You are on page 1of 18

1|Page

Feeding and Caring for Babies with G6PD Deficiency or Favism Feeding your G6PD Deficient Baby By far the best milk for a baby with G6PD Deficiency is breast milk. The mother should avoid ALL contraindicated substances while breast feeding and also during pregnancy. See below. Unfortunately, not all mothers can breast feed, which can complicate matters for a G6PD Deficient baby. We have researched every baby formula we know about and ALL of them have contraindicated substances in them. Usually either soy or ascorbic acid or both. If you are unable to breast feed your baby we recommend fresh goat's milk as an alternative. You may supplement with vitamins, but unless specifically told to do so by a physician, we suggest moderation. More is not better. Natural (not man made) vitamin C is much better than ascorbic acid. Introduce solid foods as soon as your physician will allow it. The following website has some good information about goat's milk and also a recipe for home made formula. Goat's milk for babies If you are unable to get fresh goat's milk, canned goat's milk will work. If neither of them are available the next best would be fresh cow's milk and then evaporated (NOT CONDENSED) cow's milk. Evaporated milk should be diluted with water. Another possibility for milk for your baby is rice milk. One good thing about it is if you can't find it at the store, you can make your own. There are tons of rice milk recipes on the net. You will probably have to supplement with calcium and other vitamins though. Solid foods for G6PD Deficient Babies The best solid foods for your baby are those that you prepare yourself. If purchasing baby food from the store, be sure to check the ingredients carefully for soy products. Many times soy is disguised as lecithin or vegetable broth. To prepare baby food yourself, put food that you cook in a blender. It is a bit more trouble, but the food will be much better for your baby. Some Good First Foods For G6PD Deficient Babies First foods should be a single ingredient and can be given as soon as six months old. Ask your doctor for verification. Homemade foods are MUCH better and you have control over the ingredients. Salt and sugar should never be added to baby foods. Here's some examples. Rice cereal has been a staple first food for a long time. To make it, simply grind organic whole grain rice in a blender or food processor and thin with some breast milk or goat's milk. Avocados and bananas are also great first foods and they are simple to prepare and contain folate, which they need for blood production. Just remove the meat and mash with a fork. Add breast milk or goat's milk to thin or rice cereal to thicken.

2|Page

Pears, yams/sweet potatoes and winter squash are prepared by steaming or baking until tender and then processing in a blender or food processor. Add a bit of breast milk or goat's milk to thin or rice cereal to thicken. Be sure to cool to warm before serving. Apple can be made into applesauce by peeling, coring and cutting into chunks. Just cover with water and boil until soft. Be sure to stir often and do not use aluminum pans. Drain, reserving water, and puree. Add some of he reserved water to thin or rice cereal to thicken. Serve lightly warm. Canned applesauce can also be used if it contains only apples or apples and water and nothing else. Pregnant and Nursing Mothers and G6PD Deficiency It is very important that you avoid ALL contraindicated substances while pregnant with or nursing a potentially G6PD Deficient baby. Be sure to eat lots of antioxidants as well. What to look for at birth Many times G6PD Deficient babies develop jaundice (yellow skin) shortly after birth. This condition can cause brain damage (or kernicterus) if severe enough and not treated. The usual treatment is placing the baby under special lights. Jaundice can be caused by hemolysis as well as other conditions. G6PD Deficiency What is G6PD deficiency? G6PD deficiency is an inherited condition. If someone is G6PD deficient their body does not produce enough of the enzyme glucose-6-phosphate dehydrogenase (G6PD). G6PD helps red blood cells to function properly. G6PD also protects red blood cells from certain substances that are harmful to the body. If a person with G6PD deficiency is exposed to these substances, their red blood cells can be damaged or destroyed. G6PD deficiency can be serious if it is not diagnosed. Otherwise, with proper care and the right precautions, a child with G6PD deficiency can live a normal and active life. What causes G6PD deficiency? G6PD deficiency is passed on from parent to child. A child inherits it from one or both parents. It can neither be prevented or provoked. However, the symptoms of G6PD deficiency can be triggered by certain medicine, foods, and substances. Read more on symptoms and triggers below. How common is G6PD deficiency? G6PD deficiency is the most common known enzyme deficiency in humans. An estimated 400 million people around the world are affected.

