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.

Mucinex mini-melts for Kids! not compatible with feeding tube

Talli has a pretty nasty cough. Her pallative care nurse suggested Robitussin DM, however, the pharmacy only had adult Robitussin DM, so the pharmacist suggested using Mucinex mini melts. She indicated that it is like Prevacid Solutabs, so would fit into her feeding tube.

We did try it last night. Unfortunately, Mucinex completely clogged up her tube AND her button. Changing a button is anxiety provoking to Talli, so I try to avoid it as much as possible. After cleaning out the extension set while not attached to her, I realized that her button was indeed clogged. I found a bamboo skewer and gently used the tip to unclog the button. The one way valve on the button was already shot, so I didn't have to worry about breaking it.

So Mucinex mini-melt for Kids! doesn't work with a feeding tube, so stick to the liquid version. Prevacid Solutabs and Tylenol meltaways DO work in a feeding tube.

Hospital Beds

Hospital beds, what can be done to get a good night's sleep in one? I don't mean the electronic beds that raise and lower, fold, and weigh patients. I mean the ones that the parent or significant other sleeps in to be near the patient.

The hospital that Talli usually stays at has 3 different types of beds, the worse being a hide-a-bed type, that folds out from a chair with a thin, likely spring mattress. The better ones in my opinion are more like day-beds that a couches and may have removable pillows or pull out slightly from the wall. My dorm bed in college was this latter type.

Over the weekend, I spent the night with Talli in the hospital. For the past 3 years, my husband has taken the honors on almost every night (40+ nights), however, he was sick when Talli was admitted, so I stayed with her. Unfortunately, she was put in a small room that will only accommodate the worst of the parent beds. I tried a couple of things to help make my attempt at some sound sleep-
1. sleep with your head at the foot of the bed. While initially, it felt an though I was laying downhill, a couple of pillows remedied that. The mattress was less worn at the foot.

2. layer a couple of blankets. I folded 2 blankets in half under the sheet. This gave more support and evened out the feel of the springs, so one wasn't poking me in the knee or some other random place.

3. Sleep in the parent room or "quiet room". I didn't do it this time, but I have in the past. The beds seem to the newer and more comfortable in there. Even in our hospital with about 15 peds beds, there is a lounge-type room with an extra bed in it. At larger facilities, we have used their parent suite that had several beds and a full bath with sitting area.

4. Trade beds. Talli loves to sleep in the pull outs, so often she and my husband switch (with the nurse's ok). When O had surgery a few weeks ago, the nurse gave me the option of just having a hospital bed in the room to sleep together, instead of a crib for him. However, with that option, I would have needed to stay with him ALL of the time and I knew at some point I would need a crib to just use the bathroom.
When Talli was younger she also liked to sleep in the wagon they had in the unit, so that freed up her bed. When she had pneumonia once, she would only use the oxygen if she could sleep in the wagon.

Please post a comment if you have other ideas on how to get a good night's sleep in the hospital.

Soft Clothing

A few months ago, I posted about SOFT Clothing. I got some this week that I had ordered for Talli. I got the demin pants and a khaki pair. I ended up ordering a little bit too big for her, so will have to get a smaller size. I think they are true to size. She is short and a bit pudgy with a spinal dimple, so she needs something with a high rise and short legs. I think the next smaller size will fit her perfectly.

They are incredibly soft!! She is able to get them on herself, since they do not have fasteners. She only wore them for a sort period of time, since they were too long, but in that time she didn't get any hives. They do have flat interlock seams, similar to Hanna Andersson's infant clothes.

Update

I hope to be back to posting more regularly. We have been having a pretty busy couple of months. We did close on our new house in June and then I promptly left to spend a month at my parents' place with the kids. Once home, we had a protracted battle with our school and Talli ended up missing most of the first 6 weeks of school (so I was her teacher). O ended up having some surgery.

We have had weeks with 4-6 medical appointments. I try very hard not to have more than one in a week, but we lost 3 medical providers over the summer because of the physicians leaving their practices. It took several meet and greets and appointments until we could find the right fit for us. We are still looking for a pediatric GI specialist, but that is not something incredibly urgent for us.

In all of this our beloved babysitter moved away. We are on the search for other helpers.

Restocking the Emergency Box

I like to be prepared for situations that may not come up a lot, but would be pretty darn inconvient or unsafe if I wasn't prepared. An important part of this for me is having supplies for Talli to get through an emergency. Say if we had to evacuate or we had a fire, flood, or tornado, all unlikely situations, but they could come up. So I keep a couple days worth of supplies with a friend who lives about am hour away.

Today, I met up with my friend Jill. Her son also has Propionic Acidemia, but is on a different brand of medication and he only uses 2 of the 4 formulas that Talli uses. So last night we were chatting and e-mailing about what was in the last box we left at each others houses. Everything had expired or was no longer usable (i.e. diapers that were too small).

In the couple of years that we have been doing this, Jill has not had to use her supplies that were at my place. However, we have had to use what she had stored for us. We are regularly near Jill's place for religious services. Three years ago, I was coming back from Michigan from visiting with some friends and was to meet up with my husband, Talli, and M, so Rawlo could attend a religious service near Jill's house. I arrived a little late and Rawlo had already left (some friends were watching our kids), Talli's feeding had been forgotten at home. I thought that she would be ok with some sugar water in her feeding tube.

Unfortunately, Talli was sick with something and when Talli gets sick, she get sick quickly. Within about an hour, she fell asleep (a sign things are pretty bad) and wouldn't wake up. As I packed up our things to head home, she started to vomit. I made a quick call to Jill who made up a batch of no-protein formula and a friend carried Talli to my car. She continued to throw up on the quick trip to the car. We made it to Jill's house in about 15 minutes and got her some formula and cleaned her up. Thankfully, by the time we arrived back to our house she had perked up and could be roused. NOTE TO SELF: put some extra clothes in the box at Jill's place.

So, while we have only used the supplies once, that one time did save Talli from a likely hospitalization.

For those with similar disorders or any chronic medical condition that requires medication, any kind of emergency like I previously mentioned could impact the health of the individual as emergency responders (i.e. Red Cross) are usually not set up to provide for chronic medical needs. It is best to be prepared, so that you are not dependent upon others in these types of situations. For us, it typically takes at least 3 days to get formula from our supplier.

Ready.gov has information in being prepared for those with disabilities and medical conditions. This page has the most information: http://www.ready.gov/america/getakit/disabled.html