How do you take PKU Kuvan/Sapropterin tablets & does storing them in fridge help lower PHE?

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  • เผยแพร่เมื่อ 25 ธ.ค. 2024

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  • @RekojRaylor
    @RekojRaylor 5 หลายเดือนก่อน

    Hi Richard. I'm on 19 tablets per day and am lucky as I don't mind swallowing tablets whole. I swallow all 19 at once so no bad taste and very quick!
    I also store my 'current' pot of tablets in the fridge as I'm wary of the warning to keep them below 25c. I'm not sure if it helps as I find it quite hard to compare results with a blood test once a month. So many other factors can change within a month - bring on home testing! That would be a huge change for me personally and I think would lead to much better understanding of the diet.
    I'd like to store all of my tablets in the fridge from when I receive them but recently I've started getting pots of 30 tablets. When I'm taking 19 tablets a day that's a lot of pots, so I just don't have the fridge space. I feel like if I don't put all of the pots in the fridge from the moment I get them, just keeping my current pot in the fridge probably won't make much difference but who knows - I do it anyway!
    One thing that was suggested to me for further improving levels was having half the daily dose in the morning and the other half in the evening, which does seem to improve results.
    Anyway, thanks for the video. Look forward to the next one 👍

    • @Richard_FitPku
      @Richard_FitPku  4 หลายเดือนก่อน +1

      I swallow 2 at a time, and they go down pretty easy (for me). There are so ma y points in your comme t that I agree with.
      I have heard about hakfing the dose, I may consided doing this in the near future too. I would love a home testing kit, it seems so old fashion that we do not have this in place already.

  • @KristyRamirezgomez
    @KristyRamirezgomez หลายเดือนก่อน

    My 4 year old has Classical PKU, we are going to use it in powder form. We start trial in 30 days.

    • @Richard_FitPku
      @Richard_FitPku  หลายเดือนก่อน

      Great, where are you from? Adults here only get access to tablets at the moment I believe.

  • @aymanmbarki4683
    @aymanmbarki4683 4 หลายเดือนก่อน +1

    Hola soy de España mi hijo tiene pku moderada quisiera saber si sabes si tiene alguna probabilidad de que funcione para el kuvan?.gracias.

    • @Richard_FitPku
      @Richard_FitPku  3 หลายเดือนก่อน

      I'm afraid the only people would know would be his pku clinic doctors. I had to do a genetic test before I started the drug. This gave me a % of potentially rsponding to the drug, mine was a good % so they let me progress to the trial. If it was low they would not have. Every country migjt be different. I've heard the high exchanges or protein we are allowed normally potentially the better chance we have.

  • @WadoodJani-j6k
    @WadoodJani-j6k 4 หลายเดือนก่อน +1

    I need this teblets i am pakistani

    • @Richard_FitPku
      @Richard_FitPku  4 หลายเดือนก่อน

      @@WadoodJani-j6k I hope you get them soon ❤️

  • @pavlinka.p
    @pavlinka.p หลายเดือนก่อน

    Storage of medication has to do with its bioavailability. I think that is the term you’re looking for. Apparently hot/cold temperatures can alter the physical properties of the medication…. I tend to talk to pharmacists rather than doctors; pharmacists have more knowledge on these things! Extreme hot or cold can cause meds to be less potent or more potent… also the food too. Drug & food interaction. Some foods can increase or decrease the bioavailability of the drug! Example: grapefruit can prevent the metabolization of some drugs like: heroin, I know that is an extreme example. Basically the grapefruits substrates/molecules attach competitively to the protein/enzyme and binds to it permanently & blocks the medication etc., from binding to that site/receptor & so the medication lingers for a longer amount of time in the body. It takes 3-6+ days for grapefruit to be completely metabolized…. & since it competitively bound itself to the enzyme permanently, the medication/heroin cannot be processed until the body has made more enzymes….this concept is called: irreversible inhibition/competitive binding….. so the medications that are mixed together or foods that are mixed together are competing (there are competitive vs noncompetitive inhibitors). Sugar can be both competitive & noncompetitive & so processed more quickly than a protein… usually sugar is processed & metabolized first before protein….. terms to look up: pharmacodynamics, pharmacokinetics, bioavailability, affinity, pharmacogenomics….. it looks like unless packaging states to place medication in the refrigerator, it does not necessarily make the drug last longer/prevent expiration…. Some antibiotics, or other drugs like insulin should be placed in the refrigerator. Antibiotics stored in the fridge because it is a live microbe/fungus, etc… liquid forms…microbes survive, procreate/proliferate in specific temperatures. So that is another reason to follow instructions on the label!! :) placing the drug in the fridge will help with the taste of the medication. The cold temperature masks the bitterness…. But some drugs do degrade in cold temperatures. A pharmacist knows! You need to be specific when asking questions with the pharmacists. Hope that helps! 😊

    • @pavlinka.p
      @pavlinka.p หลายเดือนก่อน

      Meant to say that microbes proliferate/multiply or become stagnant dependent on temperature. It has to be optimal/optimal temps for them to multiply or survive. & will become stagnant or stop multiplying if the temperature is not to their liking. So some antibiotics need to be stored appropriately in the fridge to maintain effectiveness & stability

    • @Richard_FitPku
      @Richard_FitPku  หลายเดือนก่อน

      @@pavlinka.p thanks for the comments on the video, really interesting. The label does say store under 25c only

    • @pavlinka.p
      @pavlinka.p หลายเดือนก่อน

      @ only