3|Page

In the Philippines, around 1 in 50 children are G6PD deficient. G6PD deficiency is more common in boys than in girls. How is G6PD deficiency diagnosed? G6PD deficiency is tested through a simple blood pricking procedure. If you are pregnant or have just given birth, the Department of Health and your doctor will strongly advise you to participate in the Newborn Screening Program. It is a heelprick procedure that tests for five congenital (or inherited) conditions, including G6PD deficiency. Because it is quite common among Filipinos, your baby should be tested for G6PD deficiency as soon as possible. Newborn screening is available in many health institutions including hospitals, lying-in centers, rural health units and health centers. If your baby is delivered at home, you may bring him to the nearest health institution that offers newborn screening. Besides the Newborn Screening Program, you or your child can be tested in one of the G6PD Confirmatory Centers. You can also ask your doctor to refer you to a testing center near you. It may take several tests to completely confirm if your child is affected with G6PD deficiency. Can G6PD deficiency be cured? There is no known cure for G6PD deficiency. It is a lifelong condition that cannot be outgrown. However, a child with G6PD deficiency can live an active, healthy and normal life as long as he is able to avoid the substances that can trigger G6PD deficiency symptoms. What are the symptoms of G6PD deficiency? If your child is G6PD deficient, he will have no symptoms unless he is exposed to one of the harmful substances that can trigger the breakdown of red blood cells. Your child's symptoms will depend on what the harmful substance was and how much of it he was exposed to. In milder cases, your child may not even show any symptoms.

4|Page

In more serious cases, hemolysis (or hemolytic anemia) -- the accelerated destruction of red blood cells -- may happen. If so, he may have these symptoms: pale skin (among darker skinned children, check the lips and tongue for paleness) fatigue shortness of breath rapid heart rate jaundice (yellowing of skin and eyes) especially among newborns dark, tea-colored urine If your child is showing these symptoms, take him to the nearest hospital Emergency Room immediately. He may need hospitalization and medical care. When the trigger has been removed or treated, the symptoms usually resolve themselves within a few weeks. What are the triggers that cause G6PD deficiency symptoms? The foods and drugs that can trigger hemolysis are known as oxidative substances. These triggers may come in the form of: an illness, such as a bacterial or viral infection some painkillers and fever-reducing medication some antibiotics (particularly those with "sulf" in the generic name) some anti-malarial medicine (particularly those with "quine" in the generic name) some chemicals and substances like menthol and moth balls some food and drinks like flava beans, legumes and soya These triggers should not be eaten, ingested, touched or inhaled. The Newborn Screening Reference Center has provided a comprehensive list of food, medicine and substances to avoid.

How do I care for my G6PD deficient child? The best thing you can do to protect your child from triggers. Make sure he is not exposed to triggers for hemolysis in the food, drink and medicines he is given. Stay well-informed about the food and medicines which contain oxidative substances. Keep your list of symptom triggers (oxidative substances) in a convenient place. Make several copies: one to carry with you, one for a permanent place in your home, and several others to readily give out when necessary. Always double-check food, beverage, and medicine labels

5|Page

against the list. Make sure that your relatives, caregivers and health care providers know that they have to be careful about your child's condition. Brief everyone who will be caring, feeding, and treating your child about G6PD deficiency. Provide them with a list of oxidative substances to avoid. Tell them about the symptoms to watch out for. Teach them to be vigilant about avoiding exposing your child to possible symptom triggers. Take your child to his pediatrician for regular health-care maintenance checkups to keep him healthy and free from infection. She will monitor your child's levels of red blood cells. She may also prescribe diet supplements such as iron and folic acid. Do not ignore infections and persistent fever. If your child is sick, bring him at once to his pediatrician. Your pediatrician can give you more information about caring for your child. She may also refer you to a support group if one is operating in your area. In Manila, the Institute of Human Genetics (of the National Institute of Health) at the U.P.-Philippine General Hospital conducts regular activities designed to spread awareness about G6PD deficiency and other genetic illnesses. You may also get in touch with the National Institute of Health for your concerns. As your child grows up, talk to him about G6PD deficiency. Honestly explain what the condition is about as simply and gently as you can. Let him know that he can be just like his friends as long as he takes care to avoid the food, drink, medicine and substances that may make him sick.

http://www.babycenter.com.ph/a1043402/g6pd-deficiency#ixzz2LrINjLI4 G6PD Medicines to avoid Contraindicated Vs. Allergy G6PD Deficiency does not cause individuals to be allergic to the substances below as commonly believed. An allergy causes itchy eyes, runny nose, etc. whereas in a person with G6PD Deficiency these substances cause red blood cells to burst (hemolysis) and can lead to hemolytic anemia. G6PD Deficiency Drugs to Avoid and Other Contraindicated Substances Low Risk drugs, medicine, foods and other substances below MAY be safe at normal theraputic doses. People with Class 1 G6PD Deficiency (ie: Mediterranean variant among others) may still react to low risk substances. Consult with your physician before using any of these substances.

