Saturday, February 4, 2012

The New Plan

A lot has happened this past week, so I will give a little recap of each day's events.


Tom, Allie, and I went to a cardiologist appointment for her to get her heart checked out.

She had an EKG, where they measured the electrical signals coming out of her heart: she passed with flying colors! Then, she had an ultrasound of her heart. They found a small heart defect known as as an atrial spetal defect.

The heart works by pumping blood from the body into the right atrium (the blue side in the diagram to the right). Blood is then pumped into the lungs where the blood is oxygenated and it returns into the left atrium (red). Then the oxygenated blood can return into circulation around the body.

Before a baby is born, the mother is oxygenating all the blood for the fetus and the fetal heart uses a shortcut and skips the right atrium. This shortcut is possible because of a hole that is present between the right and left atriums. When the baby is born, this hole should close up and the right atrium should take over the job of oxygenating the blood. However, in about 25% of people, this hole does not close of perfectly. This can causes things like heart murmurs and heart palpitations.

In Allie's case, the flap that was supposed to close the hole is just a little off. It wasn't entirely clear that there was even any blood moving through this alleged hole but the doctor wants to keep an eye on it to make sure it isn't creating a problem. The cardiologist assured us that this is not a major concern as most people never have any symptoms from it. In fact, said that she has a hole substantially larger than Allie's and it has never caused her any problems. Regardless, Allie is getting another heart ultrasound in a year to see if the hole has closed itself (40% of cases close completely on their own).

I had to go to work after the appointment, so Tom took Allie home and babysat her all day. Judging by this picture that I found on our camera, they had an exciting afternoon of playing baby hide and seek.

"Where's Allie?"
Allie moves into view from behind an object.


Allie had a busy morning. She had an appointment to get her head-shaping helmet refit and then an appointment with her occupational therapist to get a resting hand splint made to help stretch out Righty while she sleeps. She then went home and took a nice long nap.

When Allie woke up from her nap, she started having the same types of jerky seizures that she was having before. I took a quick video of one, sent it to her neurologist to update her, and then Allie started lunch. I was disappointed to see the seizures return but it wasn't entirely unexpected. There had been a few breakthrough seizure sets lately and we were starting to reconsider the Prednisone and Topamax cocktail that she was on. We were even considering having Allie admitted into the hospital that day to start a new more intense treatment.

Allie was sitting in the high chair tossing back her Cheerios when she suddenly slumped over and froze. She stayed like that for a couple seconds and then she popped up and grabbed some more Cheerios. I wasn't entirely sure that she wasn't just playing with me but she definitely got my attention. Then she did it again except she froze off to the side and I could see in her face that she wasn't playing. I immediately started packing Allie's bag while I was on the phone with the Children's Hospital having her neurologist paged. The doctor got on the phone and said to bring her in to the emergency room right away.

We spent a few hours there until we got moved to a room and prepared for a long stay.

The plan was to start Allie on an injected steroid called ACTH. This is the most intense medication that they have for Infantile Spasm. It requires twice daily injections, typically 3-5 days in the hospital, and has a litany of scary side effects including a dramatically lowered immune system, increased blood pressure, kidney failure, and bloating. We had been hoping that we wouldn't have to use this medication but at this point we were ready to try anything.

Allie's ACTH for the 6 weeks that she will be on it costs $90,000 and had to be shipped in from a specialty pharmacy in Florida.

Her neurologist had told us that Allie would get the initial injections from the hospital's very limited supply of ACTH and that we would need to bring our own in as soon as it arrived for the remaining injections. This seemed like a reasonable plan.

Later that night, the neurologist who was working our floor came in and said that Allie would not be receiving any of the hospital's ACTH since we had our own being delivered the next day. Jonathan and I sat there in total disbelief. Could this doctor really be saying that they would not treat our daughter because we have medication coming a day later? Once the shock wore off, my tears followed. What ensued was a near-hysterical crying fit as I sobbed and tried to convince, beg, and plead them to help Allie. The doctor just kept saying "we only have one vile of the medication and we want to use it judiciously." After what I guess was about 10 minutes of my sobbing, he agreed to ask his boss to weigh in on the decision to deny Allie treatment. Soon after, the doctor came back and said that Allie will get her first dose that night from the hospital's supply.

Apparently this issue was that three kids had come in that night to be treated for Infantile Spasms and each vile of medication is supposed to be attributed to only one patient even though each vile contains enough ACTH for about 2 weeks. Allie's medication was arriving sooner than the other kids so this doctor had decided to prioritize the other patients. But, his boss spoke with the pharmacy and had the vile partitioned so that all three kids (including our Allie) got the treatment that they needed until their own medication could arrive.

That night, Jonathan went home to await the early morning delivery of Allie's ACTH and I stayed in the hospital with Allie.

Friday was mostly just a day sitting around in the hospital. Bonnie flew into town late on Thursday night so Allie had the surprise of hanging out with Gama all day. We saw the neurologist from the night before around a few times and made sure to give him the stink eye.

Allie had quite a few visitors. Her PT and OT both came to visit. She also met with a Ophthalmologist who gave her an eye exam and said she looks good but they will have to do more tests as she gets older. A lot of stroke kids have vision issues. A developmental psychologist also came to meet her and get some information for an upcoming appointment.

Since we have to give the medication to Allie by injection, both Jonathan and I had to demonstrate that we were capable of giving her a shot. Jonathan took Friday night and passed, leaving Saturday morning for me.

That night, Jonathan stayed in the hospital and I went home and got some sleep.

Bonnie and I got to the hospital early and had another uneventful hospital morning awaiting my injection test. I passed! To our surprise, they let us leave the hospital. They said that Allie's vitals seemed stable and her blood/urine tests all looked good. We will be having a nurse come to our home twice a week to check Allie's blood pressure and glucose levels.

Her neurologist recommended that we pull her out of daycare while she is receiving ACTH because of her compromised immune system. Quite a few ACTH patients have died from infections and there is no reason to take a chance with a building full of germy kids.

When we got home today, Allie was so excited. She was chatting and playing and being a totally normal kid. Her face is starting to bloat from the treatment, which I take to be a sign that her body is starting to work with the medication. We were told that it takes between 3 to 14 days for the medication to take full effect. This is the best medication there is and is largely successful (one clinical study showed that ACTH stopped Infantile Spasms in 87% of patients).

We are anxious and nervous and scared, but also hopeful. It is horrible watching Allie go through all this but she is still our regular baby. We haven't noticed any of the cognitive decline or personality change that often accompanies IS. Hopefully we caught it early enough and this will just be a stepping stone in her recovery.

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