August 24, 2012
This week marked the one year anniversary of Matthew's FPIES diagnosis....what a difference a year has made! We don't feel lost, confused, or like the sky is falling anymore :) Sure, we've had our ups and downs, but we've got this FPIES thing down! We deal with it everyday, but it's in the background and not something we dwell on. In the past year Matthew has gone from two safe foods to twelve, and from two trigger foods to five. But he's also met (or exceeded) all of his developmental milestones, and remained the same happy, super-smiley boy we've always loved :) We haven't done this on our own...at last count he has one pediatrician, two allergists, two GI's, two geneticists, one endocrinologist, and one dietitian keeping an eye on him to make sure he's uber-healthy :) Our family and friends have also been wonderful about supporting us and keeping Matthew safe. We feel so blessed to have all of these wonderful people in our lives! And yes, we definitely met our deductible this year (back in February), in case you were wondering ;)
This week was also the one year anniversary for the FPIES food survey I put together. It now has the info on over 212 FPIES kids' trigger and safe foods! I never imagined how many families my little survey would help, I was just hoping to help Matthew :) The survey definitely gave me more confidence when trialing foods with Matthew, and still helps guide our choices of what (and what not) to trial. I get notes every once in a while from families who have found the survey useful....in fact, just the other day a mom messaged me and said she keeps a printed copy of it in her purse! It's so awesome to hear that our efforts to keep Matthew healthy have touched so many more lives than we ever could have imagined.
I like to focus on the positives, but looking back, I think I would have sobbed a year ago if someone had told me that we would only have twelve safe foods at this point. Don't get me wrong, when I think about having twelve foods now it seems like a huge number! I can't even list all of Matthew's safe foods off the top of my head anymore because there are SO many :) But a year ago I thought we'd be somewhere above 20 safe foods at this point...but that was before I knew about the food aversions that go along with FPIES. Matthew has overcome so many obstacles to get to where he is, and we are so proud of how much he has accomplished in the last year :)
His latest passes have been swiss chard, onion, and chicken! Yep, that's right, Matthew has an actual MEAT! He's still not the biggest fan of it yet, but you can't really blame him. The texture of meat is unlike anything else, so we're slowly working on getting him acclimated to the wonderful world of carnivorous consumption :) And Matthew's newest trial is wheat! We've definitely been having fun with this one....waffles, pancakes, corn fritters, chicken nuggets, and real honest-to-goodness carrot cake! Please pray for wheat to be a pass...I'm already dreading the three day break at the end of the trial. Wheat just opens up so many fun foods that I'd really hate to go back to the non-wheat days. Sure, he had corn four, quinoa flour, and potato flour already...but wheat wins hands down! So, fingers crossed :) After that we're going to tackle almonds, because I'm pretty tired of this breastfeeding gig, and would love to work toward adding almond milk to Matthew's diet. Without dairy, soy milk, or coconut milk as possibilities, we're only left with almond milk, hemp milk, or sticking with formula until he outgrows FPIES.
Now to the metabolic front...we went to Mayo Clinic in June to try to get more answers about why Matthew has consistently high blood ketone levels. At Mayo the doctors like to work as a team, so tackling the ketone issue also meant revisiting the FPIES diagnosis. It was so weird to sit there, almost a year into our FPIES journey, discussing symptoms and hoping that the doctor you're talking to knows enough about FPIES to make an informed diagnosis. Bottom line, Matthew has now been diagnosed with FPIES by five different physicians, so he most definitely has FPIES :) And all of his doctors agree that FPIES has nothing to do with blood ketone levels, so we are indeed dealing with two separate issues. But we still don't have answers as to what exactly the ketone issue is. They ran SO many tests, but at Matthew's age it's just hard to get conclusive answers. Most of the tests they ran didn't even have a "normal" range for his age group.
In the end, Matthew's geneticist at Mayo, Dr. Kirmani, said that Matthew more than likely has some type of ketone utilization defect, but until he is 4-5 years old, we won't know exactly what it is or at what level it will affect him. Matthew needs to get older...old enough for the tests to mean something and old enough to out-grow FPIES and have a normal diet. Until he's eating like a regular kid, we just won't know how severe the defect is, because Matthew's limited diet may actually be masking some of the symptoms. He may have a defect that never throws his body into crisis enough to end up in the hospital...or he may have a defect that would have shown more severe symptoms on a normal diet. We just won't know until he's older. That was hard to hear, but it's actually been a little liberating as well. If we can't get answers until he's older then it does us no good to dwell on a diagnosis until a few more years have passed. It's still on our radar, but we aren't focused on it the way we used to be.