6|Page

We try our best to keep this list up to date, but the constant influx of new drugs, medicines and research makes this task quite difficult. To be safe the following classes of drugs, medicines and other substances should be avoided if at all possible. 1. 2. 3. 4. 5. 6. 7. 8. NSAIDS (Asprin, Ibuprophen) Tylenol Quinolones Drugs metabolized through the liver or known to cause blood or liver related problems or hemolysis Sulfa drugs Petrochemically derived substances (This is a long list and gets longer every year. Many artificial foods, dyes and vitamins are included in this list.) Moth Balls and anything containing naphthalene. Artificial Food Coloring (Methylene and Toluidine blue)

When in doubt, the safest course of action is to avoid the substance. If you react to something not on this list, avoid that substance in the future. Watch carefully for hemolysis signs at all times. To learn more about a substance (including information links, synonyms, notes, etc), click on the substance name. To just see a list of synonyms, click on the Synonym link.
Acetaminophen C8H9NO2 Analgesics / Antipyretics Low Risk - Synonyms Antazoline C17H19N3 Antihistamine Low Risk - Synonyms

N-(4-hydroxyphenyl)acetamide

N-benzyl-N-(4,5-dihydro-1H-imidazol-2-ylmethyl)aniline Acetanilid C8H9NO N-phenylacetamide Analgesics High Risk - Synonyms Antipyrine C11H12N2O Analgesics / Antipyretics Low Risk - Synonyms

2,3-DIMETHYL-1-PHENYL-3-PYRAZOLIN-5-ONE Acetylphenylhydrazine C8H10N2O Antimicrobial High Risk - Synonyms Ascorbic Acid C6H8O6 Vitamin Low Risk - Synonyms

1-Acetyl-2-phenylhydrazine, Acetylphenylhydrazine

(R)-3,4-dihydroxy-5-((S)- 1,2-dihydroxyethyl)furan-2(5H)-one Aminophenazone C13H17N3O Analgesics High Risk - Synonyms Aspirin C9H8O4 Anti Inflammatory High Risk - Synonyms

4-dimethylamino-1,5-dimethyl-2-phenyl-pyrazol-3-one

7|Page
2-acetyloxybenzoic acid Colchicine C22H25NO6 Astemizole C28H31FN4O Antihistamine Low Risk - Synonyms Anti Inflammatory Low Risk - Synonyms

(S)-N-(5,6,7,9-tetrahydro-1,2,3,10-tetramethoxy-9-oxobenzo[a ] heptalen-7-yl) acetamide

1-[(4-fluorophenyl)methyl]-N-[1-[2-(4-methoxyphenyl)ethyl]-4 piperidyl]-benzoimidazol-2-amine

Dapsone C12H12N2O2S

Sulfones High Risk - Synonyms

Beta-Naphthol C10H8O 2-Naphthol

Miscellaneous High Risk - Synonyms

4-(4-aminophenyl)sulfonylaniline

Dimercaprol C3H8OS2

Miscellaneous High Risk - Synonyms

Celebrex C17H14F3N3O2S

Sulfa Drug High Risk - Synonyms

2,3-Dimercaptopropanol

4-[5-(4-methylphenyl)- 3-(trifluoromethyl)-1H-pyrazol-1-yl] benzenesulfonamide

Diphenhydramine C17H21NO

Antihistamine Low Risk - Synonyms

2-benzhydryloxy-N,N-dimethyl-ethanamine Chloramphenicol C11H12C12N2O5 Antibacterial High Risk - Synonyms Dopamine 2,2-dichloro-N-[1,3-dihydroxy-1-(4-nitrophenyl)-propan-2-yl] acetamide C8H11NO2 Anti-epileptic Low Risk - Synonyms

4-(2-aminoethyl)benzene-1,2-diol Chloroquine C18H26ClN3 Antimalarials High Risk - Synonyms Doxorubicin C27H29NO11 Chemotherapeutic High Risk - Synonyms

N'-(7-chloroquinolin-4-yl)-N,N-diethyl-pentane-1,4-diamine

Ciprofloxacin C17H18FN3O3

Quinolones High Risk - Synonyms

10-(4-amino-5-hydroxy-6-methyl-oxan-2-yl)oxy-6,8,11-t rihydroxy-8-(2-hydroxyacetyl)-1-methoxy-9,10-dihydro-7H-tetr acene-5,12-dione

