Click HERE for Jonah's Health History Part Two
Part One runs birth to Sept 2023/ Part Two starts Dec 2023
Since this is our digital scrapbook, I wanted to make this entry so if I ever can't remember when something happened, I have it organized in this post.
9-14-10
Jonah born.
9-17-10
Jonah discharged from hospital and goes to his foster home.
9-18 thru 10-12-10
We visit 2x/wk at the foster home. So hard to say good bye each visit and leave him.
9-30-10
Jonah sees cardiologist who explains Jonah has PFO: A patent foramen ovale (PFO) is a hole in the wall that that separates the heart’s two upper chambers (atria). All babies have this opening (called a foramen ovale) before birth to allow blood to bypass the lungs. Shortly after birth, the tissue usually grows together and closes the hole.
Cardiologist is quite sure this hole will close on its own and says we should have Jonah rechecked in 6 mths.
Teaser: hole does close on its own! I don't have the date of this 6mth followup, but that was the report we received at that appointment. Yeah!
9-30-10
Because of foster mom's concerns about Jonah gasping/choking while eating and being very gurgly following feeding, we take Jonah to
ENT #1 who diagnoses Jonah with GERD and Laryngomalacia:
congenital softening of the tissues of the larynx (voice box) above the vocal cords. This is the most common cause of noisy breathing in infancy. The laryngeal structure is malformed and floppy, causing the tissues to fall over the airway opening and partially block it.
ENT#1 orders a speech consult and swallow study since Jonah is having difficulty eating.
As a result of this swallow study (10-27-10), ENT#1 recommends suggests Jonah have surgery right after Thanksgiving 2010 to have extra tissue removed and open his airway (supraglottoplasty).
10-12-10
Termination of Parental Rights Hearing. We bring home to our house that afternoon!
10-27-10
Jonah has renal ultrasound and sees urologist for the first time. This is because Jonah was diagnosed in utero with severe left kidney hydronephrosis and mild right kidney hydronephrosis:
swelling of a kidney due to a build-up of urine. It happens when urine cannot drain out from the kidney to the bladder from a blockage or obstruction.
For Jonah's first year, he will have regular ultrasounds and appointments with urologist to assess if the kidneys are going back to normal size on their own or if surgery will be needed.
Sometime in Oct/Nov 2010
We seek a second opinion about Jonah's throat surgery from ENT #2. He says to give Jonah more time for the Laryngomalacia to clear up on its own. Plan is to do another swallow study in December to assess if Jonah's feedings etc are going any better.
Jonah also begins with physical and speech therapy thru the Birth to 3 program for our county. I can't remember if occupational therapy starts right at this point or a bit later...
12-23-10
2nd swallow study shows improvement! Plan is to continue to hold off on the throat surgery.
3-11-11
Jonah gets pneumonia. We bring him into the ER at our local hospital and from there a Children's Hospital picks him up to bring him that hospital. Jonah was a very sick little baby. Inpatient stay from Mon-Sat. Start of daily inhalers and chest thumps.
This was very interesting timing in our life since I had just had surgery for my breast cancer 3 weeks prior. I was unable to lift Jonah yet at this time. Nurses at Children's were amazing as they had to give me extra help with caring for Jonah.
6-29-11
Jonah gets pneumonia again. This time we are able to have our inpatient stay (2-3 nights) at the local hospital since Jonah is not quite as sick but still needs IV antibiotics. This second bout necessitates a referral to pulmonology. This becomes a long relationship...
Pulmonologist diagnoses Jonah with reactive airway disease, recurrent aspiration pneumonia, and atelectasis of right lung.
Sometime in June 2011
Surgery for Jonah's kidney/bladder issues seems more and more imminent. Condition is not improving on its own. Urologist will need to reimplant the ureter to bladder connection in hopes of creating a wider opening to help with urine flow. Because I need my 2nd constructive surgery from breast cancer in August, we finally reveal to urologist what has been going on in our lives the past few months (surgery, recovery, and chemo). He is shocked that we are dealing with this. He assures us that Jonah's surgery can wait until after my surgery in August. So surgery scheduled for 9-25-11.
