Osteoporosis Checkup and almost birthday time!

Mason had a great holiday season. Family time, parties, church, book club, art class, and homeschooling filled the season. He also started physical therapy each week with a walker. His birthday is on Wednesday. I'm not sure where the last 5 years have gone but this sweet boy is a joy to know.

In medical land he had his yearly checkup last week for his osteoporosis. The DEXA scan shows that his bone density is better than it was a year ago - hooray! It also is still in the negative numbers so we will keep going with his infusions. I am waiting to hear from his doctor whether he will continue with IVs every 6 months or if we will space it out further.

On the same day he had his DEXA Mason had his IV infusion. He and I got to watch a couple movies and eat lunch while the medication was going in. He's a trooper.

Later this month he has appointments with colorectal and his full team appointment at Myelo clinic. Next month he'll see his orthopedic surgeon for a check up on his scoliosis. It's that season again where we evaluate what is working, what is questionable, and hopefully this time around we'll have nothing unusual going on!

Merry Christmas and Updates

The year is nearly over.  We have been enjoying lots of family time during the Christmas season.  This is a picture we took of the kids Christmas morning. 

One fun memory of Mason I want to record here:  This year he has been adamant that we bake a cake to celebrate Jesus' birthday (Christmas).  Even better, he is sure that Jesus loves chocolate cake the best - so that is what we needed to make.  It was delicious and Mason loved helping to mix the ingredients.

Mason continues to have a few concerning issues and we have appointments coming up for those (vision/eyes, foot contracture) as well as checkups with his entire team of specialists all in the next few weeks.  Developmentally, however, he's doing fantastic.  We are getting ready to celebrate his birthday and I'm not quite sure how he's about to turn 4 years old!  He is articulate, opinionated, and funny.

The most recent medical happening was last Monday when he had his next IV infusion for his osteoporosis. He does really well with the medication, which we are grateful for.  It did take 4 attempts before they were able to get an IV started, which stinks.  His veins are just not great, no matter how hydrated he is.  We always make IV attempts in his feet first so that he doesn't feel it.

I'll update again soon!

Quiet Blog, Quiet Life

Honestly, I have been slack on blogging here but have no huge updates.  I'll try to hit the main things:

1. Mason is in good health generally.  He's happy, growing, learning, and so full of personality.
2. He still has occasional headaches.  We wonder if they are related to his bifocals.  Neurosurgery checked him over and the shunt and everything else looks fine.
3. Bowel management (enemas) is going great.  We've moved his enema to afternoons instead of evenings and love having our evenings free for family time and relaxing.  He is generally cleaned out well with the balloon enema and has no accidents.  It takes us about 50 minutes from starting the fluid to getting off the toilet.  He does still require miralax daily for the enemas to be successful (keeping poop soft enough that it will come out). 
4. Feet/legs and bracing - This is one area things are not working in.  He outgrew the standing braces (HKAFOs) but at the same time his right foot has contracted and so his shorter braces (AFO, begin just under the knee) don't fit either.  And the bracing doc can't fix braces enough for that, we're needing to see his orthopedic surgeon and possibly looking at another surgery to release that foot tendon again.  As I'm having baby #9 this week we've chosen to wait a bit on pursuing the appointments that will lead to that decision, we'll revisit it come January and are working on stretches to see if we can change things in a good direction with the foot instead (which is what his surgeon prefers we try first). 
5. Mason's osteoporosis has improved!  We had his yearly checkup with the nephrologist and a bone density scan and while his numbers are not yet normal, they are better.  We will continue the IV infusions of Zolodronic Acid, spacing out to every 4 months instead of every 3 for the next year. 
6. Large Motor Development - Mason is at a pretty stable place right now.  He can climb into and out of his wheelchair, climb a flight of stairs, get into and out of chairs/on and off furniture, and shows large amounts of strength in upper body.  His balance when sitting is decent, and we don't expect it to improve beyond what he can already do.  He generally can do anything he really wants to do, whether using his wheelchair or not, and if he can't figure out a way he is not shy about telling us what he wants to do and insisting we pick him up/carry him/ help him. 

