CF & PKU

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anonymous

New member
My son (6 1/2 months old) was just diagnosed with CF, We actually just got back from the doctors with his genetic tests. His sweat test was negative and genetic was positive. We haven't seen a specialist yet, that isn't scheduled until next week.

He has multiple problems including epilepsy and has been in and out of the hospital many many times since he has been born. One of my major concerns with cf is by what I'm reading is CF people must eat plenty to keep weight on. Right now he is chunky and very happy. However the problem is that he also has PKU (google pku) which means he has to follow a very strict diet, He can't have most meats or anything with even little amounts of protein, everything must be weighed in grams and has to continue taking special formula to replace most of what he would eat for the rest of this life.

How is this going to affect his CF?

Thank You
 
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anonymous

New member
Welcome! Sorry to hear that your son has been diagnosed with cf along with pku. Goodness you must be overwhelmed! I did read about pku on google. You need to have your cf doctors meet with your the other specialists who treat your son. That way they can help you with his diet. If your son is chunky now that is great, hopefully that will continue for a long time. Some with cf (about 10%) do not need enzymes to gain weight. If there is a special formula that could help him grow you might just need to continue that.
Sharon, mom of Sophia, 4 and Jack, 2 both with cf
 
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anonymous

New member
Thanks Sharon, His special formula actually contains everything he needs except it has an incomplete protein. So he can usually only have organic fruit and veggy baby food. The doctors do say that he burns calories too fast and have already considered putting him on supliments. They might just do that now though.

He is on Pulmicort and Xopenex and singular already for what they thought was asthma and zertec and some other new med for alergies. I have severe asthma and allergies which I have to get shots for, so we didn't think anything of it. Since he was put on the asthma treatments I have always been patting his back and sides of his chest when he weazes and starts coughing, Is that a good thing to do?

He has already been in the hospital at a few months old for a bowl ubstruction, he was put in IV meds for it however the doctors (He has 6 or 7 now) just thought it was because his PKU levels where low which cause his imune system to not be able to fight off an infection.

Right now he is also on some pretty strong meds and bacteria suppliments due what the doctors call bad bacteria in his colen which is what they thought caused some problems with his bowel.

Does this stuff sound familiar for people with CF?

Thanks
 
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anonymous

New member
Dear Annon -
Have you ever been tested for CF yourself ? Just curious. From your e-mail. ...... "I have severe asthma and allergies which I have to get shots for, so we didn't think anything of it. ..."

..." Since he was put on the asthma treatments I have always been patting his back and sides of his chest when he weazes and starts coughing, Is that a good thing to do? ".......

"Clapping" (as it sounds by your description) his chest is very helpful. This will aid in his removal of any mucus that may be clogging his airways. I tend to weeze only when something is on its way up through my airway. Clapping was the simplist form of respiratory therapy a lot of us older patients grew up with before any of the more recent innovations. My two brothers and I grew up having our parents "clap" us each morning - all three of us have CF (Ages 35, 30 and 28). A family ritual.

It sounds as if you are doing all that you can which is wonderful. Keep your chin up. I agree that meeting your CF specialist and others will definitely aid in his treatment. Best of luck - Christina
 
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anonymous

New member
I have not been tested with CF, One of my brothers also has severe asthma and allergies and my dad had the same problem. Actually both of my brothers are really healthy we just get bronchitis every year but that is most likely cause we all smoke. I figure if I had cf I would have died by now because I smoke, which I better quit before my son comes back from grandma's which is sunday.... I clap him because I assumed everything was asthma related and that helps break up the mucus in the lungs which I learned when he was in the hospital for RSV.

Thank You
 
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JRPandTJP

New member
Sounds like you are doing a great job trying to figure all this out. It is a lot to digest all at once. My heart is with you.

My son has CF but no real respiratory issues. His is mostly GI, and thankfully that is mild as well once we got everything figured out. We do alot of protein and a very well balanced diet (all organic if possible) with no dairy. Since you can't add protein to his diet, you may want to ask them about adding omega 3 fats to his diet in small amounts if he begins to show signs of low or no weight gain as he get older. We cook only with olive, canola or walnut oils as they help keep inflamation down throughout the whole body (anything high in omega 3 is great). We also use to put a little MCT oil in his first fruits and veggies when he was around 9 months old. There are also some carbohydrate supplements you can use to boost calorie intake, but you'd have to ask them about that as well with the PKU.

Some CFers only exhibit respiratory symptoms with little to no pancreatic insufficiency (which causes the majority of weight issues if I'm not mistaken). Is he on enzymes? There is a chance weight won't be an issue...it just depends.

Our Dr's didn't recommend any "clapping" or resp theraphy until he was 2 because it can aggravate tummies and reflux (which he had pretty bad as a baby), so not sure about that so young. Maybe others can speak to that.

We also have had our son on acidophilus (which is probably the "bacteria" medicine you mentioned). It helped alot! We use it everyday.

The single most important thing you can do for his lungs is what you already said above....stop smoking and keep him away from all smokers. THis is a huge factor in keeping his lungs well supported and clear of inflamation/toxins.

Take care of yourself as best you can...we are here for you.

Warmly,
Jody mom of Julia 6 w/o cf and Ben 22 months w/cf
 
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anonymous

New member
The biggest thing i'm worried about is his lungs at this point. Do you all recommend air filters? Any suggestions on what is best? What about bedding etc? I'm also thinking about getting my older son tested for CF, He has no real respiratory issues unless he gets sick then he has a hard time breathing, However his diapers.. WHEW I can't remember the last time he had a hard bowel... I'll also mention CF to my doctor and see if he suggests getting me tested.
 
J

JRPandTJP

New member
We use the IQ Air system. We have one in our main living room area and one in our room where we sleep (Ben and I) that is. Once he is moved to my daughters bed room it will follow him. My dad researched all this for us and they purchased them as a gift. They are rated as one of the top and are used alot in the homes that "Extreme Home" uses for kids with immune issues (not the cfer necessarily have immune issue just that the machinces are really good). No ozone concerns and very quiet. We have pulled one into the kitchen when I have burned something (it's only happened once I swear it ;-) and it clears the air in minutes. We have also used it in a room when we've had to paint and same thing. It helps with my seasonal allergies as well.

Here is the web site <a target=_blank class=ftalternatingbarlinklarge href="http://www.iqair.us/...my">http://www.iqair.us/...my</a> dad didn't tell me the cost but I know they can be pricey. However they are light weight and one could easily be moved to another room at night.
 
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anonymous

New member
I will definitally look into that. Hopfully one of the two insurances my son has will cover it. He also has PKU and other problems so we may end up getting disability insurance.

Also, with all the symtoms I will most likely get myself tested. I have to get allergy shots every 2 or three months (kellog). If I don't get them I end up not being able to breath. Along with that I have severe asthma, bowl problems.

Thanks everything for the help, I'll keep everyone updated when Nick has his doctors appointment.
 
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