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  • Dangerous behavior

    mieze - - Late Diagnosed PKU

    Post

    There are tablets, but you need to take a lot of them. I know that a few adults take them since they really do not like to drink the formula, but most people interested in the tablets are put off because of the amount they need to take, which is like 140 tablets per day! I don't think you will get your brother to eat 140 tablets every day. I don't know which medical companies are available in Iran, it differs in every country. But I know that there are several companies in Europe which make the …

  • Dangerous behavior

    mieze - - Late Diagnosed PKU

    Post

    The behaviour you described is typical for untreated PKU. Your brother cannot metabolize the amino acid phenylalanine (phe). In high concentration it acts as a neurotoxin, which is why it hinders brain development in children. Even though the brain development of your now adult brother has finished, it still is a neurotoxin, it still damages the remaining functions of the brain. And that is why the behaviour of your brother gets worse. He will lose some of the abilities he has learned, his aggre…

  • A low-protein diet is not healthy for you! You should under no circumstances eat low-protein yourself!!! You'll make yourself malnourished. You need all the vitamins, minerals, proteins etc you can get, so your body can produce enough milk for your child. No matter how many protein you eat, breast milk will always have the same amount of phe. You cannot reduce the protein in your breast milk by eating low-protein yourself. This is only done by mothers WITH PKU. Please, start eating normal right …

  • Shirataki Konjac Nudeln/Reis

    mieze - - Low-protein products

    Post

    Ich glaub, wir haben solche Nudeln auch schon einmal getestet. Die sind nicht neu auf dem Markt. Sie sehen weiß aus, nicht sehr appetitlich, muss ich sagen. Schmecken tun sie nach gar nichts und fühlen sich auch ein bisschen seltsam an, sie schnurpsen etwas beim essen, so als wenn man nicht ganz durchgekochtes Gemüse isst. Ich finde sie kein Vergleich zu den phe-armen Nudeln, die es sonst gibt. So viel Phe haben die auch nicht, dass man sie mit diesem geschmacklosen Zeug ersetzen muss.

  • We don't have such specialised shops. Food like pasta, rice and bread is mostly ordered from online dealers. Vegan cheese, spreads and cold meat can be bought in larger supermarkets and in the "Reformhaus", which is a kind of organic-health-food-store. I remember buying this gluten-free, low-protein bread (called Steinofenbrot from the company Hammermühle) in Berlin in a Reformhaus at the train station Friedrichstraße. As cheese I recommend the Wilmbersburger vegan cheese which has zero protein …

  • Your GP can send your blood to a laboratory, but it needs to be one that can check the phe levels. Not all laboratories have the necessary equipment. Futhermore, PKU is such a rare disease that many GPs have hardly any knowledge about it apart from the fact that it exists. The paediatrician of my son sometimes asks me how to handle a certain situation since she is aware that I am more knowledgeable about PKU than she is. Get in touch with your national PKU society. They can tell you which PKU cl…

  • Mental problems can be related to PKU, but they don't have to. The thing with PKU is that the high level of phe in the blood stream (and thus the brain) hinder the proper development of the brain. Just because the development is finished at some point does not mean that the phe is less of a neurotoxin. I just does not cause such severe damange anymore. Now, with mild forms of PKU there is a certain rest activity with the enzyme that metabolizes phe. That means you can eat more protein than someo…

  • Video on PKU

    mieze - - General topics about PKU

    Post

    Well done. It contains the necessary information and explains everything in easy words. And I wish I could draw like that.

  • Periflex and what else??

    mieze - - Child PKU

    Post

    Sadly, there is no foolproof way to give the formula. Every child is different. My child drinks his formula with just water and despises any other brand, mixture or flavour. Even though some of the others are much milder, he only takes this pure (and truly disgusting) stuff, just because he is used to it from birth. If you can't find a mixture that pleases her, can you "reschedule" her formula? Give her an ordinary breakfast first and her first formula of the day two hours later? Taking the form…

  • I always take the ring finger or the pinkie of the left hand. That little prick can be annoying for several days and if its done on a finger constantly used (mostly the pressure of grabbing or holding something), it hurts more often. This way that excruciating wound is not disturbed so often.