I think that's it!  He currently loves pirates (Jake and the Neverland Pirates, Peter Pan, and pirates in general), is beginning to enjoy building with Legos, still loves playdoh, and talks up a storm.  He sleeps through the night in his toddler bed (we cath him at midnight but he sleeps through it).  He does not like loud noises (very normal for someone with hydrocephalus and a shunt) but he does like music and dancing if the music isn't too loud.  He does not like to sing.  He does like playing instruments (we have piano and guitar) and is asking for drums for Christmas.  We'll see...

Bowel Management Bootcamp - Day 6

Yesterday was a very full day so I didn't get to sit and type up our report.  After a weekend of doing the new foley balloon enemas with Mason and having no accidents I was interested to see what the x-ray would show.  The doctors said it looks great, that the current plan is emptying his colon where it should.  Hooray!  So this is what we stick with for now:

• Liquid solution of 400mL saline and 30mL glycerin
• Time putting fluid in/holding fluid: 10 minutes
• Time sitting on toilet total, including fluid time: 60 minutes

Mason will have an x-ray today (Tuesday) and then on Wednesday he will go to Children's Hospital for a final x-ray and meeting with the colorectal team to finish out his week of Bowel Management Bootcamp.  Then we just keep doing what we are doing.  We will also probably play with the length of time Mason sits on the toilet after this week is over.  During the program they ask you to be very exact in what you do to control all variables.  However we're noticing that Mason doesn't seem to have any fluid coming out after about 45 minutes sitting.  So we'll drop our sit time to 50 minutes and see if he remains accident free.  Why?  Because sitting on the toilet for an hour is a really long time for a 3 year old!  He does amazingly well at being patient, playing games with us or watching cartoons, but if we don't have to sit for exactly an hour then we won't.

The other big thing for Mason yesterday was his fourth IV infusion of medication for his osteoporosis.  It ended up being a long-ish afternoon.  We left at 11am and returned home at 3pm.  It would not have taken so long but one of the things we always require is that they call down the IV team to place Mason's IV.  His veins tend to collapse so we have a standing order in his file that they are to call IV team.  We also have them place IVs in his feet (unless all those veins blow out on a bad day).  So a large chunk of our time yesterday was spent waiting for the IV team to make it to Mason.  They finally got to him about an hour and 45 minutes after his appointment time.  The regular nurses offered to try and start the IV when they heard from IV team that it would be an hour and a half wait.  We declined, telling them we were happy to wait and that we always come knowing we will need to wait.  You could see they were skeptical. That is when I tell them that Mason's record is blowing 6 veins and finally getting an IV placed in the 7th vein.  We're not willing to mess around and are happy to wait for the people who literally spend all day placing IVs to make this as easy on Mason as possible.   

Well, that's really all I have to report today so I'm off to wake Mason and head to x-ray!

New Braces at Last and other general updates.

 

 All pictures in this post are from today - Mason showing off his new braces.

Mason's days have been pretty normal with no major updates for over a month so this blog has been quiet for a while.  Since he turned 3 in January we have seen some fun fine motor development.  Mason now colors purposefully instead of scribbling.  He colors in different parts of a coloring page, still going outside the lines, but you can tell he was coloring something specific.  He is also beginning to draw his own pictures and tell you all about them. 

While we have been waiting for new HKAFO braces to be built Mason has spent his days usually evenly split between using his wheelchair and not.  He has gotten stronger and one of the best evidences of that is his ability to climb.  He now can climb from the floor onto the dining room chairs and up onto the table.  Last month Mason had his second IV infusion for his osteoporosis.  It was done outpatient and they were able to get an IV started in his foot after a few attempts. 

Today was a happy day because Mason got his new HKAFOs.  We drive to the orthotist's office early in the afternoon and after a few hours of building, adjusting, and trying on the braces were ready to come home with us. 

In a week or two we will go back and the orthotist will make any further adjustments we think Mason needs.  That could be adding padding, straps, or cutting and trimming things.

Mason is so happy to be able to use his Rifton Dynamic Stander again!

In one other bit of news Mason is happy that he is going to be a big brother again this coming fall.  He is a great big brother to Samuel and a good little brother to his 6 older siblings.

Mason's Getting Stronger!

