Otis had a follow-up appointment with his neurologist and Keto team at Children's Mercy yesterday, and for once we left feeling really good about it. O's current neurologist, Dr. A, is really great at being completely open and honest with us without being super dismal about it, unlike like the last neurologist, Dr. L. We liked Dr. L okay, but he would always come into the room shaking his head and saying something like “oh, this is not good, this is not good.” (Uh, yeah, dude, we KNOW it’s not good. Please just skip the gloom and doom and give us the facts, mmkay?)
We had a ton of questions for Dr. A and the dietitians, all of which were answered in great detail and with great patience. Dr. A told us that his main goal is to give Otis the best quality of life possible, and that he would like to try to accomplish that with as few drugs as possible, which we were thrilled to hear. We read so many stories about kids with IS being on as many as 5 different anti-seizure meds. Dr. A said that while the right combination and dosage of anti-seizure drugs would stop O from having seizures, the cost would be having a complete zombie for a kid. What good would being seizure-free do him if it meant that he would be sitting catatonic all day every day? We would rather Otis have the occasional seizure here and there while still being able to learn and play and enjoy life. So the goal is to get his seizures down as low as possible with the Keto diet, the right supplements, and low doses of no more than two anti-seizure meds. If the seizures go away completely, AWESOME--but at this point we're happy with just getting the numbers and the disruption to O's life down as low as we possibly can. After months of counting and crunching the numbers very carefully, we've found that he's been having about 450 seizures per week (minus the weeks when the Vigabatrin caused the numbers to spike to more than double that) since September. If we could get them down to where he was having around 10 or so per day, we would be absolutely ecstatic. That's not to say that we're giving up hope for him to someday be seizure-free--that will always be our Mondo Beyondo dream--just that we realize that that may not necessarily be in the cards for Otis. Only time will tell. In the meantime, patience.
Dr. A also adjusted the Keto diet ratio to 3.75:1 (that's fat calories to carbohydrate calories), which he's been building up to over the past couple of weeks (maximum Keto diet ratio is 4:1), and prescribed melatonin to help him get better sleep, a new seizure med, Topamax, which we’re not dreading too much, and an additional B-vitamin supplement (folinic acid), just in case a deficiency in that is contributing to his seizures. We learned that in the very rare case, a folinic acid deficiency can be the cause of IS. If this is the case, the seizures would completely stop within a week or so of beginning the supplement. We're not getting our hopes up on that one, but it would be cool if that were the case. We’re also going to do another EEG soon to see where he is now compared to where he was in October, when he had his last EEG. The EEG will also tell Dr. A whether or not Otis has begun having other types of seizures besides the "partial" seizures that are characteristic of IS.
Dr. A also ordered a whole bunch of lab tests to be done to check for other possible causes, including possible genetic causes. The testing is going to be part of a research study, so we won’t be billed for any of it. We were originally told that testing of this sort would cost us around $3000, and that it would be very unlikely that insurance would cover it. If they find that Otis is somehow genetically predisposed to IS, then that will be a trigger for Ryan and I to have testing done so that we can know if one of us is a carrier for something weird, or if something about our mixed DNA is the problem. It won’t affect the way they treat O’s condition, but it will be helpful to us for future family planning. We should have some results in about 6 weeks.
Dr. A warned us that since they're running such a huge range of tests, some of them might uncover some other unrelated results that we might not be too excited about—liver or kidney problems, etc. Not fun, but I guess it would be good to know if that’s the case, so that we’re not surprised with any additional health issues later on down the road. They had to draw quite a bit of blood from the poor little guy yesterday which was not fun, but luckily the lab tech was awesome and only had to stick him twice--in the arms, and not in the legs or head as they've had to do in the past.
In recent weeks, Ryan and I had done some research and contacted a doctor--a neurosurgeon and IS specialist at the Children's Hospital of Detroit. Dr. Chugani's name had come up over and over again in the online IS groups and forums to which I subscribe as the go-to guy for Infantile Spasms. He's won awards for his work with IS, and has a reputation for being very approachable and accommodating to parents. All of the IS parents who recommended him said that they had e-mailed him directly and received a near-immediate response. It was no different for us, once we finally found the time to sit down and compose an e-mail to him. (O's medical history is kind of difficult to summarize.) He responded right away saying that he would like to evaluate Otis, as well as having him get a PET scan and a 4-6 hour EEG. The PET scan would help to identify any focal points in the brain that his MRI may have missed.
