"Don't share food", said with a stern expression, while you are giddy inside

Those with Propionic Acidemia often have anorexia. They have no desire or limited desire to eat, so often depend on feeding tubes. I don't know of any affected individuals who have swallowing issues, they just don't eat.

I had a sweet kind of peace when we stopped feeding therapy when she was about 5 years old. She had eaten as an infant and then as a toddler she ate quite a bit. She got her g-tube at 6 months old, which relieved a lot of stress about her getting the food that she needs, especially when she is sick.

Talli does have a good relationship with food, thanks to all of that therapy. She eats when she wants. She is developing table manners. We rely on how much she eats as a guide to how well she is feeling. She gets in food jags, much like toddlers do and wants to eat the same thing all of the time. A few months ago she wanted to eat plained buttered noodles, every day. Now she has moved on to Wavy Lay's potato chips.

Because of food allergies and germs food sharing is not a good idea. But with things like potato chips that have 2 grams of protein per serving (she only gets 14 grams/day) we need to make sure that we know what she is eating. So as I give the kids a snack I tell them that there is no sharing and they repeat the rule back to me.

This afternoon, I just got a chuckle after M had settled down because Talli had swiped all of his chips and eaten them. What a normal sibling thing to do! Since the bag of chips was empty, Talli agreed to buy M a small bag of chips and she apologized. It is also very ironic because M, who is 2, but looks almost 5 asks every day, "Can I have Talli's______?", after she has finished with a meal.

Cherrybrook Kitchen

I am so excited! Today I received in the mail 3 coupons for free Cherrybrook Kitchen products. Cherrybrook Kitchen is an allergy friendly and also low protein food company. They sell cookies, brownies, baking mixes, frostings, and they have a gluten-free line as well. They are sold on-line, but also at health food stores, Target, and locally for us, Jewel-Osco/Albertson's.

They have a rewards program called "CBK Rewards" where you send in the bottoms of the boxes with the UPC code for cool stuff.

A Cup-a-cake cupcake holder is 4 box bottoms
A free coupon is 8 box bottoms
An iPod Nano (8GB) is 125 box bottoms
There are more, this is just a sampling.

We really love the mixes. When Talli couldn't have wheat we would use the wheat-free mixes and everyone in the family ate them.

Needless IV lines & Oral Med Syringes

Multiple brands of needleless IV systems can accomidate oral med syringes into their hubs. With increasing use of needleless IV lines there is risk of mistakes in using oral med syringes in those lines. Here is an older article listing the problem: www.ismp.org/hazardalerts/inject.asp

Baxa Corp has several new oral med syringes that are incompatable with needleless IV lines. They also have syringes that are orange and purple to differentiate them from IV syringes.

Be aware in a hospital setting that oral med syringes (non-sterile) are not used in your child's IV line. You are their biggest advocate.

What my other child needs to see a specialist?!

Baby O had his 12 month check-up this past week. It was a bit delayed because M had an appointment the day we were suppose to go. He has always been on the smaller side, but he has fallen off the growth chart in length, weight, and the very critical head size. His pediatrician wanted him to see an endocrinologist. Since Talli sees one, I scheduled an appointment with him.

The endocrinologist has already told me that work is his life-- he has office hours 6 days a week at various Chicagoland locations. We started seeing him when he was on staff at Loyola University. We were able to get in this morning.

We are changing up his diet a bit-- more fat and we will be adding extra calories to his bottle. He suggested 22 calories/oz and after discussion he said to go up to 30cals/oz if he tolerates it. Since we have polycose & prophree around and we can do the math in our heads, we will add that in to the formula. Since his growth was poor, the pediatrician said to not take him off formula. He had a bunch of labs drawn and we will see what comes up.

I think after Talli something like this comes up and we just shrug our shoulders and go on. Food is always an issue in the house, so my big hope is that celiac disease is not the problem, but that is so easily managed. If he needs ongoing follow-up, we can easily schedule Talli's & O's appointments for the same time. The doctor brought up finances at the appointment and I told him if it was a problem I would let him know, $50 co-pays stink, but we do plan for them.

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.

