Day +60
WBC 7.6
This will be a long one, so be patient . . .One day in the hospital can be so BUSY! . . .
First the lung news: Max is breathing well. He still sounds a little "junky" and he still has a wheeze, but he sounds MUCH better than a couple of days ago. His effort to breathe is down as well, and that's nice to see. Margaret found a website of another kid who had Hurler in Canada. He passed away sadly after some breathing difficulty long after he seemed to be out of the woods. He was on the vent for a few days and passed away. While every kid is different, it is some achievement that Max has been able to make it off the vent three times. Keep it up Max! We don't want him to back on. Roger, one of Max's favorite respiratory therapists, asked if he should take the vent out of the room, since he thought it might do well to leave Max a "warning" but I told him I like the symbolism of it leaving the room. When he rolled it out, I shouted after him, "And don't bring it back." I hope it is so. Last night I found an eyelash on Grace's cheek and let her blow it away with a wish, and she said "I wish for my brother to get all better."
Next the gut news: No news, really, but they have decided to do another flex-scope and look up his butt again. He's made some progress, but he's far from looking better. This new look should be in the next day or two.
Swallowing news: Today Max had a swallowing test. While he has been drinking well, the docs wanted to be extra sure that Max wasn't aspirating (food/ liquid going down into lungs) when he swallowed. We took him down to radiology and he drank a liquid that had been mixed with a barium powder. The drink is white, but as they watch him under the x-ray, it's black. He swallowed about 30 times and only one time did anything enter the "vestibule" but he cleared it. In other words, NO ASPIRATION. Then, for the heck of it really, they gave him some vanilla pudding mixed with the same stuff and he swallowed that down too. So, his swallowing is going well and he may be able to move towards more solid foods sooner than we thought. But it will depend on the health of his gut.
Skin news: Max had a biopsy done on some of the rashy skin at the bottom of his belly. The dermatologists feel it could be skin graft vs. host disease, but it didn't start or display itself like it did in the past so the transplant docs disagree. We'll learn more in the next day or two.
Finally, the most intriguing and worrisome news, brain news: Max may be suffering from PRES (Posterior Reversible Encephalopathy Syndrome). I'll try my best to explain it. This is the malfunction of the lining of the brain near the back of the head. This area affects vision and can cause seizures. The key letter in PRES is the R because it is Reversible. This most likely is from CSA (his cyclosporin, the drug used to help reduce GVHD) toxicity and high blood pressure. They've been doing a good job of keeping Max's blood pressure down, but his CSA level is hard to monitor because he's been so sedated and drugged up for so many reasons. Now that he's off so many drugs, it appears like he is suffering withdrawal or is rather delusional. His eyes are not focusing on anything and he's not responding to light. He's not using his left side of his body much, and he's moving his right hand randomly. We've been saying every time he gets off the vent that he's "out of it" and this is the best explanation so far. So tonight he's starting an anti-seizure medicine which may take the edge off of what is happening and may stabilize him a bit. He also started monoxidil for his blood pressure. You might recognize that drug as Rogaine. It turns out that the discovery of a new drug for blood pressure helped grow hair - go figure. Kind of funny that Max is starting this when he's still really bald. Tomorrow, they are going to give him an EEG to measure brain function, and he may get an MRI as well. They may have to play with his CSA level, to try and find a dose that is less toxic to him. Since this drug helps fight graft vs. host disease, Max's gut needs it. But it just might be too much for his brain.
This is all so scary. And typical of Max. Now that his breathing and stooling have made slight progress, we learn of something new. This, like most things, is going to be a long journey to solve so the hard thing again is to be patient. Let's hope that their first guess is the right guess, and it has the least long-term consequences.
LAST BUT NOT LEAST:
Thank you Genzyme for your wonderful dinner and for being so thoughtful in our time of need. These people are the makers of the amazing Enzyme Replacement Therapy treatment Aldurazyme that his helping a number of MPS1 patients who have a less severe form of Max's disease. Thank you so much, your dinner fed us and another family on a perfect night where the Ronald McDonald House did not have volunteer cooks.
Thank you Katie and Gretchen from Optimus! Your costume for Grace is perfect and just what she wanted! We are grateful.
Thank you Uncle Marko! Your handmade book for us will be treasured for years to come. Your artwork once again has brought smiles and not a moment too soon.
Thank you - to all the people we keep forgetting to thank! When our heads stop spinning we will try to remember more people. We have such generous friends and family, and we are so blessed to have you all.
Please pray for Max and for all the other Hurler champions and hope that they can bring Max's personality and joy of life back to us! We miss our dude!
