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Post by maripier on Nov 10, 2016 22:55:23 GMT -5
Hi everyone! My little girl Zoé 5years old have a diagnostic to fbpase deficiency at 18m (so sorry my english is bad!!!) IM live in Québec.Canada) 😂 but IM so happy to found this siteWeb I know is different to HFI ... but the diet is very similar..IM so lost and I not have à lot of support to my dietetist
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Post by colormist on Nov 11, 2016 8:47:47 GMT -5
Welcome to the group!!
We're here to help you if you have any questions. I think we might have a few members who are dealing with that exact same condition as well!
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Post by ukbill on Nov 14, 2016 14:26:38 GMT -5
WHat are the dietary restrictions and we will see if we can help  Keep smiling all will work out. |
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Post by maripier on Nov 16, 2016 14:29:43 GMT -5
Zoé Was transered to intensive carence at 3day at birth. Severe métabolic acidosis. We are able to leave hospital se Was à month without anyway diagnostic. But I had to feel her at each 2hours to avoir hypoglycemia and à follow up tighten in genetics.
At 4month Zoé always had high blood lactate,hepatomegalia and hypoglycemia at 3h of fasting. At this timer à Strong suspicion of à glycogenosis it is done à biopsy of thé liver. Thérèse Was indeed à accumulation of glycogen. So We started à lactose free diet and low sugar,but not as restrictive as hfi or fbase. A blood test Was send to à noter country in search à mutation for glycogenosis. The symptoms of hypoglycemia lactate and hepatomegalia had decreased à little but not enough... in 6 or 7month after We started thé diet ... final after 1 year positive fbpase deficiency.
Now se must feed every 4hours.. a glass of Milk and 30g of raw cornstarch. At night se hasard à continuous feed per gastrostomy. I know its à different diet from other countries are doing.. hard to find documents. Here in Québec they are most 50 personnes with à glycogenosis type 1a so they tend to follow the enteral feeding and cornstarch collation.
In terms of food. Permitted food are less 0.2g of fructose per 50g of food (ex: 1/2 white potato. Spinach raw 250ml or 50ml baked.. so juste 2 portion per day.) IM not sure if youre understand ?! It so difficult to me to explain in english 😂 (I would to thank Google translate .. even if sometime if looks doubtful)
I will come back later with more details to diet so thank you for your support and sorry for the translation I hope it make you laugh 😄
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Post by ukbill on Nov 17, 2016 17:27:22 GMT -5
Ahh no problem. Cornstarch milk shakes yes they are good for many conditions. Commonly used for Glycogen Storage disease mostly but good for supplying an 8 hour slow release boost feed for anyone with frequent Hypoglycemia events. If I have a heavy manual day planned I make a milk shake with yogurt and milk and 3 or 4 table spoons of Corn Starch.. do not drink a hot cup of tea or coffee straight away! or you will be feeling full for 3 or 4 days while the mass of cooked cornstarch dissolves slowly..  Your English is better than my French for certain.. I can order food and beer but little else in French .. 0.2g of Fructose per 50g food is easy enough to get to. Fish, meat, eggs, white rice, pasta, mushrooms and herbs give you quite a lot of options food wise. Has she any restrictions regarding fat or fibre intake? |
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