Allergy friendly foods & Peanut Free Planet

While Talli does not eat much, having safe food around is always a concern. She is currently eating 100-200 calories a day, but since some she is sensitive enough to some allergens that being in the room with them or touching a contaminated surface and then biting her nail can cause a severe reaction we are always cautious.

Tree nuts are one of her most serve allergies, peanuts not so much, but peanut allergies tend to get worse with time. She is also on a very low protein diet, so that adds quite a bit of a complication. Cambrooke Foods is known in the metabolic community for good tasting low protein foods, however, and this is the big one for us, they make peanut and egg containing products, so we assume their whole product line is contaminated. Usually, when I call a low protein food vendor about allergies and cross contamination I hear in a quick raised voice, "Ma'am, we make foods for metabolic disorders not allergies." And my response is..., "My child has both." It has been far easier to get the protein counts on allergy friendly foods, some of which are very low protein than find safe low protein foods.

Here are a few companies that we enjoy:
Applied Nutrition- Low protein chocolate (only appealing to the low-protein crowd) that is allergy safe http://www.medicalfood.com/ They do have cute Easter shapes.
Ener-g- They do manufacturer with some nuts at some facilities, so a call to them is usually needed http://www.energ.com/ We all like their pretzels and Gourmet crackers.
CherryBrook Kitchen- Allergy friendly baking products- cake, cookie, pancake, and brownie mixes and ready made cookies. http://www.cherrybrookkitchen.com/ Save the box bottoms as you can redeem them for mixes, t-shirts, cupcake holders, and an ipod. Target carries some of these as does Jewel/Albertsons and Whole Foods.
Enjoy Life- cereal, cookies, granola bars, etc. Some items are too high in protein for us to use. http://www.enjoylifefoods.com/
Divvies- Cupcakes, popcorn, cookies and candy. http://www.divvies.com/ Amazon sometimes sells them as well. Our local peanut-free restaurant stocks them as does Disney World.

Ok, so the last one is Peanut Free Planet http://www.peanutfreeplanet.com/. We have ordered from them a few times. I am preparing for our annual Easter Egg Hunt that we host and was trying to come up with candies to put in the eggs. I also give out prizes as well. I was thrilled to find chocolate foil covered eggs to go into the plastic eggs as well as an inexpensive chocolate bunny to give out as a prize. So here is my order:
Amanda's Own Chocolate Baby Bunny $2.69/2oz (2, one for a prize and one for ODS's basket)
Comet MiniChick milk Chocolate Egg Bag $19.99/2.98lbs
Gimbal's Cherry Lovers 9 Cherry Favorites (for DH & DSs baskets)
Sunbutter $4.49/1lb Nearly the same taste as peanut butter and slightly better taste than Trader joe's brand

The chocolate is likely something Talli won't like the taste of because she is used to the low protein version, but at least it will be safe around her.

Happy Egg Hunting everyone!

Child Life Specialists & IVs

On Friday, Talli was scheduled for her regular visit to our local hospital for IVIG. However, just a few minutes before we headed over, we got a call from one of the nurses that they were "bursting at the seems with cases of RSV". She asked if we wanted to come in on Saturday morning when the nursing staff would hopefully be able to see her right away. She thought if we came in as scheduled it would take a while to get a room on the floor, as they were into two over flow units, and probably 6-7 hours before her IV would get running.

When we scheduled it we had asked the unit's clinical leader if their new Child Life Specialist would be able to be with us while Talli got her IV. We had met her in December shortly before Talli was discharged. She had some ideas on how to make things go smoother in getting the IV established and some techniques to get Talli to relax. After the staff called me, they quickly called the Child Life Specialist and called me back to say that she would come in on Saturday to help with Talli.

IVs are a particular difficultly with Talli, which is putting it very lightly. With Propionic Acidemia the veins become very fragile and can break or "blow", an added problem is that when sick or fasting the fluids that are needed to put through the IV and the rate at which they need to run to be effective can irritate and damage the vessels such that they "blow" and a new one needs to be put in. Then there is the frequency problem- There are just a lot of IVs and blood draws needed. When she was an infant/toddler she was hospitalized every 4-6 weeks for 3-14 days. Occassionally, we would stay in the ER for 12 hours, but typically we would have to return again within the next 24 hours because it was not enough. For blood draws, they were daily, even several times a day, in her first month of life, over time it has tapered down to about once a month, unless there is some wrong. Then there is the problem that she just has shotty veins, they are tiny, they hide, and they are just scarred from getting poked a lot.