Another reason we're more at peace with the ketone issue is that the geneticist at Mayo actually explained what having elevated ketone levels in Matthew's bloodstream means. We hadn't found a doctor who would do this for us, and internet searches are useless since Matthew has normal blood sugar levels. So (I'm putting on my Science teacher hat here!), usually your body uses the carbohydrates you eat, and breaks them down to get the energy you use. When there are no carbohydrates available your body breaks down your fat to obtain energy, and the energy molecule your body gets from breaking down fat is a ketone. The ketones are then sent to your brain and your heart to keep you going until you can eat more carbohydrates, and the ketones are supposed to be used as fast as they are produced. So, in normal circumstances, you should have a blood ketone level under 0.6 mmol/L even if it's been a few hours since you've eaten. Out of curiosity, we checked Abby's (Matthew's 3 year old sister) ketones one morning when she woke up. She hadn't eaten since dinner, so 13 hours after eating her blood ketone level was 0.1 mmol/L. Matthew had to fast for some of the blood tests they ran at Mayo...when we took his blood ketones 8 hours after his last meal, his level was 1.1 mmol/L. So, we know his ketones levels are higher than the norm, but what does it really mean to his overall health?
With the way Matthew's levels spike a few hours after eating, Dr. Kirmani believes there is some issue with how Matthew's body uses the ketones he produces. This means his brain is saying "I need energy," which tells his body to start using fat to make ketones. The ketones are then sent through his bloodstream to the parts of his body that need energy, but not all of the ketones are able to be used. So, the question is, what percentage of ketones is Mathew actually able to use? Is it 80% utilization, 60%, or 40%? And say it's 70%...is this just a really low part of the normal range that will adjust as he grows older, or is there an actual problem with how he uses ketones? Since Matthew presents as a pretty healthy kid who is developmentally on track, Dr. Kirmani believes it's a low level utilization issue, although he did express concerns that Matthew's limited FPIES diet could possibly be masking a larger utilization issue. The geneticist also said that the ketones themselves won't harm Matthew...they are only an indicator that something else is going on. He'd rather us watch Matthew's behavior as an indicator than blood ketone levels. Of course, that's where it gets tricky because the symptoms of a metabolic crisis are identical to an acute FPIES reaction. But luckily we still have the monitor, so if Matthew starts vomiting we have to check his ketone levels to determine if it's FPIES or the metabolic disorder.
So, for now we wait and see. And we're ok with that...we have a wonderful team of doctors who are concerned about Matthew and who will continue to check on his health...that's all we can ask for as parents :)
This week was also the one year anniversary for the FPIES food survey I put together. It now has the info on over 212 FPIES kids' trigger and safe foods! I never imagined how many families my little survey would help, I was just hoping to help Matthew :) The survey definitely gave me more confidence when trialing foods with Matthew, and still helps guide our choices of what (and what not) to trial. I get notes every once in a while from families who have found the survey useful....in fact, just the other day a mom messaged me and said she keeps a printed copy of it in her purse! It's so awesome to hear that our efforts to keep Matthew healthy have touched so many more lives than we ever could have imagined.
I like to focus on the positives, but looking back, I think I would have sobbed a year ago if someone had told me that we would only have twelve safe foods at this point. Don't get me wrong, when I think about having twelve foods now it seems like a huge number! I can't even list all of Matthew's safe foods off the top of my head anymore because there are SO many :) But a year ago I thought we'd be somewhere above 20 safe foods at this point...but that was before I knew about the food aversions that go along with FPIES. Matthew has overcome so many obstacles to get to where he is, and we are so proud of how much he has accomplished in the last year :)
His latest passes have been swiss chard, onion, and chicken! Yep, that's right, Matthew has an actual MEAT! He's still not the biggest fan of it yet, but you can't really blame him. The texture of meat is unlike anything else, so we're slowly working on getting him acclimated to the wonderful world of carnivorous consumption :) And Matthew's newest trial is wheat! We've definitely been having fun with this one....waffles, pancakes, corn fritters, chicken nuggets, and real honest-to-goodness carrot cake! Please pray for wheat to be a pass...I'm already dreading the three day break at the end of the trial. Wheat just opens up so many fun foods that I'd really hate to go back to the non-wheat days. Sure, he had corn four, quinoa flour, and potato flour already...but wheat wins hands down! So, fingers crossed :) After that we're going to tackle almonds, because I'm pretty tired of this breastfeeding gig, and would love to work toward adding almond milk to Matthew's diet. Without dairy, soy milk, or coconut milk as possibilities, we're only left with almond milk, hemp milk, or sticking with formula until he outgrows FPIES.
