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"

Low Protein Foods on Amazon.com

Talli gets into food jags. I few months ago she was eating a LOT of french fries, so much that we had to limit how much she was eating per meal. Then it was Energy Options Candy Bars. Now it is Ener-g Gourmet Crackers. The problem is that I never know what food she is going to take to and for how long. It is easy with fries, since everyone else will eat them and they are readily available.

I only ordered 1 box of the Ener-g crackers when I ordered some low protein/allergy-free foods last week. Well, yesterday she ate 5 of the crackers and would have eaten more if there were more. The box only has 18. I called and visited several local stores and even though Target, Meijer, Jewel, Whole Foods, Fruitful Yield and Dominicks all have a gluten-free sections, none have the crackers. So in looking on-line this morning, I found that Amazon has Ener-g products in their Substribe & Save section. They ended up being only $2.43/box + free shipping, while Ener-g.com has them for $4.18/box +shipping.

So she will have to wait until Tuesday afternoon to get more crackers- I how she will still want them by then.

What Not to Say to a Mom with a Seriously or Terminally Ill Child

I came across this article today by Rachel Carpenter http://www.associatedcontent.com/article/1645757/what_not_to_say_to_a_mom_with_a_seriously_pg3.html?cat=25 and it is so true. These are things you should not say to a mom of a seriously ill child.
#1 "No matters what happens. it's God's will. [sic]"
#2 "I just know he/she will get better."
#3 "Are you needing help?"
#4 "I know exactly how you feel. My baby was in the hospital with RSV last year."
#5 "I had a friend go through this too. It was really tough for them, and now the child has lifelong disabilities."
#6 "I just don't know how you do it."
#7 "You need to relax. You need to be strong for your other children."

I definately get #6 a lot. I don't know how I do it either. Many days I want to stay in bed and pull the covers over my head, but I can't. There are feedings to do, appointments to make and go to, and kids to feed. If I looked pulled together it is because I rely on Diet Dr. Pepper and try really hard not to cry in front of my kids.

Occasionally, I get #3, and as the article points out, it is difficult to say 'yes' to the phrasing of that particular question. YES! YES! I always need help. It is horrible dragging 3 kids to an appointment, especially to offices that aren't kid friendly (most), have small rooms (many), it is an appointment where I actually need to hear what the doctor is saying (typically), or they run a couple of hours behind schedule (much more than one). I could definately use someone to cook us a meal that is low lactose (O), kiwi-free (M), peanut, egg, nut-free, etc. (Talli), low protein & low fat (Talli), and high protein & low carb (me). Meal planning is enough to want to crawl under the covers as mentioned above. ;).

A reminder to always check...

I know that everything should be checked with Talli: verify medicatins are correct, the right formula is given, packages of food are checked for allergens, IV fluids are correct, etc., etc.,

A couple of years ago, she was in the hospital and TPN had been ordered. It was a non-modified amino acid version, the kind anyone else would get. Some visitors were leaving when the IV bag was hung and as I walked them to the door, I checked the bag. The protein count on the bag wasn't what I had expected, so I alerted a nurse. The pharmacist had made a mistake and 24 hours worth of protein was in a bag to run for 4 hours. I am sure that no long term damage would have been done, but it likely would have prolonged her stay. Everyone was very nice and apologetic over the mistake and a new bag was sent up within the hour.

Yesterday, Talli got her delivery of some Energy Options candy bars. She has been eating a lot lately and we had run out of the mint and while chocolate versions. Energy Options candies are made to be completely protein free and are peanut and tree-nut free. To foster her indepenence, we usually have the bars in a designated place for her. Instread of having the imprint of "Energy Options" on the bar, it said "PhenylAde". Appled Nutrition makes both Energy Options candy and a bar called PhenylAde that is an amino acid modified food to replace formula for someone with PKU. The bar contains 10g protein equivalant, lacking only phenylalanine. So if Talli ate one, she would be getting 100% of her protein for the day.

The bars are getting sent back to the manufacturer, so they can verify where the problem lies. Is this actually a PhenylAde bare that got into an Energy Options package and box or an Energy Options bar with the wrong imprint. It was a reminder that everything does needs to be checked before it goes into Talli to keep her as well as she is.

I did check all of the mint ones by pressing through the wrapper to read the imprint on the bar.

Education Resources

I came across this information and just needed to share!

Baxter makes a couple of formulations of IVIG. Talli was going to start on one of them, but we found out they contain glycine. Talli's glycine level is already very elevated, which is a complication of PA. Baxter provides the following at no charge at www.mygardian.com/gardian/living-with-pi/resources.html

The blue bear, named IGI V, is included in the Therapeutic Play Kit. It contains the bear, medical supplies to give the bear and IV and remove it. There is a notebook for the child to record how the infusion went, a guide to theraputic play for parents, and a story book for kids. Even if the child just goes into the hospital occasionally and doesn't receive IVIG, this would be an excellent tool kit in helping them to cope.

We used IGI V during Talli's last hospitalization. She put an IV into the bear, including talking about how it was important not to get it too close to thumb for video game playing.

IGI V's Medical Records is included in the Theraputic Play Kit or you can order it separately.

The ABC's of Kids Like Me is an ABC picture book and some of the pages are medical related. For example, 'D' is for Doctor. The second half of the book is blank so you can create your own personalized ABC book.

This is My IVIG Book is a children's story book about getting IVIG. It includes a blank book (you could easily customize the cover by covering up IVIG with hospital or doctor), paper and stickers.

The last thing is for parents. There are 2 Notebooks in the Wellness Kit with divider pocket folders and blank pages to keep track of medical information, bills, etc. You could also customize for your metabolic child with the Care Notebook that the Propionic Acidemia Foundation put together. It is available under 'sites of interest' at http://www.pafoundation.com/. It is available in English and Spanish.

PA Consensus Conference

Tomorrow, I fly out to Washington, DC for a PA Concensus Conference sponsored by Children's National Medical Center. There will be a small group of metabolic doctors attending, as well as liver transplant experts, a neurologist, and representatives of parent groups. I will be representing the Propionic Acidemia Foundation, along with my dear friend, Jill Chertow Franks.

We hope to make great progress in creating documents with recommendations on screenings and monitoring. It is expected that the information gathered and discussed will be published.