Parenting PKU

Where to even start... Since July we have been challenging Luke's daily phe intake to reach the maximum his body is able to process, and when we hit that number we were going to trial him on the medication Kuvan. We have had the medication since the start of August and the dietitians had said it would likely take a week or two to find Luke's max... Well fast forward to the end of last week and we are still  not there yet! In July, Luke was in-taking around 17 grams of protein a day (860mg of phe) which was an amount we had become accustomed to, it meant he had a bit of freedom when it came to having gluten-free breads or muffins instead of PKU specific ones, it meant a Kinder Surprise egg every now and then wasn't out of the question, but we still were working with much less protein then a child on a normal diet. When we initially started Luke on solid foods (all the way back in 2015) he was on an intake of 7 grams of protein a day (350mg of phe), so we already felt lik...

I am going to admit it took a lot of writing, re-writing and then re-writing again to put into words all that I wanted to say in this post. It is a hard one in a lot of ways for me but I do find it very cathartic to write this all out. I admit that I struggle daily with Luke's PKU.  Okay, you maybe thinking that it is normal and fine for me to struggle with it, and if you think that then you would be right... Logically I know this... I know that a life long medical diagnosis is going to have a life long struggles. However to me it is hard to admit that after 3.5 years I still have much to learn and yes, I do have daily struggles. I like to be the strong one, the problem solver, and when it comes to Luke's PKU I want to be completely in the know and honestly I prefer to be completely in control... well no one told my sons body that (or him for that matter!) The struggles  Will he eat a big meal, a small meal, or will he straight up skip this meal? Keep...

It has been quite a while since I posted a blog post, I didn't realize it had been since November, and I have some exciting things to catch the blog up on. As always thanks for reading and caring for our sweet little boy!! Wait... what about school? As Luke is edging closer to kindergarten age and time seems to be flying by we realized we had to make some choices about preschool. Preschool was something (pre-PKU) we had just assumed our child would attend. The question was how comfortable were we with the idea of our three-year-old, well almost four-year-old, attending a playschool where the kids have snack together... cue the fears and general feelings of discomfort.  Some playschools in our area do a rotating snack week where families take turns bringing group snack for everyone for the week and some playschools had each child bring their own snack each day to eat at the table all together. Well for Luke the obvious choice would be the sending a snack each day for him to e...

Obviously we are very open about Luke's PKU and we do this in the hopes that this will make it natural for Luke to hear about his condition. To me a very important thing is that he knows he does not need to hide it, ever. We have talked about high phe/low phe foods since we found out about his PKU and especially since Luke started eating solid food.  It is something that was encouraged by his metabolic team and it really made sense to us. Plus with  me running a day home and other children around, I hoped they too would understand the importance of being careful with food. Well it seems that this method was very effective as our smart little three year old knows to ask if the food is high phe (red light) or low phe (green light), and also he knows many of the foods that are very high. Some of the things he says are: "Oh no, that is high phe and will hurt my brain" "Is this my PKU cheese or high phe cheese?" "Is this a red light Mommy?" ...

Anger & Frustration Well the good news I posted about here  was that we were thrilled to find out that Luke's food was now going to be fully covered by Alberta Health Services. I placed a large-ish order of new foods for Luke but held myself back from "going crazy" with the order. Turns out this was a mistake.  The foods that were listed were never meant to be free... meaning there never was a change in coverage. I am not 100% sure on the details as to how this mistake happened, it sounds like an error with the metabolic food distributor.  They have not sent an invoice for the cost of the foods I ordered, and honestly I cannot imagine how they could. However foods that are very pricey are ones that Lucas loves, including the animal pasta that we tried for the first time from the free order.  I have known of this for a awhile now, as the website was corrected not long after I received our order. I struggled to find a way to post about this in a way tha...

Good News #1 The first bit of good news came via a phone call from Lucas' dietitians this week letting me know that we had the green light to starting challenging his tolerance again. I say "again" because awhile back we challenged it and eventually reached an intake high enough to balance his blood phe numbers and his tyrosine levels. Luke has been in the safe zone phe level-wise, it is just his tyrosine numbers seem to stay low and tends to drop off easily. They, the dietitians, say the low tyrosine levels leads them to think we are not fully utilizing his natural protein intake, meaning if we give him a higher tolerance then his enzyme activity should keep his phe numbers safe but also show us an increase in his tyrosine levels because his body will now be making more by breaking down the phe.  So long story short  we are pushing his tolerance which means higher amounts of natural protein (phe). Now it is not like we are adding meat or cheese or anything like t...

Today was such a busy but great day for the three of us. Luke, Jeff and I headed to the Stollery at 9am to meet with his metabolic team.  The appointment  The dietitians always start things off with weighing and measuring Luke to see how well he is growing and if you have been following the blog you may know Luke has kinda been a bit light on the scale. However today we were happy to find out he is doing really well both height and weight wise! He is in the 50th percentile for both!! Then we talked about his diet some, touched on his formula intake and got the green light to try him with a new juice type formula (which will be good for summer holidays) Then in came Dr. S, she is his pediatrician/metabolic doctor. I love this lady! She is great at offering guidance and suggestions that will make things easier for us as he gets older, things like having him help us make his formula, help us weigh and measure his foods, those sorts of things. The exci...

