One Year Ago Today

For those of you that don't know, I began blogging on carepages March 7, 2009. This was the day after Riley was diagnosed with ALD, which was very devastating news to both Joel and me. I can still remember so clearly the emergency room doctor and the endocrinologist who had been treating Riley taking us into a private room off of the ICU while Riley slept and telling us. I thought instead of doing a new post, I would re-post that very first post from almost one year ago. It is unbelievable how our lives, and especially Riley's, have changed in one year.





3/7 The day after our worst day ever

We were moved out of ICU last night into a regular patient room. We were all exhausted after yesterday. They came and got Riley yesterday morning a little after 9am to take him for his MRI. The MRI was ordered after the CT scan was abnormal when he came in on Wednesday.

I'll back up. Riley has been complaining for a couple of weeks of being tired and not feeling good, but can't tell us what doesn't feel good. Like "normal" parents, or so we are told, we would say "we are tired too, but you still have to get up and go to school", etc. Well Wednesday, he did the same routine. He came in my room after getting up and laid in my bed, moaning and groaning about being tired and not feeling good. I told him if he was really sick, I would keep him out of school and take him to the doctor, and he said ok. So, since we couldn't call for an appointment until 9am, I asked if he wanted to ride by my work. This perked him up, basically because we usually have very good snacks at the office, and he thought he that was a great idea. By the time we got to the office, he was not as perky, although he did ask about the snacks! Ms. Teresa (the other Teresa in our dept.) brought Riley some breakfast casserole (thanks Sharon for the leftovers), and that kept him satisfied for a while. He feel asleep in my office floor, which I knew was unusual. Meanwhile, we couldn't get in to the doctor until 10:45, so I let Riley sleep and did a little work. Before I could leave to take Riley to the doctor, I was called in to a meeting, which was a blessing in disguise. I had to call Joel to see if he could leave work and come take Riley to his appt. He did and they did blood work and didn't find anything wrong, so basically, we thought he was faking. Joel decided to keep him out of school the rest of the day, so he took him for some lunch and then they went home.
I had told Joel I would leave work around 4pm and come home so he could go to an appt he already had scheduled. Shortly before time for me to leave, I got a frantic call from Joel that Riley was having a seizure - this is why it was a blessing in disguise - that Joel was home with Riley and not me. Riley has had seizures in the past and every time, Joel has been there. I don't think this is just by chance. I freeze when this happens and Joel jumps into gear. Joel was on a conference call after putting Riley down for a nap. Joel is a terrible pacer while on the phone, and was pacing around the house, and he peeked into Riley's room. At first, he didn't see Riley in his bed, so he opened the door wider, and Riley had fallen out of the bed face-first. He was face down on the floor with his feet and legs still in the bed, and he had vomited. We don't know how long he had been like that. When Joel got to him he was seizing. This is the scariest thing to go through, but it's happened before and we thought it was the same as in the past, although the seizure did last much longer. I left work with Heidi driving and we met the ambulance pulling out of the neighborhood as we were pulling in. Joel was already in the ambulance so they wouldn't stop for me, so we had to go separately to the hospital. The seizure continued in the ambulance until they gave Riley Valium. I'll pick up with the emergency room but have to take a break now...

