Sleepy?

Recently my little monkey has struggled with her sleep, fighting it and getting so worked up that it take awhile to get her to nap... Once she sleeps she's a very light sleeper and until fully settled she's very jumpy.

After a bit of a nap she wakes up still seeming tired and not fully with it. At night she then becomes very hard to get to sleep properly until about 1am... Crying and getting really worked up, she never had much energy at all...

This is a very recent thing... Does this sound like your little one? Fear not!

PKU'ers struggle with energy levels due to the small amounts of protein, throw weaning and teething into the mix and you have a very energy deprived little one...

But things won't always be this tricky I noticed something on our prescription letter I had come across so looked it out, found out it's an energy supplier to be introduced into our PKU'ers routine. This will help make a difference as they grow as they will be getting less from the small amounts of protein...

So all I will say is if a sleep pattern changes and your PKU'er acts out of character don't worry this is to be expected... But let's not pin PKU on everything 😂😂 we don't want to excuse any naughty behaviour on PKU... But don't panic if sleeping patterns change, I've been told that this is pretty common in PKU so do not worry

I hope this helps you all in the future when tired and worried that you'll never get those lovely 8 hour sleeps back they will soon enough!

Weaning - Food Diary

Breakfast
At 8am I have my first bottle of cow & gate and chill

At 10am I sit in my highchair for some yummy fruit sometimes strawberries, sometimes peach... However when my rusks arrive I will have rusks and milk yummy!

Lunch
At 12pm I get another bottle of cow & gate, when I have established three meals I will no longer get cow & gate but measured amounts of protein foods like peas at dinner time... Ummm not sure I'm gonna like peas otherwise I'm making this whole weaning thing to easy for mummy and dada

At 2pm I get put back in my highchair (I like it I'm like the queen demanding my food) I normally don't have lunch but it's new and interesting and this is where I get fruit yummy! I'm just waiting on those rusk thingys then it will all change.

Dinner
At 4pm I have another bottle of cow & gate yum yum, I will of course be loosing this privilege soon once my mummy and dada change it to something else... I heard them say mash potatoes, whatever that is...

6pm we're finally at dinner time. At the moment I have vegetables like sweet potato, califlower, carrot... But when my pasta arrives I will have pasta with vegetables mixed in, this sounds interesting I must say...

8pm I get a bottle of milk or sno-pro and then I fall asleep and dream. Of course things will change soon, I won't have that Anamix milk I'll have gel, squeezies or first spoon starting off once a day (breakfast) moving on to three times a day (before each meal) then of course I will have my measured protein instead of cow & gate and drink water and juice instead of milk... Mummy and dada promise I'll get my bottle of milk before bed every night... I'll hold them to that!

Of course being a baby human my times do change a but for my cow & gate but mummy and dada still make sure my food is the same time everyday, they did say something about that changing when I'm not on cow & gate to 9am, 1pm and 5pm so that I get bathed and in bed by 8pm after my bottle of milk of course.

Well that's my diary for now... When I'm even older it will completely change again!

Thanks for reading
Amelia Xx

When It All Changed

To tell the true story of Amelia's journey we have to start at the very beginning...

January 5th 2016 Amelia just 5 days old had her heel prick, all children have this check done at this age. However this became a crucial check for Amelia. Like most people I didn't fully know much about the 5 day check, all I knew is you don't hear anything unless something is wrong.

On January 7th I got a phone call while out at the range shopping with my new beautiful baby and my husband. I felt amazing I enjoyed my new purpose as a mummy to this little baby. However when I got the call my heart sank a little, all they told me was that Amelia had Phenylketonuria and that we needed to get to Bristol hospital the following day as it was urgent.

On the 8th January we learnt all we could about PKU we embraced it and have since found it easy to come to terms with. I still get a pain in my heart every now and then but I know that my little girl will flourish and become a gorgeous healthily girl full of fun and loving memories. We couldn't however have become so relaxed about this without the awesome support we have received from Bristol and Truro hospital and Amelia has an amazing team ready to help whenever we need it.

