Meet our new friends: MACE and Mitty

Many people have asked me about Jonas and how he is doing.  I know that the surgery he had in June is confusing and his current status is just not simple to explain.  So here's my attempt at summarizing the surgery  along with what led up to it and how his recovery is going.  I'll try to break it into categories so you can easily skip over certain parts and read what you might be interested in.

Medical Background:

Jonas has Spina Bifida and as a result has a neurogenic bladder and bowel.  Simply put, his nerves do not properly communicate with these organs causing a variety of difficulties.  We have catheterized Jonas every 3 waking hours since he was 2 days old.  Even with cathing him on a very ridgid scheudule, he still developed reflux into his kidneys and the beginnings of hydronephrosis (kidney damage) by the time he was 6 months old.  He was started on a medication to help relax his bladder and allow it to grow properly so that his urine wouldn't be forced - due to high pressures - up into his kidneys.  The medication worked well at reversing the hydronephrosis and so far his kidneys have been kept healthy.  His bladder health is on the fence - not the best and not the worst.

His bowels also do not receive the proper nerve communication resulting in either constipation, or the opposite, depending on the combination of medications we have him on to control this.  Needless to say, it is always changing and something we are always trying to stay a step ahead of.

Decision Process:

A few years ago Devin and I learned about a procedure called the MACE.  Instead of our standard protocol of giving Jonas a nightly cone enema to help him stay clean, this would be an enema from the top of his intestines down.  To read the details of the MACE, you can go here.  Essentially, the surgeon uses his appendix to create a tube from his upper intestine to the skin.  A stoma (or hole on the skin) is created through which we will insert a tube that will deliver the saline solution into his intestines.  This water will flush through and help him evacuate his bowels as he sits on the toilet each night. 

Why put him through the surgery for this?  There are two primary reasons we made this decision. First, we would love for Jonas to be "socially continent" meaning he can wear underwear and be accident free during the day.  As Jonas gets older this is especially important for his well being.  Being accident free is just not something we could achieve with only using the cone enema.  Second, the MACE enema would be feasible for Jonas to do independently when he is older while the cone enema would not be something he could easily do on his own. 

The surgeon explained that the ideal age to do this surgery is 6-8 years old for a variety of reasons.  After years of dealing with bowel issues, this seemed like the best option.  We knew that the healing process would be long so a summer surgery would be ideal. 

During the consult with the urologist where we were discussing the MACE procedure, he also brought up a similar bladder surgery.  There are many different names for this depending on small differences in the surgery but the one Jonas had is called the Mitrofanoff procedure.  For details on this, go here. 

The Mitrofanoff also uses the appendix to create a tube from the bladder to the navel.  This way, Jonas would be catheterized through his navel rather than his urethra.  Our surgeon, Dr. Siam Ottomasathein (Dr. Si) was very careful to explain that the natural way is BEST! Once we introduce this unnatural channel, we are also introducing a number of other potential problems.  After a 45 minute discussion where he sufficiently scared us, he said that even with the potential problems, he felt this was the best thing to do for Jonas.  Again, the primary reason being future independence and that his urethra is very difficult to cath due to a tight sphincter.  This is not something we see Jonas being able to do independently, especially considering his fine motor delays.  We also feel that as Jonas grows he will appreciate the privacy that will come from cathing through his navel rather than needing to pull down his pants - especially if he is requiring help at school or other settings.  We also learned if we wanted to do the bladder surgery, it is best done at the same time as the bowel surgery.  This meant we needed to decide on both, we couldn't put the bladder decision off until he was older.

Believe me, this was not an easy decision and one we thought and prayed about for months.  In April 2014 we felt good about our decision and met again with Dr. Si.  He drew this example on the board and helped us even better understand the surgery and decision process.  We were told that we would regret this surgery for the first month, but would never look back after that.  I appreciate honesty in situations like this and knew we needed to be mentally and emotionally prepared for a very long healing process.  Surgery on the bladder and  bowels is never easy and especially so when the nerves do not function well.

