babies without any doctor’s prescriptions As the Bo Ying Tan contains
Calculus Bovis and Margaritas we should not give this to
babies with G6PD Deficiency The ingredient of over the
counter herbal supplement for babies indigestion varies greatly It’s better to avoid giving this to babies If there really is a need you should seek advice from a qualified
Chinese medicine practitioner Besides the food mentioned
in the green card is there any other food
that my baby should avoid? Whenever you buy anything you should check ingredients for any
triggers stated on the green card For example certain brands of vermicelli and
mixed beans may contain fava beans If you are not sure check with the manufacturer or doctor directly We have to avoid using moth balls but how about camphor? According to the latest research findings natural camphor will not trigger breakdown of red blood cells in
people with G6PD Deficiency However the purity of camphor containing products in the market varies Therefore it is better not to use them Can we also use scented aroma fabric softener and insecticide? Exept those triggers
mentioned in the green card there is no current
evidence showing that aroma fabric softener food or products containing methol chamomile tissue and diapers insect repellents mosquito coil and medicated oil etc can damage the red blood cells
in people with G6PD Deficiency However you should check the ingredients before using them Should you have any doubts you’d better consult the
manufacturer or your doctor If a baby with G6PD Deficiency does not have any problems after eating
the food which should be avoided can he eat that food in the future? Even though a baby
with G6PD Deficiency has no adverse effect after taking
those food that may trigger haemolysis you shouldn’t let your baby eat them again The degree of damage to the red blood cells depends on other factors like the person’s health condition and the amount of triggers exposed to Therefore for safety’s sake the baby should avoid taking those
foods for their entire life When he goes to school is there any problem if his classmate’s
uniform has a moth ball odour? It’s common that the uniform of
classmates carries odour of moth balls However the dose of moth balls is much lower and it would not lead to haemolysis If your child smells the odour of moth ball he’d better get away from it More important is the affected person and their care giver should not use moth balls for their clothes Do you have any other questions? No that’s very clear already Thank you for your explanation Please remember to come to our workshop on G6PD Deficiency next month Good morning parents I am Nurse Ho Welcome to today’s workshop on
Glucose-6-Phosphate Dehydrogenase Deficiency a condition commonly called
G6PD Deficiency or Favism Today I’ll focus on the inheritance pattern of the condition and answer some of your questions Do you know that G6PD Deficiency is the most common metabolic
disease in Hong Kong? Around 4.5% of baby boys
and 0.5% of baby girls in Hong Kong have G6PD Deficiency Nurse Ho my wife and I do not have G6PD Deficiency why does our son have it? G6PD Deficiency is an X-linked
recessively inherited condition X-linked means that gene responsible for
this condition is on the X chromosome one of the sex chromosomes Being recessively inherited it means that not all generations
will have this disease Boys only have only
1 X chromosome When this carries the gene he will get G6PD Deficiency On the other hand girls have two X chromosomes Therefore if only one of her X chromosome carries the G6PD Deficiency gene and the other X chromosome is normal she will only be a carrier of
the gene, but with no disease. If both of her X chromosome carry this gene she will get G6PD Deficiency That is why most of the people
with G6PD Deficiency are males In your situation you do not have the G6PD Deficiency But your wife is likely a carrier of the gene So if we have a baby in the future will that baby inherit
G6PD Deficiency? If mother is the carrier
of G6PD Deficiency and father is unaffected for each pregnancy there is a 50% chance that a son will have G6PD Deficiency and 50% chance being unaffected If the baby is a daughter then there will be 50% chance that she will be a carrier of the deficient gene and the rest will not be affected My son has G6PD Deficiency if he gets married in the future will his children inherit
the same condition? For people with G6PD Deficiency their children may or may
not inherit the condition It depends on the genes
of their partners If the father is G6PD Deficient and mother is unaffected then for each pregnancy all sons will not be affected while all daughters will be
carriers of the deficient gene If the father is G6PD Deficient and mother is a carrier of the gene then for each pregnancy there is a 50% chance for a
son to be G6PD Deficient and 50% chance he will be unaffected On the other hand there is a 50% chance for a daughter to be G6PD Deficient and the rest will be a carrier What about my daughter? She has G6PD Deficiency Will her baby have the condition? If the mother is G6PD Deficient and father is unaffected then for every pregnancy all sons will be G6PD Deficient all daughters will be carrier In addition pregnant ladies should inform their doctor about her family
history of G6PD Deficiency for antenatal counselling Although there are no preventive measures for the condition during pregnancy early detection and with
the right precaution can protect babies from any harm
caused by acute haemolysis In this case does my baby need regular blood
testing and follow up consultation? Provided that people
with G6PD Deficiency can ensure lifelong avoidence
of the food stuffs and products mentioned
in G6PD green card it is not necessary to have regular blood
taking or follow up consultation I have one more question If my child grows up and his immunity becomes stronger can he recover from this disease? G6PD Deficiency is an inherited disease it is not the problem of the immune system Therefore the baby will not outgrow this with age or when his
immunity is stronger If babies with G6PD Deficiency
can avoid exposure to triggers they can lead a healthy and
active life as other children Therefore please always take with you the green card and inform medical staff about
the G6PD Deficient status of your baby during consultation Also let your child know that he has the condition and teach him to take
precautions to the food or products mentioned on the green card Do you have any other questions? No, thank you very much Mummy please don’t put the green card on the sofa Have you forgotten what
the nurse has said? We should always bring
the green card with us In addition we have to bring along this card during every consultation Oh, sorry Could you put the green card in my hand bag please? Okay Afrer watching this scenario you should understand more about G6PD Deficiency Let’s go over the main points 1. Children should avoid exposing
to triggers of haemolysis that is damage to red blood cells 2. Should there be any signs and
symptoms of acute haemolysis please consult doctor immediately 3. Always bring along with you
the G6PD Deficiency card 4. Inform medical staff that your baby has
G6PD Deficiency during consulatation 5. Let the child understand G6PD
Deficiency at the earliest possible age 6. Teach him to avoid food and
products which can trigger haemolysis although G6PD Deficiency will not be
recovered when the child grows up If the affected person can pay
attention to the above precautions he can also enjoy good
health like other children Finally if you wish to learn more information about G6PD Deficiency or to receive telephone counselling you can dial the following hotline 2361 9979 or visit the following websites www.cgs.dh.gov.hk or www.fhs.gov.hk