1-cyclopropyl-6-fluoro-4-oxo-7-piperazin-1-yl-quinoline-3-ca rboxylic acid

Ethanol C2H6O

Disinfectants High Risk - Synonyms

8|Page
Ethanol C13H18O2 Low Risk - Synonyms

2-[4-(2-methylpropyl)phenyl]propanoic acid Furazolidone C8H7N3O5 Antibiotics High Risk - Synonyms Isobutyl Nitrite C4H9NO2 Miscellaneous High Risk - Synonyms

3-[(5-nitro-2-furyl)methylideneamino]oxazolidin-2-one

Nitrous acid, 2-methylpropyl ester Furosemide C12H11ClN2O5S Diuretic High Risk - Synonyms Isoniazid C6H7N3O pyridine-4-carbohydrazide Gadopentetate dimeglumine C28H54GdN5O20 Contrast Agent High Risk - Synonyms Lamotrigine C9H7Cl2N5 Antidepressants High Risk - Synonyms Antibiotics Low Risk - Synonyms

4-chloro-2-(furan-2-ylmethylamino)-5-sulfamoylbenzoic acid

2-[bis[2-[carboxymethyl-(2-oxido-2-oxoethyl)amino]ethyl]amin o]acetate; gadolinium(+3) cation; (2R,3R,4R,5S)-6methylaminohexane-1,2,3,4,5-pentol

6-(2,3-dichlorophenyl)-1,2,4-triazine-3,5-diamine

Glucosulfone C24H34N2Na2O18S3

Miscellaneous High Risk - Synonyms

Levofloxacin C36H42F2N6O9

Anti-bacterial High Risk - Synonyms

Glyburide C32H28ClN3O5S

Antidiabetic Agent High Risk - Synonyms

(-)-(S)-9-fluoro-2,3-dihydro-3-methyl-10-(4-methyl-1-piperaz inyl)-7-oxo-7Hpyrido[1,2,3-de]-1,4 benzoxazine-6-carboxylic acid hemihydrate

5-chloro-N-[2-[4-(cyclohexylcarbamoylsulfamoyl)phenyl]ethyl] 2-methoxy-benzamide

Lisinopril C21H31N3O5

Antihypertensive Agents Low Risk - Synonyms

Henna C10H6O3

Miscellaneous High Risk - Synonyms

(2S)-1-[(2S)-6-amino-2-{[(1S)-1-carboxy-3-phenylpropyl]amino }hexanoyl]pyrrolidine-2-carboxylic acid

4-hydroxynaphthalene-1,2-dione

Magnevist C28H54GdN5O20

Contrast Agent Low Risk - Synonyms

Ibuprofen

Analgesics / Antipyretics

2-[bis[2-[carboxymethyl-(2-oxido-2-oxoethyl)amino]ethyl]amin o]acetate; gadolinium(+3) cation; (2R,3R,4R,5S)-6-

9|Page
methylaminohexane-1,2,3,4,5-pentol 1-carbamimidamido-N,N-dimethylmethanimidamide

Mefloquine C17H16F6N2O

Anitmalarials High Risk - Synonyms

Methylene Blue C16H18ClN3S

Miscellaneous High Risk - Synonyms

[2,8-bis(trifluoromethyl)quinolin-4-yl]-piperidin-2-ylmethan ol

3,7-Bis(dimethylamino)phenazathionium chloride

Menadiol Sodium Sulfate (Vitamin k4 sodium sulfate) C11H8Na2O8S2

Miscellaneous

Mirtazapine C17H19N3

Antidepressant Low Risk - Synonyms

High Risk - Synonyms 1,2,3,4,10,14b-hexahydro-2-methylpyrazino[2,1-a]pyrido[2,3-c ][2]benzazepine

Menadione C11H8O2

Miscellaneous High Risk - Synonyms Moxifloxacin C21H24FN3O4 Anti-bacterial High Risk - Synonyms

2-methylnaphthalene-1,4-dione

1-cyclopropyl-7-[(1S,6S)-2,8-diazabicyclo[4.3.0]non-8-yl]-6fluoro-8-methoxy-4-oxo-quinoline-3-carboxylic acid Menadione sodium Bisulfite Miscellaneous (Vitamin K3 sodium bisulfite) Nalidixic Acid C11H8O2NaHSO3 High Risk - Synonyms C12H12N2O3 High Risk - Synonyms Antibacterial

