PEG PAL

So the recipe that I referenced in my last post did not come to fruition the way I thought it would, so it will be a bit longer before that appears.

As you can probably see from the title of my post, today I wanted to write a bit about PEG PAL.  I recently was accepted into the Phase III trials for PEG PAL.  The study that I am participating in will look at appropriate dosing of PEG PAL.  Basically, they are trying to find the lowest possible dose that can be given for PEG PAL to be effective.

PEG PAL is an enzyme substitution therapy for PKU that is currently in its third and last phase of trials before it goes to the FDA for approval.  PEG PAL would convert phenylalanine into two other chemicals (which I do not remember at this moment) that the body can process normally.  It is different from phenylalanine hydroxylase, which is what non-PKU people produce, so it will not convert the phe into tyrosine.  However, the effect would essentially be the same as if my liver produced the phenylalanine hydroxylase, minus the tyrosine.  I am super excited about what this might mean for my diet, as well as for stability of my blood phe levels.

The clinic that is doing the study informed me the process is slow-going to start taking the medication.  For four weeks, I will take one injection per week of PEG PAL, and then start taking it twice per week, then four times per week, until I am taking it daily.  The reason for this is because the body considers the medication a foreign element and tries to fight it off, creating an allergic reaction response.  By administering low doses of the medication once per week, the body can build up its antibodies and eventually the allergic reaction disappears (very similar to allergy shots, if you have ever had them).  

I was hoping to be a patient that did not have an allergic response.  Um, not so much.  I was warned that joint pain and a rash were the most common side effects.  My knees were achy all day long yesterday, as well as my hips.  Luckily, I woke up this morning with minimal aches, and *knock on wood* I haven’t had a rash.  My hope is that my body builds antibodies quickly so that this gets better, not worse.

Despite some achy knees and hips (I sounded like a little old lady there!), I am still so excited to be a part of this trial.  The hope is that PEG PAL will allow for a decrease in blood phe levels as well as an increased phe tolerance, and if that happens, a few achy days might just be worth it.  🙂

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Supplements and PKU

Hello there!  I am planning to write a post later today about a recipe that I have made “PKU friendly”, but in the meantime, I wanted to write a post about supplements.  Please keep in mind that I am neither a dietitian nor a doctor, and that the supplements that I take are what works for me, but might not work for everyone.   OK, with that said, here are my thoughts on supplements.

Maybe it is because of PKU, but I tend to enjoy reading up on the latest nutritional research and what is best for our bodies (kind of a nerd that way, I guess).  During my reading, I have come across the idea that a well balanced diet is the best way to get nutrients.  When you think about the PKU diet, we are missing lots of vitamins, minerals and *ahem* amino acids other than phenylalanine.  Now, this is where the formula comes in.  I switched in early 2013 to the Vitaflo PKU Coolers 20, and I LOVE them.  They are a pre-mixed, easy to drink formula.  My previous experiences with other formulas did not work for various reasons (lophlex LQ was way to acidic for me and caused nausea, even when I drank it with water), but the Vitaflo coolers have been awesome.

The supplements I take daily.  3 Vitaflo 20 Coolers, and left to right, two capsules of tyrosine, one B Complex vitamin, and two flax seed oil capsules.

The supplements I take daily. 3 Vitaflo 20 Coolers, and left to right, two capsules of tyrosine, one B Complex vitamin, and two flax seed oil capsules.

Currently, I take 1000 milligrams of tyrosine per day.  Throughout my life, my tyrosine has bounced around and sometimes required supplementation.  I started taking tyrosine in May after my doctor informed me that my tyrosine levels were low.  I noticed a HUGE difference taking the supplement.  In people with PKU, we tend to have low tyrosine because we don’t convert phenylalanine into tyrosine like non-PKU people do.  Low tyrosine can result in several side effects, and the most notable for me was fatigue.  I feel like I have so much more energy with the tyrosine!

I also take a B-complex vitamin daily.  I started taking this one on my own, but my doctor agreed that it was a good idea, since the PKU diet does not include many sources of B vitamins (except the formula).  If you take a B vitamin, I recommend taking it with a meal, so it won’t upset your stomach.

Lastly, I take two flax seed oil supplements daily.  I wanted to take a supplement that provided DHA, which is an essential fatty acid that plays a role in brain health.  I also knew that the PKU diet does not really allow for foods that have omega fatty acids (such as fish, etc.).  I also knew that I was not very thrilled about taking fish oil.  I have been told by friends and family members that take fish oil that it can lead to “fishy burps”, and that sounds a wee bit repulsive to me.  However, flax seed oil seemed to be a nice compromise.  I get healthy fatty acids in my diet and I don’t have to worry about burping up fish oil.

 

Welcome to my blog!

You know, I have never done this blogging thing.  However, because of recent life circumstances, I thought this might be the best time to start.  Why, you ask?

Well, I have always enjoyed writing, for starters.  I remember my desire to be the first six year old to publish a book.  No joke.  However, that dream was not realized, and I went on to a different career path.  I went to graduate school and became a clinical social worker, and worked as a therapist with children and adolescents.  And I LOVE what I do on a day-to-day basis.  Still, there was always the nagging thought that I wanted to write- the belief that I have things that I want to say and that I am pretty darn good at putting my thoughts into written words.

Well, fast forward to June 2013.  My wonderful husband got an amazing job offer that would take us back to the town where I grew up.  I was excited to move back to the place where my parents and brother live, but I was sad to say goodbye to my friends and my wonderful job as an outpatient kids therapist with wonderful coworkers.  My last day was at the end of July, and since then, I have been looking for a job in my new/old town.  I really began mulling over the idea of doing more writing while I wait in that special purgatory where you send out multiple resumes (daily) and are waiting for social services agencies to call you back (and let me tell you, they are notoriously slow).

Now, I’m sure you’re wondering how you wound up on a blog about PKU and here I am musing about my career choices.  GETTING THERE!  I promise!  I just tend to be long winded 😉

I also have classical phenylketonuria, or PKU.  PKU is a metabolic disorder in which my body cannot metabolize phenylalanine, an amino acid in protein, properly.  When phenylalanine builds up in the body, it can cause brain damage, seizures and retardation (I will get into the specifics of how PKU works in a later post).  I was extremely fortunate that my PKU was diagnosed through newborn screening, and I have remained on the special PKU diet for my entire life.

However, there are still numerous challenges that adults with PKU face daily.  I continue to follow a low protein diet daily, as well as drink a special formula, which contains all of the amino acids except for phenylalanine, as well as vitamins and minerals that are missing from my diet because I don’t eat meat, dairy, legumes or anything high in protein.  I am in the first generation of PKU patients that are on their diet “for life”.  We are figuring out how to navigate relationships with family and friends, work, and romantic relationships while maintaining this PKU lifestyle and trying to stay healthy.

That’s where this blog comes in.  I would like to utilize this blog as a place to discuss my concerns with staying healthy on the PKU diet, as well as creating a space for PKU adults (as well as the parents of PKU kiddos) to connect and to learn from one another.

With that being said, I invite you to stop by once in a while- you might see something you like!

Cheers!