We get asked a lot how Daniel is doing. It's a difficult question to answer briefly or as part of hi, how are you. Dan has good days and bad days, but during each one, he gets a little bit worse. When I think of the answer to how is he doing, the first response in my head is, he still has MLD and he is still dying. It's not what I say but that thought and the reminder of my son's terminal illness, colors any answer I give, makes the response just a little bit harder to say, and gives me a sad smile that, for my companion in the conversation, is supposed to be pleasant but that really says that I am falling part on the inside.
So what I normally say is that he is doing ok. Sometimes I will add in a bit of what's happened recently, pneumonia, problems sleeping, and, on occasion, the joy of hearing a word he has not said in a long time such as mom, dad, or, I swear he said this two days ago, hurry up.
I know you all care about Daniel and really do want to know how he is. Here is the complete and total response of how he is doing. You will almost never hear this because it that almost never fits into a conversation, unless we are seated down one-on-one and you say you really want to know.
Overall, I can't say that the disease progression has plateaued but the weekly changes are not as drastic as they used to be. Daniel cannot sit up or roll over anymore. He has occasionally head control. He can still move his head from side to side but holding it up is increasingly hard for him. If he does it for longer than 10 seconds, it's a good day. He can still move his arms but he only does it when he really wants something now. Sadly, he almost never points anymore. Pointing was his primary way of communicating what he wanted. He does not have that degree of fine motor control anymore. He mostly used his hands as fists now. On the positive, we haven't noticed any problems with hearing or vision yet.
Daniel's ability to move or speak on a day to day basis varies. He was very sick the week of Thanksgiving. He had a fever of about 101 and he was completely uninterested in moving. We talked to our nurse and she thought it was a virus. We waited for it to run its course but on Thanksgiving Day his fever spiked to 103.6. We went to the ER and even though his lungs sounded clear - he had a doctor's appointment on Monday and Wednesday that week so there was lot of lung listening - the x-ray showed that he had pneumonia. After two days of antibiotics, he perked up. We heard Mom and Dad. We were thrilled! We hadn't heard those words since the summer.
The pneumonia got better but our most persistent problem has been Daniel's leg spasms. The peripheral neuropathy that accompanies MLD has been destroying Daniel's feet for over a year. He has always fought wearing AFO's or any stretches. The muscles in his lower legs are becoming so tight that his right foot is almost impossible to get straight. Daniel's leg will shake and he will scream with pain. There were several days where he seemed to cry all day and nights when he doesn't sleep. Valium and Oxycodone wouldn't work in quelling the pain.
We tried Neurontin, thinking that this maybe this was related to neuropathic pain. It was hard to tell sometimes if Daniel was getting upset and causing the spasms or if the spasms came first. The drug seemed to help but after several days, Daniel became very lethargic and non-responsive. Since this was happening at the same time he was getting sick with pneumonia, it was hard to tell what was making him so sleepy. Regardless, by the time he was diagnosed with pneumonia, we had stopped the Neurontin. He about two good days as the pneumonia was clearing up. Then he started having spasms again. I suggested several meds to our doctors but everyone seemed to want to wait for the Botox injections before starting new meds.
The Botox injections....that should be a post in itself. The very short version is Daniel had them done locally on Friday. He has two casts on right now that he hates. The casts are there to put the foot in the proper position so when the Botox, that weakens that super tight muscle, kicks in, it's effect is maximized. Daniel and I feel the same way about them. I absolutely hate them and want them to come off. I really don't want him to be upset like this. Dan, however, is a proponent and rightly thinks we should wait as long as possible to take them off. He wants to, again rightly, maximize the long-term benefits of the Botox. Daniel every day is a little more used to the casts and I have to admit that it's for the best he keeps them on. They come off Monday.
Sometimes, especially lately when he has been sick, Daniel is content to just sit and do nothing. This is super weird and very hard to get used to. It's this, more than anything else, that tells me just how far the disease has progressed. Imagine a three-year old just staring into space, watching TV, and almost non-reactive to attempts to engage him. I am sure that most of this is the fever or a virus, but it scares me. That's what he is like as I write this.
