When my husband was about 8 or 9, his specialist told his mother that he could resume a normal diet. He is now 23 and recently went to the Dr. for a checkup and brought his PKU up. Our family Dr. said that there wasn't any reason for him to go off of the diet when he was a child and that he needed to test his blood to see what his Phe levels were. We were told that the normal range is between 30-80 and my husband got his results yesterday. His is at 1197. To me that seems so high I don't know how he is even functioning normally. However, I don't know much about this. We've been married almost four years and the only thing I know is that he had a strict diet when he was a child. I just need some general help. I'm worried about his health and also upset that his previous Dr. told him it was okay to resume a normal diet without following his progress. Has anyone had this same issue? Also, how do we adjust our diets now to be acceptable with PKU? I don't want him to have to eat salad every day but I'm so lost at this point. I also have Ulcerative Colitis and some things I think will be okay for him may not be okay for me.
Thank you in advance for your help.
If I am correct 30-80 ummol/L is the normal range for phenylalanine levels in people who do NOT have PKU. Doctors recommend that people who have PKU keep their phe levels between 120 and 360 ummol/L. Some doctors will allow adults to go up to 600 ummol/L, but below 360 is still the safest route.
Is your husband seeing a special doctor specifically for his PKU along with a metabolic dietitian? Most general physicians do not know enough about PKU to treat it. Do you live in the US? If so you could check out this link: http://www.macpad.org/page.aspx?id=92187 This will provide you with information on where the nearest metabolic clinic is to you.
The diet that he followed when he was a child was called a low protein diet. In most cases this diet excludes meat, dairy, beans, nuts, and most grain products. The diet consists mainly of fruits, veggies, snack foods in measured amounts, and specially made foods. These specially made foods are foods that are made to imitate things such as breads, pastas, and baked goods without having a high amount of protein in them. He also needs to look into finding a metabolic formula. Ask your husband about this, he might remember drinking a drink mix when he was little.
A lot of adults who have PKU have had this issue. Many years ago doctors believed that children could discontinue the low protein diet at about six years old, because that is when the brain is fully developed. They did not believe a fully developed brain could be harmed. Today doctors now know that to prevent future issues it is necessary to follow the low protein diet for life. Unfortunately this doctor was probably just follow normal procedure at that time.
Breanna Hardy 18 CPKU
I'll tell you a little about my experience.
I remember when i was younger i got told that i could start eating normal food at age 12 cause my brain was fully developed. So thats what i did, it was great eating those first time Hamburgers etc from McDonalds etc. It was only when i was 17-18 years old that i knew something was'nt right, i started having panic attacks, was very anxious and depressed and i hated the way i was thinking, i always thought the worse of something, i was only young had everything in life and i never understood what i had to be so sad about, intill i met my New doctor and dietician they put be straight on a low protein diet and after a few month of a very strict diet, i was a totally different person, depression dissapeared the panic attacks gone, and i was my own cheerful confident person. Although i ain't saying its the same with everyone but there's loads of other syptoms that high levels do that we don't notice.
High levels can make:
Hard to Concentrate
Moodiness and Aggressive
Tremors, twitches in eyes
aching Bones or Joints etc
Your Husband may not have any of these syptoms, but by returning to the low protein diet will make him alot healthy, more energetic etc
My doctor always told me in an easier form that i could understand was that now our brains are fully developed eating Protein or PHE is like Poison to our brain, and it can affect us all Emotionally (more for women) Neurologically, mentally, and phsycology (sorry about spelling).
hope your husband returns to diet
Thanks for all of the help guys! We have already started him back on the PKU diet. I cooked my first PKU friendly meal last night and it turned out really well. I was like a proud parent.
My Husband went to our family doctor this morning and they are working on getting him an appointment with a specialist. The closest one to us is over three hours away so we will have to take off of work and spend the night. Our family Dr. went ahead and prescribed him with Kuvan, however, we can't find a pharmacy around here that can even get it. Not to mention its about $4,000 a month without insurance. I hope that our insurance covers it. I will post more when I know more.
Nicole, my husband does have a lot of those symptoms you listed. I'm very excited about the idea of him being able to function better. I can't wait until we can get him back on track where he needs to be.
When you do get the chance see the specialist speak to him/her about Kuvan. As far as I know Kuvan needs to be ordered through a special pharmacy. You cannot just pick it up at your local Walgreens. For example, in my case my parents order my Kuvan through Medco and it is delivered to our door. Your husband will also need to see if he responds to Kuvan. Some people respond, and Kuvan helps them lower their phe levels. Other people do not.
