On February 27, 2013 at 7:10 pm, Calvin started having a bigger, more physical seizure. I picked him up and watched him (since there is nothing I can do to stop seizures). He continued to seize, so I gave him an emergency dose of anti-seizure medications rectally at 7:24pm. That didn’t work, he seized actively/physically until 8pm, at which point the seizures calmed down a bit and got less rhythmic and more sporadic. He was still seizing/twitchy and had been awake, refusing food and seizing continuously well into the next day. I did not take him to the emergency room because normally he comes out of it after the medication is given. The next morning, after having been awake all night, refusing food and seizing, we made the decision to take him back to Georgetown Hospital, on the advice of his neurologist. At 2 pm we arrived at Georgetown and were immediately admitted to the PICU (they had a bed waiting for us.)
They started an IV for fluids and gave him 3 doses of Ativan to try and stop the seizures, none of which worked. The nurses and doctors were running around in a sort of panic mode because of the continuous seizing and because he seemed almost lifeless and was unresponsive to touch or stimulation.
When we first got there, the nurses took several temps that were about 100.4, so they were concerned that he had a bacteria/virus. They started him on 2 IV antibiotics and an antiviral to try and fix that. They also did an LP (lumbar puncture) to check his spinal fluid for meningitis, which came back negative. That did show, however, that he had elevated white blood cells, which could indicate an infection, which may have been what triggered the uncontrollable seizure to begin with. They hooked him to an EEG machine to watch his brain activity, and he is remained hooked up to the EEG until March 5th, at which point they removed it temporarily to let his skin breathe and heal. A few days after we got there, the fluid they had been giving him started to build up in his body and he looked very "puffy" and bloated all over. They put a feeding tube (NG tube) down his nose to give him nutrition that way, and they stopped the IV fluids. He still had the IV in, because they were giving him his medications through that.
The EEG results were showing his brain function as extremely disorganized and muddled. The neurologists kept telling us that it was hard for them to pick apart and determine what is and what is not a seizure because the waves are so scattered. The neurologists were telling us that his seizures keep increasing; the EEG showed 75 in a 24 hour time period, then 80, then 115 (on 3/3) then 125 seizures. Calvin was seizing continuously for the first week or two that we were there in the PICU. They were giving him doses of Ativan whenever he was clinically (physically) seizing for more than 5 minutes, and those doses lasted about 2 hours, at which point he would go right back to seizing.
The neurologists were suggesting several different medications and courses of treatments, all of which are "not ideal." They wants us to consider a 6 week course of steroid shots to help seizures;1 shot 2x per day. They tried two new medications (Fosphenytoin and Vimpat) {in addition to the 3 he's always on}). The neurologists stopped the Fosphenytoin a few days after they started it because they determined it wasn’t working. They also started him on the Ketogenic diet again (they tried this when he was in the NICU and it did not work) on March 4th, that is supposed to stop/diminish seizures. They changed his feeding tube from an NG to an ND tube, which just means it’s now in the duodenum (first part of the small intestine) instead of the stomach and they have him on continuous feeds rather than bolus (5 oz. at a time) because they thought it was too much for his stomach to handle and it was bloating him and he would be more comfortable.
On March 4, 2013, we met with Calvin's clinical geneticist and she told us that she got verbal/email confirmation from the clinical study that they included Calvin in (when he was in the NICU) that show he has mutations in 2 genes that were isolated for Microcephaly Capillary Malformation Syndrome. This confirmation doesn’t really mean anything though; it doesn’t tell us how to treat him or how to stop the seizures...all it does is give us a name. She said that I have a copy of one of the genes and so does Ryan (Autosomal Recessive), so that’s how it got passed on to Calvin. The difference is his are mutated. This also means that future babies have a 25% chance of having the same syndrome. The geneticist also wants our permission to publish their own paper/research study for the medical community about Calvin and their findings. This paper will include detailed pictures, not to be published for the general public, only for internal use or in the medical community.
