PKU GEL CHALLENGES

K has been teething very badly. He only had two teeth appeared before he turned 1 years old but since his birthday he had four teeth all at once coming through. At points he was so much in pain, he would throw himself onto the floor. He would even push me away if I tried to cuddle him. This is so unlike of K, he loves his cuddles.

With all this going on, he refused the gel with passion. He was in terrible tears, screamed like I poured some chemical that burnt his mouth. He would not take his gel at all, so we could not move onto protein food. I spent all day every day trying to get him eat his 2 full sachets of PKU Gel. It was extremely stressful. I felt so hopeless and had few 'I don't know what to do but to cry' moments. We asked for help and advice from the dietician and the paediatrician on our visit to GOSH. They explained the behaviour which made a lot of sense but I still felt little daunted as it confirmed back that I had to go through the screaming, tears without showing any stress and just had to persevere. Apparently, children at this age (1 year) refuse food, period. Normally parents walk away from the situation and try feeding them later but with PKU child the fight goes on because they have to eat their gel and protein, no choice. You cannot walk away so you have to be strong and persevere. We discussed the option of trying a different substitute with the dietician. We were going to try Anamix Junior powder which you can mix into his milk as an option. This sounded very promising as K loves his milk. I hoped he would not notice any difference to his milk and drink this will take away a lot of the stress. The other benefit for us would be that if he took his substitute with his milk, he will have more room for the 8 exchanges at meal times as he struggles to eat all 8 exchanges a day.

We received our sample pack of Anamix Junior a week ago. They were flavoured so K immediately could tell, it wasn't his usual milk so refused to take it. I tried lots of different tactics but every time he refused so it wasn't as easy as I anticipated. Luckily, he's been much easier at taking his PKU Gel so that is a huge relief. We have now received unflavoured ones, I tried again with mixing into his milk, he refused. He could tell straight away. I stuck to it and he finally took the bottle. Phew! I think I just have to build his taste to it. 

KUVAN - Government Consultation

There is currently an NHS consultation for KUVAN treatment to be made available through NHS in UK. Deadline is 23rd April 2015. If you haven't  already please go fill in the form.


INSTRUCTIONS:

Click on link here

1. Scroll to bottom, click on “Complete the public consultation here”
2. Complete “About You”
3. Subject of response is “Clinical Commissioning Policy”
4. Select “Sapropterin for Children Clinic Commissioning Policy” (4th from bottom)
5. Click yes - top and no at bottom - then submit.

If you want to put something in the comment part at the end you could cut and paste something like this…

"It has been well documented that The European Commission granted a marketing authorisation valid throughout the European Union for Kuvan (BH4) on 2nd December 2008. Kuvan is now being freely prescribed and used in countries such as Netherlands, Spain, Germany, France and Italy not to mention Turkey, America and Canada - however the NHS is still resisting. There is sufficient efficacy to support the claim that Kuvan is safe and improves blood phenylalanine concentrations, relaxes diet therapy and may improve quality of life for many PKU patients."

You can also find the instructions on NSPKU Facebook page. Just look for the post on their page.

FATE SAUSAGES

Cooked sausages

K enjoying his PKU Sausages

Yesterday I decided try out a new PKU recipe. I made sausages using Fate All Purpose Mix following the recipe from Fate Cookery Book. It was a real success, K loved it. I have to say I kept putting it off from fear to be disappointed but I am now relieved. Here's some pictures.

TEETHING AND IMMUNISATIONS

K had his 1 year vaccines last Friday. He has been a little agitated and Monday he was very poorly with fever as well. Poor thing, managed to eat all his 9 exchanges and 2 sachets of PKU Gel despite teething pain and fever. He has been a very brave little soldier.

Today we received his latest blood test results. His levels is around 475. It has been outside of the range for few weeks so we decided to drop it to 8 exchanges this week. Fingers cross his level will neutralise.

1 YEAR OLD

I have been meaning to post here but it has taken me this long to find the time to do it. K turned 1 years old 2 weeks ago. We celebrated his birthday at home with grandparents. Unfortunately daddy was poorly in bed.

I was not prepared for his birthday celebration with PKU as it revolves around eating, eating and eating... I attempted a cupcake with Mevelia cake mix. Our 5 year old girl (without PKU) loved them but K has tried a little but I don't think he appreciated it. I think it was purely down to being new to cakes. Never mind he is only 1, many birthdays to come.