As another "food centered" holiday approaches I can already feel my mind racing. Trying to think of ways to make Luke feel like he has choices at dinners and that he can feel included in all the Easter goodies. Chocolate is high phe, so I will be going to Micheals this weekend to get some chocolate molds to fill with his low-phe chocolate, I will bring some to dinners and some will be for his Easter basket. I am also going to try my hand at his special buns - he is a carbohydrate lover like his Mommy so I know that I will likely have success having him eat those.  I don't want to make it sound like we don't have marvelous families because we do! Our families understand, they always help us include Luke friendly foods and want to make the holidays special for him. We are truly blessed to both have supportive parents and siblings AND extended family who love to shower Luke with love & fun!  Luke is not trusting when it comes to food, this makes him VERY reluct...

Sample packs arrived with new flavors for Lucas to try! Good News Luke's phe levels had gone out the top of the safe zone last week, more then this Momma would like! This meant last Friday we adjusted how much phe (protein) Lucas intakes in a day from foods, lowering the amount. This is done so that his body has a chance to stabilize the balance of his enzymes and the amount of phe he can break down effectively.  Well the decrease had the desired effect and Luke is once more in the safe zone!! *cue happy dance* Now just to get Luke to keep improving on drinking up the full amount of his formula each day! 

I think I have mentioned before that a HUGE  part of Luke's diet & phe management is his formula. It is like a PKU "protein shake" in that it has all the parts of protein that he needs but that he misses out on with his restricted diet. He has a set amount of formula that he must  drink in a day and if he goes a little over on drinking extra then that is alright. It has zero phe in it so will not cause him to be over his phe intake, in fact it will help him feel more full if he has eaten his days worth of phe and needs a bit more to fill his belly. When we switched over from the infant formula to the new, more age-appropriate, formula I worried so much about Luke rejecting the new one. I have read so many posts on the Facebook group about kids refusing to switch over, of parents stressing as their kids went days without drinking any formula and I was so worried. It's a big fear of mine for Luke to stop drinking his formula. Another situation I read a lot about ...

Right now you maybe looking for someone to tell you that it will be okay, and it will be.  Or maybe you are looking for a picture of what a day in the life of a PKU baby/child is like. Or maybe you are just curious as to how someone else is handling it or how they handled it when they found out. Well perhaps you will find an answer here or maybe not but either way please feel free to comment or ask me questions.  *            *           *   That moment they told us he had a condition with a big long name that I couldn't even pronounce I felt something inside of me break, I don't mean to sound melodramatic but maybe it was everything else that was going on but when they said he has  Phenylketonuria I felt my world freeze. I came home and let all that emotion pour out of me, I cried and I worried and I didn't even really understand what I was worrying about because I barely heard or understood what they ...

As 2016 is coming to a close I am very much looking forward to the very exciting things that the new year will bring our family. One of the big things we are looking forward to is Lucas doing a drug trail with a medication called KUVAN. What is KUVAN & why should we try it? (much of this is copied from the http://www.kuvan.com website)  This medication trial offers us the chance to see if Lucas is a responder to KUVAN. KUVAN is a form of the cofactor (or helper) BH4 that already naturally exists in your body. In people who respond to treatment with KUVAN, it works in the body just like BH4 does to help stimulate residual PAH enzyme activity to convert Phe to tyrosine and lower blood Phe levels. KUVAN has been prescribed along with a low–Phe diet to lower blood Phe levels in many people with PKU of all ages and types In many people with PKU, KUVAN increases the activity of the PAH enzyme that isn’t working properly. In other words, the PAH enzyme that isn’t working “wakes up” a...

Things I have learned as a PKU Mom  ( and as a Mom in gereral ) Never let your supply of formula run out (dry or the stuff you have pre-made) order and make LOTS! Lancets (the things we use to do the heel poke) are expensive but it's worth it to have the right one! Do not cheap out on a shaker bottle for making formula, it will leak or explode and that is  so not okay Plan ahead as much as possible - it makes life easier But plan for things to change People are amazing at offering love and support, ask for help when you need it but make sure to pay it back when you can There are people you can talk to somewhere in the world going though almost the exact same thing you are, sometimes it is nice to talk to someone who knows exactly what you are dealing with Don't hide or feel embarrassed about having to ask for different food choices but also try not to let it take over your enjoyment of what is offered It hurts some days to think this is a life long...