All I think about is if Amelua was born when this early screening wasn't done she wouldn't have the chance she has today, I take that as a blessing and remind myself that it could have been so much worse. I'm very lucky that I have Amelia in my life, I wouldn't change a thing. We are on this journey together.

When I have my next baby, the screening will be even earlier after 48hours of being in the new world the heel prick will be done to find out even earlier is they also have PKU... And between you and me as bad as it may sound to other people, I would hope that they also have PKU... I know for people reading this not in this situation will find it very hard to understand, it sounds like I'm wishing an illness on my not even here child but it would hurt even more to see them both being brought up differently... But who knows what will happen...

All I know right now is I have a daughter with PKU, she was screened early enough for us to come to terms with her condition, early enough to start the monitoring and do something to stop any damage happening.

I also know that Amelia Rose Meaney may have PKU but she has no limits, she will achieve whatever she wants most most of all she is awesome and so much braver than anyone I have ever met.

I love my daughter no matter what!

Fruit & Vegetables

Fruit allowed freely in the diet

- Apples
- Apricots (fresh & dried)
- Bilberries
- Blackberries
- Cherries
- Clementines
- Cranberries
- Currants
- Damsons
- Figs
- Ginger
- Gooseberries
- Grapes
- Grapefruit
- Guava
- Kiwi
- Kumquat
- Lemon
- Lime
- Loganberries
- Lychee
- Mandarin
- Mango
- Melon
- Mixed peel mulberries
- Nectarine
- Olives
- Orange
- Papaya
- Peach (not dried)
- Pear
- Pineapple
- Plum
- Pomegranate
- Prunes
- Quince
- Rasins
- Raspberries
- Rhubarb
- Satsuma
- Strawberries
- Sultanas
- Sharon fruit
- Star fruit
- Tangerine

Vegetables allowed freely in the diet

- Artichoke (globe and Jerusalem)
- Aubergine
- Beetroot
- Cabbage
- Carrots
- Cassava
- Cauliflower
- Celeriac
- Chicory
- Courgettes
- Cucumber
- Endive
- Fennel
- French Beans
- Gerkin
- Herbs - all free
- Lady Fingers (okra)
- Leek
- Lettuce
- Marrow
- Mushrooms
- Mustard & Cress
- Onion
- Parsnip
- Pepper (green/red)
- Pumpkin
- Pickled Vegetables
- Radish
- Squash (butternut, acorn, spaghetti)
- Swede
- Sweet Potato
- Tomato
- Turnip
- Watercress
- Water Chestnuts

Fruits & Vegetables that need to be limited to 1 serving per day

Fruit

- Bananas
- Banana Chips
- Dates
- Figs (dried only)
- Peaches (dried only)
- Physalis
- Mixed dry fruit (1 small packet a day)

Vegetables

- Asparagus
- Beansprouts
- Broccoli
- Plantain
- Runner Beans
- Seakale

Weaning - Stage Three

Low protein finger foods

By the age of 7 months your baby might want to start feeding themselves. This should be encouraged with the use of low protein finger foods.

Low protein finger foods:

- Lightly cooked free vegetables i.e soft carrot sticks
- Soft pieces of free fruit i.e pear, peach, banana
- Low protein rusks
- Toasted fingers of low protein bread
- Low protein pasta
- Low protein cereal
- Low protein biscuits

Remember never leave children unattended when eating

Low protein milk

At this stage you might want to start introducing a low protein milk. Currently there are 4 on the market: Prozero, Sno-pro, Loprofin and Taranus Dalia PKU drink. These can be used in recipes to replace milk and from the age of 9 months they can be introduced as a milk replacement drink.

Weaning - Stage Two Continued

Introduction of Exchanges

If we fed a measured amount of 1 protein exchange of food we will have to decrease the infant formula or breastmilk by 1 protein exchange. The aim will be to swap out 1 protein exchange at a time. This can be done every 1 to 2 weeks or slower depending on your baby.

