JONAH'S SEVERE VOIDING DYSFUNCTION DIAGNOSIS

 As I talk to physical therapists and have pysch consults for Jonah, I found I needed to put the history of Jonah's toilet training all in one place to make it easier for me to explain his situation.

JONAH'S SEVERE VOIDING DYSFUNCTION DIAGNOSIS

BACKGROUND INFORMATION

Before Jonah was born, he was diagnosed with a bladder/kidney defect (left kidney nephrosis) that would have to be surgically repaired when Jonah was one year old.  Since that surgery, we have had follow ups with the urologist to make sure the repair was still working.  All our follow ups have been great reports. After yearly checkups from 2012 thru 2016, since things were looking so good, our urologist said we didn't have to be seen for 5 years- 2021!

 

3 YEARS OLD (2013) TO SOMETIME IN 2018

 

We tried many methods of potty training.  We attended several seminars on potty draining a child with Down syndrome.  We read many books and articles about the same.  We even did 2 phone consults with an expert from Chicago, Alice Belgrade.  

None of this led to success.  Jonah would sometimes pee for us on demand, Jonah would sometimes initiate and pee on his own, but neither of these things were ever consistent.  And Jonah never kept his pull up dry during the day. 

 

Night-time pull ups were usually VERY wet in the morning. 

 

SOMETIME IN 2018 (Jonah 8 years old)

 

Jonah's special ed teacher offered to do a 3 day intensive toilet training  at school.  Her thought was to train Jonah in the setting he spends most of his time in and then hopefully he will transfer those skills to the home setting.

 

While she did not have total success as she hoped, she got Jonah the furthest he had ever been!  From age 8-10, Jonah would pee on demand, he would sometimes initiate, he would sometimes have a dry pull up for a 2-3 hour stretch during the day.   In regards to BMs, Jonah continued to make those in his in pull-up.  The teacher's focus during the 3 day intensive, was only on potty training.

 

 

SUMMER OF 2020

 

Since Jonah was peeing on demand and sometimes going BM in the toilet, our goal for the summer was going to get Jonah use to wearing underwear with a pad insert to protect against wetting.  At this point, Jonah was keeping most of his daytime pullups dry, with occasional drippage in between toileting.  We were excited about Jonah wearing underwear when he went back to school.

 

AUGUST 2020

 

We started to notice a very strong and bad odor to Jonah's pee. 

 

After 2 weeks, we took Jonah in for a urine culture to check for a bladder/UTI infection.  Both samples came back as contaminated, not a clean sample.  However, our pediatrician decided to focus on Jonah's constipation (which is always an issue for Jonah which is why he regularly uses Miralax), and see if that would help clear things up.  At this point Jonah was feeling good, not complaining of any pain, and was not running a fever.

Then on Sun, 8-23, he ran a low grade fever and seemed to be complaining of pain on his left side.

However, Monday through Wed, no fever and not much complaining of pain. He actually seemed fine.

Thursday he was very tired and just wanted to sit by me and cuddle.

Then Friday evening, he ran another low grade fever and seemed very uncomfortable.  

So Saturday morning we brought him in for another urine sample.  This one came back with protein, blood, and white blood cells in the urine-all signs of infection.  Ended up being aerococcus urinae bacteria which is very rare and seen usually only in elderly men with prostate or catheter issues.  Obviously Jonah doesn't fit this profile. 

The rarity of the bacteria caused our pediatrician to reach out to Jonah's urologist at the Children's Hospital.  

After a ultrasound and x ray at Children's, we found out Jonah's left kidney is severely enlarged and severe enlargement on the right also.  In other words, we are back where we were when Jonah was born, if not worse because BOTH kidneys are enlarged L

Now the million dollar questions start:  Did the repair stop working and cause the UTI infection?  Or did the UTI infection cause the kidney to back up and stop draining properly?  Why is Jonah holding his urine and not emptying his bladder completely?  Because of the UTI or because there is something not working between the bladder and the kidney?  

All questions that need to be answered. 

So that is why Jonah is having surgery on Friday.  Partly to have a camera go in and take pictures and investigate what is going on.  Partly to stretch the bladder to ureter connection and place a stent to make sure things drain properly.  And partly to insert a temporary catheter so that we have a plan B if Jonah doesn't void the way he should until the doctor knows what is exactly wrong.   

 

 9-4-20

Surgery a success!  Stent placed in left ureter to help drain that kidney.  Dr  thinks the right kidney will adequately drain on his own.  Suprapubic catheter placed as a safety measure until we know Jonah is adequately voiding on his own. 

 

SEPTEMTER 2020 thru OCTOBER 2020

 

Jonah experiences a lot of pain and discomfort from the stent and catheter.  Jonah  gets frequent infections in the catheter.   

We even brought him to the Children's ER one night because he was running a low grade fever and vomiting and in so much pain.  They tested his urine, but said it's common to have bacteria from the catheter.  No antibiotic prescribed.  Jonah sent home still in pain and holding a barf bag for the ride home.  Very frustrating.  Apparently antibiotics are only prescribed with catheter bacteria when there is a fever.  Jonah's fever was low grade and only lasted an hour.  Not enough to warrant more aggressive treatment.

