3 Different Diets + 1 Family = Learning Curve.

Posted on November 16, 2016November 16, 2016 by Amanda Horner

Madelyn is 8 months old as of November 4th. She is doing amazing.

monthlys6

Madelyn can now walk around the table and from couch to couch while holding on.
She climbs the baby gate
chases copper and figaro.
She says mama / Dada and hi !
She is getting better at sleeping in her crib, though we still co sleep at the beginning of the night.
She is learning to wave and clap
She hums / sings to music
She likes to pull mama’s hair and jewelry.
She has 2 bottom teeth and working on another one soon.
She loves to stand
She pushes her toys around while walking.
She stands at the toy bin and pulls all the toys put then puts them back in
She pulls her self up onto everything.
She’s very busy and vocal
She is easily entertained by others and loves to watch other kids.
She has a new baby cousin. A boy! Who she will stare at and smile..
She loves to put anything Into her mouth .
She’s fast!
She celebrated her first thanksgiving and Halloween this month.

She still wears size 6 months clothing.
She got a new 3 and 1 car seat and jogging stroller this month to.
Her favorite foods are fruits and vegetables. Specially carrots and bananas. She prefers to feed herself. She tried many new foods this month like pancakes / toast / sole fish and rice.
She got her first cloth diaper.
She loves her elephant pillow and toys.
She had her hair up in a pony today for the first time.
She loves to sit and play in the bath tub every night as part of our bedtime routine.
She still loves her swing.
She does not like eggs or asparagus. She does not like her crib but it’s getting better.
She does not like the jolly jumper.
Our baby is growing and changing so much that I’m constantly astounded and trying new ways to challenge her and keep her busy while teaching her new things. Trying my best to keep up !
She weighs 14.6 lbs
I love her smiles and the way she giggles and the sounds she makes when she’s talking to you. I love the way she gives open mouth kisses and says mama and Dada.
I love our cuddles and watching her sleep or watching her play with her toys and interact in her environment . She so happy and full of smiles and giggles !
#daddysgirl #mommysworld #babygirl

At 6 months we started weaning. We started with home made purees due to her dairy allergy. I made everything from scratch. We started with baby organic oatmeal and slowly started adding purees , introducing one new food every 3 days. but with in a couple weeks we started to introduce baby lead weaning and she took to it so quickly and it was clear she prefers to feed herself. She is such a great eater. I was so worried about choking and feeding her this way. I did my research , I read books and talked to other moms and joined groups on fb.

now at 8 months old , she is currently sitting next to me in her high chair eating oatmeal bars that I made her the other day.

She already has such a variety of foods. She is not a picky eater at all like I was. I am always amazed buy how strong her little jaw and gums are to be able to eat foods with out many teeth! One thing I love about baby lead weaning is that she is learning and challenging herself and developing skills and fine motor skills. By feeding herself she is control. She eats as much or as little as feels like at the time. She controls what is going into her mouth and how much. IF she puts to much in her mouth at once, then she gags and she learns not to put that much in her mouth. So far she has not choked on anything. she sometimes gags and clears it herself. I always sit beside her while she is eating. I watch her closely.

Babys gag reflex is a lot closer to her mouth then the back of a throat in an adult. So by feeding herself shes engaging this reflex and preventing choking. Babies who are fed purees on a spoon , we the parent place the spoon past this reflex and when they move to solids, the reflex has moved further back with age and they dont gag as quickly and it can lead to chocking.

Madelyn’s diet so far includes :

Toast and jam

Fruit – specially berries, banana, mangoes, peaches, pears , apples, papaya and avocados.

Steamed vegetables – Carrots are her favorite. Broccoli , cauliflower, Roasted vegetables like yams, potatoes and squash, sauteed veggies like mushrooms, peppers, onions , tomatoes, garlic .

Oatmeal

Rice

pasta

quinoa

fish/ sole and cod mostly.

chicken

sausages

pancakes

scrambled eggs with dayia cheese

we use coconut milk as a substitute when cooking or baking.

She is on a special formula for her milk protein allergy. She drinks nutramigen. We special order it from Vancouver just like my formula.

We omit dairy and milk from her diet. I dont cook with it and she has not had cheese or yogurt. I make most of her food from scratch or fresh ingredients to avoid it. Any thing store bought i am very careful to read the ingredients so it does not contain milk soy or whey.

Most days I am cooking 3 different meals at breakfast and dinner. She does not eat lunch yet. Cole has a regular diet, no allergies. Then Madelyn’s food , and my low protein. Though admittedly i have really let mine slide since the days i use to bake or cook or meal prep for me are now for her.

This has lead me to deal with some mixed emotions and thoughts surrounding having pku.

The other day I was in the kitchen making Madelyn some new foods to freeze. I was making her home made oatmeal bars ( oatmeal , water and fruit then baked in the oven) and some quinoa fruit balls ( 1 1/2 cup quinoa, 1/2 rolled oats and fruit of our choice, rolled into balls then baked in the oven for 15 mins) and i realized that I cant eat as healthy as her. I eat healthy for me, but nutrition wise, my diet is not as healthy. I cant even modify her recipes or make them PKU friendly. I dont even have anything similar in taste of texture. I have made such an effort to ensure Madelyn is eating healthy and having a variety of nutritious foods. A well rounded diet and striving to give her the very best so she does not develop issues with food like I did. I want her to have a positive relationship with food. I dont ever want her to feel how I feel. I realized i didn’t want her to feel shame when she eats. It made me sad, cause I thought back to my youth and remembered when i use to hide and eat alone. Because I felt shame. I hid in my room at meal times . and refused to eat at school in front of my peers as they always asked why my food looked weird or why i drank that stinky milk.