Good news today is that Lucas got to go up a bit in his phe tolerance, he is now at 600 - 610mg phe per day. Woohoo a nice early birthday present for Lucas. Well by request here is a list of foods people have been curious about the phe content! One egg (boiled) = 715mg phe Mars Bar (full size) = approx 140mg phe Grilled Cheese Sandwich = approx 610mg phe (if made with a kraft cheese slice) Budweiser Beer = 72mg phe Spaghetti (noodles only) 1 cup cooked = approx 440mg phe Ritz Crackers (1 cracker) = 10mg phe Celery with cheddar cheese = 8mg phe for celery & 1375mg phe in 100gr of cheese Tim Hortons Muffins = 275 - 330mg phe  Lucas eats 150mg of phe at each of his 3 meals and then about 75 mg of phe per snack.  So as you can see there is a reason why dairy, meat, and gluten are no go areas for Lucas on a regular basis! If you have any questions or want to know a different food let me know!!!

Well as most know we have been trying more and more of Luke's low-protein foods, I had blogged about him trying some of his little pasta circles  here . Well they have gone over really well, as has the low protein version of rice! Luke really enjoys it if I mix the rice with coconut yogurt (plain) and a bit of cinnamon, almost like a little guys rice pudding!  We tried the Eggz next and it was a bit more of an adventure then the pasta! Eggz powder comes in a bag and has simple instructions - add "non-dairy creamer" and let sit, then cook one side and flip. So easy.... yeah not so much. Okay the first one was a flop. I thought it would be more like normal eggs and be soft enough for him to nibble on, instead it was much to firm on the outside and not what I would consider safe for a little. So I decided to scramble them up like normal eggs, which of course they didn't do the way I am used to but this time I had softer pieces for him to try a...

Doh! Well I know it was bound to happen at one point or another!  I calculated Luke's phe on the fly and got it a touch wrong, darn it! Saturday when I tallied up his final numbers in the evening I realized I had gone about 20 mg of phe over his limit.  At first I was super upset and mad at myself but I calmed down and told myself that it's done & I cannot change it now. Really with his low levels last week this should not make a difference. It is less then 4% more then his usual amount, so it is not like I doubled it. Well I did the heel poke this morning and tomorrow first thing I will take it into the Lab. I will be very curious (and slightly anxiously) awaiting these results!

Lucas having some applesauce with my cousin Amanda in BC   One of my worries when we thought about solids was that the "easy" feeding methods would not be something we would be able to use, things like food pouches and self feeding foods like rusks and puffs. Well that proved an unfounded fear as pouches that are only one type of fruit or a mix of low-protein veggies and fruit are ones I can pretty easily estimate the amount of phe. The puffs and rice rusks are low in protein, especially considering how little they weigh, which is wonderful because they make great distractions when trying to cook a hungry little guy some food. Back Left to Right : Bread mix for a bread machine, case of phenyl-free formula, Mushroom Burger Mix Front Left to Right: Amino-acid supplements, Lo-Pro Anellini, Lo-pro Rice So now that Lucas is ready to start having "more solid" type foods I ordered up what I consider the next step in his diet. One of the things I wanted t...

Well today I placed an order for what seems to me is the "next stage" of foods for Lucas. I have been putting off moving Luke to more firm and diverse solid foods until I ordered him up some of the low-pro options that will a big part of his everyday diet. Things like making him bread and putting the "peanut" butter on it, it won't be real peanut butter because 3 tablespoons of creamy peanut butter has his entire days worth of phe!! (crazy right?!?) So how do I order up this food? I go to a special website and it provides me with all their options of mixes, baking needs, soup bases, pastas, rice, cereal, cookies and more. I select what items I need to order (including his formula) and it provides me with a grand total. So far we have been able to order up all free items. These are items that are paid for by the government and I have had a couple people ask me why would it be covered? Well Lucas' food is a medical food  due to the fact that the protein has ...

Very happy to report that Lucas has safe zone levels (finally!). As of Fridays blood spot he was 252 which is my personal favorite area for him to be in at, as it means we have some "wiggle room" on each side if his numbers start to vary again. For this to be considered balanced we would like to see three consecutive safe zone numbers. Why? That would mean we have found a tolerance that his body is able to keep up with. The good news too is that he is tracking just fine on the weight charts and we can stop worrying quite so much about his weight gain. Before it was once a week weighing him and measuring him, now we are down to every 2 weeks!! Hope everyone is having a great July!!

Hey all!! First a small update on Lucas: we still do not have his phe levels balanced out but we are working hard with the dietitians to get past this current growth spurt! I have a feeling, call it Mommy instinct, that his next levels we get back on Tuesday will look much better. But I wanted to drop some quick facts about PKU that people have asked me recently. I love when people ask me about Lucas' current levels and ask me PKU questions so don't be embarrassed or worried about asking me anything about it!!! Luke will never out grow his PKU We may decide to try one of the current drug options in a few years to improve how much food he can eat but it is not something that is cured (yet......) Everything Luke eats and drinks  must be recorded and counted in a daily diary Some foods are very low phe naturally (apples, grapes, pears, and more) so he can eat a lot more of them then some foods that are higher (potatoes, peas, cereals) and some proce...