What to do for the PKU

We frequently get questions concerning PKU, due to its rarity and the oddness of it. I thought pictures might help, so I photographed the processes involved in Adam’s PKU related care.

Daily routine: Administer the medicine.

First I have to cut his medicine pills in half, due to the fact that he only requires a half dosage a day. Before we got the pill cutter we were using a knife, which obviously has a larger margin for error, and with such expensive pills, this was the preferable option. 

I then put that half pill into the mortar and pestle and grind it into a fine powder. 

As you can see, this medicine stains everything deep yellow. We have learned to administer it just before or after a bath, when we have yet to put an outfit on him. Even his spit up will stain his blankets yellow if it's within an hour or so of having been administered. 

Then we add a wee bit of water. I've now started sucking up the water into the syringe first, so I have an exact amount, THEN I put it into the mortar. This prevents accidentally getting too much water and having to give the poor kid numerous syringes... 

After shooting the measured water from the syringe into the mortar, I mix it with the powder until it dissolves. Then I suck it all back into the syringe. 

I attempt to administer the medicine around the same time every day, however we are often very busy and our schedule does not always allow us to be at home during that time. As such, in the instances we won't be home, I suck it up into the syringe and place it into a small tupperware. There it travels quite nicely and fits well in the diaper bag, ready to pulled out when the time finally arrives. 

Here I am also practicing my photography skills (I joined the photography club!) I am focusing on a close object and blurring the background for effect. This also shows the daily amount Adam receives. He's gotten quite used to it and the process is much quicker now that he expects the taste.

Weekly Routine: Draw Blood

A device containing a needle for heel
sticks bottom right, and the sheet with the
circles at top we are supposed to fill with his blood.

         Yes, you read that correctly. One of the more recent developments in our care for Adam. At our last visit to the Padova hospital, they decided to show us how to take the blood sample at home so it could be mailed in, allowing us to skip the frequent, long drives and days spent waiting at the lab at Padova. I liked the idea of less travel to Padova, however I was not fond of having to draw the blood ourselves. They gave us a few starter tools and then said we could get the rest of the tools from our lab on base. And when I say they showed us how to take the blood sample, I mean they talked us through it, neither the nurses or my hubby or I actually drew blood, so it was not a hands on learning or a demo, though I had seen it done many times before.      
           When I left I was still rather surprised. They are letting us take BLOOD samples at home? You can send blood IN THE MAIL??? Who knew. I didn't feel like this was something we could get away with in the states...
          After discussing it with the pediatrician on base, we went over to the lab and asked for the supplies needed, heel sticks and bio hazard bags and such. The lab technician commented on how they had never received such an odd request nor heard of this being done before. We replied that we, similarly, had not heard of doing such a thing before either.
        Every week we are supposed to take Adam's blood and mail it in. My hubby and I decided to designate Saturdays as the given day we would do this. When the first Saturday we were supposed to draw blood rolled around, I made a blood drawing station on the washer and dryer in our bathroom. I laid down a towel for Adam, and set up all of the supplies my DH would need. I didn't want to be a part of it, and my hubby was fine with that. We had seen the nurses do it by themselves and he figured he could too. I handed him the baby and walked away. After some time and a few good screams later, he opened the bathroom door and declared he needed my assistance.

A Heel Warmer used to encourage blood flow.

           I entered the bathroom to find a rather distressed baby, a blood speckled towel, and several used test sheets. The test sheets are very particular in that you must acquire enough blood to seep through the thick paper and can be visible on the backside. However you must be careful not to let the blood come out of the dotted line. They were very clear about us not sending in any test samples that were not in the lines. Getting the blood into these specifications is difficult, and my hubby's first few attempts failed as he needed more practice. He asked me to hold down the baby while he attempted it again on a new one.
          I held the poor squirming kid down while my hubby squeezed his heel and carefully attempted to get the blood into the parameters designated. Eventually we were successful, and successive weeks of blood drawing have improved from the first.

We then put on a cotton ball and bandage wrap until bleeding stops.  

            The test sheet is placed into a Biohazard bag, which is then placed into a regular mailing envelope and sent to the Padova hospital. (I then got to learn how to navigate the Italian post office. That was an interesting experience... but I now know how to work their post versus the American style post office they provide us on the base) Once at the hospital, the blood is evaluated and they only contact us if there is a problem with the levels. So far so good!
          The blood drawing is an experience to be sure, but all parents must play nurse to their children in some fashion. Some parents may be fortunate enough to get by with just band aids and hydrogen peroxide on their medical care resume. Our medical requirements extend a little further than that, but we're okay with it. It's all in the job description and we welcome every new experience that adds to our learning and understanding.