This is our family’s journey on finding out my children (and husband) have an inherited metabolic disorder called Fructose 1, 6-bisphosphatase deficiency.

My husband and I have been blessed with two wonderful sons, ages 8 and 6, after 10 yrs of infertility. Since the moment each of our sons were born it seemed as though they were STARVING! As babies and until they were around 2-2.5 yrs old they never slept through the night without needing to eat. I did not know then that there was a reason for this until my oldest was around 2 yrs old, and the first low blood sugars started.

Both of our boys always had exceptional checkups but, according to the doctors, had sleeping problems (rolling my eyes). I was ok with my boys waking up to eat at night, if their bodies needed it then I would provide it for them. Around the age of 2 my oldest finally no longer needed a bottle at night. Not long after, he woke up in the morning with his first low blood sugar episode. He woke up that morning shaking and was unable to walk on his own. With my education and experience as a medical assistant I knew right away it was low blood sugar. He pounded down two pieces of bread and a glass of milk (and probably more food but I can’t remember). Within 20 minutes it was like nothing had happened and he had all the energy in the world. Talk about freaking Momma out! I became concerned about diabetes and took him to the doctor. He was tested for diabetes and anything else possibly concerning that could have caused the low blood sugar. Everything came back normal. My husband would also get low blood sugar from time to time and thought maybe it was genetic. We decided to make sure our son was getting better protein in his diet every day.

Also around the same age, our youngest stopped needing to eat at night. Lo and behold, he had his first low blood sugar episode. I remember feeling super overwhelmed and anxious for the wellbeing and health of my children due to these low blood sugar episodes. I had been earnestly praying to God for answers and for him to guide me to the knowledge of what was going on with my children, and causing the low blood sugars.

After one particular low blood sugar episode, where both of my boys had low blood sugar at the same time one morning, my husband told me that the boys had peas in their dinner, and that every time he had peas it would give him low blood sugar. Peas are a low glycemic food, so for peas to cause low blood sugar was very odd to me, but I believed it was significant. I searched on the internet for reasons that peas could cause low blood sugar. To my amazement, there were only 2 possible causes. Hereditary fructose intolerance, and Fructose 1, 6-bisphosphatase dificiency. I was filled with peace. Hallelujah! and praise God! I knew I had found the answer.

I researched both conditions and found that the only one that made sense was Fructose 1, 6-bisphosphatase deficiency. It is an inherited metabolic disorder in which the body cannot properly make glucose. The liver is supposed to convert fructose into glucose. With this disorder the liver struggles to do so. Glucose is the main type of sugar in the blood and a primary source of energy for the body’s cells. If not properly treated someone with the disorder can go into ketotic hypoglycemia and even coma. When my children would have high fructose foods, such as peas, it would cause them to get low blood sugar. Other high fructose foods that my children are particularly prone to low blood sugars with are applesauce, mangos, dried fruits, fruit juices, fruit snacks, honey, agave, molasses, high fructose corn syrup and of course SUGAR. The next time I took my boys to the doctor I told him what I had researched and what I was doing for my children to manage the condition (which I will go over in this post). He told me there were no tests to do and that I had figured it out on my own and was doing everything that would already be recommended.

The reason both of my children couldn’t sleep through the night without needing to eat until they were around 2 yrs old was because of their metabolic disorder! If I had tried to get them to sleep through the night as babies, when their need for food and glucose was so great, their lives would have been in danger.

Over the years I’ve found how to know when my children are starting to get low blood sugar, and what I can do daily to prevent it!

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How to know your child is getting low blood sugar

  1. Keto breath: With this disorder it is important to be eating complex carbs because they slowly digest and help you to have a steady flow of glucose. Not having enough complex carbs leads to the depletion of glycogen levels. (Glycogen is made up of glucose molecules coming from carbs). If your child is low on carbs their breath will smell fruity or like nail polish remover. (My children’s breath will smell like nail polish remover). Whenever my children are not feeling well I will have them blow in my face so I can smell their breath to know if they’re getting low blood sugar. I will also pay particular attention to their breath when they wake up in the mornings, or if they wake up in the middle of the night for any reason.
  2. Weakness: When my children are getting low blood sugar their bodies will feel weak. The few times when it got particularly bad it was hard for them to even walk.
  3. Nausea: The few times my children have had really low blood sugar they also felt like they were going to throw up, and even have once. It is easy to mistake this for food poisoning or the stomach flu. But with this disorder, even if it is food poisoning or the stomach flu, it is absolutely essential that they EAT! Most of the time when I wondered if they were sick, all I had to do was give them a spoon full of sugar, a low sugar yogurt for protein or half a protein shake, and 1-2 pieces of bread and 20 minutes later, it was like nothing had happened! They were all better.
  4. Shaking: When your blood sugar starts getting significantly low it can cause your body to shake. First with your hands and then even your whole body as it gets worse. I experienced this with my children during their worst episodes. Also, when my husband gets low blood sugar his hands will start to shake and he knows he has to eat something asap.
  5. Dizziness: Along with the weakness can come dizziness, and when really bad, fainting. Only one of my children has ever fainted from low blood sugar and it only lasted a few seconds. It was first thing in the morning when low blood sugars from this disorder are most common. We got him the food he needed and he was better in 20-30 minutes.