  • values too low?

    mieze - - Child PKU

    Post

    @Paul Normally, I'm on the cautious end myself. I often argued not to raise the tolerance of my son or to at least limit the increase when the doctors were of the opinion that the blood levels were so stable we should try a few mg more. Every parent gets a feeling for how their child will react to changes and I know to be cautious. But even I would increase more than 10 mg with blood levels as low as 0.1. The problem often is the communication between doctor and patient. We had the same problem …

  • values too low?

    mieze - - Child PKU

    Post

    2.4 mg/dl is definitelly not too low. Everything between 1 and 4 is perfect. I would never go so far as 6 mg/dl. A person without PKU has about 1.6, therefore, 2.4 is really not too low. You should try not to pass over 4 mg/dl too often. Better stay on the safe side. Nevertheless, 0.1 indicates a much higher protein demand. I agree her growth spurt requires a much higher increase than 10 mg. You can add 20 mg to increase her tolerance if her value is on a constant good level. A blood level of 0.…

  • This is interesting information though I'm not sure how significant this study can be since there are no healthy 60, 70 or even 80 year old PKUs. Though they can study how the brain reacts on short term now while a subject is young and healthy, they cannot know how the brain will develop after several decades of abuse or when other age-related issues are added. Maybe this self-healing ability wont' work after several decades. I would be cautious nevertheless. In the German part of this forum the…

  • Aquafaba

    mieze - - General food topics

    Post

    This is pretty new stuff. There is no sure nutricial information about it yet. Some sources say it contains a lof of protein, some say it is low on protein. Therefore, I would be cautious to use it. If you need a last minute egg replacement, take a banana. Apart from being a useful egg replacer it also gives a nice taste.

  • You can get maltodextrin. It is an almost flavourless powder you can put into drinks to add calories. Maria's suggestion of looking at your drinks is very good as well. It is a problem for many people who want to lose weight, that they discount the amount of calories in their daily drinks. Especially fruity stuff looks healthy but contains rather a lot of calories, smoothies are gaining a bit of a bad reputation because of that. If fruity drinks contain too much protein for you, you can still go…

  • That is not really a big fluctuation, especially since your daughter does not have a fixed daily intake of phe yet. As you breastfeed her, the amount of milk she drinks is different each day. Therefore, her blood levels can vary. Just keep checking regularly. The daily tolerance doest not necessarily grow evenly with your daughter. I can happen that she never needed a diet because she never reached the limit of her tolerance before and at one point she might exceed it as she eats more food the o…

  • Please, don't be afraid. I know that it is overwhelming at the beginning, but you will get used to it. You don't need to know everything at once. Just like with any other baby you will start slowly, milk and formula and then mashed foods and so on. You will learn everything, I promise. And just as you said, you can see here that you will have a completely healthy child. She can do anything she wants, she just can't eat everything. Just like a diabetic or someone with an allergy. Your daughter ha…

  • Mentorship program

    mieze - - Maternal PKU

    Post

    Such a mentorship program would be helpful for new parents as well. They are often completely confused about their situation, despair of having a child with a potentially harmful condition, have been scared by doctors and cannot imagine how they should be able to cope.

  • Apart from keeping a diet, people with PKU definitely need to take their formula, no matter their age. Most people are never told that there is more to PKU than just limiting the phe intake. One important function of PKU formulas is providing your body with tyrosine. Tyrosine is another amino acid and is necessary for the production of dopamine, a neurotransmitter, which is important for a lot of neuronal activity. Several diseases are associated with a dopamine imbalance: depression, Parkinson'…

  • milk alternatives

    mieze - - General food topics

    Post

    In the beginning we used mostly Vitaflo since it is devoid of phe. But we found it to be rather expensive and it had a destinctive flavour that "contaminated" many meals. We since then switched to rice milk. It is comparatively cheap, readily available in many shops and has a very low amout of phe.