Only two more to go!

Spent another day at Fletcher Allen getting the boys infusions done to increase their bone density.  Over a two-year period we have to go every three months and today was #6!!  We go again in February and then again in May and we'll be done.

We thought Nurse Lauren was going to work today but she woke up with a cold.  So it was just me and Nurse Julie.  Of course my timing of everything was off but we made it out of here only about 5 or 10 minutes later than planned.

Hit registration, which took a while, and then we went to the Comfort Zone.  They put us in a big room so we could be all together so that was good.  Casey went first.  After a lot of looking and warming up various parts, they tried his foot (where it was successful last time) but it was a no go.  Then they got his left antecubital on the first try, whew!  Casey kept everyone on their toes talking about his Google maps and songs and such.  They got Colin on the first try in his left antecubital as well.  Another whew!  His poor little hands were dripping with sweat but he was congenial.  The Comfort Zone nurses are used to working with kids so we had lots of joking around and some singing, oh my.

Headed up to the Children's Center.  Some waiting.  Check vitals.  Head to the Bay.  We waited to make sure their pre-infusion labs were good and then we got them out of their chairs and settled onto the beds. 

Colin all snuggled and watching a Charlie Brown's Thanksgiving while getting his infusion.

Casey is Google Mapping and chatting on his DynaVox while his infusions run.

Tylenol given.  Saline bolus given.  Get the word that labs were okay on both so the infusions run for 2 hours.

Casey had the Child Life Specialist, Jenny, to himself for a while and he loved it.  They have developed a relationship over these visits and enjoy each other.  She is a good sport.  She takes Casey's orders for this or that movie and tries to figure out what he wants. "Man, movie, funny...Christmas." They finally figured out he wanted the Jim Carrey's version of How the Grinch Stole Christmas and they didn't have it anywhere in the hospital!  They both got a chuckle out of that.  And, it's not on Netflix, either because she went and got an ipad and tried.

Colin was pretty easy today.  Some ows and one change of movie because he decided he didn't like it but other than that it was just routine.

Saw Dr. Zimacus, the endocrinologist.  Talked about maybe jump starting Casey's puberty with testosterone injections.  That's all I'll say here :)

Our only downfalls were:  both Julie and I forgetting to give the boys their 1 o'clock feeds, and Julie forgetting to bring an extra Trilogy battery.  Not bad for a long day at the hospital.

We actually had infusions done by 2:30 or so.  Packed up, in chairs, with coats on by 2:40.  Got the van by 2:50 and home by 3:20.

Again, I am soo thankful for our van, Nurse Julie and our hospital being so close and being so good to us.

Got home and started getting settled and Paul from Apria came with Colin's new heater.  VERY long story short, Colin's heater crapped out on Thursday. Sent a script for new one to Apria on Friday.  Apria came back saying they can't provide such a thing and to find a new DME.  PFFFTTT!  Luckily, we have two.  What would we have done if we didn't have 2????  Colin could NOT have gone from Thursday to Tuesday with out a heated humidifier.  Anyway, Apria couldn't figure out how to get one/bill one so they gave us one they had.  I appreciate that but find it totally wacked out that they couldn't figure out the paperwork and how to bill for one.  So many stupid changes going on.

While Paul and I were talking in the hallway, some drama was taking place in the living room.  Gene was lifting Casey of out his chair thinking that everything was unhooked.  Nope.  Feeding tube was hooked to pump which was in the backpack on his chair.  So, out came Casey's feeding tube with the balloon fully inflated!  Ouch!  Nurse Julie jumped to help and somehow tripped over Jaxon who thought she wanted to play so he started running around like a mad dog, and Julie landed in a not pretty way, but didn't land on a boy, so that's a good thing (they are laying on mattresses on the floor in the living room) but she did manage to break the end of the plunger piece of a syringe--who knows!  All is good.  Gene put Casey's g-tube back in--with only "a little dog hair attached to it" he said, ha ha.  I missed the whole scene!