Now to the metabolic front...we went to Mayo Clinic in June to try to get more answers about why Matthew has consistently high blood ketone levels. At Mayo the doctors like to work as a team, so tackling the ketone issue also meant revisiting the FPIES diagnosis. It was so weird to sit there, almost a year into our FPIES journey, discussing symptoms and hoping that the doctor you're talking to knows enough about FPIES to make an informed diagnosis. Bottom line, Matthew has now been diagnosed with FPIES by five different physicians, so he most definitely has FPIES :) And all of his doctors agree that FPIES has nothing to do with blood ketone levels, so we are indeed dealing with two separate issues. But we still don't have answers as to what exactly the ketone issue is. They ran SO many tests, but at Matthew's age it's just hard to get conclusive answers. Most of the tests they ran didn't even have a "normal" range for his age group.
In the end, Matthew's geneticist at Mayo, Dr. Kirmani, said that Matthew more than likely has some type of ketone utilization defect, but until he is 4-5 years old, we won't know exactly what it is or at what level it will affect him. Matthew needs to get older...old enough for the tests to mean something and old enough to out-grow FPIES and have a normal diet. Until he's eating like a regular kid, we just won't know how severe the defect is, because Matthew's limited diet may actually be masking some of the symptoms. He may have a defect that never throws his body into crisis enough to end up in the hospital...or he may have a defect that would have shown more severe symptoms on a normal diet. We just won't know until he's older. That was hard to hear, but it's actually been a little liberating as well. If we can't get answers until he's older then it does us no good to dwell on a diagnosis until a few more years have passed. It's still on our radar, but we aren't focused on it the way we used to be.
Another reason we're more at peace with the ketone issue is that the geneticist at Mayo actually explained what having elevated ketone levels in Matthew's bloodstream means. We hadn't found a doctor who would do this for us, and internet searches are useless since Matthew has normal blood sugar levels. So (I'm putting on my Science teacher hat here!), usually your body uses the carbohydrates you eat, and breaks them down to get the energy you use. When there are no carbohydrates available your body breaks down your fat to obtain energy, and the energy molecule your body gets from breaking down fat is a ketone. The ketones are then sent to your brain and your heart to keep you going until you can eat more carbohydrates, and the ketones are supposed to be used as fast as they are produced. So, in normal circumstances, you should have a blood ketone level under 0.6 mmol/L even if it's been a few hours since you've eaten. Out of curiosity, we checked Abby's (Matthew's 3 year old sister) ketones one morning when she woke up. She hadn't eaten since dinner, so 13 hours after eating her blood ketone level was 0.1 mmol/L. Matthew had to fast for some of the blood tests they ran at Mayo...when we took his blood ketones 8 hours after his last meal, his level was 1.1 mmol/L. So, we know his ketones levels are higher than the norm, but what does it really mean to his overall health?
With the way Matthew's levels spike a few hours after eating, Dr. Kirmani believes there is some issue with how Matthew's body uses the ketones he produces. This means his brain is saying "I need energy," which tells his body to start using fat to make ketones. The ketones are then sent through his bloodstream to the parts of his body that need energy, but not all of the ketones are able to be used. So, the question is, what percentage of ketones is Mathew actually able to use? Is it 80% utilization, 60%, or 40%? And say it's 70%...is this just a really low part of the normal range that will adjust as he grows older, or is there an actual problem with how he uses ketones? Since Matthew presents as a pretty healthy kid who is developmentally on track, Dr. Kirmani believes it's a low level utilization issue, although he did express concerns that Matthew's limited FPIES diet could possibly be masking a larger utilization issue. The geneticist also said that the ketones themselves won't harm Matthew...they are only an indicator that something else is going on. He'd rather us watch Matthew's behavior as an indicator than blood ketone levels. Of course, that's where it gets tricky because the symptoms of a metabolic crisis are identical to an acute FPIES reaction. But luckily we still have the monitor, so if Matthew starts vomiting we have to check his ketone levels to determine if it's FPIES or the metabolic disorder.
So, for now we wait and see. And we're ok with that...we have a wonderful team of doctors who are concerned about Matthew and who will continue to check on his health...that's all we can ask for as parents :)