Because of these difficulties it takes on average 3 attempts to get an IV line in. On rare occasions, it is just one attemple, on others it is more than the number of fingers on one hand. So it is understandable why a small child would be upset by getting an IV. Once the nurses or doctor tries for an IV, she starts wiggling, screaming, spilling, and kicking. However, after the nurse counts "1 2 3" and pricks her skin she holds still. She will then give instructions to the nurses regarding how they should tape and put a house and board on her. Then comes the flush, the final test to make sure that IV is actually good. A flush is usually a small amount of saline (salt water), so they can see that fluid is going into the vein like it should, if the IV is not in the right place or the vein has "blown" they will either get resistance or a lump or bruise will show up under the skin. Often times, the nurses put self imposed limits on the number of attempts they will try for an IV. If they can not get it in those attempts, they call for someone else, which could be a 15min to 2 hour wait. So the process and trauma could get repeated and repeated. Again, she has gone through this enough to know that if it is not good, which chances are that it is not, she will get a nice bruise which does hurt.

So back to Child Life Specialists (CLS), they usually have a Bacheler's or Master's degree in Child Life. There are multiple programs throughout the country for training. They do play therapy with children on getting procedures, distract them while a procedure is occurring, train staff on ways to minimize trauma and anxiety in the child, they often coordinate and train volunteers, and organize and purchase supplies for the play room. Our local hospital had just hired a new CLS a few months ago to work part-time. She met with us in December when it was close to discharge time, but did get to observe Talli getting 1 dose of the H1N1 vaccination. Earlier in January, she spoke with the Art Therapist that has been coming to the house through Pallative Care to come up with some more ideas.

So Saturday morning, she came in and sat with us for a couple of hours. She talked with Talli about what would happen with the IV and blood draw (she needed 6 tubes of blood tested) and what Talli's role was- Hold still and not spit. We were incredibly blessed that it only took 1 attempt to get the IV in and they were able to get all of the blood that was needed.

Here are some things that were done to help minimize Talli's anxiety and keep her still voluntarily:
-CLS met with us previously (in December) and came with cool toys and a terrific stuff whale.
-CLS talked to Talli prior to the IV about what was going to happen and reminded her about her two responsibilities- holding still and not spitting.
-CLS brought new toys to distract Talli during the IV.
-Nurse counted "1 2 3" then stuck her with the needle, so she knew what was coming.
-Talli held onto some soft playing dough (NOT playdough brand as is has LATEX in it) with the hand not getting the IV.
-I sat in the bed with Talli on my lap.
-The arm getting the IV was propped on two pillows at her side.
-We muted the TV, so she could focus on me or the CLS talking to her.
-Continuous encouragement from tech, CLS, and me about sitting so well.
-Wrapped "prize" was given to Talli once the IV was all taped up.
-Arranged with the nurse prior to the IV placement to have fluids running after the IV was in to minimize flushes.
-Brought from home a bag of toys (plastic slinky, mini pinwheel, grow capsules, playing dough) that is only for use in the hospital and she doesn't have to share with her brothers.

One of the problems in general is that CLS time and services can not be billed to insurance companies, so their pay and their resources have to come out of a hospital or clinic's bottom line. Talli have been in numerous hospitals throughout the country in our travels and moves and we have found these facilities to have good Child Life Programs (CLP), things may have changed since we have been there last-
-Primary Children's Hospital in Salt Lake City, UT- the best CLP we have experienced
-Lutheran General Hosptital in Park Ridge, IL- they seem a bit stretched with the new pediatric unit that opened in 2009, but are also good
-Edward Hospital in Naperville, IL- this is our local hospital and the CLS seems to be doing good work thus far
-Oncology/Hematology clinics at Hope Children's Hospital and Central Dupage Hospital-we didn't see a CLS there, however, you can see their hand in how the clinic runs with volunteers in the lobby, snacks in the lobby, video game consoles in the lobby, a treasure box, staff walking patients to the blood lab and putting them in the front of the line for a draw.