Big

We had our BIG urology appointment yesterday at Riley.  I told Owen "we were going to see Dr. Kaeffer the doctor who helps him take care of his bladder, to talk about how he could wear Thomas underpants."  He was excited about that but said "I don't want to go in the tunnel room."  Took me a minute.  But he meant the CT scanner.  Bless his heart.  It is interesting as he's getting older helping him understand all that he has to go through.  I assured him "he didn't have to go in the tunnel room.  This was a different doctor.  His shunt was just fine."  Bless his heart.

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The appointment went well.  We are so thankful for the doctor/surgeon we have there.  He is very kind, thorough and brilliant really.

Owen had a quick Renal Ultrasound (checking on his kidneys and bladder.)  He did great and got a Mater car.  He was beyond thrilled.

He also did great at FUds (Floroscopy Urodynamics).  Got to watch a movie.  He chose Kermit.  It gets pretty uncomfortable at the end when he bladder is filled to capacity.  Basically, they gradually fill the bladder with dye and take periodic x-rays.  The x-rays along with several other monitor sensors tell them things about the size, shape, thickness, activity of the bladder.  Pretty interesting actually. Almost peed all over me.  Glad that didn't happen!

He got another Cars car, Finn, for being brave here.   Let's just say thanks to the prizes today I'll be returning a Christmas present before it's even wrapped.  What happened to those cardboard kaleidoscopes?!

Then we spoke with Dr. Kaeffer.  It became pretty clear what we need to do.  I've been researching options and listening to others' stories for a long time now and kind of knew it was inevitable.  So it was good to hear him confirm that this really was the best option.

Here's the plan... Owen will have the MACE and the Mitrofanoff with a Bladder Augmentation and Bladder Neck Repair.   

I'll try to give you the layman's cliff notes version, minus the terribly drawn powerpoint we were privy to.  (get a graphic designer, Dr. K!)  Imagine little brown ovals of poop... really?

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The MACE - (the poop one) - Dr. Kaeffer will use Owen's appendix or part of his small intestine to make a little channel (like a straw) from his belly button (literally IN the middle of his belly button) to the top of his colon.  This will allow us to put a small catheter in the stoma (that's the name of the opening. you won't even see it in his belly button.)  and flush out his entire colon from the top down once a day.  

The MACE will keep him continent for stool.  Because of his spina bifida Owen has a neurogenic bowel - which just means the nerves don't work right.  Motility and function are different.  He can't sense when he needs to go and he has no ability to hold it in. 

"Why does he need the MACE if the cone enema is working so well?" I asked.  Answer:  The other part of this surgery (which I'll describe below) is 8-9 hours long with a 7-10 day hospital stay.  Yeah.  Adding the MACE to it only tacks on an hour. Recovery is the same.   Okay great, getting a two for one is still not a good enough reason for me.

The thing that convinced me was that while the cone enema (which obviously works from the bottom up and only cleans out the lower two-thirds of the colon) is working well now for a 30 pound 4 year old, that might not always be the case.  It may work great until he's say 13, has a growth spurt, experiences some Tethered Cord, loses some bowel function and suddenly he can't stay clean.  To go back in later and do the MACE would be an equally long surgery and involve forming more scar tissue and another 7-10 day hospital stay and long recovery. 

So we might as well do it now.  Don't have to use it, could still do the cone.  But why would you?  At least it will be done.  It also offers more independence and dignity to the process for him.

A little more info for those interested:  MACE is an acronym for Mitrofanoff for Antegrade Colonic Enema. Mitrofanoff is a French urologist who popularised an operation whereby the appendix was implanted into the bowel or bladder to create a one-way valve. Sometimes the "M" is used to represent Malone, who was the first surgeon to report this surgical approach to the management of constipation and faecal incontinence.

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The Mitrofanoff, Bladder Augmentation, and Urethral/Bladder Neck Repair.   

The Mitrofanoff - (the pee one) - is similar to the MACE in that Dr. Kaeffer will use Owen's appendix or part of his intestine to make a channel from his bladder to his lower abdomen (kind of below and to the right of his belly button.)  The Stoma, or opening on the skin, will look similar to his healed G-tube hole if you've seen that.  Owen will be able to cath through this opening instead of through his penis.  

Why?  Well, Owen leaks ALL THE TIME.  Because of his spina bifida he has a neurogenic bladder, which means the nerves don't work right causing his bladder to be thick and small - more like a leather bag than a thin, pliable balloon.  He can't feel when he needs to go nor control the flow.  

He leaks mostly because his bladder neck sphincters are loose.  This is the reason for the Urethra/Bladder Neck Repair where Dr. Kaeffer with stretch and narrow the Bladder Neck to the point where it will no longer allow urine to pass. You can also no longer catheterize through the urethra .It's the only way to keep him dry and also the reason he needs the Mitrofanoff to provide another way to remove the urine. 

