Tuesday 28 April 2015

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. 

Wednesday 15 April 2015

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.




Friday 10 April 2015

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.