The Most Annoying Part...

Some people may think that the most frustrating thing about being the parent of a special needs/medically fragile child are the child's needs themselves. The constant fear of hospitalizations, the struggle to reach milestones, the endless quest to find the right mix of medicines.

However, while these things are indeed frustrating and tiring, what's even more tedious are the day to day struggles that we parents deal with. Here are a few samples of recent conversations. (Names have been changed to protect the innocent.)

Medical Supply Company

8 AM
Mom: Hi, my daughter's feeding pump doesn't seem to be charging.
Rep: I'm so sorry about that. I can get another pump out to you today.
Mom: Great. How quickly will it arrive? She uses the pump for lunch around 1 pm.
Rep: No problem, the pump should be there in 3-4 hours, so no later than noon.
Mom: Great, thanks.

12:30 PM
Mom: Hi, I'm calling about a replacement feeding pump that should have arrived by noon.
Rep #2: Sorry, let me check on that. (long pause) Oh, it looks like the replacement pump won't arrive until at least 3 pm.
Mom: I was told that it would arrive by noon.
Rep #2: Sorry about that m'am.

Pharmacy

Pharmacy Tech: Hi, I'm calling about a refill order that you placed for your daughter.
Mom: Yes, I'll be at the hospital tomorrow and can pick up the compounded medicine.
Tech: Oh..well, you should have enough of your current medicine to get you through the next 3-4 days.
Mom: Yes, I do. But, I will be at the hospital tomorrow and can pick it up rather than go through the hassle of delivering it.
Tech: Oh, it's no problem. We'd rather not overlap since the compounded medicine is only good for 28 days...so we'll deliver it to arrive at your home on Tuesday morning.
Mom: OK. That's fine.

(Next day)
Tech: Hi m'am. We have your daughter's medicine here for you to pick up.
Mom: This is Michelle*, right? We spoke yesterday about you delivering it on Tuesday morning.
Michelle*: Oh, right...but I saw on our clipboard that it was to be ready today, so we got it ready for today.
Mom: Well, I don't know about your clipboard, but we are now home from the hospital, and I'm not coming back to pick it up. You'll have to remake it and deliver it for Tuesday.

Prescription Drug Benefit Company

Dad: Hi, I'm calling about my daughter's compounded solution of Prevacid. It seems that the Pharmacy is having trouble running it through insurance because of a specific ingredient they have to use.
Rep: Which ingredient?
Dad: Ironically enough, it's just purified water. They can't use any of the sugary syrups due to my daughter's special diet, and the other ingredient - sodium bicarbonate - is on backorder. So they just want to substitute purified water. However, your computer system is not accepting the purified water as an approved ingredient.
Rep: You are right, the computer is not telling me it's an approved ingredient.
Dad: What is your common sense telling you?
Rep: I'm sorry sir, the pharmacy will have to fax in the label for the purified water and our pharmacist here will have to approve it and an NDC code needs to be entered into the computer for it to be approved.
Dad: Can't you just put an override in the system so my daughter can get her medicine?
Rep: I'm sorry, we can't do that.
Dad: How about I run a bottled water over to the Pharmacy for them to use?
Rep: I'm sorry sir, this is our protocol and it will take at least 15 days to get this resolved.

(15 days later)
Dad recaps conversation above.
Rep: I'm sorry it seems there is no way to get this entered into our computer.
Dad: Really? We did everything you said. The pharmacy sent the information, and you're still not able to approve it?
Rep: We just can't get it in the computer as an approved ingredient.
Dad: So, you're telling me that because your company can't figure out a way to get it in your computer my daughter must go without her medicine.
Rep: I'm sorry, sir.


Add about thirty other similar conversations and there you have it...the most annoying part.

Note: A third character, Inner Monologue, has been left out due to the dialogue being laced with profanity.

One Year Ago. Hindsight.

One year ago, we were preparing for what would be the biggest procedure of Sadie's life. On February 23rd, 2012, she had part of the right side of her brain removed. Similar to a hemispherectomy, the surgery removed her right frontal and parietal lobes of her brain. The hope was that by removing these areas of seizure focus, she would experience fewer seizures and have a better quality of life. The full surgery story can be found here.

It feels a little surreal to be approaching the one-year anniversary of her surgery. In some respects, it feels like it happened so long ago. It's been a rough year for Miss Sadie - a long recovery in Cleveland followed by two subsequent brain surgeries - a cyst fenestration in August to alleviate hydrocephalus, and a shunt placement in December when the hydrocephalus returned.

We knew going into the surgery last year that there could be a range of outcomes - anywhere from significant reduction in her seizures to no reduction. While we did see some initial reduction in the months following the surgery, the seizures seem to have found a new focus area on the other side of her brain.

Is this disheartening, yes. Does it make us question our decision? Of course. But we know that these are the unfortunate decisions that we as parents of a medically fragile child have to make in order to improve her quality of life. They are decisions that we wish we never had to make. We simply have to move forward and continue to do everything we can to make sure she has the best life possible.

On that note, a bit of good news is that her pee strike seems to be coming to an end - yay! After having to cath every four hours for a few weeks, she finally decided to start peeing again on her own. I was never so happy to see a wet diaper. Champagne was popped and celebration ensued. She is now peeing on her own about 90% of the time. There have been a few times every other day or so, when we have to go in to get the pee, but it seems that her bladder has regained it's function.

Ultimately, who knows why she started retaining her urine. Although the pee strike seemed to coincide with her treatment on the seizure medication, Banzel. While urinary retention is not a listed side-effect, Banzel proved to be an awful medicine for Sadie. It intensified her acid reflux - causing her to vomit a few times and actually seemed to make her seizures worse. Once we started weaning her off the medicine, she had fewer seizures. Case made that every girl with Aicardi Syndrome is so different, as Banzel has been a great medicine for many of Sadie's Aicardi sisters.



Activating her light box during therapy

While the pee issues seem to be getting better, the constipation issues continue. We are following the treatment plan of Sadie's GI Motility Specialist who has ordered an anorecal manometry study. She will undergo this test next week to determine whether her muscles "down there" are operating properly to pass stool. Fun stuff.

Another bit of good news, is that Sadie completed her steroid treatment a few weeks ago - whew! We were hoping that getting her off steroids would help with her sleep issues. Unfortunately that does not seem to be the case. As a matter of fact, her sleep patterns have been getting progressively worse lately. She has been a bit hyperactive lately and is unable to nap during the day. At night, she is able to get to sleep, but staying asleep has been an issue. On a typical night, she is up between 3-6 times. The entire house is sleep-deprived.



How the Vagal Nerve Stimulator (VNS) works

We had been holding off on investigating the sleep issue in great depth, thinking that the sleep problems were related to her seizure activity - hoping that better seizure control would equal better sleep. After almost three years, and only a handful of nights where she's slept the entire night - we're ready to pursue an in-depth sleep analysis. More to come on that.

We have also scheduled an appointment to discuss a Vagal Nerve Stimulator (VNS) for Sadie. A VNS is like a pacemaker for the brain - helping keep the brain waves from getting too crazy. VNS has been a highly effective tool for girls with Aicardi Syndrome, and we can only hope that it is just as helpful for Sadie.

So, lots going on in Sadie-Land. She is back in the swing of things with all of her therapies. And overall is more alert and attentive - giving out lots of smiles, which makes this crazy journey a lot more enjoyable!