I developed weight issues and still struggle. I dont want to pass these onto my daughter.

Now that Madelyn is eating more and more, I am finding myself struggling with resentment towards having pku. To being angry and frustrated. Baby lead weaning books recommend we all eat together or eat the same thing and that whatever i am eating should be safe for my baby to eat should she want to share or try from my plate. But I cant share my foods with her.

This morning that became more evident. She wanted some of my toast. My low protein bread. With honey on it. So one babies cant have honey before age 1, 2 its white low protein bread and not very good for her. So I had to tell her no and she cried. She didn’t understand and it hurt my heart.

I dread having to explain to her one day, that mommy cant share her food, though we teach children to share most everything but food. Or why mommies food looks different , or why i cant eat what she is having. or when she reaches her little hands up trying to share her food with me and i pretend to eat it but dont actually. One day she will notice. One day I will have to explain to my baby girl that mommy is different and tell her about PKU. She wont understand at first, there will be alot of questions and I will probably have to tell her several times as she grows up.

I think back to when I was pregnant and my tolerance went up so high and I could eat real foods and the healthy and nutritious foods I had to choose from. The healthy choices and options that suddenly became an option for me . Like oatmeal, oats, real bread and whole grains and rice. Not white sugary and full of carbs o nutrition. I realize now that those options are only available to me when i am pregnant.And I suddenly realize now why treatments like kuvan and peg pal are sooooo important. I am considering trying to make it through the kuvan trail again to see if i am a responder. If I am not , then hopefully one day I can try peg pal. I didn’t think i would ever want to. I use to think the diet and formula was enough and I wasn’t worried. But now I think , given the opportunity , I would jump at it. I want to loose 6o lbs and I want to eat healthy and clean and still feel full. Something I struggle with on the low protein diet. I want to eat like my daughter. I want to eat oatmeal again. I am learning so much more about nutrition when I thought I already knew so much and I am feeling more limited then ever and wanting to feel “normal!” I dont know anyone or myself can loose weight on this diet and still feel full and satisfied. I over eat because i burn through the low protein foods and fruits and vegetables way to quickly. I have way over the recommended calorie intake for a women of my age. But when I think about restricting myself , going back to limiting myself, going back to tracking calories and protein and phe . I think about how hungry I am. I feel guilty that I was able to excel when i was pregnant and stick to it so amazing for 10 months only to fall back after. Then I think to myself, pregnancy was easier. She was taking everything she needed. As my pregnancy progressed or when I got hungrier my tolerance went up. I had flexibility and choices even when restricting. I wasn’t stuck at 375 mg of phe and 1600 calories for the whole 10 months. I was having like 2000 calories and never gained more weight then what baby needed. Now I cant loose the weight and am stuck here.

So maybe if I responded to kuvan or to peg pal , I could have a more regular diet, with healthier options like oatmeal, whole grains,and proteins that could fill me up more with out packing on the weight or filling up on low protein pasta and empty calories!

So I get it now, I get why we are trying to fight, why we need this passed. I get it now, being on kuvan or peg pal will give me the chance at a healthier life , with flexibility and quality of life with out revolving and focusing on foods. With out every aspect of life being impacted by food. It is freedom. normalcy. Wouldn’t it be nice if I was a responder despite having classical pku and one day when we have coverage I can eat the same as my daughter, loose the weight, be more active and lead a healthier life style and set an example for my daughter and our family I could be proud of, that i have always drempt of. When I think of the future, I think of being that soccer mom, an active mom, a healthy mom who can keep up with my kids. That has the flexibility to grab a quinoa ball on the go, or a protein bar, drink a protein shake, have a multi grain bagel or sandwich , real rice, nuts and seeds? I cant ever see myself ever eating meat. or a meat alternative like tofu. Yuck! I am not big on chocolate or cheese either, or crap foods. But yogurt ? that might be nice. Oh and not to be pregnant to eat normal lol.

This is my battle, my eternal struggle. I am sure I will adapt and meet these challenges in raising Madelyn as we get to them. Being responsible for my baby girl is a whole world of new lessons. I just hope I can keep teaching her, leading her , inspiring her and challenging her to be her very best. Most days I dont feel like I know what I am doing but I am trying my best to keep up to her. I never thought about the choices id have to make for her till she is old enough,. something as simple as what to feed her can have such a huge impact.

I can sympathize what is like for a parent with no diet challenges to raise a baby with PKU. I bet you feel the same as I do most days. Its a learning curve for us all and we just do our best. I am sure you are doing your best to. I tell you one thing, my beautiful girl is growing up so fast and everyday is something new. This first year has gone by so fast. Some days I am like wow , she is 8 months old, then others its like she is ONLY 8 months old. How will I teach her to be kind , to be a good person, to love and be respectable and give respect. to be honest, humble, compassionate. How will i show her this? The decisions I make for her now , will impact a life time. Starting with Learning to walk, to talk, to run, and one day their will be puberty and other obstacles to navigate. Oh the joys of parenthood. Non PKU or not, the challenges are the same. how we deal with it not only shapes our children, it shapes them to.