 

Things for Mason have been fairly quiet since I last posted here.  He breezed through the week following the IV infusion of osteoporosis medication with mild symptoms.  At the same time I, Mason's mommy, have been dealing with my own medical emergency, which you can read more about on my regular blog here and here.  As things have settled down we have been thrilled to see Mason making a lot of progress in his physical development. 

If you remember, months ago Mason had tendon surgeries for his right hip and heel.  Two months of casts and broken bones from those casts later he was finally able to move around again, only to discover he had lost both strength and ability.  The cut tendons in his hip have affected his ability to move that hip/leg.  The lost strength in combination meant my sweet boy could no longer climb up steps, transfer into and out of his wheelchair, or do anything using similar muscle work.  We have done weekly physical therapy with a new PT and developed some exercise play routines here at home with the therapist.  Mason's hard work is paying off! 

 

Here he is, climbing our flight of 13 steps so he can go play upstairs in the bedrooms.  Without help (other than a spotter to make sure he doesn't slip and fall).  He's so proud of his ability.  He loves the freedom to go play upstairs without being carried there, especially right now as the large tub of Legos have been brought out of the attic.

Unfortunately for this momma heart he has also decided he can climb DOWN that flight of 13 steps, so I regularly hear him calling, "I come down.  Legs, mom.  Legs first."  Then I rush over to find him backing up on his belly to go down legs first.  He's getting better at it.  At this point he needs someone with him for every step down because if he gets distracted he starts moving too fast and his bottom moves faster than his legs and then he falls backward.  Where we catch him.

I know in my head this growing independence is a good thing.  I cheer him on.  He's learning to do things we want him to do.  I just wish he were in a padded suit while attempting them! 

The First IV Infusion for Mason's Bones

 

I have just a short time here at the computer to share updates on Mason so here we go!  On Monday Mason and I headed to the hospital for his first IV infusion of Zoledronic Acid to help strengthen his bones.  This first dose was to be done inpatient so they could monitor his reaction.  We showed up cheerfully and got into his room, put on PJs, and waited for the IV team to arrive. 

I warned them before beginning that Mason is a hard one to stick, his veins tend to clot or collapse.  Unfortunately this was in full force Monday.  The first IV team attempted three placements in Mason's arms.  All 3 failed.  They could not even get enough blood to fill one tube for his labs, much less the five tubes he needed before placing the IV.  The IV lady left in tears for my poor, sweet boy who was in pain and asking if he could go home or if she would go away please.  A while later another IV team arrived to try.  They used one of Mason's feet and after two attempts (#4 and #5 for the day) they still had no success.  This IV team lead said she would be back later after tracking down a machine that might help.  The only good part to this attempt was that Mason cannot feel his feet.  They were bruised right away but he didn't feel anything.  The nurses also put a call in to Mason's nephrologist Dr. A to tell her what was going on and recommend she begin considering alternatives. 

Mason ate lunch (pancakes, chocolate cake, milk) and fell asleep for a nap.  During his nap the IV team lead returned with an Accu Vein.  She checked both feet and made one attempt in his left foot (#6, no success).  She made attempt #7 in his right foot and finally got a useable vein.  Blood was collected for labs, the IV placed, and Mason slept through the entire process. 

Nurses started the infusion, which was only 1/4 of the normal dose.  Half an hour later it was done and we had to just watch for a reaction.  Mason was on blood pressure, breathing, and cardiac monitors for the next several hours.  He did just fine.

Late that evening Mason was cleared to leave his bed and go to the playroom.  He was ecstatic. 

He and I had the whole playroom to ourselves.  The only rule was he had to stay in his wheelchair because of the IV lock still in his foot.

Two hours later I convinced him it was time for a snack back in his room.  He was getting pretty silly and sleepy.

He slept well that night and we spent time playing with playdough and watching some dvds to pass the morning.  He had visits from his doctor, nurses, and several other medical professionals, had labs done, and all was well.

Finally he was released to go home, with one last ice cream bowl from his nurses for the road.  :) 

Since returning home Tuesday Mason has done remarkably well.  We have been expecting a high fever and flu like symptoms (achy bones) but so far everything has been mild.  Mason will have another infusion in 3 months and then every 3 months after that.  If the IV continues to be a struggle there is an alternative medication he could do orally but it is not as effective as the Zoledronic Acid.  We will have to wait and see!