1-ethyl-7-methyl-4-oxo-[1,8]naphthyridine-3-carboxylic acid Menthol C10H20O Miscellaneous High Risk - Synonyms

Naphthalene C10H8 Naphthalene

Miscellaneous High Risk - Synonyms

2-(2-Propyl)-5-methylcyclohexanol

Mesalazine C7H7NO3

Anti Inflammatory High Risk - Synonyms

Nimesulide C13H12N2O5S

Anti-Inflammatory Agents High Risk - Synonyms

5-amino-2-hydroxy-benzoic acid

N-[4-nitro-2-(phenoxy)phenyl]methanesulfonamide Metformin C4H11N5 Antidiabetic High Risk - Synonyms

Niridazole

Miscellaneous

10 | P a g e
C6H6N4O3S High Risk - Synonyms Pefloxacin C17H20FN3O3 Anti-Infective High Risk - Synonyms

1-(5-Nitro-2-thiazolyl)-2-imidazolinone

1-ethyl-6-fluoro-7-(4-methylpiperazin-1-yl)-4-oxo-quinoline- 3carboxylic acid Nitrofurantoin C8H6N4O5 Cytotoxic / Antibacterial High Risk - Synonyms Pentaquine 1-[(5-nitro-2-furyl)methylideneamino]imidazolidine-2,4-dione C18H27N3O High Risk - Synonyms Antimalarials

Nitrofurazone C6H6N4O4

Cytotoxic / Antibacterial High Risk - Synonyms

1,5-Pentanediamine, N-(6-methoxy-8-quinolinyl)-N'-(1methylethyl)-

1-amino-N-[(5-nitro-2-furyl)methylideneamino]methanamide

Phenacetin C10H13NO2

Analgesics High Risk - Synonyms

Norfloxacin C16H18FN3O3

Antibiotics Low Risk - Synonyms

N-(4-ethoxyphenyl)ethanamide

1-ethyl-6-fluoro-4-oxo-7-piperazin-1-yl-1H-quinoline-3-carbo xylic acid

Phenazopyridine C11H11N5

Miscellaneous High Risk - Synonyms

3-phenyldiazenylpyridine-2,6-diamine Oxidase, Urate C1521H2381N417O461S7 Aspergilus urate oxidase C19H20N2O2 Low Risk - Synonyms Miscellaneous High Risk - Synonyms Phenylbutazone Anti-inflammatory

4-butyl-1,2-diphenyl-pyrazolidine-3,5-dione Pamaquine C42H45N3O7 Antimalarials High Risk - Synonyms Phenylhydrazine N,N-diethyl-N'-(6-methoxyquinolin-8-yl)pentane-1,4-diamine C6H8N2 Phenylhydrazine Para-Aminobenzoic Acid C7H7NO2 4-Aminobenzoic Acid C15H12N2O2 Low Risk - Synonyms Miscellaneous Low Risk - Synonyms Phenytoin Anticonvulsants High Risk - Synonyms Miscellaneous

11 | P a g e
5,5-diphenylimidazolidine-2,4-dione C20H24N2O2 Low Risk - Synonyms

(2-ethenyl-4-azabicyclo[2.2.2]oct-5-yl)-(6-methoxyquinolin-4 -yl)methanol Primaquine C15H21N3O Antimalarials High Risk - Synonyms Quinine N-(6-methoxyquinolin-8-yl)pentane-1,4-diamine C20H24N2O2 Low Risk - Synonyms Antimalarials

Probenecid C13H19NO4S

Analgesics / Antipyretics High Risk - Synonyms

(2-ethenyl-4-azabicyclo[2.2.2]oct-5-yl)-(6-methoxyquinolin-4 -yl)methanol

4-[(dipropylamino)sulfonyl] benzoic acid

Stibophen C12H4Na5O16S4Sb

Miscellaneous High Risk - Synonyms

Procainamide C13H21N3O

Cardiovascular Drugs Low Risk - Synonyms

2-(2-Oxido-3,5-Disulphonatophenoxy)-1,3,2,Benzodioxastibole4-6-Disulphonate

4-amino-N-(2-diethylaminoethyl)benzamide Streptomycin C21H39N7O12 Proguanil C11H16ClN5 Antimalarial Low Risk - Synonyms 5-(2,4-diguanidino-3,5,6-trihydroxy-cyclohexoxy)-4-[4,5-dihy droxy-6-(hydroxymethyl)-3-methylamino-tetrahydropyran-2-yl]o xy-3-hydroxy-2-methyl-tetrahydrofuran-3-carbaldehyde Anti-bacterial Low Risk - Synonyms