Please see the list below for some first home cooked protein exchanges

List of protein exchanges (1g of protein)

- 80g of boiled/mashed potato
- 45g of boiled rice
- 25g of peas (fresh/frozen)
- 25g of boiled spinach
- 20g of baked beans
- 10g of weetabix (not before 6 months)

Using exchange free foods

It is important to keep using exchange free foods and low protein prescribed foods to complement the exchanges and to provide energy for your baby to grow.

Second stage protein substitute 

At this stage your baby's intake of PKU Anamix Infant may start to decrease as they are eating more solids. It is very important that they receive a set amount of phenylalanine free protein so it will be time to start them on the second stage protein substitutes. There are a few different products available: PKU Anamix First Spoon, PKU Gel and PKU Squeezie.

Weaning - Stage Two

Starting Exchange Foods

The next step will be to add in protein into your baby's food. This is done in the form of protein exchanges. The term protein exchange is used to define 1g of protein which contains 50mg of phenylalanine. Currently your baby's protein exchanges are being provided by their infant formula or breastmilk. When we start to introduce 1 exchange from food we will have to decrease your baby's intake of infant formula or breastmilk by 1 exchange.

Calculating Exchanges

How to calculate a 1g protein exchange from a food label:

100 divided by total protein in 100g of product = 1 protein exchange (amount of food in grams)

Example

Nutritional label per 100g of vegetable baby jar

Energy - 50kcals
Protein - 2.4g
Carbohydrate - 9.4g
Fat - 0.3g

Look at the protein content per 100g (found on the nutrition information label)
e.g 2.4g per 100g

Divide this protein figure into 100 and this will give you the amount of food for a 1g protein exchange e.g
100 / 2.4 = 41.6

Round up or down to the nearest 1g = 42g

So 42g of this vegetable baby jar = 1 exchange

It is important to calculate foods such as baby rice from the protein per 100g of dry product. The weighed exchange amount can then be made up with PKU Ananix Infant or water.

Example

Nutritional label per 100g of dry baby rice

Energy - 378kcals
Protein - 7.4g
Carbohydrate - 85g
Fat - 0.9g

Look at the protein content per 100g (found on the nutrition information label)
e.g 7.4g per 100g

Divide this protein figure into 100 and this will give you the amount of food for a 1g protein exchange e.g
100 / 7.4 = 13.5

Round up or down to the nearest 1g = 14g

So 14g of this baby rice = 1 protein exchange

Scales

It is advised using digital scales to weigh exchanges of food accurately. When buying scales look for ones that can weigh in 1g increments and that are light weight and portable. They can be purchased from catalogue stores, chemists, large department stores and supermarkets. Try and find one that can measure 0.1g and up for more accuracy.

PKU Weaning Stage One

The first foods you will offer your baby are what we call exchange free foods as they are very low in protein. When you start introducing solids choose a relaxed time of day for both you and your baby. Start with 1 meal a day, initially it will be only 1-5 teaspoons and gradually build this up to 3 meals per day.

To make your meals choose from the foods below:

Fruit

Apples
Apricots
Banana (no more than one small one per day)
Mango
Pear
Peach

Vegetables

French beans
Carrots
Cauliflower
Parsnip
Swede
Sweet potato

Low Protein Prescription Foods

Anamix, low protein rusks (these can be softened with water or PKU Anamix infant or PKU start formula)
Promin, low protein pasta meal

Very Low Protein Baby Jars
If a baby jar has 0.5g or less protein per 100g it is allowed as a free food in the diet

Aspartame
Aspartame is an artificial sweetener which contains phenylalanine and is therefore not included in the diet. It can be found in fizzy drinks, squash, cordial, puddings, chewing gum, and artificial sweeteners such as canderel. On food labels it will appear as: Aspartame or E951 and it should be stated that it is a source of phenylalanine. Please note that aspartame can also be found in medication so it is important to check this with your GP and pharmacist.

Protein
The protein in your baby's diet is being provided by the phenylalanine free formula PKU Anamix Infant and the infant formula or breastmilk. It is important that at this stage these volumes stay the same as prescribed by your dietitian. Once you have established 3 free meals per day the next stage is to introduce protein into the meals in the form of exchanges and reduce the amount of infant formula or breastmilk accordingly.

