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?"