1-(4-chlorophenyl)-2-(N'-propan-2-ylcarbamimidoyl)guanidine

Sulfacetamide Pyrimethamine C12H13ClN4 Antimalarials Low Risk - Synonyms C8H10N2O3S

Sulfonamides / Sulfones High Risk - Synonyms

N-acetyl-4-amino-benzenesulfonamide

5-(4-chlorophenyl)-6-ethyl-pyrimidine-2,4-diamine

Sulfacytine Quinacrine C23H3OClN3 O Antimalarials C12H14N4O3S High Risk - Synonyms

Sulfonamides / Sulfones Low Risk - Synonyms

N'-(6-chloro-2-methoxy-acridin-9-yl)-N,N-diethyl-pentane-1,4 diamine

4-amino-N-(6-methoxy-2-methylpyrimidin-4-yl)benzenesulfonami de

Sulfadiazine Quinidine Antiarrhythmic Agents / Antimalarials C10H10N4O2S

Sulfonamides / Sulfones Low Risk - Synonyms

12 | P a g e
4-amino-N-pyrimidin-2-yl-benzenesulfonamide C6H8N2O2S 4-aminobenzenesulfonamide Sulfadimidine C12H14N4O2S Sulfonamides / Sulfones High Risk - Synonyms Sulfapyridine C11H11N3O2S Sulfonamides / Sulfones High Risk - Synonyms High Risk - Synonyms

4-amino-N-(4,6-dimethylpyrimidin-2-yl)benzenesulfonamide

4-amino-N-pyridin-2-ylbenzenesulfonamide Sulfafurazole C11H13N3O3S Sulfonamides / Sulfones High Risk - Synonyms Sulfasalazine C18H14N4O5S Sulfonamides / Sulfones High Risk - Synonyms

4-amino-N-(3,4-dimethyl-1,2-oxazol-5-yl)benzenesulfonamide

2-hydroxy-5-[4-(pyridin-2-ylsulfamoyl)phenyl]diazenyl-benzoi c acid Sulfaguanidine C7H10N4O2S Miscellaneous Low Risk - Synonyms Sulfathiazole 4-Amino-N-(diaminomethylene)benzenesulfon Amide C9H9N3O2S2 High Risk - Synonyms Cytotoxic / Antibacterial

4-amino-N-(1,3-thiazol-2-yl)benzenesulfonamide Sulfamerazine C11H12N4O2S Sulfonamides / Sulfones Low Risk - Synonyms Sulfonylurea 4-amino-N-(4-methylpyrimidin-2-yl)benzenesulfonamide C21H27N5O4S Low Risk - Synonyms Antidiabetic Agents

Sulfamethoxazole C10H11N3O3S

Sulfonamides / Sulfones High Risk - Synonyms

1-cyclohexyl-3-[[p-[2-(5-methylpyrazinecarboxamido)ethyl]phe nyl] sulfonyl]urea

4-amino-N-(5-methyloxazol-3-yl)-benzenesulfonamide

Sulfoxone C14H16N2O6S3

Antibacterial High Risk - Synonyms

Sulfamethoxypyridazine C11H12N4O3S

Sulfonamides / Sulfones Low Risk - Synonyms

[4-[4-(sulfinomethylamino)phenyl]sulfonylphenyl]aminomethane sulfinic acid

4-Amino-N-(6-methoxy-3-pyridazinyl)-benzenesulfonamide Tamsulosin C20H28N2O5S Sulfanilamide Chemotherapeutic 5-[2-[2-(2-ethoxyphenoxy)ethylamino]propyl]-2-methoxy-benzen esulfonamide Sulfonamides High Risk - Synonyms

13 | P a g e
C14H18N4O3 Tiaprofenic Acid C14H12O3S Anti Inflammatory Low Risk - Synonyms Trinitrotoluene C7H5N3O6 Toluidine Blue C15H16N3SCl Miscellaneous High Risk - Synonyms Tripelennamine C16H21N3 Antihistaminic Low Risk - Synonyms Miscellaneous High Risk - Synonyms Low Risk - Synonyms

