PKU.com Community Discussion Forum Parent support Low Levels in CPKU Preemies HELP PLEASE

Viewing 15 posts - 1 through 15 (of 21 total)
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  • #7444
    Avatar of Nikki
    TucksMommy
    Member

    WHY are my sons levels going from 750 to 13??????? I dont understand and the doctors have not been able to shed light on the subject for me. His levels shoot up high and then they go down in the teens. Either way is bad apparently, what happens with low levels because the doctors show they are concerned when they are low, but isn’t that a good thing. He was over 2700 at birth until about 3 – 4 weeks. Has anyone gone through this, they say it is normal but how can that be normal. I am a mess and I haven’t been able to talk to my mentor mom the clinic set up I don’t know why I can’t talk in person about PKU to anyone but my docs dietician and the nurses that were there in the NICU for the first five weeks. Can anyone help me before I lose it, please. I am clinging on by God’s good graces right now and the precious look my children give me. I don’t want to seem negative, I love my children, but I have post pardum from a preemie and he has been diagnosed w CPKU doc said he cannot have protein almost at all, tetrabiopterin deficiency and heart defect… he had a uti also, what’s next. I’m looking for an answer at a time…

    #7447
    Avatar of Nikki
    TucksMommy
    Member

    Nevermind…I read another persons input and it seems like it’s normal. I was just wondering because my 3 month old does not respond to anything or smile he doesn’t do anything but cry and it scares me, but I’ll just ask his docs again and again until I get my answers…

    #7448
    Avatar of Jennifer
    neurotic mom
    Member

    I had no idea that levels could be that high?!!!Really, they were 2700 and 750? nBelieve me, I know how frustrating the level fluctuation and not getting any concrete answers can be. I don’t have a classic pku baby, she’s hyperphe, but hopefully, having someone who has been through the incredibly troubling, upsetting, and aweful wait and see game may help. What are the doctors telling you? Does the fact that the baby was a premie and has a heart issue make a difference? Please let me know. I hope I could help in any way even if I have to ask my daughter’s nurses for you.

    #7449
    Avatar of Breanna
    BreaMarie91
    Member

    NeuroticMom,
    I just thought I would let you know that I think Tucker’s mom is counting his phe levels in different units then you are. You are probably using the smaller units (mg/dl) which is why the larger units (umol/L) seem enormously high.
    When Tucker’s level was 750umol/L it is 12.5mg/dl. 13umol/L converts to about 0.21mg/dl and 2700umol/L is 45mg/dl. To get from umol/L to mg/dl you divide my 60.
    I hope this helps get rid of your confusion . nBreanna 17 CPKU

    #7451
    Avatar of Nikki
    TucksMommy
    Member

    I’m sorry, she’s right, I noticed that, my doctors convert levels in a different way than what I’ve seen here. That was extremely high, but it is calculated differently, thank you for explaining that because I wasn’t sure about the level exchange either until you explained that. Thank you, his levels are up and down because he is a preemie, but he is 3 months now and they are at 0. something now, in your conversion, in his he is between 35-187 lately. The higher is good but what heppens with lower levels? Does anyone know if they are safe?

    #7452
    Avatar of Nikki
    TucksMommy
    Member

    I’m not sure whether his heart condition affects his PKU, we just found that because he was admitted for not eating and having a uti and upper resp. infection. When they did the chest x-ray they found it. It is all very troubling and frustrating, at least I know I’m not alone because that is how it feels with PKU- not too many people can understand. Thank you for responding, I am kind of desperate for some answers right now, and although his docs are great, I haven’t gotten them all and any parent knows that not having answers is devastating. Sorry for the negativity, just feel very alone with PKU…

    #7453
    Avatar of Nikki
    TucksMommy
    Member

    Brea – I tried to send you a message to thank you because it is great to finally be able to convert the levels, I had no idea how to do that. And I wanted to tell you that I have read so many of your posts and I think you are an amazing person to help parents with these hard times and questions. I cannot tell you how much relief I have gotten from reading your posts – it has made me feel that Tucker will be okay because you are okay and you seem like a very smart and caring and sensitive young lady. I think you are brave and honestly just an amazing person – you are a PKU hero for me…thank you again.
    nLost… nTucker CPKU 3 months

    #7454
    Avatar of Breanna
    BreaMarie91
    Member

    Thank you. I’m hoping to become a medical geneticist someday, so I’m glad that before that even happens I’m able to help other PKU families .
    Low levels can be dangerous. People with PKU still need some phe to grow properly. The way my dietitian explained it to me is that if we don’t get enough phe our bodies will begin breaking down the protein in our muscle to get the phe that we need. I personally have noticed that when my levels get too low I am always tired.
    The only way to get his levels to increase is to continue adding more phe into his diet by increasing the amount of breast milk or baby formula that he is allowed. On the positive side if his levels are low that means is phe tolerance will increase and he’ll be able to eat more then he has been! nBreanna 17 CPKU

    #7455
    Avatar of Nikki
    TucksMommy
    Member

    Well, that is good and bad to hear. That’s awesome that you have such a great dream/plans. Youse seem like you will be a freat doctor/geneticist. You will be able to relate to people and that makes someone suffering from a disease feel a sense of comfort and trust in their care. I hope that you fulfill that dream and from what I’ve seen from you just on here I’m sure you will, and you are a very good big sister too!!
    I spoke with his metabolic doc and dietician this afternoon and they said that because he is growing so fast he is using all of the phe that he gets, but you are exactly right – he might end up having mild PKU or atypical PKU. We won’t know for a while but I am definately praying for that, but most likely it is just because he is still preemie. Unfortunately the low levels negatively affect his tetrabiopterin deficiency and I don’t know ANYTHING about that condition or what it does, I just know that it can be bad.
    You are very smart and you know a lot about PKU! Your mother must be very proud!
    Thank you again!!