If cost is an issue again talk to your husbands specialist. There are two organizations, BPPS (Biomarin Patient & Physician Support) and NORD (National Organization for Rare Disorders) that can help with the cost. My family has very good insurance, and our co-pay for three months of Kuvan is $50. BPPS actually contacted my family when I first began taking Kuvan, and they reassured my parents that if even this small co-pay was too much they would assist us. They are dedicated to making sure that those who can benefit from Kuvan have access to it no matter what your financial status is.
I hope this helps!
Breanna Hardy 18 CPKU
Thanks so much Breanna! My husband's regular Dr. prescribed him Kuvan so that he could go ahead and begin it while he was waiting to get an appointment with the specialist. We ran into the problem you were referring to – no one around here can get the medicine. So we had the Dr's office call around as well and they found a Walgreens about 30 minutes from us, a place we travel to often so it really isn't out of the way, that can get it. I just hope our insurance will cover it. Our co-pay will be extremely cheap if they do cover it. I have also been reading about the programs you listed. It's really good to know that they have something we can go to incase our insurance will not pay for it. Thanks again for the additional advice. If you think of anything else, please send it my way :). I also have a question. Is there a way to know how many grams of protein my husband can have a day? Or is it based entirely on the PHE in everything? I'm just trying to figure out what he can have until we go to the specialist. Do you have any snack ideas? Also, the low protein pastas and rice have to be ordered. Is it good? Worth it? I also found a PKU friendly recipe that called for wheat starch. I can't find a store anywhere that carries it. Have you ever heard of it and is it something you have to special order as well?
Does your husband by any chance remember how many mg of phe he was allowed as a child? Each person with PKU is different, so it is hard to say how much phe he can tolerate. In my personal experience before I began Kuvan I was allowed 300 mg of phe, which is approximentally 6 g of protein (50 mg of phe=about 1 g of protein). With Kuvan I am now allowed 900 mg of phe. On the other hand I know others who have PKU that are allowed much less than this, and others are allowed much more. It really does differ on an individual basis. His specialist may start him at what he was allowed when he was a child and the adjust it from there. The only way your husband will be able to figure out how much phenylalanine he is allowed is by restricting his diet, doing a blood test, and modifying his diet according to his levels. Treatment for PKU is a lot of guess and check.
As far as snacks go almost all kinds of fruit snacks (fruit roll ups, fruit by the foot etc) are ok. Hunts lemon pudding, most chips and popcorn in moderated amounts are ok, fruits are usually ok, most fruity candies as long as they are not sugar free/contain asparteme.
I personally love the low protein rice and pasta. I grew up eating the low protein rice and pasta, so naturally I am used to what it tastes like. Can I ask what state you live in? I am just asking, because I know some states do provide the metabolic formula and/or some staple foods such as pastas, rice, baking mixes, etc. Here in Wisconsin formula and foods such as pastas, rice, baking mix, peanut butter etc are provided through the state. As far as I know the wheat starch does need to be specially ordered. Here are some websites you may want to look into: cambrookefoods.com, myspecialdiet.com you can also purchase cook books and a phe book (which lists the amount of phe in a variety of foods) from this website, pkuperspectives.com, dietspec.com, dietforlife.com, and http://www.listserv.emory.edu/archives/pku-support-l.html this is a mailing group that thousands of people with PKU and their family members are a part of. If you would like to subscribe to it the third link down should read Join or leave the list (or change settings) click that and go from there.
Breanna Hardy 18 CPKU
Thats great that your husband has already started his diet
And well done you for making your first low protein meal, hope your husband helped out though
I live in the UK, so i could'nt tell you what Kuvan is etc, we get a drinking formula called PKU express which are in sports pouches, there amazing
I am personally on 4g of Protein, which we call 4 exchanges,its all completely different over here. We don't pay either.
I snack mostly on Fruits, LP Crackers with salsa sauce etc.
I hope you enjoy more Low Protein cooking
Thanks again yall!
Okay I doubt he remembers how much PHE he could have as a kid. I'll see if his mom can remember. It's been a really long time. We live in Georgia. I ordered the PKU cookbook and am still waiting on it to come in. We've been cooking vegetarian meals and altering them slightly. Chris cooks this one soup Pilgrims Potato Soup that we found on a PKU website and taking it to work for lunch.
We did find out that the earliest appointment we can get with the closest specialist in our insurance network will be some time next year. So I guess we're going to just limit his PHE-Protein intake and wait and see.