5-[(3,4,5-trimethoxyphenyl)methyl]pyrimidine-2,4-diamine

2-[5-(benzoyl)thiophen-2-yl]propanoic acid

2-methyl-1,3,5-trinitrobenzene

3-Amino-7-(dimethylamino)-2-methylphenothiazin-5-ium Chloride

N-benzyl-N',N'-dimethyl-N-pyridin-2-yl-ethane-1,2-diamine Trihexyphenidyl C20H31NO Antiparkinson Agents Low Risk - Synonyms Vitamin K1 1-cyclohexyl-1-phenyl-3-(1-piperidyl)propan-1-ol C31H46O2 Phytomenadione Trimethoprim Antimalarials / Antibacterial Low Risk - Synonyms Miscellaneous

G6PD Deficiency Food To Avoid

Some of the foods commonly eaten around the world can cause people with G6PD Deficiency tohemolyze. Some of these foods can be deadly (like fava beans). Some others can cause low level hemolysis, which means that red blood cells die, but not enough to cause the person to go to the hospital. Low level hemolysis over time can cause other problems, such as memory dysfunction, overworked spleen, liver, kidney and heart, and iron overload. Even though a G6PD Deficient person may not have a crises when consuming these foods, they should be avoided.

Fava Beans and other legumes This list contains every legume we could find, but there may be other names for them that we do not know about. Low level hemolysis is very hard to detect and can cause other problems, so we recommend the avoidance of all legumes.

Sulfites

14 | P a g e

and foods containing them. Sulfites are used in a wide variety of foods, so be sure to check labels carefully.

Menthol and foods containing it. This can be difficult to avoid as tooth paste, candy, breath mints, mouth wash and many other products have menthol added to them. Mint from natural mint oils is alright to consume.

Artificial blue food coloring other artificial food color can also cause hemolysis. Natural food color such as found in foods like turmeric or grapes is okay.

Ascorbic acid Artificial ascorbic acid commonly put in food and vitamins can cause hemolysis in Large doses and should be avoided. It is put into so many foods that you can be getting a lot ofAscorbic Acid without realizing it. See Ascorbic Acid for more information about iron absorbtion and ascorbic acid.

Vitamin K This is from drugbank: "Menadione (vitamin K3), which is not used as a nutritionalsupplemental form of vitamin K for humans, has been reported to cause adverse reactions, including hemolytic anemia. Large doses have also been reported to cause brain damage. Vitamin K administered to newborns with G6PD Deficiency has been known to cause adverse outcomes including hemolytic anemia, neonatal brain or liver damage, or neonatal death in some cases."

Tonic water

(contains quinine, a contraindicated drug which causes hemolysis in G6PDD people).

Bitter Gourd and Garden Egg Bitter Gourd is also known as Bitter Mellon. These are common foods in some parts of Africa and Asia.

Some Chinese herbs

15 | P a g e

particularly Rhizoma Coptidis (huang lien), Calculus Bovis (neu huang), Flos Chimonanthi Praecocis (leh mei hua), Flos Lonicerae (kam ngan fa) and Margarita oranything containing them. Glucose-6-phosphate dehydrogenase deficiency is an X-linked recessive hereditary disease characterised by abnormally low levels of glucose-6-phosphate dehydrogenase (abbreviated G6PD or G6PDH), a metabolic enzyme involved in the pentose phosphate pathway, especially important in red blood cellmetabolism. Individuals with the disease may exhibit nonimmune hemolytic anemia in response to a number of causes, most commonly infection or exposure to certain medications or chemicals. G6PD deficiency is closely linked to favism, a disorder characterized by a hemolytic reaction to consumption of broad beans, with a name derived from the Italian name of the broad bean (fava). The name favism is sometimes used to refer to the enzyme deficiency as a whole, although this is misleading as not all people with G6PD deficiency will react to consumption of broad beans. G6PD deficiency is the most common human enzyme defect. The most common medical problem associated with glucose-6-phosphate dehydrogenase deficiency is hemolytic anemia, which occurs when red blood cells are destroyed faster than the body can replace them. This type of anemia leads to paleness, yellowing of the skin and whites of the eyes (jaundice), dark urine, fatigue, shortness of breath, and a rapid heart rate. In people with glucose-6-dehydrogenase deficiency, hemolytic anemia is most often triggered by bacterial or viral infections or by certain drugs (such as some antibiotics and medications used to treat malaria). Hemolytic anemia can also occur after eating fava beans or inhaling pollen from fava plants (a reaction called favism). Favism may be formally defined as a haemolytic response to the consumption of broad beans. All individuals with favism show G6PD deficiency. However, not all individuals with G6PD deficiency show favism. For example, in a small study of 757 Saudi men, more than 42% showed G6PD deficiency, but none reported symptoms of favism, despite fava in the diet.[2] Favism is known to be more prevalent in infants and children, and G6PD genetic variant can influence chemical sensitivity. Other than this, the specifics of the chemical relationship between favism and G6PD are not well understood. Symptoms Dark urine Enlarged spleen Paleness Rapid heart rate Shortness of breath Yellow skin color (jaundice) Note: Severe hemolysis may cause hemoglobinuria(hemoglobin in the urine).