--mike
This will be a long one, so be patient . . .One day in the hospital can be so BUSY! . . .
First the lung news: Max is breathing well. He still sounds a little "junky" and he still has a wheeze, but he sounds MUCH better than a couple of days ago. His effort to breathe is down as well, and that's nice to see. Margaret found a website of another kid who had Hurler in Canada. He passed away sadly after some breathing difficulty long after he seemed to be out of the woods. He was on the vent for a few days and passed away. While every kid is different, it is some achievement that Max has been able to make it off the vent three times. Keep it up Max! We don't want him to back on. Roger, one of Max's favorite respiratory therapists, asked if he should take the vent out of the room, since he thought it might do well to leave Max a "warning" but I told him I like the symbolism of it leaving the room. When he rolled it out, I shouted after him, "And don't bring it back." I hope it is so. Last night I found an eyelash on Grace's cheek and let her blow it away with a wish, and she said "I wish for my brother to get all better."
Next the gut news: No news, really, but they have decided to do another flex-scope and look up his butt again. He's made some progress, but he's far from looking better. This new look should be in the next day or two.
Swallowing news: Today Max had a swallowing test. While he has been drinking well, the docs wanted to be extra sure that Max wasn't aspirating (food/ liquid going down into lungs) when he swallowed. We took him down to radiology and he drank a liquid that had been mixed with a barium powder. The drink is white, but as they watch him under the x-ray, it's black. He swallowed about 30 times and only one time did anything enter the "vestibule" but he cleared it. In other words, NO ASPIRATION. Then, for the heck of it really, they gave him some vanilla pudding mixed with the same stuff and he swallowed that down too. So, his swallowing is going well and he may be able to move towards more solid foods sooner than we thought. But it will depend on the health of his gut.
Skin news: Max had a biopsy done on some of the rashy skin at the bottom of his belly. The dermatologists feel it could be skin graft vs. host disease, but it didn't start or display itself like it did in the past so the transplant docs disagree. We'll learn more in the next day or two.
Finally, the most intriguing and worrisome news, brain news: Max may be suffering from PRES (Posterior Reversible Encephalopathy Syndrome). I'll try my best to explain it. This is the malfunction of the lining of the brain near the back of the head. This area affects vision and can cause seizures. The key letter in PRES is the R because it is Reversible. This most likely is from CSA (his cyclosporin, the drug used to help reduce GVHD) toxicity and high blood pressure. They've been doing a good job of keeping Max's blood pressure down, but his CSA level is hard to monitor because he's been so sedated and drugged up for so many reasons. Now that he's off so many drugs, it appears like he is suffering withdrawal or is rather delusional. His eyes are not focusing on anything and he's not responding to light. He's not using his left side of his body much, and he's moving his right hand randomly. We've been saying every time he gets off the vent that he's "out of it" and this is the best explanation so far. So tonight he's starting an anti-seizure medicine which may take the edge off of what is happening and may stabilize him a bit. He also started monoxidil for his blood pressure. You might recognize that drug as Rogaine. It turns out that the discovery of a new drug for blood pressure helped grow hair - go figure. Kind of funny that Max is starting this when he's still really bald. Tomorrow, they are going to give him an EEG to measure brain function, and he may get an MRI as well. They may have to play with his CSA level, to try and find a dose that is less toxic to him. Since this drug helps fight graft vs. host disease, Max's gut needs it. But it just might be too much for his brain.
This is all so scary. And typical of Max. Now that his breathing and stooling have made slight progress, we learn of something new. This, like most things, is going to be a long journey to solve so the hard thing again is to be patient. Let's hope that their first guess is the right guess, and it has the least long-term consequences.
LAST BUT NOT LEAST:
Thank you Genzyme for your wonderful dinner and for being so thoughtful in our time of need. These people are the makers of the amazing Enzyme Replacement Therapy treatment Aldurazyme that his helping a number of MPS1 patients who have a less severe form of Max's disease. Thank you so much, your dinner fed us and another family on a perfect night where the Ronald McDonald House did not have volunteer cooks.
Thank you Katie and Gretchen from Optimus! Your costume for Grace is perfect and just what she wanted! We are grateful.
Thank you Uncle Marko! Your handmade book for us will be treasured for years to come. Your artwork once again has brought smiles and not a moment too soon.
Thank you - to all the people we keep forgetting to thank! When our heads stop spinning we will try to remember more people. We have such generous friends and family, and we are so blessed to have you all.
Please pray for Max and for all the other Hurler champions and hope that they can bring Max's personality and joy of life back to us! We miss our dude!
--mike
posted by Mike Ciacciarelli
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