Bladder Augmentation - This involves Dr. Kaeffer splitting Owen's bladder like a clam. Then removing a section of small intestine and opening it up to create a square (picture cutting a toilet paper roll open).  Then attaching this square patch to the open bladder - doubling its size.  Does that make sense?  

Basically, the augment solves the problem of a too small bladder.  It will decrease the pressures at which urine is held because there will be more room to store it.  This will be important since he won't be able to leak and we don't want pressures to get too high between caths.

As you can imagine it does mean we'll need to be more diligent about cathing in a timely manner.  Every 3 hours during the day.  Although I think the timing will have more to do with what his bladder will hold safely and how much he drinks.  What goes in must come out!

We asked if the stoma will leak. He said it shouldn't.  The channel actually is placed inside the bladder against the bladder wall and as the bladder fills it actually pinches the channel closed (like stepping on a garden hose).  Clever.

A little concerned about the change from a very lax cathing schedule to a more urgent situation, but it will be worth it for him to be dry and in big boy underpants.

Also, I didn't realize that after the surgery he should no longer need Miralax or Ditropan (or Detrol).

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Ditropan update:  They were totally fine with trying Detrol.  It does not cross the blood brain barrier.  "Why don't we start with that from the get go then?" I asked.  Answer: Insurance.  Yeah.  gag.  they bulk at the price and so want you to try the cloud inducing, over heating, agitation inducing, sleep affecting, appetite lessening Ditropan FIRST before they'll okay a switch. Nice.  

Now it's yet to be seen if Detrol will cause Owen no problems, but my advice if your kid is having adverse reactions to Ditropan.  talk to your doctor about it.  It as like they were just waiting for us to say something about it.  I don't think they would have brought it up. 

Owen has been off the Ditro for 3 weeks and it's like a cloud has lifted.  "Oh, there you are, Owen!"  He is so much happier, less agitated, less irrational, talks more, better concentration, hits rarely, get's self-control quicker when frustrated, sleeps better, eats better.  Not gonna say he's perfect now. And many of these behaviors though possibly influenced by Ditropan were learned habits that are not broken overnight.  It's subtle in many ways.  But very evident to us his parents nonetheless.  It's been great to find the Owen behind the Ditropan.  I absolutely hate that he's been on that since birth.

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Wow.  If you're still with me I am impressed. Back to the surgery... 

Bottom line.  This is major abdominal surgery.  9-10 hours in the OR.  We scheduled it for April 24.  He'll actually go in the 23rd to get his bowels cleaned out (that sounds like fun doesn't it?) and surgery will be the next day.  He'll be inpatient for 7-10 days.  Then have catheters in the stomas to help them heal for 6 weeks.  Way long right?  Dr. Kaeffer admitted he keeps them in longer than most, but has good results doing so.  

We chose April (even though he'll miss some school) so that he'll be healed up by summer.  Most people want it in June so they are ready for school in August, but that also means their summer is shot.  I'd rather do it now while we're in a good routine and be healed up for vacations and swimming.  No sense in ruining the other kids' summer.  Plus it's also a hard time to get scheduled around that time. This surgery is a full day's work after all.    We tried to get it during my Mom and Kim's spring break but Dr. Kaeffer is on a medical missions trip to Guatemala that week.

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It's the longest surgery he'll have had (9-10 hours).  I think his Tethered Cord/Decompression was 5-6.  I'm not as nervous about that as I probably should be.  

I'm relieved to have it scheduled.  To finally know that the end of diapers is in sight.  

I'm thankful that it was so clear what needs to happen.  

I'm thankful for a brilliant and kind surgeon who cares about Owen's whole person not just his medical issues.

OH!  I forgot to mention... he on the spot set us up to speak with two other families who had had these procedures immediately after our appointment!  Two girls, also with spina bifida.  One 15 and with a more severe case, and in a wheelchair.  She was adopted from Bulgaria, and her sister (also with SB) from Romania.  The other girl was 10 and wasn't wearing her AFO's (there were in the brace shop) but walked with a perfect gait. They said her lesion bubble (L3) covered her whole back!  astounding.    It was amazing to see a micro sample of the wide spectrum that is spina bifida right there before our eyes.   

It was very helpful to talk with them and their parents.  They showed Owen their stomas. He didn't seem to think this was strange at all.  And loves to meet other people with spina bifida.  It's like it tickles him that there's somebody else just like him.  I love that.

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It was a long day.  I'm glad it's over.  just relieved to have a plan.  after 5 years of wondering what that plan would be.

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And Wound Care for the Blister:  The nurse came and looked at his heal.  said it looked like it was on it's way to healing with a ways to go. Gave us some fancy silicone band-aids to wear around the clock.  Told us to wait until it was completely healed and to take a look at the braces again.  I'll be anxious to see what Ortho says at clinic the 20th.  My gut says it's his feet.  They are getting to be a mess.    So no braces for the foreseeable future.  Bummer too, because his new KAFO fitting is Tuesday.

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thanks for your prayers.  they were felt yesterday.  We do not take for granted the love lavished on Owen by our Heavenly Father through each of you.