SHEFFIELD CHILDREN'S NHS FOUNDATION TRUST G6PD DEFICIENCY

					 SHEFFIELD CHILDREN’S NHS FOUNDATION TRUST                               drugs used to prevent and treat malaria are to be strictly
               G6PD DEFICIENCY                                           avoided as are the drugs listed in the “You should avoid the
                                                                         following” section.
 Name:
                                                                         Some people have a severe form of the condition with
                                                                         breakdown of red cells even without exposure to the above
 DOB:
                                                                         triggers. These patients are said to have Chronic Non –
 Blood Group:                                                            Spherocytic Haemolytic Anaemia. They sometimes require
                                                                         blood transfusions.       Whatever the severity of G6PD
 Consultant:                                                             deficiency, all patients are at risk of crises when their
                                                                         Haemoglobin drops to dangerously low levels due to a viral
 Results (steady state):                                                 infection that stops the bone marrow from producing red cells
 Hb           (g/dI)                                                     (aplastic crisis). Aplastic crisis is dangerous and you should
 Retic Count (x109/I)                                                    contact us immediately in the event of sudden tiredness with
                                                                         a pale appearance. Most crisis will require transfusion of
What is G6PD?                                                            blood to correct the severe anaemia.
G6PD is short for glucose 6 Phosphate Deyhydrogenase. It is an
enzyme, which is found throughout the body. Some people have             Problems for the unborn child
an inherited deficiency of G6PD, which results in low levels of the      G6PD deficiency does not cause problems for the unborn
enzyme in the red blood cells, and this may cause occasional             child.
problems for them. Certain drugs, viruses and food substances            Jaundice in the new-born baby
cause the problem which breakdown G6PD deficient red cells and           Mild jaundice (increase in a blood pigment called bilirubin) is
cause anaemia.                                                           common at birth due to immaturity of the liver. G6PD
How is it inherited?                                                     deficiency increases the risk of severe jaundice in the first few
Both males and females inherit G6PD deficiency but males are             days after birth and this can result in damage to the brain if
more severely affected. Individuals at greatest risk of inheriting it    not detected and treated in time.          When you become
are those who’s family originate from the Mediterranean, Asia,           pregnant, alert the midwife and doctor in charge of the
Africa, Caribbean and South East Asia. For example G6PD                  antenatal care to this risk as they may wish to make special
deficiency is found in 1 in 12 Cypriots, 1 in 5 Africans, 1 in 10 Afro   arrangements to monitor the newborn.
Caribbeans, 1 in 5 Indians, 1 in 30 Chinese and 1 in 5 Thais.            When should you seek medical advice?
There are many different kinds of G6PD deficiency, but the kinds         If you become unwell with pale and yellow looking skin,
found in people from the Mediterranean and South East Asia are           backache and dark urine you should either contact us directly
usually more severe than those in other groups.                          0114 271 7000 (ask for Haematology) or go to your GP or
When can G6PD cause anaemia?                                             nearest casualty and present this leaflet.
Individuals with G6PD deficiency are usually completely healthy          You should avoid the following: - Maloprim, Primaquine,
until exposed to certain drugs, foods and infections. These are          Pentaquine, Pamaquine, Nalidixic acid, Nitrofurantoin,
listed in the “You should avoid the following” section and exposure      Sulphonamides, including Septrin/Bactrim, Dapsone. Avoid
to them results in a breakdown of red blood cells and anaemia (low       close contact with, Moth balls (Napthalene). Avoid eating
concentration of haemoglobin). Fava beans (broad beans) and              Fava Beans (Broad Beans), Chinese herbal medicines.
Information Leaflet number:                       71 (as/06/12/06)
Author/Contact:                                 Jenny Welch
                                                                     G6PD DEFICIENCY




For Further advice contact:
        Sheffield Children’s NHS Foundation Trust,
             Main hospital number: 0114 271 7000


Patient Advice and Liaison Team
Sheffield Children’s NHS Foundation Trust
Western Bank
Sheffield
S10 2TH

Tel: 0114 271 7594
Trust web site address: http://www.sch.nhs.uk