They've both been given Tylenol just to ward off any aches or ouchies and are watching a movie.  Early to bed for both.  Casey always seems a little pale and woozy after his infusion but Colin is as perky as always.

I have to go clean up their room (looks like a whirlwind was in there!) and put away things from our trip today and make formula.  Oh, and I have to try to ready the 'aunt' room because Peggy will be arriving on Thursday!!!!

4 Pamidronate infusions down; four more to go

We made it through another long day at Fletcher Allen Health Care getting Pamidronate infusions.  The boys are getting these in hopes of increasing their overall bone health and, hopefully, avoid breaks.  The boys have each had a couple of broken bones but we truly have been lucky in only having a few compared to many kids that suffer multiple breaks.  And, all the breaks we've had have been before we started the infusions.

Anyway, once again it took us longer to get the boys ready for the day, up in their chairs and out the door but we made it to the Children's Specialty Center by 9:50 which was only 5 minutes past my goal :)   We had to wait a few minutes before we were called back, but not very long.  Vitals taken and weights recorded and then we went and settled into the infusion bay.

We got Casey onto the bed and comfy.  He certainly had his moments of grumpiness and loud vocalizations but he also cooperated and read some of the Spark notes on My Sister's Keeper with me.  The child life specialist also came and spent some time with him chatting and changing the TV and movies.  We all had our turn entertaining him!

Colin stayed in his chair but next time we'll get him out so he can be repositioned better.  He gave us a lot of "ows" but mostly because he didn't like that he had to keep his arm as straight as he could.  the nurses got right to warming up both boys arms with hot packs and towels.  The IV nurse got his vein on the first 'pick'!  She drew the pre-infusion labs (calcium and phosphorus) and also Vitamin D that I had requested since we've upped the boys daily dose in hopes of getting their levels up.

Dr. Zimacas came in and I updated him on the boys diets/supplements.  He'd like them to be getting more calcium so I'm going to ask our nutritionist to sit down with me and work on the boys diets.  It's been about almost two years since we've taken a detailed look at everything.  The boys nutrition labs have been good so I'm not wanting to change much but I'm sure it will be good to go over everything again.

Both boys were examined by Dr. Z.  May need to get an ultrasound of Colin's 'package' but he said it's low on the priority list and not to worry too much.  We may need to look at jump starting Casey's puberty but we've still got time so nothing to do in the short term.

We will schedule the boys for a dexascan next time to see if we can get a picture of their bone health.  Years ago we had them done and the results were not very valid.  With Casey's fusion hardware and how we positioned Colin they didn't get great pictures.  This next time we'll look at different areas to scan on the boys and hope we can get a baseline so that when we're done with our two years we'll take another scan and hopefully see that we've been making a positive difference.  Of course, not having any future breaks will be the real test, and the best reward!

All in all, everything went pretty smoothly.   Casey got pretty chokey and full of secretions at the end and Nancy had to trach suction him several times.  Next time we'll bring up our cough machine or order one from respiratory.  MAYbe we'll have the new, portable version and it will be easy to carry instead of having to bring the big ole honker on the luggage rack.  I gave Dr. Lahiri a list of things to put on a script for the Respironics T70 Cough Assist.  I've been waiting for the branch manager at our Apria to look into getting one but I don't think he's looking very hard.  If he has the script from the doc he'll have to start doing some fancy footwork, I hope.

We got home at about 3:30--took awhile to cough Casey in the valet parking area and get them loaded up and on our way home, but we did it!

Casey clicked some channels in the yellow room for a while.  Colin went right to his mattress with a big "AAAHHHHHH" and has been happy with his shows.  Brooke is here for the evening so she's wii-ing with the boys.  Colin is intent on watching and Casey is spacing out watching some football and being very quiet.  Casey seems to be affected a bit more by the Pamidronate than Colin.  He'll be back to his feisty self in the AM. 

Sorry no pics!  I could have taken some with the ipad but didn't think of it until now :)

Pamidronate Infusions, ugh

So we're here.  Casey went in first.  Infusion Bay is very busy today.  They only had space for one boy at first so we volunteered Casey.