Old School PKU

Being as it's PKU awareness month I took on the PKUphotoaday challenge... Today's challenge is a hard one, all the others have been easy, light hearted and quite fun to do... This one is my take on what the challenge is asking for... So please grab your tissues, prepare yourself but most of all be thankful for the advances in diagnosis and treatment we are lucky to have now.

Old School PKU

I looked into how different things were for people with PKU, I'm only talking 20-30 years ago. There was no screening at 5 days old... This meant high levels of brain damage, there were lots of children with development issues being put into institutions because of this, they had speech problems and were not able to look after themselves.

When screening did begin there was as much treatment nore was there clear understanding, reading stories of other people now in their 40s, struggling to get back on their diet as they were told at different ages they didn't need to be on it anymore, this still cause development issues and behavioural issues too.

Even looking 10 years ago at the choices of food for PKU'ers, it must have been a very hard diet to stick to with only 1 or 2 companies providing very minimal low protein or completely free from foods.

But we look at now 2015... I am blessed, pleased, releaved that this is the year my daughter was born, she had a diagnosis at 7 days old, her diet started straight away, the knowledge and monitoring continues to today, 4 months on... The support is amazing. One thing is for sure, yes it was a shock but I know I wouldn't be as calm, level, balanced or relaxed about PKU as I am now, and I still well up thinking about how lucky we are but how horrible it must be for families who go through this before the advancements in the treatment of PKU.

I raise awareness for this reason, it isn't as scary, it is dangerous but only if you don't follow suggestions, stick to your diet the best you can and you will live a full life... No one and nothing should limit you just because of PKU... It's three letters, not the end of the world... Your strong, your awesome!

Let's all help others realise just how lucky they all are, because things could have been so very different...

Poem for Pops

My pops gets to eat steak

He even gets a chocolate birthday cake

He doesn’t have to measure the chips

But if you ask me, it will go straight to his hips

My pops smiles as he eats his roast dinner

But I think I am going to be thinner

He eats lots of cheese and drinks all the milk

But I have hair that is just like silk

I think he sneaks slices of bread

I can hear him as I lay in bed

You might think this unfair

But my pops hasn’t got a whole head of hair

He says he eats it to stop my temptation

I get lots of fruit I love that sensation

My pops plays with me and my cousins too

I may have PKU

But I am lucky I have a pops as awesome as you

A Letter For My Little Girl

Dear Amelia,

I look at you and feel such pride, I still find it hard to believe that your my awesome little girl. What did I do to deserve such a brave, amazing, gorgeous little girl like you? And yet I still feel a huge amount of sorrow and guilt when I look into your bright blue eyes. I have accepted your condition and embraced it, lighthearted blogs, information and research has made this easy for me to accept. However how can I accept something like this so easily when it's you that has to live with it...

Your wobbly gene means that you will be monitored for the rest of your life, we don't know how you will react when you cannot have steak, chicken or fish... I'm interested to see what your food will be like at your wedding (if you decide to get married of course) will your partner have PKU? Will you rebel and sneak a mars bar when no one is looking... Will you feel guilty will it make you poorly... I want to protect you but I can't stop you living.

I love your smile, your growls, your beauty and character even after week after week of blood tests, the monitoring, the anxious wait we go through just to know your levels are in range... The longing for your milestones to be reached, for you to remain a positive, playful and funny little girl as you grow and your journey is set.

I love how we are learning this with you and as we watch you grow and my little baby turns into a little girl, how we will adapt so it isn't no you can't have chocolate, but here have this chocolate instead... But I'm sorry you still cannot have the steak, chicken or fish.

You are my gorgeous girl, yes you have PKU but without it you wouldn't be you, it does not limit you, it does not define you, you are awesome because you are you! And I will love, support and stand by you as we start this interesting and sometimes challenging journey together... I understand if you want to cry and shout that it's not fair, I'll understand when you yell that you hate me when I say no you cannot have steak and chips for tea as I will know deep down its for your own good, deep down you'll know that too...

