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!

Thursday, October 9, 2014

Happy Shuntiversary Mason!

In the Spina Bifida and Hydrocephalus world you celebrate some unusual holidays. Today is Mason's Shuntiversary!  One year ago today the shunt in Mason's brain was fixed for the sixth time.  Pictures from the next morning are here.  He was not even two years old and had gone through more shunt revisions than anyone close to his age that we knew.  Every shunt revision is a brain surgery. 

The shunt is simply tubing and a valve.  It's job is to siphon off the extra fluid from the ventricles pf the brain when the pressure gets to high, draining it down into Mason's abdomen where the fluid can be reabsorbed.  You see, Mason's brain isn't positioned normally in his skull.  It actually grew pulled toward the back, with the bottom of it down in his neck vertebrae.  His goes down to C3.  It gets in the way of the fluid that should be draining down the spinal cord.  This is called Chiari II Malformation.  Mason's Chiari is currently asymptomatic, not causing any symptoms, which is a huge blessing.  When children have problems with their Chiari they face things like breathing and swallowing issues, among other things.  (I know you're wondering if Mason could develop symptoms - yes.  It is possible.  We pray he never does, but as he grows there is a chance he could become symptomatic and need surgery to remove part of his skull and neck vertebrae.)

Back to the shunt - we're grateful for it. Without it Mason would die as the pressure built up in his skull and damaged his brain.  Today, for the first time, Mason has had a working shunt for an entire year!  Will this be the first October of his life to NOT have brain surgery?  I'm praying it is!

Wednesday, October 8, 2014

More Questions: Therapy and Educational Options

Time for another reader question to raise awareness for Spina Bifida! 

"What are your plans for school?  Will the school district help with Mason's therapies and equipment?"

This is something we've been talking about recently.  Mason receives his physical therapy through the Help Me Grow program right now.  Once a month they come to our home, show us what to work with Mason on, and answer our questions.  Then they leave us to do the day to day work ourselves.  When he turns 3 in January he will no longer be eligible for the program. 

We've had our very first discussions with Help Me Grow about transitioning Mason out of their program and more discussions are on the horizon.  They were shocked that we would not consider sending Mason to preschool so we could take advantage of free physical therapy and get him started on academics.  To be honest, this did not surprise me.  In our area it is unusual for any child not to attend preschool, but more so if your child could be doing speech, physical, or occupational therapy.  The entire Help Me Grow program is structured to make a seamless transition for the child from their program into the public school preschool programs.  As a homeschooling family we have not chosen to send our children out to a preschool program or to a public school for their education. 

For Mason this means two things.  First, we will seek out private physical therapy when needed.  We could drop him off at the school for therapy each week but we would not be welcome to participate nor would the school want us there.  To them we are a distraction. We prefer to know the exercises and activities the therapist does so that we can include them in our daily routine at home. Which is more effective?  1-2 hours of physical therapy a week or a daily physical therapy routine?  Obviously, the consistent daily effort is more effective.  Our plan is to seek a physical therapy provider who will help us develop a daily program addressing Mason's needs, then evaluate and adapt it as needed.

Mason's needs change.  Right now he cannot climb up the stairs.  Two months of wearing casts and not being allowed to climb has left Mason weaker.  A hip surgery has left him needing to learn new ways to climb as his leg now works differently.  Once a week therapy is the least effective way for Mason to regain the strength he needs.  One hour a week of helping him with a new climbing pattern will be ineffective.  He needs a daily workout, encouragement, and help practicing new climbing patterns.  We can do that at home!  Putting him in preschool isn't effective as he would still only have one hour at most of physical therapy a week.  The rest of the time he would be restricted to his wheelchair to do 'school work', exactly the opposite of what his body needs.

The original questioner also wondered about equipment.  The school district would not help with the cost of any equipment for Mason. We have to pay for equipment ourselves if insurance does not cover it.

The second aspect of the school question is the academics.  If you've read my other blog, Our Busy Homeschool, you have seen the rich opportunity homeschooling offers.  I know that homeschooling is the best option for our family.  Learning is a natural part of life and we treat it as such.  We do not believe that the 'one size fits all' educational model in our country is as effective as the personalized tutorial education homeschooling offers.  I truly think each family needs to do what works for them - be that private, public, or home education.  I'm grateful to live in a country where each family has options in the educational arena and the freedom to pursue them! 

Have you been enjoying these posts where I answer reader questions? If you have any questions related to Spina Bifida and it's effects on the whole family just ask! 

Tuesday, October 7, 2014

How Does Mason Handle So Many Appointments?

Today's reader question for Spina Bifida Awareness month:

How does Mason do with all the doctor's visits and therapies? 

He's cheerful!  As a matter of fact I whine and complain more about it then he ever does.  The hour drive each way is my least favorite part of appointments.  Sitting in waiting rooms, dealing with medical professionals and having to explain his medical history yet again, having students or residents who think they know more than mom - it all drives me batty.  Mason wheels through it all with a smile on his face.

He is wary of strangers, especially if they speak loudly.  He isn't a fan of male doctors and nurses unless he knows them well.  This wariness spills over into everyday life.  If you see us out and about at church or elsewhere Mason will probably be silent and ignore your attempts to talk with him.  He'll wheel away if you come too close while you are talking to him.  It is quite a difference from life at home where he talks non-stop, asks questions, and tells people what to do.  As I type this he's saying the following to daddy (I'm typing his exact words):

"You use that screwdriver fix mouse?  Mommy, Daddy need that screwdriver fix his mouse.  What this?  Batteries?  I going to charge this.  Daddy we need this battery.  I can't open it.  We need scissors."

Seriously, the boy didn't say two words when he turned 2 years old in January and now he speaks paragraphs at a time.  But have a stranger talk to him and he'll clam up.  Once he gets comfortable he'll talk again but it takes a while and it helps if you talk quietly to him.

We try to do a few things to help smooth this medical appointments roller coaster. 
  1. Music and audio books in the van. Mason likes music and enjoys listening to stories.  He's easy in the van which is a total blessing.
  2. Things to look forward to.  We talk about what we'll do, who we'll see, or where we're going.  For example today was, "We're going to take pictures with your Curious George toy and get stickers."  That would be the x-ray for the bone density scan.  This Friday we'll talk about seeing Margaret, our nurse coordinator at Myelo Clinic, and walking to see the fish tank at the hospital. 
  3. Food and drinks.  We ALWAYS pack a cooler with snacks, a meal, and drinks.  Even a quick appointment of 45 minutes has us gone from home nearly 3 hours with the driving.  Our average appointment is 4.5 hours out of the house but the many cast change appointments in the last two months averaged 5.5 hours each.  Mason and Mommy bring food so we're always able to snack during long waits or on the drive home. 
  4. Paper, pen, and playdough.  We bring along a small baggie of things to do but these three almost always are Mason's top choices. 
  5. Kindle Fire with videos.  We have a Kindle Fire just for Mason.  He uses it each night during his hour long enema.  He also brings it along to appointments.  The only rule right now is he doesn't use it on the drive, it is used when we are actually at the appointment.  He occasionally paints on a free app, but usually he just turns on a video to watch. 
Will Mason always be this easy going about his many doctor's appointments?  We will have to wait and see!