Julie and I got him out of his chair and comfy on the bed.  IV came right in to get him started.  He needed some warm packs and towels because his hands and arms were freezing, but it's about 8 degrees out so I'm not surprised.

IV Nurse Micki got his IV in pretty quickly (for us, anyway).  Success on first try in left wrist.

Colin was another story.  He ended up with 3 picks, total.  One successful, finally.  I was not happy but I didn't actually see when they were poking.  Colin was a trooper although he did "OW" a lot and had shed a tear, but no full blown crying.  It took the two IV nurses trying but they were successful in his left antecubital.

Colin was very happy watching movies.  His first one was Tron.  After that I tried reading City of Orphans with him but didn't get too far.  He is going to be in big trouble with Priscilla tomorrow because we didn't read as far as we were supposed to.  Funny how during the movie he hardly "owed" at all but when I was reading he was full of "ows." 

Woozy Colin.

Casey went through periods of being good and being very needy.  He had a grand time yanking the Child Life Specialist's chain :)  Jenny was a good sport about it and played along with him.  He was telling her that he wanted a funny movie/show about animals that is normally on during the day.  He had her scouring the Internet looking for this show--that doesn't exist, lol!  I think she was impressed with his DynaVox use and tried to honor his requests. 

Grump and woozy Casey

Casey was done right at 2:00.  The nurse had called ahead to Dr. Hubbell's office to say we might be a tad late.  We only had to wait for a minute before they took us back and Dr. Hubbell came in right away.  Casey has only one tube which is in his right ear (he's been like this for a couple of years now).  The left ear looked perfect.  His right ear had lots of 'debris' that Dr. Hubbell sucked out.  Julie and I both wish that the suction tubing was clear so we could see what gunk came out of him, lol!  He also looked in the back of his throat because Casey had said it hurt yesterday but it looked nice and healthy to him. 

Interesting aside here.  Yesterday Casey told Julie several times that his throat hurt.  She looked in his mouth and felt around but didn't see anything concerning.  Then, at bed time, I could hear something in Casey's throat when I suctioned his mouth.  It took a lot of work--coughing, saline and pressing on his tongue--but I got a pretty large plug out that was very stringy.  I wonder if that was bugging/hurting him?????   With Casey, it's hard much of the time to know when he's being serious or just telling us tales, so this was interesting to me. 

Colin was still infusing when we were done so Julie and Casey packed themselves up and went downstairs to check out the gift shop.

Finally, Colin was done infusing and had his saline flush and was done by 3:00. 

Headed down to find Casey and Julie.  Got the van.  Loaded up.  Julie drove home.

It was a very weird drive home.  Casey was quiet as a mouse and we are NOT used to that!  I kept asking Nancy if they were okay back there. 

These infusions sure wipe the boys out.  Casey even more so than Colin.  Casey looks pale and puffy and tired.  Colin looks a little pale but is quite full of it and is yelling at Aunt Chris while she plays wii.  Casey goes from being very quiet to fussing loudly, doing a little talking on his DynaVox. 

I felt very comfortable driving the van.  It sits higher than our old van and the nose is longer but it was pretty easy to drive.  YAY!!  It will take us a little bit longer to get used to tying down the boys chairs but we did pretty well.  It would help if it wasn't as cold so we could be more comfortable while we work on positioning and all that.  But it's nice to have these Q'Straint QRT Max tie downs.  It literally takes one hand to secure them and one foot and a hand to undo them.  So much nicer than our old ones that I don't even know the name of!

Another round of Pamidronate Infusions

2 down (the ones we did several years ago don't count :( ) and 6 to go.  After the 2 year mark we will re-evaluate.  FYI, Pamidronate is in the bisphosphonate family and is used as prevention and treatment of osteoporosis.  It's in the same family as Boniva that is touted on daytime TV by Sally Fields.