As we began to work with Dr. Chugani's intake RN to get an appointment set up over Ryan's spring break, we learned that the contrast used for the PET scan is a radioactive glucose...which means that it would be incompatible with the Ketogenic Diet. We finally decided that we are not yet ready to take Otis off of the diet, as with a bit more time and fine-tuning, we may find that will start to work for him. Plus, after the week-long hospital stay and all that it took to get him transitioned onto the diet 3 months ago, we're just not ready to throw in the towel on it yet, especially if there is still a chance that it could work for him. Dr. Chugani's RN assured us that they will keep O's records on file just in case we change our minds and decide to come see him in the future.
We felt comfortable discussing this with Dr. A, who, when we brought it up to him, told us that he is good friends with Dr. Chugani. He explained to us that Children's Mercy has a PET machine and could do the test, and also explained to us the reason why he hadn't recommended a PET scan for Otis. He said that all 3 of O's previous EEGs had indicated that the seizure activity was not stemming from one focal point, but that the erratic activity was coming from all over. He said that because of that, all a PET scan would tell us was that there was something abnormal about O's brain metabolism (all over), which we already know. That was really reassuring to hear--good to know that we're not putting off something potentially urgent. Dr. A also told us that while we will of course keep in touch over the phone in the meantime, he would like to see Otis back in 3 months. By then, O will have been on the Ketogenic diet for 7 months, at which time we will know whether or not it is working to help control his seizures. Dr. A said that some kids show a response to the diet right away, while other kids tend to be "late bloomers" and begin to show a response after having been on it for awhile, and possibly after some fine-tuning. This was also reassuring to hear.
Dr. A also told us that if we are still not seeing any improvement from the diet or the drugs by the next appointment, he would like to talk to us about the benefits of getting a second opinion, which he thinks can be really valuable and sometimes necessary. Hearing that from him really lifted a huge weight off of our shoulders. Hopefully we won't have to go that route, but if we do, it's nice to know that we'll have the support and blessing of our home doctor instead of feeling like we're sneaking around behind his back!
Just in case you're curious, here is what O’s med schedule looks like now:
Morning:
Clorazepate (seizure med)
Topamax (seizure med)
Piridoxine (B6 supplement)
Folinic Acid
Vitamin D3 drop
Zantac for reflux
Mid-day:
Clorazepate
Nighttime:
Same as morning, minus D3
Melatonin
Miralax, since the Keto diet makes him super constipated...which triggers more seizures
The Zantac comes in gelatin capsules which are specially made for us by a local compounding pharmacy, since Otis can no longer have the syrup. The D3 comes in drops which we just add to his bottle--one drop a day--and the Miralax is a weird white silica-looking powder, which we also just measure and shake into his bottle at night. The seizure meds, melatonin, and supplements come in tablets which we crush up and give to him either in his bottle, mixed up in baby food, or drawn up with water into a syringe and squirted into his mouth. He's onto us these days, though, and has learned to clamp his mouth shut and turn his head when he sees us coming at him with the syringe. I can’t say that I blame him—I’m sure that stuff tastes awful!
Otis is still working very hard on neck and trunk strength. He's doing much better at holding up his head, and is doing pretty well at supported sitting, although he absolutely hates it! Our tiny-K coach has given us little arm splints to put on him while sitting or doing propped-up tummy time over a boppy pillow so that he can see what it feels like to support his weight on his hands and get used to doing that. He likes to keep his elbows bent and his hands (especially his right one) held in toward his chest, so although he hates the splints, we think that they really will be helpful. We've also picked back up on helping him use his vision. He's got cortical visual impairment, which means that although his eyes are very healthy and work just fine, the visual messages he's receiving aren't being relayed to his brain, so he doesn't "use" his vision. We've started getting creative about getting him to notice stuff around the house and really use his vision. We've hung shiny red Christmas garland above his changing table and taped shiny red mylar to the wall for him to look at while we're changing him. We've also begun wrapping his bottle with a shiny and glittery red Christmas bow. There's other stuff we use for vision play--light wand, light box with different colored transparencies and a spinning/pinwheel attachment for contrast, silver sequined tube top, green sequined fabric, gigantic red satin blouse--shiny or sparkly stuff, stuff with movement, and lights really seem to catch his attention.
Otis is also cutting two new teeth--his top incisors--and has been chewing on his fingers and pacifier quite a bit. It probably won't be too long until we have to start working with his dieticians on coming up with Keto-friendly foods that he can chew on. We think that introducing finger foods may also help get him interested in reaching and grabbing for stuff.