Ideally, he could use tissue from Jonas' bladder to create the bladder tube.  This would be our #1 preference.  If the bladder was not big enough or healthy enough, using the appendix would be option #2.  If there was not enough appendix, he would have to use some intestinal tissue.  We all wanted to avoid this option if at all possible but this was something the surgeon would decide on during surgery so we would just have to wait and see - not to mention trust Dr. Si to make the best decision possible during surgery.  I'll avoid details, but there are a variety of consequences related to each option.

Before leaving his office, surgery was scheduled for June 17.

The surgery:

Three days after our consult with Dr. Si, I received a huge envelope in the mail from his office.  It contained details regarding the two surgeries as well as instructions to prepare.  I sat there and read about the bowel clean out process and learned that Jonas would have to be on a clear liquid diet for 3 full days prior to surgery and be in the hospital the day before surgery to prep.  I know this sounds like a minor detail compared to everything else, but as I read that I started to cry - which would be the first of a few cries during this process. :)  I didn't want to create a negative approach to the surgery and considering Jonas' anxiety, I wasn't even planning on talking about the surgery until the day before the hospital.  He is not the type that deals better when we talk about things for a while to prepare him, that backfires with Jonas!!  But how else would I explain why he couldn't eat anything but clear liquids?  I knew this would not be easy and since Jonas doesn't even weigh 40 pounds, I also knew he'd miss those calories.

The morning of the clear liquid diet started out well.  I bought a variety of fun jello flavors and let the kids help make them.  Jello for breakfast? Everyone was all smiles. 

By lunch, Jonas was in tears.  It was not a fun three days, I'll leave it at that. My favorite part of the sample diet is how chicken broth, consomme and bouillon are listed as options.  I'm sorry but my 7 year old is not going to appreciate the culinary differences there.  Those are hardly different choices!

We checked into the hospital the morning of June 16th for a day of attempted distractions.  It was hard for Clara to say goodbye. 

We welcomed the visitors we had that day very much!  Devin's brother Jeff and his cute family came by as well as my parents and Clara and Nell.  We did our best to keep Jonas calm and happy while on his liquid diet for the third day!  He was done with jello for good by now!  The hospital toy room helped distract Jonas as he gave dad a shot and then flat ironed his hair. :)  Gotta look good in the hospital!

Clara was a fun visitor.  She did a great job cheering Jonas up.

Uncle Jeff, Aunt Melissa, Rex and Asher helped pass the time that first day.

 We also had one more final consult with Dr. Si this day and made sure everyone was on the same page and that we fully understood what would be taking place.  Dr. Si was awesome at explaining things to us and we felt as good as possible at this point.  Here was our basic overview.

This is how we fell asleep that night...

Tuesday morning we bathed Jonas and he was prepped for surgery.  Surgery started at 11:30.  We knew the surgery could vary in length depending on what option Dr. Si needed to use so we were prepared for anywhere from 3-5 hours.  Here is Jonas as we wheeled him down to the OR. :(

We had a few calls from the OR letting us know that he was stable and doing well but nothing to let us know how much longer.  Trying to be patient...

FINALLY at 6:30 Jonas was coming out of surgery.  I understand that many parents have dealt with longer surgeries - even Jonas has - but this felt very long as we wondered what Dr. Si would end up doing.  We really really wanted to avoid option #3 and were so anxious to find out!

Dr. Si came into the waiting room and asked us to join him in the consult room.  He told us that everything went well.  He also told us that he was able to use the appendix!  We were very happy to hear this.  He explained that he had a dream about Jonas. In the dream, he saw that Jonas had this awesome, healthy bladder and a huge, healthy appendix.  We sat on the edge of our seats wondering what he had actually found.  So I asked him, "Well, were they as healthy as your dream?".  His answer?  No!  He explained that his bladder is not as healthy as he hoped.   This could mean a bladder augmentation in the future - we REALLY REALLY do not want to face this!!!  I'll spare those details for now.  You're welcome.  He also said the appendix was big enough for the two surgeries but not as big as he hoped.  His appendix was just shy of 8 cm.  He needed 2 cm for the MACE leaving 5.75 cm for the Mitrofanoff.