They did another MRI shortly before we were discharged that showed more atrophy of his brain and brought more bad news. The PICU attending doctor and the chief neurologist sat Ryan and I down on several different occasions and told us that we need to seriously think about the fact that Calvin is on the decline and at any moment he could have another "crisis" or massive seizure/event that could end his life. I asked the neurologist, based on what he knows about Calvin's brain and the rate at which it is shrinking, what type of life expectancy he thinks Calvin will have. His response was "its going to be extremely short." They recommended that we enroll Calvin in hospice care when we get home and just enjoy what time we have with him, however short it may be. After those conversations, Ryan told me to call my parents and get them to come out and spend some time with Calvin. I told him that I didn't think Calvin was going to die in the next week- I just felt in my heart that he wouldn't and he just looked at me and said "I don't know babe....just call your parents." Up until that point, I felt like all the things the doctors were saying were worst case scenario, and they HAD to say that. I felt like it was their job to prepare us for the worst, but that we weren't necessarily there yet. When Ryan said those words to me, it hit me hard. That was the first point that I broke down HARD and completely lost it. I'm not supposed to, at 27 years old, have to call people and tell him that they need to come spend time with my son because he is going to die. So, I stepped outside on my way to get food and called my dad. That was one of the hardest phone calls of my life. "I don't know Dad, you just need to come out. I don't know when he's going to die, but I want you guys to have time with him regardless. Maybe he wont die anytime soon, maybe he'll die tomorrow. I just don't know."
We signed a Do Not Resuscitate order for Calvin, with much heartache and many tears. This decision did not come lightly, and it is one that Ryan and I have made based on what we know about Calvin's syndrome. We know his prognosis is bad: he is never going to get better, he will only continue to decline. We want his quality of life to be fair, we don't want him to suffer. Who are we to artificially keep him alive, because of our need and desire to have him here with us? I believe that if he is not meant to be with us, then he will be in a better place and it's not my decision as to when and where that happens. You may not agree with that decision, and you don't have to. Its the hardest decision I have ever had to make, but its one we made out of love. Never before in the course of our 8 year relationship have I seen the type raw emotions that my husband had in the course of this hospital stay. And I wish I never had....I hate that this is happening and I hate that he hurts.
March 5th was the first day Calvin seemed to be better/different. He opened his eyes open for a couple hours and was talking/cooing for a bit that morning, which he hadn’t done since the seizures started on February 27th. Not sure if the Vimpat or the diet is working at this point. Also on March 5th, the nurses were telling us that he did not have any ketones in his urine yet, which means he is not Ketotic, which is the point of the Ketogenic diet (no ketones=diet is not working yet.) Eventually we got Calvin up to 80+ Ketones, which means he is ketotic. The next weeks were spent trying to get him off the feeding tube and back on bottle feeding, continuing medications and watching his brain waves on the EEG. We finally got discharged on March 21, 2013 (21 days after we were admitted) from the hospital. We took Calvin home with the feeding tube still in his nose, because we had to, in order to get insurance to cover the formula he needed for the diet. Otherwise, the formula was $250+ per can (that lasted 4 days). He was however, taking every feeding and all his medication via bottle.
A few days before we were discharged the neurologist recommended that we begin the course of steroid shots for Calvin and we agreed, as sort of a last resort, since there essentially no more options as far as medicines to help him. So, the nurses taught Ryan and I how to reconstitute (mix) the medicine, draw it up into the needles and inject the steroids into his thighs. It started as two shots at a time (Ryan would inject one thigh while I simultaneously injected the other thigh) once a day and then the nurses weren't comfortable with the amount of fluid being injected at one time, so it was broken up into 3 shots. I spoke with his neurologist about it because I wasn't happy about that, and it turns out the dosage we had been giving him (based on what one neurologist wrote the prescription for) was way too high (about 3x more than he needed) so once it was corrected, it was down to one injection twice a day. I absolutely hated having to give him those injections. I cried every single time, for probably a week and a half. He was such a trooper, but it was so hard to have to stab him with that stupid needle twice a day, even though it was supposed to be helping him. We (I) did those shots for 4 weeks after we went home, at which point we stopped them on advice of his neurologist because they didn't seem to be working and/or getting the results we were looking for.
We were discharged on March 21, 2013.