Managing the diet, That's my PKU life

Kids today.

Posted on August 21, 2016August 21, 2016 by Amanda Horner

Though I am very happy to see how far treatment and quality of life for PKU has improved over the years , even just in my short 30 years .

Then there is another part of me is jealous of PKU Kids today. So much is geared to ward todays children. Parents are much more involved and advocating to make sure they do not miss out and lead a normal life and have a good relationship with food and a healthy life style.

There is a much greater sense of community , thanks to social media. There are more resources for support and families are connecting more.

I did not meet anyone else with PKU until I was 13.

Today youth can log onto fb and look for recipes, share meal ideas and photos, take part in discussions, ask questions, seek advice and connect with people there age. Parents and caregivers can join groups and have the same resources available to them.

There has been advancements with tools for tracking diet like How much phe and apps.

Families are coming together to socialize and hosting events.

Even the foods have improved. We have so many more choices and imitations of regular foods that kids dont have to feel left out eating anything different from there family and peers.

You want a cheeseburger like the rest of your friends? Here is a camburger and dayia cheese? Want to roast hotdogs while camping with family? Here are brooklyn dogs? Pizza? icecream? pancakes and waffles? bagels and pasta?

Lets not forget the formula? Chocolate, strawberry, vanilla, orange, tangerine, swirl, GMP protein. Powders, liquids, pre made mixes, bars, sachets, gels, shakes, puddings! Various brands and low protein companies. Variety ! Why not mix it up and have 2 different ones?

We have choice now!

Choices I did not have . Choices I missed out on. That I feel if I had of had from an early age I never would have cheated on my diet! I never would have hid my food from my peers, or thrown it in the garage when I got to school. I may never have had levels above 12 . I would be saved from high level symptoms and hospitalizations.

Kids today dont need to face the battles I did. They wont feel different or left out.

So when I am at events and I hear parents lobbying to the government for more advancements such as kuvan so they have the quality of life they deserve , I stand in support but I also think.. What about the adults?

How do we achieve the quality of life we deserve when many of us have already learnt these bad habits, have a bad relationship with food? struggle with compliance? when we are not use to some of the low protein foods because we didn’t have them so we developed a taste for ” regular foods” ? How do you make yourself eat the low protein rice that might as well be pasta made in the shapes of grains when you have the taste and experience of real rice?

Or what about the adults who have no aces to low protein foods, because they have either aged out of the government funding, or they have no private insurance?

My heart aches for the adults like me who ride the roller coaster of com pliancy. Who are stuck on the merry go round of high level symptoms or battle with side effects such as eating disorders or mental health issues developed by not staying on diet?

This is why I decided 8 years ago to dedicate my life and my voice to being an advocate for the adults. Sometimes its very frustrating being the only one up trying to get the attention we need. In my experiance so many adults have shyed away from anything to do with the diet. Its like pulling teeth to get them to part take in social events, come out to seminars, partake in the community. Try new low protein foods and recipes and find there way back and value their health.

I see adults blame everything wrong in their life on their high levels from relationships to work and jobs and any failure and I get frustrated , and want to scream come to the events, get involved and be inspired and get back on diet. It is so much easier now.

Or when a maternal PKU mom only returns to diet long enough to have her baby then goes right back off again because the temptation of a real cheese burger or meat is to alluring. She is not use to the textures and tastes of the imitation low protein options. Or insurance wont cover her now that shes had her baby. I fear for her and the impact that bad levels will be on her to parent.

My thoughts always go back to if only they had the opportunities kids today have. If only we had all these options and choices as kids , we would be repping the benefits now. The next generations will learn from us and it will continue to improve.

Being born today with PKU is not a curse. Today is a great time to have PKU and it will only get better.

In the mean time… how do we help the adults? How can we bring them back on track? and improve their quality of life too? I want to hear from you ! Please write me at my email or leave a comment to have your answer featured on a part 2 blog post.

Managing the diet

High PHE Level Syndrome

Posted on August 17, 2016 by Amanda Horner

Even though, I have been on a well controlled diet with acceptable levels within range for many years, every now and then I still struggle. AS I have talked about in the past 3 posts and make reference to through out my blog , as a teen I struggled immensely and suffered from High PHE level syndrome for years. High PHE level syndrome is prolonged levels in a high range and the symptoms brought on by the high levels.

Now when I have high levels i am very sensitive to the slightest change and I can recognize it in my body with in days. I can feel it the same day if I have eaten to much protein.

I have been asked a lot lately what id feels like. So I thought id share a post today about what high levels someone may experience and how it feels to me.

Headaches- Different from tension headaches or migraines. When my levels have been to high and I have a high level headache. This headache feels like a full head pain. Like being knocked on the head. Strong behind the eyes similar to a sinus headache but wider. It radiates at the top of my head and it makes me feel tired . Sensitive to light. I tend to hide out in my bedroom more .

Brain Fog- I use this as a broad term to cover many different feelings. For me having brain fog means that I have trouble focusing , retaining information, finishing trains of thought. simple problem solving. It also effects my ability to learn . I also have problem recalling past information and facts. I also have a very hard time with math.