16 | P a g e

Diagnosis The diagnosis is generally suspected when patients from certain ethnic groups (see below) develop anemia,jaundice and symptoms of hemolysis after challenge to any of the above causes, especially when there is a positive family history. Generally, tests will include: Complete blood count and reticulocyte count; in active G6PD, Heinz bodies can be seen in red blood cells on ablood film; Liver enzymes (to exclude other causes of jaundice); Lactate dehydrogenase (elevated in hemolysis and a marker of hemolytic severity) Haptoglobin (decreased in hemolysis); A "direct antiglobulin test" (Coombs' test) - this should be negative, as hemolysis in G6PD is not immune-mediated; Treatment The most important measure is prevention - avoidance of the drugs and foods that cause hemolysis. Vaccinationagainst some common pathogens (e.g. hepatitis A andhepatitis B) may prevent infection-induced attacks.[11] In the acute phase of hemolysis, blood transfusionsmight be necessary, or even dialysis in acute renal failure. Blood transfusion is an important symptomatic measure, as the transfused red cells are generally not G6PD deficient. Some patients benefit from removal of the spleen(splenectomy),[12] as this is an important site of red cell destruction. Folic acid should be used in any disorder featuring a high red cell turnover. Although vitamin E andselenium have antioxidant properties, their use does not decrease the severity of G6PD. Alternative treatment Vitamin E and folic acid (both anti-oxidants) may help decrease hemolysis in G6PD-deficient individuals.

) If the father is unaffected (healthy) and the mother is a carrier (no clinical symptoms): One daughter out of two will be a carrier One son out of two will be G6PD deficient B) If the father is G6PD deficient and the mother is unaffected: All sons will be unaffected C) If the father is G6PD deficient and the mother is a carrier:

17 | P a g e

One daughter out of two will be G6PD deficient

D) If the father is unaffected and the mother is G6PD deficient: All sons will be G6PD deficient E) If both father and mother are G6PD deficient: All daughters will be G6PD deficient

TIPS FOR PARENTS Any boy suffering from Favism should be informed of his deficiency so he can help himself avoid the intake of the forbidden foodstuff in various situations where he is not under your supervision (such as school). You should also inform school supervisors and, where applicable, also the school caterers who provide school lunch. Best is to carry with you the To Avoid list and show it to all those who may treat your boy. How do you care for babies with G6PD deficiency? Parents should take proper precautions when caring for a child with G6PD deficiency. The main thing is keep your child from being exposed to triggers of G6PD deficiency symptoms. Newborns with G6PD deficiency may suffer from prolonged and more severe levels of jaundice. Often, if the levels are too high, mothers are advised to stop breast feeding their infants for a short period of time by paediatricians as breast milk might interfere with the livers ability to conjugate bilirubin. After being discharged from hospitals, breast-feeding mothers should avoid consuming certain types of food and medications which might induce anaemic hemolysisin G6PD deficient babies. The list of food and drugs to avoid is fairly similar to the ones a G6PD deficient person should avoid. Analgesics, anti-malarials, sulphonamide drugs should be strictly avoided. the number of drugs that should be avoided is fairly comprehensive. To be on the safe side, make sure every medication for your child is acquired via doctors prescription. Both breast-feeding mother and child must avoid fava beans (broad beans). Fava bean remains the most common food to avoid for G6PD deficient individuals. Keep your child away from mothballs. Do not let them touch or get close enough to inhale the scent of mothballs. If your newborn inherits some hand-me-down clothes, make sure to wash the clothes clean before your baby wears them. Youd never know the clothes may have been stored in close vicinity to mothballs. Choose milk formulaes which have no traces of soya or soybeans.

18 | P a g e

Observe your childs diet by carefully introducing legumes such as peas, beans, lentil or various types of beans. Some people with G6PD deficiency avoid legumes, but not all. Most people shows no symptoms. Soy products, blueberries and red wine are also trigger symptoms of G6PD deficiency but again, does not necessary affect all people. Peppermint oil should not be used around your childs face. In fact, breastfeeding mothers themselves should refrain from using peppermint oil too.