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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