Behavior- I get agitated quicker. Overwhelmed and irrational. I feel angry for little or no reason. Or I cry easily. I take things the wrong way and become very emotional. I call it feeling crazy, and all over the place. Like my head is spinning a mile a minute around me I cant keep up.

Mental state- I have struggled with depression for 7 years. 2008-2015. Due to multiple injuries and circumstances but made worse when my levels are higher. Also when my levels are high I have anxiety. Mostly about driving or being alone. I hate doing things alone or going somewhere alone. I wont drive certain distances, or go out after 3 and not after dark either. I get panic attacks too.

Verbal- I tend to ramble more, my writing is effected in such that i am more repetitive . Friends and family can tell just by talking to me that i am off or not making sense. it is like I have a harder time articulating what I mean to say. it makes sense in my mind but dosent come out the way it was in my head.

Fine motor skills- I find my reaction times are effected and my reflexes. They feel impaired.

Physical- I find my chronic pain is increased . It feels like an all over pain . My muscles feel heavy and inflamed. My joints ache. I feel like muscles are pulled or ripped. I have a very hard time relaxing.

When you are feeling like this it can be very hard to pull yourself out of the merry go round . Thankfully I have a wonderful support system and am apart of an amazing community and have built friendships and relationships that have gotten me though and held me up when i have fallen and helped me find my way again. I know with my friends and family and my team behind me I can get back on track and I will !

Managing the diet

Chances

Posted on August 16, 2016November 9, 2018 by Amanda Horner

How many first, second, third + chances can one person have? Is there a limit? No!

Here I am again sitting before this screen writing my confession. A scene that seems all to familiar and equal parts shame. The same struggle, the same bad habits, the same failure.

High levels. High PHE levels. My current level is 14.5 . I am feeling it. You yourselves can probably tell just by reading my words. I find I am more repetitive trying to describe my point. Or I drag on and on about the same thing. Rambling. Which is pretty much the previous sentence in different words.

Why was this so much easier when I was pregnant? When I was pregnant my levels where the best they have ever been. I was able to maintain them to. By the end of the 10 months ( yes pregnancy is actually 10 months not 9 ) It was second nature and I went into the post partum period with confidence about maintaining my awesome control well into the future.

But one bad choice lead to old habits and a downward slope. One lazy over tired un planned bad meal choice lead to another and then the slope got steeper.

In fact I know I have already written a post about struggling to stay off track after Madelyn was born.

Things were suppose to be so much better PKU wise . My Madelyn providing the motivation to keep on the wagon. To lead by example in a healthier life style.

We have low protein food coverage now, and I have always drank my formula. So why is this so hard?

The high levels are clouding my ability to problem solve about how to start to get back on track. It seems daunting. Not to mention how hungry I am!

Then there are the physical symptoms. The headaches. Oh god the headaches. They really do put a damper on your mood. I have been much to lazy to long. Quick and easy meals.

Larger and larger portions. To much white minute rice. To much mashed potatoes. Not enough fruit and vegetables. No Salads what so ever. Stuck in old patterns of eating the same thing day after day. Sick of boring foods but to lazy or tired to meal prep and plan a head. Or lack of time. Its amazing how fast your day goes by between feedings and nap time and play time. I can only imagine how it will change as she grows even more. Or when she starts to crawl! Then I will really be in for it.

Now that she is starting to eat solid foods it is even more important that I learn to branch out and in coles words ” not be a lazy cook” dare to experiment.

I have always been so scared to experiment , being such a picky eater , i was worried if I didn’t like it , it would be wasted and we all know how precious and expensive food is. Specially low protein food.

I need to re focus going forward and make my first goal to get these levels down ASAP. I cant even begin to describe how it feels to see and feel levels this high after having levels under 3 for 10 months. I knew they where high based on how I am feeling ,but seeing it.. well thats the real shock to the system. Seeing it in the email from the dietitian made it right there in my face. I could not hide from it, deny it , or ignore it. There was that big bold number staring out at me. Calling to me and reminding me of my failure to stick to my goals and to do right by Madelyn. She deserves me to be at my very best so I can give her the best possible care and healthy life too.

After I get them down, and while getting them down, I need to find new ways to cook and prepare meals. To make things more exciting. To put my time management skills to the test and plan things out. Take the time to try new things. One of my biggest challenges will be healthy pasta recipes since it will be my biggest staple for dinners and I am bored of the 3 ways I always cook it. Also new ways to eat vegetables and incorporate them and fruit into my day to day. Other then just eating them raw with dip.

So this is where I am again. This is the challenge ahead of me , with yet another chance to get it right.

As I always say ” one meal at a time, one day at a time” and ” every day is a fresh start and a new chance to do better than yesterday” words I need to learn to live by and not just speak.

Day 1 :

Breakfast:

Bettermilk

Low protein cambrooke omelette

Lunch :

bettermilk

Raw Vegtable platter

Dinner :

Bettemilk

Roasted veggies and Aprotein Penne Pasta.

Managing the diet, PKU Photo A Day

May 3rd 2016 #PKUPhotoADay

Posted on May 3, 2016 by Amanda Horner

PKU infographic and facts:

wheel

The Diet and Formula are the oldest and most well researched and tested treatments for PKU.