 

 

NOVEMBER 2020

 

Finally after 2 months of us trying to tell the doctor how miserable this little boy was, the doctor put him on a daily long-term low dose antibiotic to prevent anymore infections.  Jonah starts to feel much better!  He does continue to complain of pain from the stent, but not nearly as much as during September/October.   

 

Suprapubic catheters need to be replaced every 6 months so surgery  is scheduled for 12-17.  During this surgery, the doctor will also decide whether he can remove the stent if he feels the left kidney is adequately draining again on its own.

 

The original plan after 2 week recovery from 9-4 surgery was that we would begin cycling with Jonah.  This means the catheter would be closed for 2 hours, allowing Jonah's bladder to fill with urine.  Then every two hours we would open the catheter and see how much urine Jonah built up during that 2 hours.  If Jonah ever asked to pee, we would let him but during this first phase the goal was simply to have his bladder get use to holding urine again.  

The next phase was requiring Jonah to try and pee every 2 hours.  After his pee attempt, we would then open catheter to see if any pee left.  Then the 2 hour increment would stretch to 3 hours and then 4 hours. 

 

Because of Jonah's pain and constant infections during Sept/Oct, the doctor decided to not cycle Jonah and let the catheter be open and draining 24/7 until his 12-17 surgery. 

 

While this made life more comfortable for Jonah, it also put a huge delay in the process needed to get Jonah off the catheter.   

 

DECEMBER 2020

 

Andrew, Stephen, and I get COVID.  The boys and Lydia, thankfully, do not get it. 

 

However, the 12-17 surgery has to be delayed 6 weeks because of COVID in our household.  This is Children's policy. 

 

Doctor has us continue with 24/7 drainage until his rescheduled surgery on 2-11-21, thus another delay in the cycling process.

 

 

2-11-21

 

Stent is able to be removed!  Catheter tube replaced.  Now hopefully Jonah can start the cycling process.

 

FEBRUARY 2021

 

Jonah hardly pees on his own as we start cycling.  Not giving us much hope that he can be safe without the catheter.

 

MARCH 2021

 

Doctor offers us some ideas to help Jonah pee better:

1. Flomax medication to help relax peeing muscles

2. Targeted physical therapy to help strengthen Jonah's pelvic floor muscles

3. Psych consult to start addressing behavioral issues (bad habits) that might be contributing to his difficulty peeing. 

 

SO FAR IN APRIL 2021

 

The Flomax medication seems to be improving things for Jonah.  He is peeing more, and Dr. is impressed with his pee log numbers so far.

 

Physical therapy starts 4-23, and psych consult is 4-20.

 

Also on 4-7 Jonah had a spinal MRI to make sure he did not have a tethered spinal cord that may be causing loss of sensation to pee.  This MRI came back normal so that rules out a physical cause for his voiding dysfunction.

TO NOTE:
There are some things about Jonah

that make diagnosing difficult:

 

1. Jonah does not present "textbook".  Often Jonah does the complete opposite of what the books say a person will experience.  This makes it hard for all of us, parents and doctors, to assess what is really going on with him.

 

2. Jonah has a very high pain threshold.  Jonah can act like he is fine and then all of a sudden present as very sick.  Then in a few hours or the next day, he will act normal again.  Very hard to assess what is truly going on.

 

3. Jonah has a goofball personality which means his primary goal in life is to have fun.  So he is not the most reliable source when we try to ask him what hurts or what is wrong.  Sometimes he just acts goofy instead of being serious about what is wrong.  Sometimes he likes to pretend something is wrong when it isn't.  

 

4. Jonah is nonverbal so he can't tell us what is going on.  He can gesture and sign and try to say some words to us, but we don't always understand.  If we ask him questions, we run up against point #3 which is his goofy unreliable personality.

 

5. Jonah has weird things happen to him medically.  This is not our first rodeo with Jonah: pneumonia, kidney infections, tooth absesses, strep, thyroid, respiratory issues, allergic reactions to meds, odd bloodwork etc.  As I often say to Jonah, "Do you look for trouble or does trouble find you, Jonah?"

 

 

 

 

Hard to believe it's August already.  Summer is slowly but surely winding down.

We started August by having Jonah wear underwear during the day instead of pull ups!  This is a major accomplishment!

We have been toilet training Jonah for the past 6 years.  Yes, you read that right- 6 years!  It has been a long frustrating process where we have tried every method and idea that was ever thought of for toilet training.  

What finally got us some success was a method Jonah's teacher used at school.  She did 3 intense days of toilet training and got Jonah to the point of going on demand with some self-initiating.  

Unfortunately Jonah still wets (usually slightly) between his "goes".  This is why wearing underwear has not been a possibility because of the constant clean up we would have to do.

