A story about the use of rapamycin (sirolimus)

This story highlights one of the great stories coming out of the rapamycin usage trial.  While it doesn't appear to be panning out for LM's in all areas, it's exciting to see it helping others, including this cutie pie! 

http://www.dailymail.co.uk/health/article-2171518/Boy-5-takes-steps-experimental-drug-shrinks-huge-tumour-growing-body.html

The biggest problem... isn't that big!

We're at a.... dare I say it???... a slow point with Micah's treatment. We got the go ahead to stop 3 medicines yesterday (that's 7 less doses a day), switch one med from twice a day to once (that's 8 less)... We're seriously only left with 4 doses half the week and 2.....2!!!! the other half of the week. This morning, it took me like 5 minutes to realize he doesn't have morning meds this morning!

The drain is out now, so we dont have that antibiotic irritating his stomach, and let me tell you, 7 weeks of a pretty potent antibiotic... UGH!!! Not to mention the one he's been on for most of the last 6 months... I hear parents so tentative to give abx for an ear infection or something... and I can't help but think that I wish we had that luxury to worry about that.

He's off the immune suppressant, the drain is out, and his iGG is fine (for now). What? My kid has an immune system (well, maybe not quite, but soon?!) Oh wait, it's October. Doesn't matter because he doesn't get a free pass out as long as he has a trach, in my humble opinion. The (sort of) good news is that he gets synagis again this year, it's sort of like a vaccine against RSV. UNFORTUNATELY, it's monthly shots for probably the next 6 months. This mama just about had a breakdown when I heard that he qualified again. It's a torturous shot (he was upset for about 5 seconds about a flu shot, this he'll scream for), and he knows by now what's coming. And we just got past weekly bloodwork/sticks. *bangs head against the wall* Poor kid... poor mama and papa. I'm just sick of it. Sick of bringing my kid in for torturing, knowing all the while we don't have a choice. RSV can be devastating for a trach kid. Most kids you only worry about for the first year, but it's constant with a trach kid. RSV is just a specific type of cold, nothing an adult would ever have a clue about having.

He's got his yearly evaluation for First Steps this morning. It's kind of silly because he qualifies for OT, PT and Speech just because of his medical conditions, so why bother? We don't ask for the 4th type of therapy anyway! They'll probably try to convince me he doesn't need PT... Good luck with that one!

Which leaves us with our two "big" problems, his puking and his swelling. I'm hoping somehow the puking is related to all the morning meds he was on (two abx and advil). Unfortunately I know I'm wrong, LOL. In the morning the kid just can't tolerate food. We used to be able to get about 5 oz in an hour. Now we're at 4 ounces in about an hour and a half, and he's still trying to throw up (and being successful if we don't get it all back out of his stomach in time). It's baffling. We've had to increase his feedings from 3 to 4 already to accomodate the increase in volume he needs to grow.

Okay, I'm being incredibly boring to about 90 percent of you who don't live in this world. Long story short, he's got swelling in his face that leaves his eye swollen shut for the first half the day, but it goes down which says it may not be LM, just tissue swelling... but why?

That's enough of a novel, I'd say.

Not much to update...

We're thankful Micah's leg has gotten back to normal without any intervention. I couldn't even imagine if it were broken or something... He'd be so ticked!!!
No luck getting Micah's swelling under control. We're trying a regimen of advil to bring down the "inflammation" type swelling. We've tried to drain it around the drain, but still no luck even after the doc working on it Thursday. Sad day. As the day progresses he can see out of his eye somewhat, but not usually until the afternoon. Doc wants to watch and wait for awhile, since we've been intervening so much in the last few weeks. I understand, but it's hard. Maybe, though, that buys us two or three weeks from traveling though, which would be a nice change of pace.
I can't remember if I put this last time, but I've requested some time off until the next round of surgery/procedures. Maybe at least a month when this gets sorted out. It's cold and flu season now, which isn't a great time to be out. Don't want to wait too long though, since the weather will turn south in December, probably.