He created the appendix tube for the Mitrofanoff and attached it to the best location on the back of Jonas' bladder but things did not go well.  He was unable to properly catheterize that channel and could not make it work.  So he had to undo that and reattach it to the front of his bladder.  Due to the length of the appendix, he was not able to attach the tube as low as he wanted but felt that it was low enough to prevent the pee from leaking out of the tube and out his navel.  At least with the tube attached to the front of Jonas' bladder he was able to successfully cath.  Dr. Si filled his bladder with 200mls and Jonas did not leak so he felt good about the overall outcome.  It may not have gone as smoothly as he wanted but it could have been much worse.

Just before finishing up with Dr. Si, Devin made some joke about how we would no longer need to worry about appendicitis.  Dr. Si shocked us by telling us that actually there have been cases of people still getting appendicitis!  WHAT?  I can just imagine taking Jonas to the local ER and trying to explain where his appendix is located and what function it is performing.  This surgery is not very common so I hope I never have to explain it in a moment of urgency.  Apparently, regardless of where the appendix is placed, it will always function as an appendix!  So fingers crossed tightly that appendicitis is NOT in Jonas' future!!

The recovery:

Jonas came out of anesthesia slowly and we were able to see him around 7:30. 

Once back in his room, we tried to settle in.  Jonas was a bit irritable and wanted to eat but was on clear liquids for at least another day.  Jonas has a funny memory and as soon as he started becoming conscious he was asking to hold his mandarin oranges.  These seriously became his security blanket at the hospital!  He is such a funny boy!  We were told he wouldn't feel like eating anything the night after surgery but here is a list of what he asked for:
1. Pop Pop (lollypop)
2. Popsicle
3. Apple Juice
4. His bag of mandarin oranges (he literally slept with them every night in the hospital...but never ate one)
5. My personal favorite - a cup of warm ice! He was insistent it was warm...

Now might be a good time to show a picture of our sleeping arrangements.  They were awful.  Devin and I pretty much rotated all night between our smaller-than-twin-bed and sleeping with Jonas.  Neither of us was willing to leave Jonas so we made the best of it.  Jonas really wanted to be cuddled anyway, so it worked out.

But don't feel too sorry for us...we always manage to make ourselves feel better with some good food.  A trip to Dodo's for a piece of Banana Cream pie helped cheer us up.

The next few days were difficult.  Jonas was in incredible pain and it was hard to watch.  Even with heavy pain meds, it hurt him so much to move.  We had to get him out of bed twice a day but he cried when we would move him which broke our hearts.  Again, the visitors we had were such a welcomed break and helped lift Jonas' spirits.  We felt so loved by our neighbors, friends and family.

 

A trip to the toy room or a group of visitors left Jonas exhausted...but it was well worth it!

Two days after surgery Jonas was allowed to eat solid foods again but the crazy thing was that he didn't have an appetite.  He didn't want to drink or eat much at all.  We were tasked with getting him to but we were not having much success. 

After another couple of days, the doctors decided that maybe he would eat better for us at home and so decided that he would heal best if discharged.  We were excited and terrified to bring him home (it reminded me of bringing him home from the NICU) and be responsible for his care. 

Jonas looks terrified at the thought of his parents being solely in charge!

Once home we were solely responsible for his "flushes" and the care of his tubes.  He had two tubes for us to take care of.

Jonas insisted he continue to sleep with his mandarin oranges.

First was his bladder tube which was stitched into his navel that was to stay in place for a month.  It was taped to his leg and hooked up to a bag that collected his pee. Jonas and Devin nicknamed it "yo pee bag".  This catheter was there to help hold the new stoma open and allow it to heal without being irritated by us cathing every three hours.  Not to mention, it could close up very quickly - like a newly pierced ear - so it needed the constant tube for at least a month.  We were given very strict instructions to not let this tube get ripped out!