He is now on 9 exchanges. He is still having vegetable mash such as sweet potato, butternut squash as well as petit filous, baked beans and cottage cheese.

His favourite food is bananas. He goes apes for bananas! :) He started to feed himself with spoon, he is doing so well and enjoying his food more this way. It really helps that he doesn't fuss over his gel anymore as he is happy to feed himself.

He started bottom shuffling around Christmas, he is getting around very fast nowadays. He recently started holding onto our hands and taking few steps around the house. Early stages of walking attempts...

He is looking so healthy and happy. He makes me proud everyday.

PKU cupcake

PKU GEL AND WEANING

K is now 29 weeks. He has been weaning and getting on with his diet extremely well. He is enjoying his food and has a good appetite.

K started his weaning around 17 weeks old. He is now on stage 2 of his PKU weaning. The first stage of his weaning was just introducing him to PKU substitute -which contains essential vitamins and minerals- and some free food. We were given samples of Anamix First Spoon and Vitaflo PKU Gel sachets for K to try out once a day. We found that he didn't like the First Spoon and got on better with the PKU Gel. PKU Gels come in different flavours, he built up a taste for the raspberry one so we kept to it.

PKU Gel comes in 24g sachets. K is having half portions so we weigh out 12g and mix it with 15ml of boiled and cooled water. He has this mix before his food. We built it up from once a day to three times a day. His phe levels have been pretty good so he is now having 2g of protein (2 exchanges) a day. He also takes 60ml of Anamix and about 3 to 4 times a day breast milk.

He is a very happy baby, gives us lots of smiles. I say very often; he is almost reassuring us  'it's all right mummy and daddy don't worry about me. I am happy'.

List of Free Food that he has been enjoying:

• Sweet Potato Purée
• Butternut Squash Purée
• Peach Purée
• Pear Purée
• Carrot Purée
• Low Protein Hot Breakfast (on prescription) - He loves it mixed in with mango purée

We found that baby food jars (such as Hipp Organic, Ella's Kitchen etc) have been the best way for us to control his protein exchanges. We having been measuring out 1g protein equivalent of food and offering it after he finished his PKU Gel. If he is still hungry we offer him some free food from the above list.

LOW PROTEIN COOKERY LESSON

At the weekend, we went along to an organised Low Protein Diet Cookery Lesson in our local area. It was organised by the NHS dietician and the the company called Nutricia Metabolics which produces the PKU brand Loprofin. A chef came out to show how to cook Low Protein food for the PKU children to the parents, siblings and the PKU children themselves. It was a great event. We met new people in our local area with PKU condition .

We went as a whole family so  we took our 4 year old daughter E with us too. She does not have PKU. We let her take charge of the messy dough making, plaiting bread rolls etc. She really enjoyed it. She also loved the taste of the food. We felt so happy and relieved  that the food was actually very palatable and tasty.

On the day, the chef showed us how to make:
Bread (which was very fluffy and had a very good texture)
Vegetable Pizza
Savoury Pancakes
Muffins
Cookies

This looked all good fun. I then spotted a worktop with some substitute products lined up. This was a wake up call. Don't forget the substitutes! Food making is all fun but it is only one part of it as there is measuring, calculating exchanges and getting the child take their substitutes every meal time. I wanted to find out more so I asked the dieticians about the different types of the substitutes and tried some of them. I tried a substitute pouch flavoured in blueberry. It was strong tasting and it tasted very medicinal like a cough syrup. It was bitter and very sweet at the same time. I only tried a tiny drop less than 2.5 ml but the taste lingered. I understand now why they have the substitutes before their meals. I believe it was an 85g in the pouch which the child will need to finish. It's rather alot but I am hoping K won't mind the taste. Fingers cross. I was talking to one of the PKU kids who was a teenager, she did mention that she doesn't like her substitutes withour really complaining about it. She tried alternating different brands and flavours but it wasn't making any difference. I admired her courage and keeping with her diet even though she didn't like her substitutes.

In summary, the day was good fun. It was a great chance to see the food, taste it, meet other children and their parents (strangely all mums), take away Low Protein recipes and finally involve our daughter in the process so she understands her little brother's special diet.