9-14-11
JONAH TURNS ONE!
JONAH TURNS ONE!
9-25-11
Surgery to reimplant ureter to bladder connection and to place stent in left ureter to stretch. Surgery takes from 8 am to 1 pm- longer than expected. Very nerve-wracking waiting, however, surgery deemed a success. Jonah has a 1 week inpatient stay to recover from this surgery.
Since Jonah was under general anesthesia anyway, the pulmonologist did a bronchial scope during surgery to check on Jonah's atelectasis.
Mid-October 2011
Post-op check with urologist. Everything looks great! Stent removal surgery planned for 11-11-10.
The day after this appointment, Jonah gets sick with a fever and throwing up. We suspect he may have the stomach flu. We call our pediatrician who says to monitor.
The next 2 days (Wed/Thur) Jonah does not throw up again but continues to have a fever.
Later Thursday, Jonah throws up again. We call pediatrician who is now concerned of a kidney infection and sends us to the local ER. Here Jonah is put on an antibiotic.
However, during the night, Jonah is very sick. We call the on call urologist at Children's in the early morning. He is very concerned and tells us to bring Jonah into the Children's ER asap.
Stephen and I end up taking him because Andrew couldn't get off of work.
Urology is very worried about Jonah getting sepsis. They are thinking that Jonah did not like the feel of the stent and so he start to hold peeing which then made the stent a breeding ground for bacteria.
Bacteria was very antibiotic resistant so Jonah was given some "big guns" antibiotics and doctor said to hope that they work in time...very scary!
1 week inpatient stay.
Stent removal still planned for 11-11-11.
11-10-11
Jonah gets the "flu" again, but this time we bring him right in to urologist. Another suspected kidney infection. Doctor decides to admit him for IV antibiotics and remove stent in a few days instead of the next day.
This is a different bacteria than 1st infection.
Interestingly enough, this 2nd infection comes on the day after the antibiotic from first infection was done.
Stent removed a few days later. Stayed one more night and then discharged.
What an ordeal!
Sometime January 2012
Jonah has his first ear infection.
Rest of Winter 2012
Just intermittent colds/coughs. Inhalers and chest thumping are done everyday no matter what to prevent a simple cold/cough from turning into pneumonia.
April 2012
Another ear infection, but this time Jonah has a severe allergic reaction to amoxycillin. Rash similar to Stephen's-Johnson Syndrome. Yikes!
May 2012
Jonah has nighttime fevers of 102 and hardly any sleep for any of us, but he seems fine during the day. Pediatrician can't figure out what is going on on because ears, chest, and urine look good.
End of May 2012
Finally an ear infection is diagnosed! This is actually good news because pediatrician was starting to worry about leukemia...
May 2012 through November 2012
Jonah suffers from 6 rounds of ear infections/antibiotics. The last round was Thanksgiving 2012.
9-14-12
JONAH TURNS TWO!
12-18-12
ENT#2 decides to have ear vents placed to help stop the constant ear infections. This is an outpatient surgery.
2013
Besides normal colds/coughs, Jonah remains relatively healthy for the next year!
3-5-13
Urologist says kidneys are back to normal size so we don't need to be seen every 6mths. We can now have yearly check ups!
9-14-13
JONAH TURNS THREE!
Sometime in 2012 or 2013
Sometime between and 2-3 years old I think, Jonah was fitted for orthotics. They started out as ankle and foot supports, but over the years, they have transitioned to foot supports only. He continues with these currently.
Jonah gets pneumonia, but we can treat at home with breathing treatments and daily visits to pediatrician for breathing/lung checks.
2014
Jonah continues to be monitored by pulmonologist, ENT, and urology.
4-3-14
Pulmonologist orders a sleep study. This is a common test for children with Down syndrome because they tend to have a higher risk of sleep apnea, but doctor also orders one because of Jonah's snoring/breathing issues while sleeping.