Ryan and I are so proud of our little guy and all that he has accomplished despite all of the obstacles he has faced in his short life. He is an amazing little fella who has so much more to teach us. All that AND he is out-of-control cute to boot (9-month pics to follow)!
We had a ton of questions for Dr. A and the dietitians, all of which were answered in great detail and with great patience. Dr. A told us that his main goal is to give Otis the best quality of life possible, and that he would like to try to accomplish that with as few drugs as possible, which we were thrilled to hear. We read so many stories about kids with IS being on as many as 5 different anti-seizure meds. Dr. A said that while the right combination and dosage of anti-seizure drugs would stop O from having seizures, the cost would be having a complete zombie for a kid. What good would being seizure-free do him if it meant that he would be sitting catatonic all day every day? We would rather Otis have the occasional seizure here and there while still being able to learn and play and enjoy life. So the goal is to get his seizures down as low as possible with the Keto diet, the right supplements, and low doses of no more than two anti-seizure meds. If the seizures go away completely, AWESOME--but at this point we're happy with just getting the numbers and the disruption to O's life down as low as we possibly can. After months of counting and crunching the numbers very carefully, we've found that he's been having about 450 seizures per week (minus the weeks when the Vigabatrin caused the numbers to spike to more than double that) since September. If we could get them down to where he was having around 10 or so per day, we would be absolutely ecstatic. That's not to say that we're giving up hope for him to someday be seizure-free--that will always be our Mondo Beyondo dream--just that we realize that that may not necessarily be in the cards for Otis. Only time will tell. In the meantime, patience.
Dr. A also adjusted the Keto diet ratio to 3.75:1 (that's fat calories to carbohydrate calories), which he's been building up to over the past couple of weeks (maximum Keto diet ratio is 4:1), and prescribed melatonin to help him get better sleep, a new seizure med, Topamax, which we’re not dreading too much, and an additional B-vitamin supplement (folinic acid), just in case a deficiency in that is contributing to his seizures. We learned that in the very rare case, a folinic acid deficiency can be the cause of IS. If this is the case, the seizures would completely stop within a week or so of beginning the supplement. We're not getting our hopes up on that one, but it would be cool if that were the case. We’re also going to do another EEG soon to see where he is now compared to where he was in October, when he had his last EEG. The EEG will also tell Dr. A whether or not Otis has begun having other types of seizures besides the "partial" seizures that are characteristic of IS.
Dr. A also ordered a whole bunch of lab tests to be done to check for other possible causes, including possible genetic causes. The testing is going to be part of a research study, so we won’t be billed for any of it. We were originally told that testing of this sort would cost us around $3000, and that it would be very unlikely that insurance would cover it. If they find that Otis is somehow genetically predisposed to IS, then that will be a trigger for Ryan and I to have testing done so that we can know if one of us is a carrier for something weird, or if something about our mixed DNA is the problem. It won’t affect the way they treat O’s condition, but it will be helpful to us for future family planning. We should have some results in about 6 weeks.
Dr. A warned us that since they're running such a huge range of tests, some of them might uncover some other unrelated results that we might not be too excited about—liver or kidney problems, etc. Not fun, but I guess it would be good to know if that’s the case, so that we’re not surprised with any additional health issues later on down the road. They had to draw quite a bit of blood from the poor little guy yesterday which was not fun, but luckily the lab tech was awesome and only had to stick him twice--in the arms, and not in the legs or head as they've had to do in the past.
In recent weeks, Ryan and I had done some research and contacted a doctor--a neurosurgeon and IS specialist at the Children's Hospital of Detroit. Dr. Chugani's name had come up over and over again in the online IS groups and forums to which I subscribe as the go-to guy for Infantile Spasms. He's won awards for his work with IS, and has a reputation for being very approachable and accommodating to parents. All of the IS parents who recommended him said that they had e-mailed him directly and received a near-immediate response. It was no different for us, once we finally found the time to sit down and compose an e-mail to him. (O's medical history is kind of difficult to summarize.) He responded right away saying that he would like to evaluate Otis, as well as having him get a PET scan and a 4-6 hour EEG. The PET scan would help to identify any focal points in the brain that his MRI may have missed.
As we began to work with Dr. Chugani's intake RN to get an appointment set up over Ryan's spring break, we learned that the contrast used for the PET scan is a radioactive glucose...which means that it would be incompatible with the Ketogenic Diet. We finally decided that we are not yet ready to take Otis off of the diet, as with a bit more time and fine-tuning, we may find that will start to work for him. Plus, after the week-long hospital stay and all that it took to get him transitioned onto the diet 3 months ago, we're just not ready to throw in the towel on it yet, especially if there is still a chance that it could work for him. Dr. Chugani's RN assured us that they will keep O's records on file just in case we change our minds and decide to come see him in the future.