    #7471
    Avatar of Monika
    monika26
    Member

    i also have a 3mth old and his levels have been low for most of the 3 mths like really low at 0.2 and then once they got it up to a 12 and then it dropped again to .2 so we have never really had him in range..the genetic doctors here recommended he have a DNA test done which will let them know if he has mild or severe PKU because at this point we still dont know..so we did and we are now waiting on results ..6-8 weeks

    #7475
    Avatar of Nikki
    TucksMommy
    Member

    I’m sorry, if anyone knows, I do. It’s very frustrated and emotionally draining! We did the genetic testing and it came back with one mutation seen in classic PKU and one seen in mild PKU. Good luck with the results, hopefully it comes back with mild PKU, it’s better than CPKU. That’s really all we can hope for is the mildest possible case, because we know that they have it, so there’s no changing that. Let me know how it comes out! It’s nice to know that other people are going through the same thing, that someone else can relate. Makes you feel a little less alone out here. Where are you from if you don’t mind me asking?

    #7476
    Avatar of Monika
    monika26
    Member

    i am so glad to find someone that understands what it feels like to never have your child in range..i keep track of how much he eats every day and each day he eats the required amount and yet his levels are still low..i thought it might be mild pku but once they increased his intake by like 100mg and his level shot up and then it took a week to get it back down and now we are back to square one cause we cant get them up again…its just soo frustrating cause i just want him to be ok..i really hope this dna test tells us something..ohh and im from wisconsin..how about yourself? and after you had this test done did they say if your child had mild or cpku?

    #7477
    Avatar of Breanna
    BreaMarie91
    Member

    Monika, I hope you figure out why your sons levels have been so crazy. Have you guys tried to add just a little bit of phe at a time, like maybe 25 mg of phe at a time or something rather then 100 mg?
    Also, do you mind me asking, out of curiousity, where you guys live in Wisconsin? We live in Franklin (Milwaukee county) and we go to Children’s Hospital for our PKU appointments. nBreanna 17 CPKU

    #7478
    Avatar of Monika
    monika26
    Member

    yes we have been adding only about 25mg of phe for awhile and his levels always stayed at .2 thats why they decided to add so much more at one time becuase we wanted to see what would happen and it did go up..so basically his level has been really low at 250mg a day so we tried 350 and it went up to 11 then when we tried to get it back down we were at 250 again and his level was a 9 then we brought it down to 230 and his level was 0.2 again..so now we are working are way up again. We are at 250 again and im going to get his blood drawn today and we shall see what the results will bring..It is all really strange..Oh and we live in New Berlin. so you are not that far away..im glad to meet you

    #7480
    Avatar of Nikki
    TucksMommy
    Member

    It’s so frustrating! We are going through the exact same thing with little Tuck Tuck. His levels stay so low, and then they try to up his phe intake by large amounts and he shoots up too high, and it takes a long time for them to get back to a normal range, and within a weeks time, they are back down to an extremely low level. When they have tried to go up little by little in the past couple tries, it hasn’t had much of a change, only when they overshoot. He does have CPKU. I was hoping so much that they were going to come back with different results, and tell me that he has mild PKU, but once again, I had false hopes, and big let-downs. It’s so emotionally draining to try to get him where he needs to be. Right now, the doctors are saying that he is just growing so fast that he is using up every bit of phe that he gets in him, so we are hoping for more stability once he slows down on his growth patterns. He’s still playing catch up from being a preemie. Was your little one a preemie? I don’t know if you told me that, but I don’t remember if you did. Let me know if you find out anything from your doctors about why this is happening and I will do the same, it’s nice to get feed back from someone else that is going through the same thing because I feel like we are drowning here, and poor Tucker is the one that suffers. I can always tell when his levels get too high when they overshoot with the phe because he gets this gym locker smell to him. I have actually started using baby cologne from johnsons and johnsons because I have had some smart… you know what person tell me that I need to give him a bath because he smells, and that was so hurtful I just took him and walked away because you cannot explain PKU to people that easily and after that comment I didn’t think that thry even deserved a reply. I actually walked away and started to cry. People don’t realize how the things that they say affect people. The lady at the grocery store today told me that his formula stinks also!! I apparently have bad luck with people being cordial lately. Or even being humane for that matter.
    So, let me know will you please. Thank you, and BreaMarie is a good person to talk to. She is very nice and very knowledgeable when it comes to PKU; Very sweet girl. Hope everything goes better for you, and thank you for responding, it makes me feel like we are not alone because I have not seen anyone on here going through the same thing, and sometimes I feel like we are the only people struggling with this aspect of it. Talk to you later…
    Nikki n(Tuck CPKU 3 months)

Viewing 15 posts - 1 through 15 (of 21 total)
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