However, this summer I came up with the idea of Jonah wearing underwear with an incontinence pad.  This way Jonah can be more like other boys his age by being underwear since the pads can be more discreet than pullups.

It's not the ultimate success I know, but to me it's a partial victory.  There have been many times in these past 6 years where I thought we would never even get to this point so I am celebrating!

It's been a real frustration for me because I feel so guilty like I am doing something terribly wrong that Jonah can't completely get toilet training.  I feel like it's my fault.  

However, after all the things I've tried and still not had complete success, I have to tell myself that perhaps Jonah just really has a difficult time completely understanding this process plus with his low muscle tone, I think he has a hard time controlling down there.  

On a different note, we went on a beautiful hike today at the state park nearby.  Ben was a determined hiker, staying on the path and having lots of energy to the very end of the .8 mile hike.  Jonah, on the other end, kept throwing himself to the ground acting out some movie in his head and kept saying he was tired and hot.  What a difference in personalities between these two!

Of interest in this next photo, many ash trees in our area have died due to the ash borer.  This photo shows how much of the state park is affected by this.  Look how many trees are dead.  The whole look of this state park will change as these trees come down.  We have an ash tree in our yard that is slowing dying from this.  Sad...

Emerald ash borer is an invasive wood-boring beetle that attacks all species of ash trees that exist in Wisconsin. ... In Wisconsin, the southeastern part of the state is generally infested.

I SHOULD HAVE EXPECTED THIS...

Jonah is the one with the underwear (it's clean, don't worry!) on his head.  

Words to describe our Jonah- lovable, silly, huggable, sweet, and quirky.  

So as I am going through the now 4 year process of toilet training Jonah, I am telling myself I should have expected this.  Jonah is his own little person, he doesn't always process or respond the way the experts tell me he should.  I knew training him would be more difficult because of his Down syndrome, but I didn't anticipate all the twists and turns the process would take.

Let me summarize the past 4 years of my attempts:

2-5 years old:  Jonah doesn't realize he is wet and/or dirty despite trying many different methods of trying to help him have this awareness.  However, these 3 years help teach him all the steps involved in going to the bathroom- walking to the bathroom, pulling pants down, sitting on toilet, pulling pants up, and washing hands.  

5 years old: Jonah often realizes he is wet and/or dirty and lets me know.  Continuing to expose him to the bathroom process.

6 years old: Jonah is starting to realize when he needs to go!!!  This is the beautiful stage of SELF-INITIATION which is really the ultimate goal of toilet training.  However, for Jonah, this doesn't automatically mean that he is successful in using the toilet.  At 6 years old, we are also dealing with a bit of attitude and not wanting to try new things so he often wants to continue the process in his diaper rather than make all the effort it takes to use the bathroom.  But at least we are continuing to move forward.

A wise person once told me that with Down syndrome, we should expect every skill takes double the time of a typically developing child.  This has pretty much been the case with our Jonah (except speech, we are quite delayed in that area).  If most children train between the ages of 2-3, then a child with Down syndrome will probably train between the ages of 4-6.  I read somewhere that using the bathroom is about a 27 step process.  Kiddos with DS often have to work hard to follow 2-3 step directions.  

All that to say, I am hoping we master toilet training sometime during this 6th year for Jonah.  

Now the reason I am sharing about our toilet training efforts with Jonah is because with Jonah, I should have expected a quirky twist to it.  Jonah, after all, is a crazy goofy little boy.  I mentioned that Jonah is beginning to realize when he has to go.  Well, Jonah is realizing this during the middle of the night!  Yes, you read that right, Jonah is most aware of needing to go potty at about 1-2 am.   He wakes up crying and calling my name and wants to sit on the potty and go.  How can I deny him when we are finally at the most important stage of self-initiation?  I just think its hilarious (yes, I do try and tell myself that as I am woken out of a deep sleep) that Jonah picks the middle of the night to do this.  Funny joke, God!  

I think what is really happening is that for Jonah to be aware he has to go, his bladder has to be really full.  This fullness is probably at its peak during the night.  Jonah goes to bed at 7 pm, often with a dry diaper from before supper even though we try to make him go before bed.  So after 5-6 hours of sleep, I think he wakes up with a very FULL sensation.  

Plus when you think about it, during the night, there are no visual distractions because it's dark, no people around except me, and it's quiet because everyone else is sleeping, and Jonah is in his bed so he is not distracted by playing and moving around.  Middle of the night gives him the ability to totally focus on just this one thing.  That is probably a lot easier for him than during the day when he is bombarded by distractions.  

So if this works, maybe I need to write a book about toilet training in the wee hours of the night- maybe that is how I will make my millions!

The reason I am not mentioning Ben in all this talk about the toilet training process is because Ben was toilet trained at the orphanage.  I think they start very young there- I am not sure why it clicked for Ben at an earlier age than Jonah.  Probably part of it his Ben's ability to understand the process better, but also the orphanage environment might have just forced this upon the little boy, whereas our family setting is not as regimented and demanding.