The second tube was coming out of the MACE hole leading to the intestine.  It was also stitched in place.  This was capped at the end and we would uncap it each night and flush a water solution through it in hopes that it would push out his poop as he sat on the toilet.  We started with small amount of water and would eventually build up to 500mls.  This process caused Jonas immense pain.  I have only one other time seen Jonas hurting so badly as when we did his flushes.  It was awful and horrible!  The worst part was that nothing was coming out.  The water was going in with absolutely no results.  The pain from these flushes lasted for over an hour and was an awful way to end each day. 

After a few days of this unsuccessful flushing, we were told to bring Jonas back to the hospital.  He apparently had an intestinal blockage that was not allowing the water through.

It was June 24th and I was told to bring Jonas to the hospital for an x-ray and exam.  I was also told to bring an overnight bag in case he needed to be admitted.

I was so sad to be headed back to the hospital, especially since Clara's 10th birthday was the following morning and she and I both desperately wanted to be together for our traditional birthday morning activity.

Sure enough, the x-ray indicated he was blocked.  We were admitted and Jonas got an NG tube.  Apparently most kids get used to these and once they are in place are not bothered by them.  Jonas is not most kids.  With Sensory Processing Disorder, having something down his throat was just not something Jonas was going to "get used to".  It was another awful experience as I tried to help Jonas remain calm and as comfortable as possible.  Through this NG tube, Jonas was given Go-Litely.  It is a liquid that does not enter the bladder but somehow goes directly to the intestines and will help dissolve and clean out his intestines. 

 

We finally fell asleep late and after 1.5 liters of this being "ingested" the dam broke!  It was 4:00am and I will spare you the details.  Let's just say that it took three of us - me and two nurses - TWO FULL HOURS to clean him up.  It was the biggest mess you could ever imagine - believe me!  And to top it all off, we had to clean all the tubes, tape, sponges, etc. that was supposed to be kept in place and kept clean.  Remember that charge from Dr. Si for us to guard the tubes and keep them clean and in place?  I was pretty sure this did not come under the "clean" category. I had dealt with one post-surgery infection a few years ago and was desperate not to repeat it!

I spent the next few hours in constant vigilance as I tried to change each dirty diaper before it got on any more tubes, tape or sponges so we wouldn't have to change all of those again.  Removing the tape was very painful for Jonas - especially because there was so much of it and you had to try hard not to pull on the stitches as you did so. I honestly think I changed 40 diapers before it slowed down. 

Jonas is finally all "pooped out" and is peacefully sleeping after the ordeal!

I did Facetime with Clara to see her birthday morning but she had fallen off her new bike and hurt herself so she was on the couch crying.  I don't think her birthday was off to a good start for either of us! :(

Finally that afternoon we were discharged and were able to spend the evening with Clara.  I was completely exhausted but did my very best to make the remainder of her day fun.  I fell asleep that night on the couch watching her favorite movie together - Harry Potter.

From this point forward, the flushes went fairly well.  The worst part at this point was that the stitches holding the tubes in place caused a lot of pain every time they moved which was pretty much whenever Jonas moved.  It was hard to watch. I'm sorry if this picture is unpleasant, I don't mean to make anyone uncomfortable.

On the 4th of July, we had friends in town visiting and were headed out for the afternoon.  As Devin put Jonas in his carseat he felt something funny.  He lifted up his shirt to find the stitch holding his bladder tube had ripped out and the entire tube was out.  This was not a good thing!  I called the hospital and talked to the urologist on call.  Our surgeon was out of town for the weekend.  He told us we could either meet him in the ER and he would put it back in or we could try and insert it ourselves.  For any of you who know Devin - you know what he chose!  Of course he felt like putting it in himself.  To be fair, I doubt this urology resident had much experience with this anyway and inserting catheters is something we've done lots.  Dr. Devin was successful and we then prayed we could keep it taped in place with no stitch helping us out.    Here is the text message the resident sent once we let him know we had successfully put the tube back in:

"Great!  You are the first in line next time they'll be looking for a urologist here :-) I don't think there's anything else to do as long as the urine drains."