Good news is sleep study shows oxygen levels never go lower than 94 while he is sleeping. This means no sleep apnea.
9-14-14/15/16
JONAH TURNS FOUR, FIVE and SIX!
2014-2015-2016
Relatively uneventful for Jonah besides common colds/coughs etc.
He continues to be followed by urology (after 2016, we don't have to go for another 5 years!), and pulmonology.
I think at some point, ENT signs off on Jonah because ear infections are no longer a constant issue for him.
9-14-17
JONAH TURNS SEVEN!
October 2017
Jonah is diagnosed with Grave's Disease which is an over-active thyroid. This was found as a result of our pediatrician doing a routine thyroid check during Jonah's 7 year check up. Low thyroid is very common in individuals with DS which is why the doctor ordered this screening. High thyroid is more common among than DS population than typical population but is still pretty rare for DS. Of course, Jonah...
1st line of defense medicine for Grave's Disease is not tolerated well by Jonah. His white blood cell count goes too low which makes him susceptible to infections.
So now we have to use 2nd line of defense medicine for Grave's. This medicine is not one they like to use long-term with kids so doctor will use it for the next year, hoping that maybe Jonah's Grave's Disease will go into remission at some point in next 12 months.
11-2-17
Jonah has 2nd sleep done on recommendation of pulmonologist because of Jonah's snoring/breathing issues. This 2nd study shows Jonah does have mild sleep apnea. Recommended that Jonah go back to the ENT for evaluation for removing tonsils.
1-3-18
ENT#2 says that removing tonsils is not a definite yes or no decision. The decision is really up to us because Jonah's tonsil size is right in that gray area. They recommend using a nasal spray and then us doing a sleep diary over the next 30 days to see how is snoring/breathing is.
The nasal spray coincided with some really weird hyper behavior from Jonah so we stopped using that.
We did the sleep diary, but honestly the follow up with ENT did not happen as planned because treatment of Grave's disease sort of took over...
9-14-18
JONAH TURNS EIGHT
March 2019
Jonah's Grave's disease does NOT go into remission as hoped. Jonah's endocrinologist decides Jonah needs to stop taking the medicine because it has been long enough that he has been on this med.
Our next choice is to either radiate the thyroid to stop it from working or have surgery to remove the thyroid.
Doing nothing is not an option because an over-active thyroid can eventually cause heart and growth issues in children.
We decide to have the thyroid surgically removed.
5-7-19
Thyroid surgery. Surgery is successful except the night after surgery, Jonah starts wheezing and running a temperature of 102. Breathing treatments administered, and he is put on oxygen. Our 1 night stay now becomes 2 nights. Doctors think Jonah was very groggy after surgery and didn't get good enough breaths to help clear the anesthesia from his lungs.
The next day after surgery, Jonah starts to improve although he still needs oxygen support.
Finally, no more oxygen needed and we can go home!
Now Jonah will need to be treated for Low thyroid which is a much easier medicine to work with. This will be the rest of his life with continual blood draws to make sure he is at the right level of medicine.
9-4-19
Pulmonologist does Pulmonary Function Test to make sure Jonah is doing okay. Test comes back normal.
9-14-19
JONAH TURNS NINE!
Fall/Winter
2019-2020
Jonah suffers from constant sinus infections. Pulmonologist refers him back to ENT#2.
And in fact, in early February Jonah gets strep.
7 days into the antibiotic for strep, he started running a fever and his pulse ox was in the low 90's (We actually have an at-home pulse ox monitor we bought just for Jonah). We brought Jonah back to the pediatrician who listened to his lungs and said they sounded clear. The plan is to use albuterol every 4 hours.
Jonah continues over the next 2 weeks with cough, lower O2 levels, and on/off fevers.
Ben also gets a bad cold/cough during this time although he doesn't need any help from inhalers.
The discussion starts up again about removing tonsils. We are a bit hesitant to put Jonah through surgery again after what happened after the thyroid surgery with breathing issues.
Jonah is on antibiotics pretty constantly from December through January.