When PKU was first discovered in the late 1960s , treatment started with the amino acid formula.

Secondly it was the low protein diet as the main treatment for PKU. Patients with PKU where placed on a restricted diet with limited protein. The less protein in the diet, the less phenylalanine in the blood.

They actually use to think that PKU patients could come off the diet after age 7 as they use to believe the brain stopped developing. Patients who were taken off diet at such a young age, now suffer from delays, developmental and social disabilities. Thoug they are not mentally handicapped. Being taken off diet as children has impacted their ability to be high functioning , maintain relationships, hold steady employment, impaired reading and writing skills , learning troubles, or struggle with depression and anxiety. As well as challenges maintaining diet and compliance to diet and managing lower phe levels.

It was in my generation when scientists finally had enough evidence to prove Diet should be for life. There is no cure for PKU. You do not outgrow it, and your brain never stops developing.

The Low Protein / Low Phe Diet consists of :

Mediabolic amino acid formula

Medically necessary Low protein food products that are artificially made not to have protein or phenylalanine in them. These foods range from baking mixes, Pastas, pre made frozen foods made specially by Cambrooke foods and more.

Limited amounts of Fruits, vegetables, fats , spreads, oils and spices. Some hard candies and fruit juices are also low in phe.

Foods high in phe or protein such as Meat, Dairy, Fish / Shellfish, Nuts / Seeds, Legumes, beans, peas , Soy, Tofu, eggs, grains, and regular pasta must be avoided. Also higher protein vegetables like corn and potatoes must be limited.

Low pro food / cooking, Managing the diet, PKU Photo A Day

May 2nd 2016 #PKUphotoaday

Posted on May 2, 2016 by Amanda Horner

2016-05-02 09.40.26

May 2nd 2016
‪#‎pkuphotoaday‬ bottoms up/formula

One of the main treatments of phenylketonuria (pku ) is a metabolic amino acid formula.

It was the first treatment for pku and is essentially a medication. It requires a prescription and dosage is based on body weight.

All of my life I am required to drink this formula 3 times a day
The formula is made up essential amino acids , vitamins , minerals and protein with out the phenylalanine that I am allergic to. My brain and my body rely heavily on my formula as it is essentially my life support system.

As a child our choices were very limited and my formula was made from potatoes starch . We flavored it with strawberry nestle quick.

Now for the past 3 years I drink this new gmp / whey protein
formula called bettermilk and in flavor it wit Mio water enhancers !
I’m very lucky that I enjoy my formula and have never had issues drinking it. I drink 2 packages in the morning with 16 oz water, 1 package at lunch with 8 oz water, 2 packages at dinner with 16 oz water and each time I pick a different flavor of mio too add. My favorites are the sports , blue and orange!

It took me a while to adjust to the different taste and texture of Bettermilk. In fact at first I could not stand it. I switched to it and switched back a few times. But the benefits of GMP and bettermilk made me want to give it another try. It is very good for digestive health and I have always had tummy troubles and struggle with IBS. So it has really helped me.

Many people ask me what it tastes like, I find this a difficult question since I dont have anything to compare it to. So What I can say, is that I love it! It is sort of creamy and is suppose to be an imitation of real milk. It took me a while to adjust to that because it does not have the grittiness of regular PKU Formulas.

So what is GMP?

GMP is a naturally occurring whole protein for the dietary management of PKU . I believe it is made during the process of making cheese . Is the only form of naturally occurring protein that does not contain phenyalaine in a pure form. There is some phenylalaine in bettermilk. Approx. 23 mg per package. So be sure to account for it in your daily intake!

The formula has always been fully funded by each provincial government across Canada.

#PKU #phenylketonuria #MPKU #lowproteindiet #dietforlife #PKUAwareness #lowlevels

#Lowphe

Managing the diet, MPKU Journey!

The postpartum diet.

Posted on April 30, 2016 by Amanda Horner

It is not easy for me to admit , but i guess the first step is saying it out loud. I am struggling.

Not with motherhood or depression or life in general. With my diet.

I swore i’d say on track after my baby was born. To continue to be at my best so I could give her my best. I told myself it would be easy since id had been doing amazing for 10 months of my pregnancy. For almost a year my levels where mostly under 4.

I did a level 1 week after giving birth to Madelyn and had been eating really well. Back to a intake of 400 mg of phe, lots of fresh fruit and vegetables and low protein. Than my levels came back!

March 11th – 12.0

March 23rd : 13.6

March 28th : 15.5

April 4th : 11.1

I am feeling so defeated and shocked. I was trying so hard. Very hard to accept after all my fantastic levels during pregnancy. I was weighing , tracking and still using how much phe and drinking all my formula.

It has hit me so hard since the last level that I pretty much gave up.

I dont weigh my food anymore, I havent done any more blood tests, I am not tracking what I eat on how much phe anymore.

I eat my low protein cambrooke omelets for breakfast, I eat low protein at lunch but dinner is where I am screwing up. I am eating lots of regular rice and potatoes, corn and avocados.

I am really feeling the effects of my high levels this week and struggling to reign myself back in and to ashamed to ask for help. Not that I am sure how anyone can help me anyways. I know it is something I had to do myself, I know how to do it. I just cant seem to.