I hope you will remain the strong funny character that you are already and if you decide you want to try something on the "forbidden" list you'll come to me first, I want you to experience freedom but I want you to be safe... Your my awesome little girl and I look forward to watching you grow and prove that PKU are just three little letters and that you are far bigger and better than PKU will ever be... You have no limits, you are amazing, you are Amelia and nothing will change that.

Love forever and always

Your proud mummy and daddy
Xx
🍏🍎🍐🍊🍋🍌🍉🍇🍓🍈🍒🍑🍍🍅🍆🌶🌽🍋🍊🍐🍎🍉🍇

What Is PKU?

Phenylketonuria (PKU) - is a rare but manageable condition which affects the way the body uses protein. Amelia was diagnosed with PKU when she had her heel prick at 5 days old. We had never heard of this condition before so now we are learning and raising awareness of this condition.

Children with PKU cannot use part of protein called Phenylalanine (Phe) if left untreated, Phe builds up in the body and can cause brain damage. However this condition is easy managed by following a special diet and people with PKU can still live normal lives and go on to have successful careers, go to university and enjoy a happy family life like everyone else.

How Did Amelia Get PKU?

As a parent to a child with PKU both Me and my husband have one PKU gene and one normal gene. This is known as being a carrier. Your body then chooses which gene is passed on when conceiving a baby, Amelia inherited 2 PKU genes - one from me and one from her dad.

Around 1 in 50 people carry the PKU gene and would never know that they were carriers until two people with this gene conceive a baby with PKU. Once me and Lawrence decided to have a baby there was 1 in 4 chance that our child would get PKU.

The Science of PKU

We all need protein to help build, repair and maintain body cells and tissues, like skin, muscles, organs, blood and bones.

When protein is eaten, it is broken down in the body (during digestion) into smaller pieces called amino acids. There are 20 amino acids that make up protein. Some of these are essential and cannot be made in the body so they must come from the food we eat. Phenylalanine (Phe) is one of these. It is this particular amino acid that Amelia cannot process properly because of PKU.

Normally the liver produces an enzyme called phenylalanine hydroxylase. This enzyme breaks down Phenylalanine (Phe) to another chemical called Tyrosine (Tyr) which is important for many functions in the body. In a person that has PKU this process does not happen as it should... The phenylalanine hydroxylase is not made by the liver or the enzyme does not work properly. This means phenylalanine (Phe)  is not converted to Tyrosine (Tyr) and builds up in the blood. This is the reason for a special diet to be followed. So there is too much Phenylalanine (Phe) and not enough Tyrosine (Tyr) which can cause problems. Therefore there is a need for extra Tyrosine (Tyr) in Amelia's diet.

The Special Diet

- Phenylalanine (Phe) is found in all protein containing foods (meat, milk, eggs etc)

- In order to manage PKU all protein foods must be restricted - and a low protein/Phe diet must be followed

- All amino acids have a role or function in the body. Phenylalanine (Phe) helps us with our growth, development and tissue repair.

- It is important that someone with PKU gets some Phenylalanine (Phe) but not so much that it becomes harmful.

Following the traffic light system and knowing which colour a food falls under is key.
- Red (meat, pasta, cheese, bread, fish etc) are not allowed as these are too high in protein/Phe.

- Amber (potatoes, peas, rice, frozen chips etc) contain small amounts of protein. They would need to be eaten in measured amounts - these amounts vary from person to person as each person has a different level of tolerance to protein/Phe.

- Green (fruit, vegetables, sugar etc) are naturally low in protein so are good to have.

Also people like Amelia with PKU will get special foods which have been specially made to be low in protein so special bread, pasta, cake etc are available when Amelia is older.

Monitoring

This condition needs monitoring from an early age which means Amelia has to have weekly blood spot tests to check her Phenylalanine levels and going to the children's hospital in Bristol 3 times a year. She will have a dietitian for life and as she grows up she will be referred to different people to help with her development. Both me and Lawrence will be trained to do her blood spot tests and eventually so will Amelia.

So follow our journey as we learn more about Phenylketonuria (PKU) and follow Amelia's development and learn about her special diet with us...