Oh... What to do... what to do...

I've really had enough.

It gets really frustrating when you just don't know what to do next. Yesterday the Dr. drained tons of (mostly) blood and lymphatic fluid, we'd been just getting lymphatic fluid at home, so he must have gotten a new pocket we hadn't gotten in to. Still no functioning drain, so he's just as swollen as he was yesterday. I thought I understood his philosophy on all this, but the drain AINT working.

The poor kid hasn't been able to see conistently out of his eye for the last week.

Things are just really stressful around here. We don't know what to do to get this all under control. The poor kid... He's been on antibiotics for 6 weeks now. That can't be good for his belly (or for anything). We're doing probiotics, because I have to feel like I'm doing something good for him.

He didnt seem to tolerate the rapamycin again... Even after being off it for about 28 hours, it was still in his system higher than it should have been. I think the consensus is we're at the end of that road.

We've seriously thought about just taking a week and living in Cincinnati and doing whatever it takes to get this figured out... Problem is I don't think that'd even help, because there's nothing new left to do.

This disease is far uglier than I ever imagined. And if you know Micah you'll find it no surprise he's over in the next room playing. His face is swollen like a balloon, he can't see out of one eye, he's puking just about daily, but he's playing. I wish I had his spirit.

The kid is a mystery!

Still not sure what's bothering his leg. It's not keeping him from walking, he's just a little unsteady and limping. As long as he has tylenol, he's quite a bit better. Doctor doesn't think it's a fracture, which is good (can you imagine if it was...ugh I'm not sure I could take it!) We avoided an x-ray for today, but may do one if things don't improve.

We had a laundry list of bloodwork to get done, I think 7 different tests in all! That might be a record. We should have the first round back this afternoon, and the rest back later this week. We've been in contact with various docs all weekend/today... The bloodwork will hopefully rule out infection, although it seems highly unlikely at this point. Unless is IGG comes back in the toilet we may not have to go down to Cincinnati this week! We are seeing a new ENT in FW to see if he can fix the drain situation. Also, learned we can do IVIG in FW if need be, although we seem to always need to be in Cincinnati for something. (Plus, I'm not sure if they'll do it at the same fast rate we've become accustomed to, since they don't have proof he tolerates it just fine). I'll take the 8 hours of driving and 3 hour infusion over the 8 hour infusion, LOL!

Is it me, or do things just seem really complicated right now? It's my life and I can't even keep it all straight!

Something strange.

Update: It's Sunday morning, and no real change. Tried tylenol last night, didn't seem to help. Got a baseline for where he was this morning (worse than yesterday) and am trying tylenol again. There's a fair chance we're going to stop the rapamycin, either just for awhile or maybe entirely. It can cause joint aches, and the hematologist isn't convinced it's doing anything for him. It's hard to say, since he was only on it two weeks, off it ten days, and been on it for about ten days again. Unfortunately, we can't get blood work unless we go to the ER. So, we'll go get it as soon as we get the order in on Monday AM. It can take 2 days to get the rapa level back, although we should be able to get the CBC/IGG back sooner, if they order them.

Micah's got some strange symptoms going on. His right leg seems to be causing him pain and is weak. He's limping/dragging his right foot just a little. The reasons could be endless. Luckily, the hematologist called me today (on a sunny Saturday afternoon) because it could have been an infection, which at this point is a trip to the ER. Looking less like an infection, and more like just about anything else. Hoping to figure it out soon. Could be entirely innocent, but it's been going on for 24 hours now, so who knows.

Not a post to read with your morning bowl of oatmeal.

Sorry for the lack of updates. Our freshly installed internet decided to stop working, and so far, 6 days later, Century Link has failed to fix it.