My head feels like is moving a mile a minute. Rushing. I am very emotional. I am having headaches and dizzy spells. I am very tired to. Most people would say that’s all normal since having a baby. But Madelyn is now 8 weeks old and I have had a fabulous recovery. I never experiences crazy emotion mood swings after her birth even with our breastfeeding struggles and her milk allergy. I had a few weeks of high stress but feel that I am coping much better then i would have in years past. I have felt like I have handled everything to my best and have not felt overwhelmed or defeated and have been thoroughly enjoying motherhood and my precious daughter.

Madelyn falls asleep around 10 or 11 pm and sleeps till 3 am for a feed then back to bed till 7 am so its not lack of sleep either. we have been walking a lot this week and getting out to enjoy the fresh air and lovely weather. For the most part I feel great.

I know it’s my levels. I know how I feel when my levels are off. They are off. They make my emotions much more heightened and make me much more sensitive and I begin to take things personally that would not have bothered me normally. Which is why I told all my pregnant PKU mom friends to stick to the diet after giving birth. I could not imagine caring for a brand new born in those first few weeks with high levels and feeling like this . How can I look after my daughter and not myself? How can be at your best if your levels are off. It made me angry to know end to hear these mom friends say openly how they planned to go off diet as soon as their babies where born. I wanted to scream and yell at them . I thought it was so selfish.

Now here I am.

It’s hard. It’s so hard. I am not making excuses , but I am so busy looking after Madelyn and Cole since he broke his leg and can’t do much, Plus our Dog Copper, Cat Figaro and our house and all the normal day to day that cole use to do , that I have let my own care slide.

I am the last to eat, and by the time it’s my turn I am so hungry I dont make good choices and grab something quick and high phe. I know I should do baking and have thing prepared to grab that are low so I dont have to do all the prep and cooking. But I havent had time.

My time management sucks. Honestly I have not made myself a priority. I have not made my health and my levels as high up on my list of Madelyn or Coles needs. When it comes down to it Madelyn is more important, If she needs feedings, changes , or just to be held and cuddle that’s what I am going to do. I know I can give her to cole or ask for help but I like being able to do it myself. I need to remember I am important to and look after myself to be able to give her my best, to look after her and lead by example.

I hate the way my head feels right now. Like it cant slow down. Or its moving and rushing around me when I am sitting still.

Its time to get back on track.

I reached out to my clinic and my dieticans and have a plan to reign it in in stages and steps. Not my all or nothing attitude that sets me up for failure everytime. Ive always been about quick results and doing it all at once. I need to do this right , one day at a time. One meal at a time and show myself some compassion. To not be so hard on myslef and not give up when I make a mistake. To shake it off and try again at the next meal or the next day.

For Madelyn , For Cole and for myself.

I can do this. why can’t I do this ? it was so easy when I was pregnant. I had all the incentive in the world and I still do when I look into my daughter’s eyes and she smiles up at me with her big bright blue eyes, toothless grins and coos at me! 1461953448936

I can do this. I can do this, I can do to this!

BC Residents and news, Levels, Managing the diet, News

Interview with Nathan

Posted on April 19, 2016April 19, 2016 by Amanda Horner

Back in february before Madelyn was born , i was corresponding with Nathan Kuehne the young man from Victoria who is working on the home phe testing device.

After watching his amazing tedx talk on the device that was posted to youtube back in december i had a few questions so i wrote an email to nathan , and he has allowed me to share his responses on my blog.

Amanda :

From what I understand , the test turns purple in the presence of PHE. However since we area always eating protein and a limited amount of phe is always in our blood, this would mean that the test should always read positive? What I would like to know, is have you developed a way to accurately detect and read in numbers just how much phe is in the urine? Like reading? it is not enough to just have the presence detected, for treatment, we need to be able to read how much is present. Like on the home tests being developed with blood, they have a screen that produces a digital number. Will your device be able to do the same?

Nathan:

First off, I need to preface my answers to all the questions with a bit of a disclaimer. Due to the nature of a TEDx talk, it is hard to delve into the chemistry in sufficient detail to cover all the aspects of the project. As a result, many of the obvious questions that would arise from my work, especially for a PKU patient, would not be found online in the talk, but are questions I have definitely considered and I include in my other presentations (competitions, Science Fair, interviews, ect.)

Anyway, the answer to your question is both yes, and no. You are absolutely correct, any Phe will start the chemical process and produce nanoparticles, and thus the colour change. Having said this, the reaction is not a runaway train after that (bad analogy, my apologies). Only so many nanoparticles will be produced as there is Phe in the solution. This means a gradient will be produced depending on the amount of Phe in the sample. The more Phe, the darker the solution; I’ve included a picture that demonstrates this more easily than I can in words (Figure 1).

With this in mind, I was planning on developing something along the lines of a pH chart for people to read their levels. It could be a small piece of paper or something equivalent, and it would show a gradient of purple, and what concentration each colour corresponded to. This would give people the ability to attach a numerical value for blood concentration to their visual test, and thus determine how much is present.