We felt comfortable discussing this with Dr. A, who, when we brought it up to him, told us that he is good friends with Dr. Chugani. He explained to us that Children's Mercy has a PET machine and could do the test, and also explained to us the reason why he hadn't recommended a PET scan for Otis. He said that all 3 of O's previous EEGs had indicated that the seizure activity was not stemming from one focal point, but that the erratic activity was coming from all over. He said that because of that, all a PET scan would tell us was that there was something abnormal about O's brain metabolism (all over), which we already know. That was really reassuring to hear--good to know that we're not putting off something potentially urgent. Dr. A also told us that while we will of course keep in touch over the phone in the meantime, he would like to see Otis back in 3 months. By then, O will have been on the Ketogenic diet for 7 months, at which time we will know whether or not it is working to help control his seizures. Dr. A said that some kids show a response to the diet right away, while other kids tend to be "late bloomers" and begin to show a response after having been on it for awhile, and possibly after some fine-tuning. This was also reassuring to hear.
Dr. A also told us that if we are still not seeing any improvement from the diet or the drugs by the next appointment, he would like to talk to us about the benefits of getting a second opinion, which he thinks can be really valuable and sometimes necessary. Hearing that from him really lifted a huge weight off of our shoulders. Hopefully we won't have to go that route, but if we do, it's nice to know that we'll have the support and blessing of our home doctor instead of feeling like we're sneaking around behind his back!
Just in case you're curious, here is what O’s med schedule looks like now:
Morning:
Clorazepate (seizure med)
Topamax (seizure med)
Piridoxine (B6 supplement)
Folinic Acid
Vitamin D3 drop
Zantac for reflux
Mid-day:
Clorazepate
Nighttime:
Same as morning, minus D3
Melatonin
Miralax, since the Keto diet makes him super constipated...which triggers more seizures
The Zantac comes in gelatin capsules which are specially made for us by a local compounding pharmacy, since Otis can no longer have the syrup. The D3 comes in drops which we just add to his bottle--one drop a day--and the Miralax is a weird white silica-looking powder, which we also just measure and shake into his bottle at night. The seizure meds, melatonin, and supplements come in tablets which we crush up and give to him either in his bottle, mixed up in baby food, or drawn up with water into a syringe and squirted into his mouth. He's onto us these days, though, and has learned to clamp his mouth shut and turn his head when he sees us coming at him with the syringe. I can’t say that I blame him—I’m sure that stuff tastes awful!
Otis is still working very hard on neck and trunk strength. He's doing much better at holding up his head, and is doing pretty well at supported sitting, although he absolutely hates it! Our tiny-K coach has given us little arm splints to put on him while sitting or doing propped-up tummy time over a boppy pillow so that he can see what it feels like to support his weight on his hands and get used to doing that. He likes to keep his elbows bent and his hands (especially his right one) held in toward his chest, so although he hates the splints, we think that they really will be helpful. We've also picked back up on helping him use his vision. He's got cortical visual impairment, which means that although his eyes are very healthy and work just fine, the visual messages he's receiving aren't being relayed to his brain, so he doesn't "use" his vision. We've started getting creative about getting him to notice stuff around the house and really use his vision. We've hung shiny red Christmas garland above his changing table and taped shiny red mylar to the wall for him to look at while we're changing him. We've also begun wrapping his bottle with a shiny and glittery red Christmas bow. There's other stuff we use for vision play--light wand, light box with different colored transparencies and a spinning/pinwheel attachment for contrast, silver sequined tube top, green sequined fabric, gigantic red satin blouse--shiny or sparkly stuff, stuff with movement, and lights really seem to catch his attention.
Otis is also cutting two new teeth--his top incisors--and has been chewing on his fingers and pacifier quite a bit. It probably won't be too long until we have to start working with his dieticians on coming up with Keto-friendly foods that he can chew on. We think that introducing finger foods may also help get him interested in reaching and grabbing for stuff.
Ryan and I are so proud of our little guy and all that he has accomplished despite all of the obstacles he has faced in his short life. He is an amazing little fella who has so much more to teach us. All that AND he is out-of-control cute to boot (9-month pics to follow)!
1 comment:
You and Ryan are also amazing. AH-mazing. I know we don't see much of each other, but you guys are in my thoughts & prayers constantly.
XOXOXO
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