A Long Day at the Hospital and ER

Annie had her routine annual CDC appointment today where we meet with several of her specialist and therapists. From there, her neurologist then sent us to the ER. Here's a run-down of what we learned today...

First at CDC, we met with our orthopedist. It was quick and simple. Annie's spinal x-rays look great and he's not worried about her back. He said the chances of her back getting infected again this far out from surgery would be extremely rare.

Next, we met with our urologist, but since we've been in such close contact with him this week about Annie's new UTI, we didn't really discuss anything new. We still plan to have her back in clinic in a couple weeks to do regular bladder scans after every wet diaper over a full day. So far, with Annie hydrated again, she seems to be voiding just fine. But it will be good to see how efficiently she's emptying her bladder so we can then make informed decisions about regularly cathing her.

We then met with the developmental pediatrician who also doubled as our metabolics doctor today too. We got Annie's dexa bone density scan results which are pretty good, so no changes there. We asked about a lot of little random things, like all the injection sites on Annie's thighs that have left drops of blood under her skin. Every injection site is still clearly visible, leaving her thighs looking like a pin cushion from her twice daily shots she needs for the next 3-6 months.

Our dietician did her regular consultation with us to make sure Annie's getting the right amount of nutrients, minerals, and vitamins from her formula and her additional supplements. We always talk about calorie needs and assess her weight, and make sure her diet includes enough fluid for her needs. She's been a little low, so we'll be increasing her fluid intake a bit, but not adding more calories.

Our OT assessed Annie and actually told us about a clinic at UNMC that can help us transition Annie from her pediatric providers to new adult providers once she turns 21. Although that's a few years away still, it's a big deal to us since we love her pediatric providers so much and they have such a long history with her. It will be really hard to transition to new providers who don't know who she is or understand her unique history, or have ever even heard of Aicardi Syndrome. It will be a bit overwhelming, so we're grateful to know of this resource at UNMC.

The hospital pharmacist then visited us to make sure they have everything accurately in their system with all the meds Annie is on. That's always a quick visit.

Next was our neurologist, who we've been in close touch with the last several days with Annie's seizures being out of control. We talked at length, and Annie's last Ativan dose wore off, so our neurologist got to see her seizures first hand and how they recur every 10 minutes or so. She decided to have us go to the ER once we finished at CDC, to get a loading dose of fosphenytoin to stop the seizures since the Ativan isn't keeping them away. The Ativan has been stopping her seizures, but they come right back as soon as the Ativan wears off. Once she's had the loading dose in the ER, we'll start Annie on oral phenytoin (Dilantin) for just a couple weeks until her Sabril can catch back up in her system and start working again. The phenytoin has a lot of really nasty side effects, so we don't want her on it long. We'll wean her off of it in a couple weeks and hope the Sabril is working by then. We also talked about doing smaller doses of Ativan more frequently if we are still seeing seizures on the phenytoin. If Annie's seizures are still not under control once she's off the phenytoin, we'll discuss getting another drug on board, probably Onfi. But we're really praying that the Sabril kicks in here soon and we can get off the phenytoin without having to add anything else back in the mix. We still have an MRI of her brain scheduled in a couple weeks, so that will be interesting to check, since she hasn't had a brain MRI in several years.

So once the neurologist had seen Annie's display of seizures, we gave her more Ativan. Then our PT came in to visit with us. We didn't have any PT concerns, so it was just a simple visit and we mostly just chatted about other stuff. We love our PT and how personable she is. We make good friends with a lot of these doctors and therapists after seeing them regularly for so many years. That's why it's going to be so hard to leave them when Annie turns 21 and has to transition to adult providers.

Next was a good visit with our social worker. She talked to us some more about Annie's transition to adulthood and reminded us of how we'll need to apply for SSI for Annie soon when she turns 18. Between now and the age of 21 is a big transition time. Annie will have to move from her Medicaid waiver being managed by DHHS, to the League of Human Dignity. We learned that the waiver will pay me to be Annie's caregiver when she turns 19, and we can get our attorney fees covered by the hospital's legal aid department when we have to apply for guardianship of Annie. There's still lots more to learn about this transition, particularly whether to stay with waiver services or move to DD services when she turns 21. Both programs are great but offer very different things, so we still have a lot to learn before then. Our social worker is so great to point us to different organizations in the community that are excellent resources for parents like us.

We passed on seeing our pulmonologist today because we really don't have any concerns there. Annie's breathing is suppressed while on these sedating meds, so we've just started using her CPAP when she needs it, even though she hasn't needed it for several years now. We're comfortable with using it but hope its not a long-term thing.

From the CDC clinic we went down to the ER for Annie to get her loading dose of fosphenytoin. They had a difficult time starting the IV because Annie has had so many IVs lately that all her veins are pretty much shot. This fosphenytoin has to be given in the ER because there are risks with getting this large loading dose. So Annie had to be on monitors to watch her heart rate, respirations, oxygen sats, and blood pressure. This medication can cause low blood pressure and arrhythmia. Her blood pressure is pretty low even when healthy. She started at 89/60 and it dropped to 71/48, but once it got back up to 79/55, they let us go home. All her other vitals looked good, but her breathing has become very suppressed and she's easily obstructing.

Between this fosphenytoin and the Ativan from this morning, she quickly zonked out. We stayed in the ER for a few hours to monitor her and then headed home. Wednesday evening now, she's still passed out from these meds, but she hasn't had a seizure since about 12:45!! That's amazing! But I'd like to see her a bit more alert and awake and breathing easier.

While she had her IV in, they drew her labs to get a CBC. The ER doctor came to give us the results and thought we were a little crazy with how thrilled we were that her white blood count is down to 10k!! It hasn't been in normal range for the last 4-5 weeks! So that is some of the best news we've gotten all day! Annie's hemoglobin is 10 with a hematocrit of 33. So while that's still pretty low, it's not terrible. We suspect she just needs some more time to recover in that regard.

So it's been a super long day at the hospital and we are all rather exhausted. We have some mixed feelings about today... We're so relieved that Annie isn't having seizures, but we know there's still a chance they start coming back. And it's rather unsettling to see Annie so sedated with such suppressed breathing and hear her obstructing. We were hoping that she could soon sleep alone in her room without one of us in there throughout the night, but she'll require one of us monitoring her more closely again tonight. We'll see how her regular oral doses of phenytoin affect her, and hopefully her seizures will get controlled, without any breathing suppression, so both Justin and I can start getting more sleep each night. We mostly just want our alert and happy Annie back!