Alternatives to the chart include using solution conductivity. Since the solution produces gold nanoparticles, the conductivity of the solution would increase as the amount of Phe increases. This way, I could imbed a program that converts solution conductivity to an actual value, taking away the risk associated with visual interpretation of a chart. While I acknowledge that this would be a much better method to go about the test, as a high-school student, it is slightly out of my reach at the moment to pursue and develop electronic integration to my test, therefore I am more included to pursue the chart at the moment. However, this may change as I continue to work on it.

Amanda :

my second question is : Many of the home tests in developed by the NPKUA and previously by Biomarin, have had trouble with accuracy when phe levels like mine are in the low range. Does your device operate similarly? how can you improve the accuracy for lower range levels? Idealy PHE levels in anyone should be 2-6 mg / dl so that would be considerably lower then say someone who is suffering from high levels. My highest level I ever had was 25 mg/ dl.

Nathan:

As I mentioned in my answer to the first question, there is a lot of more complex experimentation I leave out of my presentations, and this is one of them. As part of my research, I did tests regarding a concept called the Limit of Detection, or the LOD. Essentially, this is a test that determines how sensitive my test is, and gives me a value of how low the concentration of Phe can be for me to know if the colour change is because of Phe or if the colour change is due to experimental error.

My LOD was below that of the low-end range of Phe levels in PKU patients, according to published literature, meaning my test works for all concentrations of Phe. However, I am not 100% sure how the accuracy changes at those lower levels – that will take more research to determine. I am optimistic, considering the reliability of gold nanoparticle production with Phe, that the accuracy should not change very much.

Amanda:

There are 2 systems for reading PHE results. In canada we use the milligrams per deciliter and in the states or the uk we see that they use the umol/ L system . where levels range is 120-360 . What system would your device use?

Nathan:

Regardless if I pursue the chart system, or the conductivity system, since mg/dl to µM/L is a simple conversion, it would be very to make my test useable in both locations, with both sets of units included.

Amanda :

have you been approached or applied to either the NPKUA, CanPKU or the government for any sort of assistance in developing your device or plan to?

Nathan:

I have not been approached nor have I applied to any agency or organization for assistance, and this is for a few reasons. The main reason is that I am working on a slightly different project at the moment, which may replace the device I developed and presented in my TEDx talk. I am trying to port the chemical aspect of the test to a specially designed piece of paper, and as a result, remove the need for the device. Because as much as I like it and am proud of it, it is large and fairly cumbersome. As an extension, this new test would, theoretically, include the capability to test for types of cancer, Alzheimer’s, and other metabolic disorders like PKU. It is still in the research phase, and as a result, I can’t speak to whether it will be successful or not. If all goes well, this new platform will be the method of testing for PKU.

If it is not successful, I will consider returning to the device, and trying to optimize parts of that system. With this in mind, I have not sought out funding or support yet because, quite honestly, I am not sure what will work best and as a result, what I may try and develop further.

Amanda :

And lastly, if it is ok with you, once you have replied, may i publish this conversation to my blog? with your permission of course. I think these are all questions we have had since you have entered our world and it has spurred many conversation on social media.

Nathan:

Yes, I would be happy for you to share my answers. I have been in contact with a few other members of the PKU community as a result of places I have posted my talk, and I would love for these answers to reach people like them.

Managing the diet

How much PHE ?

Posted on November 3, 2015 by Amanda Horner

I have been meaning for a while to write a post dedicated to the amazing new website “howmuchphe.org” but havent been quit able to find the right words.

Many moons ago I wrote a blog post describing Virginia Schuettes PKU Food list binder as the holy bible for PKU. Now I can say, how much phe, which is essentially an online version of the food list binder is the holy grail!

As many of my regular readers know, Prior to becoming pregnant, I was fairly liberal with my diet. I did not really eat things I could not eat. I didn’t cheat with meat or high protein foods. But i was not exactly strict.

I “eyeballed” my portions and measurements. I used rough ” cup ” serving sizes and did not use my scale. I did not keep a food log or write down in such details what I was eating day to day.

I did however eat things like Rice, Avocado sushi, yam sushi, vegetable sushi, hash browns, potatoes, corn, and sometimes white wonder bread as toast. I even tried maple brown sugar oatmeal a few times.

Over the years I tried numerous times to rein in my eating habits, either on a health kick to lose weight , or attempt the preconception diet so i can prove to cole that I could do it at all. I used the food list book and a trusty old notebook.

Since becoming pregnant I bought a new notebook and began tracking immediately after that first positive pregnancy test. i had the adobe digital additions copy of the latest food list but had not been able to afford the annual cost of how much phe yet. Prior to becoming pregnant i saw no need to justify paying for it since I knew i would not use it as its attended or as often to justify the price. As soon as I had a little extra cash after becoming pregnant i went and got a pre paid credit card and signed up as soon as I could.

At first i was using it just to look up the nutritional information such as phe and calories to record the values in my notebook. I had really forgotten what the mg of phe was for the things i was eating. I feel like these past few months I have been learning the whole diet all over again. Talk about Crash course. All of a sudden I was having to do math again, hitting my targets every day. Not to high and not to low. Balancing calories and PHe together was a new learning lesson for me as well. Changing portion sizes to fit the phe i needed. IT is a juggling act and I have a newfound appreciation for parents who do this for their children every single day. It has been adjustment for me. I am so use to eating whatever I want as long as it was low protein so my portion sizes were quite large. I remember in the first trimester being worried id be hungry when i saw what the real portion sizes were but morning sickness helped with that .