Does Spina Bifida Cause Bone Density Issues? A Reader Question

I had an anonymous question on my last post that I really wanted to answer.  It was asked:

Is low bone density a side effect of Spina Bifida?  If so, why?

That is a great question!  Low Bone Density is not an automatic side effect of Spina Bifida.  However Spina Bifida is part of what has contributed to Mason's!  Because of the extent of Mason's paralysis from Spina Bifida he cannot stand or bear weight on his legs at all.  One large factor in strengthening a bone's density is USING the bone, applying weight and pressure to it. 

To combat that the plan has always been to use HKAFOs (standing braces) and a dynamic stander that holds Mason's legs straight and holds him in an upright position that would place pressure on his leg bones.  It was to be a therapeutic, several hours daily practice.  We started that process when he was a year old. Here is a post about Mason getting HKAFOs with pictures.  Here is a video of Mason learning to use the Dynamic stander.  He was 13 months old for both of those.

Unfortunately at 18 months old Mason's tethered spinal cord became symptomatic.  Over a 4 week growth spurt the stretching of the cord caused contractures in both of Mason's hips and feet.  They no longer went into a normal standing straight position.  That meant he could not wear his HKAFOs to use his stander.

What should have been quickly addressed with a tethered cord release was not.  He developed a sacral ulcer that same month that took 8 months to heal.  Until that was healed the surgeon could not do the tethered cord release.  8 months without standing or bearing any weight.  Then, once the detethering was complete and Mason healed we found that only one side of the body's contractures resolved themselves.  The right hip and foot needed tendons surgically released.  That happened this past August, at the 13 month mark of not being able to use equipment to stand.  That is over a year and in Mason's case that was enough time for the bone density to be lost and the bones to weaken.  Following that surgery he had the two month saga of casts and two broken legs from those casts. 

That brings us to today!  It is a really great illustration of how complex God created our bodies and how changes in one area affect so many others. We have seen this in so many ways with Mason.  One area affects another and what seems to be a single skill or ability is actually the interplay of myriad body parts and systems.  Imbalance in one area dominoes out to affect others. 

There are other factors that can go into osteoporosis (which is what Mason's bone density issue is called).  A lack of certain vitamins or minerals can affect the body's ability to build strong bones.  For example the body needs calcium, potassium, and Vitamin D available to build and strengthen bones.  Which is why our nephrologist has done a full blood panel on Mason to check his vitamin and mineral levels as well.  We should get those results next week. 

Thank you Anonymous for the question!

Mason's First Bone Clinic - We have a plan!

 

Today Mason had his very first visit to Bone Clinic.  He brought Curious George along for the ride.  We met Dr. A and discussed Mason's history and his bone density scan results.  We talked about treatment options based on that.  Mason is going to begin IV infusions of Zoledronic Acid.  The schedule is once every 3 months for the first year.  After that we will check his DEXA (bone scan) results again to see how effective treatment has been and decide if we want to space out the treatments or keep the same frequency. 

The very first IV infusion will be an inpatient procedure.  Mason will stay the night at the hospital to be monitored for any adverse reactions to a 1/4 dose.  If all goes well then his future infusions will gradually increase the dose and will be done as outpatient procedures, a several hour visit hooked up to an IV instead of an overnight stay.  We will be scheduling this for November.

Before we begin the infusions we need to check Mason's vitamin and mineral levels.  His body needs healthy levels of calcium, phosphorus, and Vitamin D to use the Zoledronic Acid to it's best potential.  After his appointment in Nephrology's Bone Clinic we headed down to the lab for the blood draws.  Unfortunately they needed several vials of blood the the first vein blew out after one vial, so he got a second needle in his other arm.  That one blew out too, but we got just enough blood before it happened. Mason handled it like a champ, licking a sucker and telling them to hurry up so he could go home.  :) 

While it is another appointment, another medical issue, and more specialists to deal with we know that ultimately this will help strengthen Mason's bones.  That will hopefully minimize future broken bones!  We loved Dr. A, who worked very well with Mason and answered all my questions.  I am grateful for a caring specialist who is proactively working with us for Mason's health.