Wasteful Wednesday- Food (a day late)

Sorry, I am a day late on this one.

One of the big parts of PA is the lack of interest in food and for us food jags.

Talli will go for weeks with only eating a few bites of food a day. She does always have a lunch packed for school and there is at least one dish she can have at dinner with the family. A couple of times a month, she will also choose what we are eating as a family. However, she will have food jags that are more like binges and want to eat a particular food.

It could be a whole box of Betty Crocker Fruit Snacks in one day (.8g protein and 800 kcals). That is easy to accommodate, since I can just run to the store and if she loses interest I can give them to the boys as a treat. When it is specialty low-protein foods; it can get expensive and a lot of food can be tossed if I overestimate the length of the food jag.

She has had food jags with Ener-g pretzels (~ $2.00 for a 2.65oz bag or about 8 times the cost of Rold Gold pretzels) and I overbought, several bags went bad. Also, with Energy Options candy bars where she was eating more than a bar per day ($2.94 for a 50g bar, the size of a Hershey bar) I ordered enough for 2 weeks and reorganized the pantry and misplaced them, although, it didn’t matter because she stopped eating them. I don’t think the Energy Options bars will be tossed in the future as my boys think they are great treats when they snatch some (blehhhhh!!).

It would be nice to have a local network of families to buy or swap with. OR IF food was covered we could go "shopping" at clinic and take what we need instead of trying to plan ahead and wait for it to ship. I guess insurance would need to recognize that medical foods are just as needed as formulas-- oh, wait! some don't even see the need for formula.

What is average and what does it mean?

I was met with frustration again this week when I was discussing something with a medical provider and she did not seem to understand some basic math terms that are used all of the time in the medical field. The specific point was the "median" life expectancy for those with Propionic Acidemia. Nothing has changed in the past 9.5 years and the median life expectancy remains at 3.5 years.

Median is the middle number when you are looking at a series. So in the series 1, 3, 5, 8, 9, 15, 17, 23, 49-- 9 is the median as it is the middle number. So to get 3.5 years as the median life expectancy in PA there are an equal number of affected individuals dying under age 3.5 years as those older than 3.5 years. Median is useful in describing a set of data or series where the numbers are in a far reaching range.

Mean is what people usually think of when they talk about averages, it is when you take a set of numbers and add them and then divide by how many numbers in the series. So looking at the same numbers above- 1, 3, 5, 8, 9, 15, 17, 23, 49-- Add them 1+3+5+8+9+15+17+23+49=130. Then divide 130 by 9 as there were 9 numbers in the series- 130/9=14.44

So with the same set of numbers the median and mean can be very different.

I was upset because this medical provider was telling me that propionic acidemia was not life threatening, when it is even when you are past the median life expectancy. She was essentially telling me that since she is older we don't have anything to worry about-- no, we do. We have to continue to be careful and cherish everyday because it is not known how long she could have. So shame on her for passing on misinformation from a person who should know these basic math concepts.

Pounding the Pavement for PA

On October 16th, I will be joined by several other parents and friends of those affected by Propionic Acidemia in Columbus, OH. We will be running in the Columbus Nationwide Half Marathon. Our goal is to raise $13,100 for the Propionic Acidemia (PA) Foundation, that is $1,000 for each mile of the race. My personal goal is to raise $4,000. I will donate $1 to the PA Foundation for each mile I run in training and in events. I will also be running in the Hot Chocolate 15K with my husband, Rawlo, on November 5th in Chicago.


HOT CHOCOLATE 5K 2010
Ryan, Jill's son on the left, I am in the center, and Jill, President of the PA Foundation is on the right

The PA Foundation is dedicated to finding improved treatments and a cure for Propionic Acidemia by funding research and providing information and support to families and medical professionals. I have been on the board of directors for the past 5 years and we try very hard to make sure we get the most out of the funds donated.

PA is a tough disorder. Talli has faired well compared to most others that we know of, but we do not know how her life will play out or for low long. PA can cause seizures, low muscle tone, developmental delays, cardiomyopathy (enlarged, weakened heart), Long QT Syndrome, low blood cell counts, osteoporosis, stroke, suppressed immune function, optic and auditory nerve atropy, pancreatitis, and very early death.

Please make a donation to the PA Foundation. You can do so through the Paypal link at http://www.pafoundation.com/, through my Firstgiving page at www.firstgiving.com/fundraiser/brittany-smith-2/poundingthepavementforpa, or send a check payable to Propionic Acidemia Foundation at 1963 McCraren Rd., Highland Park, IL 60035.

You can follow the progress of my training on the Firstgiving link above.


Talli October 2010



Talli October 2001, her first "crisis"

Psychological & Neuropsychological Testing

In November I attended PKU Illinois' annual meeting. During the meeting, Dr. Susan Waisbren made a presentation regarding psychological & neuropsychological testing in those with PKU. I did ask her if it applied to other disorders like Propionic Acidemia and she said it did.

My daughter has her first appoinement with a child psychiatrist to discuss learning issues and her anxiety around medical procedures. We also have a domain meeting in school next week as she is having her 3 year review.

The Genetics and Metabolism Psychology Network (GMPN) www.gmpsych.org lists reference articles and applicable testing in those with metabolic disorders. I found the information very helpful.

And there are always angels

There is another little angel in heaven.

For the past week, I have been talking and texting with another mom whose daughter has Propionic Acidemia (PA). There was also a lot of talk with a mutual friend as one of us might have a new piece of news on the little girl. At times, she would be doing better, then take a turn for the worse. Yesterday things did look better, but this morning they decided that no more could be done for her and took her off life support.

There is always sadness, survivor guilt for those of us who still have their children, and some happiness and peace for the child who does not have to deal with tubes or pokes any longer. Most of us know these things are coming as the life expectancy of PA is still only 3 years. This angel was 3.

Talli looks at me curiously when I tell her I am sad that someone died. It could be my grandmother or another child with PA. She tells me how they are with Jesus and are so happy.

May our newest Angel's mother have peace and the rest of us love our angels while we have them.