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How to feed your child so that low blood sugars are a rarity and they can still enjoy some treats

PROTEIN!!!! Protein Protein Protein! It is VERY important to make sure you are getting enough protein every day. Your body can break down protein into glucose and is the reason protein makes you feel better when you have low blood sugar. If I make sure my children are getting enough protein in their diet every day according to their body weight (too much is not good for you) I can keep their blood sugars good. If my children want a treat, I make sure the treat isn’t over indulgent. I’ll allow no more than two cookies, or a small ice cream cone, or half a candy bar. After my children have a treat I make sure they top it off with protein to ensure their blood sugars stay good. My oldest son will not eat meat. If I try to get him to eat some he will literally vomit. My youngest is not overly fond of meat but will eat some meat, and sadly he also is allergic to cashews, pistachios, hazelnuts and eggs (not an intolerance). So much so that I can’t even cook with eggs. So no eggs for protein in our family. Half of the protein they need they get during the day through their food, and half through protein shakes right before bed. Having 1/3 to 1/2 of a protein shake before bed keeps my children’s blood sugars good all night long. When my child forgets to drink his protein (even after many reminders before bed) he will wake up with low blood sugar in the morning and sometimes in the middle of the night. Because of my youngest’s allergies we use the OWYN 20g Protein shakes. They are free from allergens, plant based, have a superfoods greens blend, have all the essential amino acids, and have omega 3s. My boys like the chocolate and vanilla best. For my oldest son I like to give him a whey protein since he won’t eat meat (or cheese, or butter). In order to help insure he’s getting animal based protein. We like using Nutricost Whey Protein Powder. It only has a few ingredients and only has 25g of protein. We have a shaker bottle for him and we’ll make 8oz for him and it will be his protein before bed for two nights.

When it comes to their daily diets I make sure they are getting protein with their breakfast and that they are getting enough complex carbs every day (no white bread or sugary cereals). No/low sugar peanut butter. No added sugar Jams. Beans with jasmine or basmati rice is a must to get that complete protein! (Brown rice is high fructose). Tacos, tostadas, burritos, Hawaiian haystacks. Most days we’ll have a vegetable like broccoli, green beans, asparagus and/or carrots. For fruit we like to have apples (no applesauce), berries, occasionally a pear or watermelon but will top it off with protein because it’s high fructose. Pumpkin seeds, sunflower seeds, flax seeds, almonds, and brazil nuts are other healthy protein snacks for my boys. I’ve experimented with cooking without sugar to make treats for my family, which I’ll do another post about in the near future.

Pasta. Oh how most people love pasta, including my family, but pasta is a blood sugar danger. Hurray for BARILLA PROTEIN+ PASTA! My family can enjoy pasta for dinner without fear of low blood sugars, while still topping off with protein shakes before bed.

Yogurt. Regular yogurt is full of sugar. I make sure my children get low sugar yogurt every day. The two brands we’ve liked the most are Too Good yogurt, and Oikos Triple Zero yogurt. We’ve tried buying our own whole milk plain yogurt and sweetening it ourselves but my boys didn’t like the taste.

Consume more Glucose! With this disorder your body is deficient in glucose. Many things I’ve read about this disorder say to consume more glucose daily. It’s hard to consume glucose without also getting fructose, as sugar is 50% fructose and 50% glucose. Sounds like a daily blood sugar battle I don’t want to fight. But your body needs glucose and I want my children’s bodies to have what they need. You can buy glucose tablets to take daily and have for an emergency. We currently have Dex4 glucose Tablets. You can also buy glucose powder or glucose syrup. I’m planning on experimenting baking with glucose to see what treats I can make with the powder or syrup.

Fructose 1, 6-bisphosphatase deficiency is rare but definitely manageable. I hope my experiences with this disorder and the help God has given me will help anyone else who has this condition. God Bless.

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