I don't think Devin is seriously considering a career change. :)

The next morning we woke up and the stitch holding the MACE tube had broke.  The tube was still in place so we taped it in place and prayed it would stay that way.  The urologist wanted the permanent piece put in but the radiologist who does this procedure would not do it until Jonas was 6 weeks post-op.  So I was on constant tape duty and it was very stressful! This lasted for about a week before I walked into Jonas' room one morning and the MACE tube was on the floor next to the bed where Jonas was asleep.  ARGHH! 

Not where this is supposed to be...

I called the hospital and they had us come in immediately.  He needed the more permanent tube and it wasn't something we could do. 

The permanent tube is called a Chait trapdoor.  It is a little white rectangle that is almost flush with Jonas' skin surface.  You open the door each flush and find a small hole to insert the tube in that will flush the water through.  This is something that will need to be replaced annually and can come out relatively easily so we are always watching that closely.  Here is a picture of the Chait.  The "pigtail" portion is internal.

With the Chait in place, we now had one tube to monitor.  On July 18th, we left our family reunion in Bear Lake to drive back to Salt Lake City and have Dr. Si remove the navel tube and teach us how to care for this new stoma on a regular basis.  Shorts didn't exactly fit over the pee bag so we improvised.  Since it could not get wet we did our best to still have fun.



The appointment went well and we were all happy to say goodbye to "yo pee bag".  Jonas was so excited to be tube-less!!!

Pee bag in the trash can!!

We hoped that with the initial month behind us and all tubes removed we were on the right road to a full recovery and use of the new MACE and Mitrofanoff.

How is Jonas doing now?:

I've been asked by many people how Jonas is doing now.  Here's my attempt at answering that question.  It is a very simple question without a simple answer.

The good news:  Jonas is feeling good, pain is gone and he is back to his happy and cheeky self.

The bad news with the MACE:  At least once a week - and often more frequently - when we do the flushes Jonas sits on the potty for 2 hours and nothing comes out.  It is frustrating and impossible to predict or explain.  This isn't really what we hoped for and not functional.  On top of that we are not "clean" for the expected 24 hours more days than not.  We met with Dr. Si on August 27 and he suggested we increase the fluid volume and hopefully that will solve the problems.  We really hope so!

The bad news with the Mitrofanoff:  This is currently our biggest stress.  The first couple of weeks after his navel tube came out went really well.  We catheterized him every 3 hours and everything seemed successful.  Then he started leaking urine out his navel.  Some days he doesn't leak anything at all.  Other days he leaks 6+ times a day.  That's a lot of laundry.  Thankfully his teachers at school are literal angels!  They are willing to deal with this at school without any complaint.  His leaking is not due to a full bladder volume - after a month of charting it I can honestly say there is no rhyme or reason to his leaking.  It seems that his physical positioning has more to do with it than anything - but keeping Jonas lying flat on his back all day isn't practical. 

This weekend has been particularly hard - he is repeatedly leaking less than an hour after we've cathed him.  I was hoping for some clear answers to this problem during our appointment this past week.  Instead, Dr. Si told us it could take up to a year to work this out and that he did not want to bring Jonas back into the OR yet.  He wants to give Jonas more time to heal.  We will see the surgeon again in 3 months and go from there.  I appreciate not wanting another surgery at this point and I hope we can avoid that!! I hope we can find an alternative solution.  For now, we are dealing with constant leaking and doing our best to stay positive.

We certainly did not put Jonas through this ordeal to have a worse quality of life.  I am hopeful that the complications will be worked out but for now it seems like we may have a long road ahead before we get to that point.

I realize this post in general has a very negative vibe to it.  I don't mean to make it seem like we are dwelling on the negative.  I also wanted to recount things as they were as much for us to look back on as anything.  I would be lying if I said it was a "good summer".  It was hard but Jonas can do hard things - and he does them very well.  Jonas amazes me and helps me realize what is really important in life.  I just don't know what I would do without him.  Look at this smile!  This was taken mid August as an example of how quickly he comes around and finds the great things in life.