The plan is to reevaluate in March with ENT about whether we want to proceed with tonsil removal which can help alleviate sinus issues.
2-24-20
Pulmonologist orders another Pulmonary Function Test because of all of Jonah's issues this winter. This test is worse than test in September 2019. Doctor recommends we talk to ENT about removing tonsils.
March 2020-May 2020
COVID-19 Quarantine
Jonah's ENT appointment gets cancelled due to quarantine. However, when ENT office calls to reschedule I explain that Jonah has been completely healthy from sinus infections since staying home from school in March. We decide to put the follow up appointment on hold for now.
Pulmonologists states he feels Jonah could be at high risk for COVID19 due to his respiratory issues. He advises us to stay home as much as possible and to start up daily Flovent inhaler again. Previously we had hoped to try stopping this inhaler in the spring.
Early August 2020
Jonah starts to show symptoms of UTI however 2 cultures come back as not able to be determined. Since Jonah seems to feel fine, we decide to work on his constipation to see if that helps clear things up.
8-30-20
Jonah's UTI symptoms worsen- 2 days of a fever along with complaining of pain in left lower back. We take him in for another urine culture.
This comes back as a very rare bacteria not common in children- aerococcus urinea. The rarity of this prompts the pediatrician to contact the urologist which prompts Jonah to go to Children's for ultrasound.
Ultrasound shows stage 4 hydronephrosis (severe) of left kidney and stage 3 of the right kidney. Surgery scheduled for later this week to have stent and suprapubic catheter placed to try and reduce the dialation.
9-4-20
Surgery went good. No breathing issues from the anesthesia. 1 night stay.
9-8-20
Currently home getting use to catheter care. Only issue seems to be bladder spasms which are quite painful/uncomfortable, but hoping the new prescribed med will help.
Guessing our future holds regular ultrasounds to check on size of kidneys and then a plan to help Jonah void more efficiently so this doesn't happen again.
9-14-20
JONAH TURNS TEN!
September/October 2020
Recovery from 9-4 surgery did NOT go as planned. Jonah developed frequent catheter infections which caused him to feel awful. Also, the stent was very uncomfortable for Jonah. These were a rough 2 months for Jonah. Because of this, along with his increased risk for COVID complications, Jonah did virtual school during this time.
Finally, in early November, urologist put Jonah on a daily antibiotic to prevent the constant catheter infections. This helped Jonah to feel much better! We continued to keep Jonah home though because we wanted to keep him healthy for his 12-17-20 surgery to remove or replace stent and catheter.
Unfortunately Jonah not feeling well for Sept/Oct caused delays in transitioning Jonah off the suprapubic catheter and back to voiding urine on his own.
In fact, urologist decided to just keep Jonah on constant catheter drainage (no peeing) until after the 12-17 surgery. This caused Jonah to take many steps backward in the area of toilet training but were necessary to hopefully help his bladder recover.
10-24-20
Because Jonah had his thyroid removed in May 2019 due to Grave's Disease, he is now hyPOthyroid meaning he needs to thyroid supplements. His thyroid levels are checked every 6 months to make sure he is on the correct dose of levothyroxine.
At this checkup, Jonah's labs were quite confusing. His TSH level was HIGH which isn't unusual because this would just mean he needs a higher dose of his med. However, is T4 level was also HIGH which was weird. If TSH is high, then usually T4 is either low or normal. Endocrinologist chalked this confusing result up to a fluke and will look at things again in 6 months.
12-17-20
Jonah's surgery to replace or remove stent/catheter got postponed until 2-11-21 because 3 of us in the household got COVID. Hospital policy required this.
The good new from this was that Jonah did NOT catch COVID despite Andrew and I and Stephen having it. We tried our best to quarantine from him, but it's very hard to do that when Jonah needs much care and attention. And yet, Jonah did not catch it. This made us feel reassured for him eventually going back to school and being safe.