The first few weeks of relearning the diet where a lot less intimidating thanks to how much phe and the facebook community. I spent days learning the ins and outs of the site. I feel in love with how easy it was to navigate and use. i only wished it was more like myfitnesspal where you can record right on the site.. only to discovered a few weeks later that was in the works! I jumped at the chance to be apart of the beta testing group. It was another adjustment to give up my notebook, though I continued to use it during the beta testing just to be sure. Since how much phe has gone live with the tracking changes My life has been so much easier!!

Now I can keep a daily log of my food, record my PHE levels, my weight, height and notes. I can also export it to my email and forward it directly to my clinic. I have it saved as a web app on all my devices and set to automatically logged in. So as long as I have wifi I can access it any time. I can use my data on my phone and access it when I am out to. This has come in super handy at the grocery store when I am looking up nutritional info on a new food or something I want to try.

How much phe is seriously user friendly and has additon of the fb group for added support and direct contact to Sarah for trouble shooting. The colours are very easy on the eyes and the lay out really flows.

When you login you have the main search page , after you have enabled tracking your site opens up to your favorites page. So foods you use frequently are right there. When you open up the food you see the name of the food and the nutritional info down the main page. On the Right you can calculate by weight, portion and PHE. Enter in your prefered method. I have used all of them. I usually calculated by grams of weight but at the end of the day when i am planning my dinner ahead and I have so many points left that i need to make up, I will choose the food I want and enter in how much PHE i need to eat and it does the math for me. To do the math you use the green calculator button. I am so relieved it does the math for you. I really really hate math and am just terrible at it. I have never understood it . It’s a foreign language to me. I don’t even know all my multiplications tables and I never learnt to divide. Admittedly I still count on my fingers or use a calculator and i am never confident that its correct so this feature takes the stress right out for me.

The orange arrows is the refresh button to erase your data and refresh to defult values.

The fork and knife is how you record it to your daily log.

When you log in for the first time you can set up your profile and change your personal settings. You have the option to track as a parent or caregiver. This is so great for family members especially grandparents. You can have multiple profiles so if you have 1 or more children with PKU you need to track for you can keep them easily separate.

You then choose your default way to calculate, and enter in your daily intake goals. Set how you track your levels ( mg/dl ) or (mm/L ) , if you are on Kuvan, If you record Tyrsoine levels. The next tab is for diet. Set your PHE target, Calories, and protein equivalent. This is what you see now at the top of your screen every day when you log in. As you record your meals and each food or formula the talley changes and also shows you what remains for that day.

Mostly I just use my favorites but occasionally I come across a food not listed or need to add in extras like tyrosine. So you have a fast track option ( the lightening bolt) This lets you quickly add something if you know the PHE and calories. For me I use it to enter in my tyrosine. 0 grams protein, 0 calories, 5 grams tyrosine twice a day so this shows my clinic and me that i have already had it or if i missed a day. ( not that I have) I have also used the quick add button to enter in jumbo freezies ( phe free only calories) and becel margarine. In the note section i describe what the quick add is for .

Other features to familiarize yourself with is, the search, settings, about , and account features. Under the account tab is where you will find a graph of your values. Intake, Blood levels, weight, height , notes and exporting.

To export you simply select the date range you are looking for, and then select the information you want to export . When the file is ready it is emailed to you and then you can save it in document form to your computer, print it off for your records or forward the email to your clinic.

When you get use to it and you use it enough, you will find it is so simple to use. Not at all overwhelming, fast , convenient and takes the stress out of compliance for adults like me.

I highly recommend How much phe to anyone. I really feel it is totally worth the price to and have no issue paying the annual cost. It really is quit small for the big picture. Also it is for a good cause. The funds help continue the hard work of the how much phe team. It helps them produce the site, and also to evaluate foods, research nutritional information and work with companies to get the info and provide it for us.

I think everyone can benefit from using this website , it has seriously impacted my PKU life and has made managing my levels and diet much easier and doable. I had so much fear prior to becoming pregnant about being able to be compliant and diligent and stick to a restricted died, How much phe , I feel has made it totally possible and almost easy. I am fully confident I am doing my very best for me and my baby and that I might not have been so sucessful without this great tool . Seriously every household should subscribe . Specially children and women with PKU. I am so appreciative and thank you to the How much phe team and producers . Gracious does not even describe. If I could afford to i’d buy it for all my pku friends who are pregnant or on the preconception diet as I feel it is that important. I will continue to use this tool for the duration of my pregnancy and breastfeeding and into the future to help stay on diet and healthy.

Thank you How much PHE! I have read many articles that stress the importance of a multi prong approach to treatment of PKU. The low protein diet / medical foods, Formula, and pharmaceuticals like KUVAN. I think it should be expanded to include how much phe. As the more tools in the tool the box, the easier to manage and achieve optimal quality of life!

IF you are interested in checking it out, please visit the site and http://www.howmuchphe.org and if you have any questions please feel free to ask me . You can also join the Howmuchphe fb group for great support and direct access to the team who runs it. They have very quick responses to!

I am PKU Strong

I am PKU Strong

My life with Phenylketonuria

Category: Managing the diet

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Interview with Nathan

How much PHE ?