Friday, December 5, 2014

New Equipment for Mason

Yesterday Mason finally got his bracing!  He now has his very first pair of AFOs (Ankle Foot Orthotics) as well as refitted HKAFOs (Hip Knee Ankle Foot Orthotics).  The AFOs are green with dinosaurs on them.  Their purpose is to help maintain Mason's heel tendons stretch, keeping them in a proper position so they hopefully do not tighten and need surgery again.  Right now he wears them for an hour a day, with the goal being to work up to most or all of his waking hours. 

The HKAFOs have a different purpose.  These Mason had a long time ago but became unable to use when his tethered spinal cord caused contractures to his hips and feet.  The surgery road to fix all of that took over a year and a half because of unexpected complications.  Now that things have stabilized we're working to get these fitting properly again.  Why?

Because Mason has no ability to bear weight with his legs.  The HKAFOs hold his legs in proper position for standing.  The benefits to standing upright include strengthening his leg bones (which already suffer from osteoporosis so this is very important) and improving his bowel function (which can use all the help it can get). There are some developmental benefits as well. 

The down side to the HKAFOs is that Mason has no balance because of his level of paralysis.  He literally needs someone holding on to him to keep him from tipping over at all times. 

Enter Mason's Rifton Dynamic Stander.  This wonderful piece of equipment has straps that keep Mason from tipping over while in his HKAFOs.  It also has wheels that offer him the mobility of a wheelchair while he gets in his time upright.  The goal is to get several hours upright every day.  Right now he does 10 minutes.  It seems like such a short time but we are needing to make more adjustments to the HKAFOs and 10 minutes is giving us a good look at places where the pressure isn't right so we know what adjustments to make.  Next week we go back to the orthotist for round 2 of HKAFO adjustments. 

All these explanations are great but if you're like me you want to SEE Mason using the stander, right?  This is from today:

Mason does not remember using a stander before, so I wasn't sure what he would think of it.  He loves it and asks to do it all day long.  It is really hard to tell him that he can't yet, because we're still working to get the perfect fit on his HKAFOs!  I also thought some of you would enjoy this video because you get to hear a good bit of Mason talking.  He's such a chatter box!  I love my little man!

Tuesday, December 2, 2014

Wheelies, Picking up Toys, and a Video

How about some Mason updates? 
My cutie pie is growing and learning new things on his own schedule.  The newest skill is likely to give me gray hairs - wheelies. 

That's right, Mason is a little daredevil.  We've raised his anti-tip bars to accommodate his wheelie fun so he can tip further back. 

While these pictures are cute, wouldn't you like to see a video?  Mason was happy to do one this morning.  Enjoy!

I also had someone ask about if Mason can pick things up from the floor when in his wheelchair.  The answer is it depends!  If he has his tray on the wheelchair he cannot reach anything on the floor.  However when the tray is off he can lean down to grab things so long as they are not flat on the floor.  So a toy car would be easier to pick up than a piece of paper. 

 He was feeling extra cooperative this morning when I got the camera out so I got a few pictures of him picking up toys from the floor. 

Here is the first toy pickup.  It was pretty easy, a Duplo tree that is a few inches tall.

The second was a Duplo train piece, shorter to the floor and therefore harder to reach.  As you can see, he is able to reach to the floor, thank goodness for seatbelts!  He uses his wheel or armrest with the hand not holding the toy to help sit back up because his core is not quite strong enough to do it without one hand helping.  We're working on those muscles!

Wednesday, November 26, 2014

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! 

Thursday, November 13, 2014

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!

Wednesday, October 29, 2014

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!

Tuesday, October 28, 2014

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. 

Monday, October 20, 2014

Bathroom Issues Part 1: We're talking pee!

It is still October and that means it is still Spina Bifida Awareness month! This week I'm sharing a bit about bathroom issues. Today we're talking pee!  Because Spina Bifida is a snowflake disability, where each person is affected uniquely, let me start by sharing how SB affects Mason's bladder.  He has what is called a neurogenic bladder.  In essence, his nerves do not send accurate signals to or from these areas. 

For the bladder this means frequent spasms that thicken the bladder wall making a small bladder capacity and risking urine refluxing backward into his kidneys.  This reflux can permanently damage the kidneys. (If your kidneys are too damaged you either need a transplant or you die.) Mason's body does not feel when he needs to pee and he has no control of the peeing process (either peeing on purpose or holding it to get to a bathroom).  This will not change as he grows up.

Right now Mason is on a bladder medication called Ditropan to stop the spasms.  It has some side effects, including dry mouth, lack of sweating, and easily overheating.  The medication paralyzes his bladder to stop the spasms, which keeps the wall from thickening. 

We need to catheterize him regularly to empty his bladder now.  Based on measuring the volume of urine he holds, pressure in the bladder at different volumes, etc, we currently cath Mason every four hours: 8am, 12pm, 4pm, 8pm, 12 midnight.  Mason gets a break from midnight to 8am (yes, we get up at midnight every single night to cath him.)  He usually stays pretty dry between cathing.  If he laughs a lot or cries a lot around the time he is due to be cathed he will leak.  He wears diapers and will continue for the foreseeable future. 

Mason has already begun to take an interest in taking over his own cathing.  Right now that means he likes to help hold the catheter once it is in place and he helps remove the catheter when the bladder is empty.  Around age 5 or 6 he should be able to cath from start to finish independently.

A Bit of Back Story:

I remember the first week home from the NICU with Mason.  Cathing was so hard that I cried nearly every time.  I had this squirmy baby who wanted to be anywhere but laying on his healing back surgery and head incision from his first shunt, I was recovering from my own major surgery and dealing with postpartum hormone changes, and cathing an uncircumcised little boy posed its own challenges. Doctors were not willing to consider circumcision until Mason was several months old, had been evaluated by the urologist, and was healed from the major surgeries he had already been through.  I remember crying and thinking I would never figure this out.  Now I can laugh at it.  A little practice and cathing became our new normal.  Not only can Daddy and I cath him, we've taught two other people as backup (Grandma and big sister Makayla). 

That's all for today!  If you have any questions related to Spina Bifida feel free to leave a comment and I might just feature it in a post this month!