We went to Cincinnati Friday, they cut open two slits on his face, drained the fluid (estimated about 6-7 ounces), and put in a drain above his ear. Now, I'm not a fan of drains in general, but this is the worst kind. It looks like they put a straw in his skin, and stitched it in place. It doesn't empty into anything (so all over his hair)... It's so open it could catch flies. (It's covered, don't worry). Talk about infection risk, yuck. (As long as it's covered it should be fine, but still).

The good news is Micah was somewhat of an immune system at the moment. His white blood cells are decent (they're never good), and his IGG should be up still. We've made two trips out, one for pizza with some of our youth and one to do some visiting in Warsaw. Both good times, and he's not even sick yet! He's off the rapamycin due to the strange things it was doing to his body, although I'm sure we'll start it again soon. Boo! So, one infection risk of 4... That's the best we've done since oh... March?

We go back to Cincinnati Monday. I'm pretty tired of these visits, won't even lie. Should be quick and easy, although it's not til 5:30, so it'll be a late drive back.

Micah's doing well though, so no complaints.

The update, part 1 & 2

The update, attempt two.

As in any college paper that you lose mid way through because something crashes, this isn’t going to be as thorough as it was the first time (this is a pain I know well!)

Last week when we went down to Cincinnati, Micah needed another IVIG (immune related) infusion. His numbers dropped (ideally, at least 400, they’ll accept 350 given the circumstances) from 500 after his last one to 300 in about a month. The infusion is about 4 hours, which makes a bummer of a day.

The jury is still out on the rapamycin. It did its first task well, in about a month. It’s immune suppressing, specifically related to viral infections and pneumonia. Coincidence or not, he’s been sick 3 times since starting the medicine two months ago. With the high risk, even a cold means a trip to the pediatrician, a chest x ray at the hospital, and a round of antibiotics. YUCK! The medicine can have a secondary effect in some cases of reducing the size of the mass. Key word, sometimes. It also can take 6 months plus to see this. We're left to decide whether it's worth it. It'll never replace surgery, I'm sure but could delay it, or at least maybe part of the surgery.

Oh, baby is up. That was two parts of 5 that I had written. It’s a start.

Just another update...

We're plugging along. We went to Cincy yesterday and finally got his drain out. It had been in for 6? (can't even remember) weeks! His skin seems to be closing up nicely. We went for bloodwork and a check up, basically.
Other than that, we enjoyed Mother's Day at my moms house with the family. We're also planning on going to the zoo this Saturday, yay! Someday I'll get pics up, they just can't seem to get off my camera!

Update on the update :)

So, we have almost all the bloodwork back (thank goodness for Cincinnati's new online feature!)
Overall, the verdict is mixed. His IGG (immune related) is back up where it should be after the IV he had two weeks ago. This is to be expected, as the IV is supposed to work for at least 3-4 weeks. BUT (and there's always a but, it seems) his cholesterol is just getting further and further out of the realm of normal. We didn't have a baseline before the meds unfortunately, but even after 1 week of meds it was high (203) and now, after a month it is 232. I just hope it stops rising because we've hit the right amount of the drug in his system. His LDL cholesterol and trigylcerides are increasing steadily as well. I hate this, because I've been told there's nothing on my end that I can do. Even so, I'm trying more of his blended diet, hoping his body can deal with that better than the pediasure.

So, we finally got out of the hospital at 4pm and headed home. His IVIG went off without a hitch once it finally got going. No adverse reactions at all. But, we are faced with a new problem... The kid has high LDL cholesterol and triglycerides. (Inside I'm saying, are you kidding me! Okay, maybe I said that outloud). The kid is in the like 10th percentile for weight. The kid gets basically JUST pediasure. Everyone is scratching their heads, needless to say. It really feels like everytime we do anything, something else happens. The rapamycin can cause this, but it seems unlikely that's the only problem (it was high after a week of a starter dose). Does the kid need lipitor or something? He's not even two!
So, his IGG level was below 140 (supposed to be at least 350-400), not 240 like I thought. Not only that, this week it was 91. But with the IVIG it should be back in happy land, at least for a month. I'm really starting to pity these doctors trying to keep everything straight.
I have a tendency to play out the future in my head, but I can't even do that anymore. It used to be "do this, then this, then this..." you know, something that resembles a plan.
Here's where I sigh dramatically.