We were all pooped out by the time we got back home but things went pretty smoothly, it's just a lot of stress and I'm sure it's not a picnic for the boys to have an IV in with medicine going in...

Colin was the first to have the IV nurse work on him.  It took some hot packs and a lot of looking, looking, looking before she found a tiny vein.  She got it on the first try and was able to draw what they need to check before the infusions are given, as well as the Quantitative Amino Acid profile that we needed for nutrition.  We were very careful with his arm and hoped that the vein would last through the treatment, and it did! The IVs nurses name was Sue/Susan and had a lot of experience--will remember her for next time.  She was successful getting Colin's in on the top of his left hand. 

Colin half-hardheartedly would "ow, ow" and want me to put the hot pack over his IV but really he did great.  He picked National Treasure to watch and was transfixed the whole time.  He wants Aunt Chris to buy him the sequel.  He watched part of one of the Pirates of the Caribbean movies but we were out of there before it was over.

The IV nurse had a time finding a vein on Casey, too.  She ended up getting it in his left wrist.  Julie said that's where he had it last time.  His lasted the entire infusion as well.

Casey watched Drumline and some sports and was pretty quiet the whole time.  Julie got him out of his chair and all comfy on the hospital bed.  

Dr Zimacas (endo) came in and went over a few things.  He'll skip seeing us next infusion and see us at the one after that.

Dr. Lahiri (pulmonary) came in and I asked him to up Colin's settings on his vents/bipaps.  He was fine with it.  Colin's daytime settings went from 19/4 to 21/5.  His AVAPs, nighttime setting will stay the same.  It's set to give him a tidal volume of 200cc so his pressures will go anywhere from 19-25.

Candy came in to schedule next infusion which will be February 5th.  I really hate going to the hospital during the flu season but it's got to be done.  We have to keep on track so we can get these infusions DONE.

Found out we're due for MDA clinic on November 27th.  We'll see Dr. Lahiri, Dr. Benjamin (rehab) and Dr. Bingham (neuro) and Linda LaShure the dietician.  We had labs drawn for D3 so we'll see if the 800units we're currently supplementing with is enough.  Have to look at calcium, too.

Oh, and we weighed the boys this morning.  Colin is 49 pounds and Casey is 70 pounds.  No changes from last time they were weighed about 3 months ago, but that's okay.

All in all we were there from 10:00 and were done at 2:30.  I think that's pretty darn good.

Stopped by the gift shop.

Ordered a pizza from NY Pizza oven so we drove home through the bay.

Gave the boys some Tylenol when we got home.  Casey wanted to hang in his 'old' room so Julie set him up with his e-reader listening to Marcello in the Real World.  She had a bunch of his fish/dolphin lights on so he was happy.  He was very quiet and pale and sleepy looking.  Colin seemed pretty normal.  He watched his shows in the living room.

Headed to bed early.  Boys were out quickly.  I slept in bed with them so they would feel extra safe and comfy.  They slept really, really well.

I got up around 1:30 to check out the election results and was happy :)  Went back to bed and slept like a baby. 

Pamidronate infusions and 3 broken things

We're back from the boys Pamindronate Infusions.  We got there about 10:00 and were downstairs waiting for the van by 2:25--not too bad--and home by 3:00.

We had a good IV nurse who got a vein first try for each boy.  Whew!   Tylenol given via g-tube, labs drawn, 1 hour of saline via IV, 2 hours of Pamindronate via IV, short flush and done!

Casey was the needier one, as usual.  He wanted a movie.  He wanted a different movie.  He wanted music.  He wanted a different artist.  He wanted TV.  He wanted football on the ipad.  He wanted a different football game.  So on and so forth.

Colin was pretty happy with Nick on TV and then Honey, I Blew Up the Kids.  He was kind of vocal saying "ow, ow, ow" about his IV and "out, out, out" wanting to go home, but he only high heartrated once when he was getting his arm assessed.