I hope he is better tomorrow. If you ask me how he is doing, I will probably say ok.
So what I normally say is that he is doing ok. Sometimes I will add in a bit of what's happened recently, pneumonia, problems sleeping, and, on occasion, the joy of hearing a word he has not said in a long time such as mom, dad, or, I swear he said this two days ago, hurry up.
I know you all care about Daniel and really do want to know how he is. Here is the complete and total response of how he is doing. You will almost never hear this because it that almost never fits into a conversation, unless we are seated down one-on-one and you say you really want to know.
Overall, I can't say that the disease progression has plateaued but the weekly changes are not as drastic as they used to be. Daniel cannot sit up or roll over anymore. He has occasionally head control. He can still move his head from side to side but holding it up is increasingly hard for him. If he does it for longer than 10 seconds, it's a good day. He can still move his arms but he only does it when he really wants something now. Sadly, he almost never points anymore. Pointing was his primary way of communicating what he wanted. He does not have that degree of fine motor control anymore. He mostly used his hands as fists now. On the positive, we haven't noticed any problems with hearing or vision yet.
Daniel's ability to move or speak on a day to day basis varies. He was very sick the week of Thanksgiving. He had a fever of about 101 and he was completely uninterested in moving. We talked to our nurse and she thought it was a virus. We waited for it to run its course but on Thanksgiving Day his fever spiked to 103.6. We went to the ER and even though his lungs sounded clear - he had a doctor's appointment on Monday and Wednesday that week so there was lot of lung listening - the x-ray showed that he had pneumonia. After two days of antibiotics, he perked up. We heard Mom and Dad. We were thrilled! We hadn't heard those words since the summer.
The pneumonia got better but our most persistent problem has been Daniel's leg spasms. The peripheral neuropathy that accompanies MLD has been destroying Daniel's feet for over a year. He has always fought wearing AFO's or any stretches. The muscles in his lower legs are becoming so tight that his right foot is almost impossible to get straight. Daniel's leg will shake and he will scream with pain. There were several days where he seemed to cry all day and nights when he doesn't sleep. Valium and Oxycodone wouldn't work in quelling the pain.
We tried Neurontin, thinking that this maybe this was related to neuropathic pain. It was hard to tell sometimes if Daniel was getting upset and causing the spasms or if the spasms came first. The drug seemed to help but after several days, Daniel became very lethargic and non-responsive. Since this was happening at the same time he was getting sick with pneumonia, it was hard to tell what was making him so sleepy. Regardless, by the time he was diagnosed with pneumonia, we had stopped the Neurontin. He about two good days as the pneumonia was clearing up. Then he started having spasms again. I suggested several meds to our doctors but everyone seemed to want to wait for the Botox injections before starting new meds.
The Botox injections....that should be a post in itself. The very short version is Daniel had them done locally on Friday. He has two casts on right now that he hates. The casts are there to put the foot in the proper position so when the Botox, that weakens that super tight muscle, kicks in, it's effect is maximized. Daniel and I feel the same way about them. I absolutely hate them and want them to come off. I really don't want him to be upset like this. Dan, however, is a proponent and rightly thinks we should wait as long as possible to take them off. He wants to, again rightly, maximize the long-term benefits of the Botox. Daniel every day is a little more used to the casts and I have to admit that it's for the best he keeps them on. They come off Monday.
Sometimes, especially lately when he has been sick, Daniel is content to just sit and do nothing. This is super weird and very hard to get used to. It's this, more than anything else, that tells me just how far the disease has progressed. Imagine a three-year old just staring into space, watching TV, and almost non-reactive to attempts to engage him. I am sure that most of this is the fever or a virus, but it scares me. That's what he is like as I write this.
I hope he is better tomorrow. If you ask me how he is doing, I will probably say ok.