Some info...

So, this new medicine came with a little stronger warning from the Hematologist/Oncologist than we'd previously heard. This medicine is her 'baby' so to speak, she's overseeing the clinical study and the kids not on the study as well. We're back to being "on high alert" when it comes to sickness and germs. The first 8 months of his life were that way, and to a lesser extent, this last winter. But, back to high alert. We're thankful for the fact it's spring and there's less going around now than a few months ago. But, we were looking forward to getting out and doing group things... which isn't going to happen now... For at least a couple more months.

The docs want to know anytime he gets ANY type of illness. Luckily, our Ped knows us well and does not hesitate to see us anytime something is up. He's a great partner to the Cincinnati crew.
Therefore, we will be cutting our outings at church down at least a little and not taking him to the store as much as possible. Luckily the majority of youth stuff is outside in the summer, less likelihood of getting exposed to germs.

But... if you're sick, your kids are sick or anything like that, please keep your distance from Micah.

Anyways, enough depressing stuff, here's the cute kid. He's such a trooper! We've been to Cincinnati 3 times in 8 days, and he really hangs in there for us. It would be nice if we lived closer, but at least we're not any farther away!

Well..

I haven't updated in awhile because... wait, need to go to cincinnati.... alright, we're back. Micah just KEEPS draining. Surgery was the 3rd of March.. oh, back to cincinnati... So it's been 5-6 weeks now, and still draining... here we go again! So they put a drain back in and are trying sclero for another time.... off we go! And then another time! into the wild blue yonder... And then they'll try the rapamyacin, probably early next week. So that's our update!

End of March update

On Tuesday we went back to Cincinnati for what I'll call a "well, shoot" visit. We were all hoping that his lymphatic malformation would stop draining so much from where thy did surgery, but it hasn't. We 're at almost 4 weeks post-op and it's not even slowed down. Well, shoot! We did two rounds of injecting that usually helps shut it down but that didn't happen. The consensus is to take one more stab at it. It's (relative to other options) a lower risk option. (We've still got one vote out on this one, yet..and a rather important one!) Sounds like we'll do this the week of the 11th. They'd have us do it sooner, but we'll be gone all next week. The big change is that they want to start him on a medicine that shows some promise to stop the drainage. First, let me explain why the drainage is such a problem. Apparently lymphatic fluid carries protein, and as he loses the fluid, he's losing protein. He's been losing 4-5 ounces of fluid a day for pretty much the last month. The drainage also contains some blood, which he doesn't have any to give. His blood work wasn't stellar when we left the hospital on the 12th. So....about this medicine. The hospital has been using it occasionally for the last few years with some success, usually in cases of inoperable lymphatic malformations and other vascular issues. It's an immune suppressant, which is the biggest problem with the medicine. It's typically used as one of two or more drugs to supress the immune system in transplant patients. The medicine is a little mysterious in exactly how it works, but at this point it's all we've got. The hope is to use it for a short time (although sometimes it needs to be used for a longer time) and take him off, do another surgery and use it again if it's needed. We're pretty bummed about all of this. The medicine has always been mentioned as a possibility. I'm glad that it was, so this wasn't totally out of nowhere. That said, it still came out of left field. Other than the immune suppression, the side effects are generally tolerable. (Other than immune suppression, geez....They give antibiotics to try to curb bacterial infections, but there's still a risk of viral infections). Of course, this is a small part of the whole story. We have to decide pretty quickly about the medicine but they're giving us a few days. Of course we hope that we get the drainage stopped before we have to make any decisions, but it's not very likely.