In the emergency room, they started doing blood work. Riley's sodium came back dangerously low. Because of that, and his great winter suntan, the doctor suspected Addison's Disease. This is a rare disease that affects the adrenal glands. It is treatable through medication. Another diagnosis they were looking into was Water Intoxication. This could dilute the sodium, and Riley drinks and actually craves and begs for water. A CT Scan was ordered because of the seizure. Our first trip to radiology was a bust. Riley was hysterical. They decided to sedate him and do it later, so we were sent back to the emergency room. All this time, Riley was coming in and out, but still had not talked to us for a few hours.The first time he finally spoke, the nurse was messing with his IV and he turned to the nurse and said "I don't like you." We were all thrilled he had talked to us. He began to come out of it a little more, so we decided we would try to talk to him about the CT scan and try it again without sedating him. We tried it again and were able to get it without sedating him. When the results were read later that night, we were told there was some swelling in the white matter of his brain. The doctor working with Riley was in contact with the radiologist reading the results, and while there was some concern, Riley was not exhibiting any neurological symptoms. We were told they would do an MRI either Thursday or Friday and that he would be admitted into Intensive Care. We had always been sent home in the past when he had seizures related to fevers. Wednesday night, we all three stayed in ICU and they woke Riley up every hour to ask him questions, such as his name, age, etc. He responded appropriately. During this time, he was being given sodium at a very slow rate to raise his sodium very slowly. Doing it too fast can cause problems with the brain. By morning, they were pleased with the way the sodium level was coming up. The endocrinologist met with us on Thursday, and she agreed that everything was pointing to Addison's Disease, which was great because this was the best case scenario. At the same time, the radiologist was still concerned with the CT scan and was anxious to see the MRI results. Thursday in the ICU was rough. Riley was given some ice chips at about 5am, and that was it. He started begging for water, and was very angry that he couldn't have any. He never asked for food, but begged for water. For some reason, he blamed me for not giving him water, but not Joel. He was so frustrated and didn't know how to deal with it, so he started saying anything he could that he thought would hurt me. The best was, "I don't like the way your clothes look"! Also, he hated our dog, and wanted to kick me. He even asked Joel if he could pick him up in the bed and move him further away from me. That day, we decided to put in a PIC line in Riley's arm so they wouldn't have to stick him anymore to draw blood. They were taking blood very frequently to check his sodium. Riley was sedated to put this in and basically slept the rest of the day Thursday.

Friday, Riley was supposed to have his MRI at 8am, which ended up being around 9:30. He was sedated again for the MRI. Once again, he slept a lot of the day after being sedated. Friday afternoon, three doctors came to us at Riley's bedside and took us in to the doctor's lounge in the ICU, and told us Riley was being diagnosed with ALD, adrenoleukodystrophy. We were told this was a possibility on Thursday, but unlikely because Riley was not exhibiting neurological symptoms consistent with the disease. I really wasn't terribly worried about this until Friday. Joel and I were talking with Dr. Macky who had been with Riley for the past 48 hours. He was getting ready to get off from his shift and came over to tell us he was leaving. We were talking about how optimistic the endo was about this being Addison's, and he told us that although that was true, the radiologist was just as certain that what he saw in the CT was ALD. And then this was confirmed by what the doctors told us that afternoon. There is a blood test that is currently being done at John Hopkins that will give 100% confirmation, but the doctors felt strong enough about the MRI results to tell us that this IS what Riley has.

Needless to say, both Joel and I completely broke down. To hear that your child is so sick, with a potentially fatal disease is devastating. After a lot of crying, we both called our parents to tell them about the new diagnosis. That was yesterday, the worst day of our lives. We met with a geneticist yesterday afternoon, and she and Riley's endo immediately began contacting their counterparts all over the country. Although this is a terrible thing to deal with, we've been told by several of the doctors that, because it is so rare, this is already getting a lot of attention from all kinds of doctors and there are a lot of people working on Riley's case. We've even had a couple of doctors come by and ask to take pictures of Riley's bronze skin for teaching purposes.

We met with the endo, Dr. Rincon today. She has already put together a lot of information for us regarding the adrenal part of this disease. Riley is already taking the steroids necessary to control that orally. The two doctors are both gathering information over this weekend and are meeting on Monday to come back together and see what each one has come up with. Right now, Duke is looking like a likely place for Riley's treatment. They do a lot of work with bone marrow transplants in children in cases that are NOT cancer. We are not sure of the treatment plan, but we've been told that because Riley doesn't exhibit the neuro symptons someone with his MRI results should, he is probably going to be a perfect candidate for a transplant. In children who do exhibit these symptoms this is usually not the treatment plan.

We will probably go home tomorrow and continue the adrenal treatment orally, and come back in late next week to discuss the next step. I will keep everyone up to date. Joel and Riley are both sleeping peacefully right now, and I'm ready to join them! It's been a long day and a crabby cranky one for Riley. Hopefully getting home tomorrow will be a good change of scenery. Keep praying for our little man.