I almost cried when a little boy, maybe 3, came in and got so upset about having to have his infusion.  Must have been a maintenance drug for some type of cancer.  He was screaming "I don't WANT to do this!  I don't LIKE this!  PLEASE don't make me do this!"  Totally hurt my heart.

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Broken thing #1  Colin's new chair has squeeze handles to change the tilt and recline.  Well, Colin will tilt no more, until we get that fixed.  Apparently when Casey was loaded into the van today he crushed Colin's handle with his chair and when we got up to the infusion bay we noticed that it was broken off.  BIG bad, bummer.

Broken thing #2  Colin's newish Nasal Aire's (his bipap nasal prongs) cracked.  It happens quite often, which sucks because we can only get so many in a period of time.  We buy them out of pocket as we need them.

Broken thing #3  The van is making awful noises.  The guys at the valet parking at the hospital said it sounds like a CV joint.  Well, I said that to Gene when we got home and he laughed and said we don't have CV joints because it's rear wheel drive and has axles.  But something is obviously wrong!   Our inspection ran out the end of June...I hope Gene brings it to some reputable place and gets it checked out and inspected. 

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So, right now both boys are hanging in the living room waiting for Julie to play a little wii.  They look pale, but they are both full of piss and vinegar, so, hopefully, we won't get any of the flu-like side effects that we had last time.

Endo and hair cuts

Another appointment today.  Today was meeting with the endocrinologist, Dr. Zimacas.  It's been about 3 years since we've seen him and about 2 years or so since the boys had their last Pamindronate infusion for bone density.  We didn't have the worst experiences with the Pamidronate, but it certainly wasn't smooth sailing.  So I kind of dropped the ball on them.  One of the main reasons I stopped was that when the boys had their bone scans to check their density, the results came back as unreadable.  I can't really understand that because so many other SMA kids get scans done and their results are readable.  Dr. Z said because of their contractures and Casey's rods, that it's next to impossible to get good results, period.  And, that there is a wide margin for error each time so it's very hard to see true improvements.  Anyway, I'm not sure if I agree with that part.  I would like to try to get baselines on the boys some time but I won't push for it at this time.

Dr. Z spent a lot of time talking about the thoughts on how Pamindronate works in increasing bone density scores in certain populations of people...I won't bore you with that here.  He also acknowledged my concerns about if this will help long term and if the density increases we may see are truly increases in density that will help prevent fractures in the future.

He also made sure to let me know that this is not a 'standard practice' and it's totally up to us as to whether we want to proceed or not.  So if we decide NOT to do it, he would be fine with it.  He said if we had opted not to do Vitamin D--he would convince us and show us the proven data on Vitamin D and bone health.  But since this isn't proven, he understands that we are the ones to make the choice to proceed or not.

If we do go ahead, he really wants us to make the commitment to do it every 3 or 4 months or so, two days in a row (about half a day each).  It would include them getting IVs each time.  That is one of my biggest fears.  They are hard sticks and it's such a stressful thing for them and for us.  Sigh.  The first time we had them done Colin's IV infiltrated over night.  Ugh.  The second time we saved the IV overnight but then it wouldn't draw so it had to be redone.  They also had some flu-like side effects but I understand that each time they are administered the side effects become less.

So, at this moment I am leaning towards doing them.  I will talk with Gene some more and then decide.  If we do go ahead, I'd like to get two doses in--maybe June and October and then stay away from the hospital until spring.

He examined the boys and found them to be progressing puberty-wise.  Colin a little slower than Casey :)

Afterwards we decided to play hookey from school.  I had been planning on dropping Casey off at school and having Priscilla come for Colin but we decided to get haircuts instead.  We called Gene and he said he'd meet us at Garry's Barber Shop to help.

The boys had the same hair cutters as they had last time so they were pretty efficient and did a good job.  Gene's friend, Lori, started goading Gene to get his hair cut (it's been a LONG time) and Casey chimed in, so he folded and let her cut his hair and trim his beard. 

Yes, you are seeing a miracle...Gene is getting his hair cut (and his beard/mustache cut and trimmed)!