December/January 2020-2021
Last summer, but also several times before that, Jonah would sometimes be in incredible right knee pain if he would jump or move quickly. We decided to have him checked by his orthotics provider. He prescribed a knee brace for Jonah because his right knee has the tendency to slide/dislocate due to his low muscle tone.
2-11-21
Jonah's stent is removed! This is great news because it means his left kidney is adequately draining.
Unfortunately, the catheter could not be removed because Jonah is still not voiding efficiently on his own. Most of his urine output is done thru the catheter at this point yet.
1 week after replacing the catheter, Jonah's tube got caught on a cabinet handle and got pulled out. It had to be replaced again.
March 2021
Although we were hopeful once the stent was removed, Jonah would start peeing adequately on his own, it did not happen.
In fact, urologist says Jonah suffers from severe voiding dysfunction. This means the catheter needs to remain indefinitely.
Jonah is now going to get the following help to increase him voiding:
1. MRI to rule out spinal nerve damage (low chance)
2. Medication to relax peeing muscles
3. Targeted physical therapy for pelvic floor muscles
4. Counseling to help with behavioral issues contributing to this
3-30-21
Jonah's thyroid labs continue to be weird: high TSH and high T4????
The endocrinologist is suspecting that Jonah's immune system is having a weird reaction to the assay used by our local lab for the T4 test.
Sure enough, we redid Jonah's T4 lab at the Children's lab and result came back normal.
So from now on, Jonah's thyroid labs will have to be done at the Children's lab.
April 6, 2021
This MRI is a prudent step to take before we can explore the options to help treat his voiding dysfunction.
Mediation update: We have had a few days of better peeing from Jonah, but we still are draining about 2/3 urine compared to his 1/3 peeing.
April 7-25, 2021
Jonah is giving us some awesome peeing. We are so excited. The medication seems to really help!
Also, Jonah's MRI came back normal. No spinal/nerve damage. This is good news!
April 23, 2021
In the middle of night last Thursday, Jonah started with kidney infection symptoms: severe back and side pain and nausea and vomiting. We spent most of Friday in the ER so he could get IV fluids and antibiotics. Over the weekend, we started oral antibiotics at home for a 10 day course.
While it is very hard to see your child is pain and discomfort when they are feeling bad, we are very grateful that the cause of this kidney infection appears to be catheter related. Catheters are breeding grounds for bacteria so it is not unusual that an infection would happen.
these past 2 weeks, we have been draining the catheter less often which is requiring Jonah to have to void efficiently and safely. We thought he was giving us very good numbers, however, when he started to get sick, we were nervous this meant he was not handling voiding on his own. This would have made us very sad because it might have indicated that Jonah would need a catheter long-term.
But we are not sad because the doctor is very impressed with Jonah's voiding totals. Also the renal ultrasound done in the ER shows the left kidney is smaller in size than it was in October so it's actually improving. So the doctor thinks this kidney infection is just "bad luck" of getting bad bacteria in that catheter.
May 25, 2021
Jonah had his catheter removed today! After 9 months of dealing with it, we are done.
We continue with 2 hour pee schedule, Flomax med and daily antibiotic for another 3 months. Renal ultrasound and follow up appointment on 9-7-21.
June 23, 2021
Jonah diagnosed with Lymes disease after symptoms of fever, headache, neck pain, vomiting, and bullseye rash. 21 days of antibiotics prescribed. Doctors feel no lingering effects since it was caught early.
7-12-21
Jonah's thyroid labs come back at 44! Normal range should be .5 to 4.50. Needless to say his thyroid med is being increased, and he will be tested again in late August.
9-7-21
Jonah's 3 month follow-up appointment did not go as we hoped. Jonah's kidney is about the same size it was at the 4-23-21 ultrasound in the ER. Today's ultrasound of the baldder also showed Jonah is still not voiding as efficiently as he should.
Some new techniques to try and get him to pee more. Return in 3 months for another ultrasound.
9-14-21
JONAH TURNS ELEVEN!
JONAH TURNS ELEVEN!
10-11-21
Starting in Aug into Sept, we started to notice more aggressive and angry behavior from Jonah: defiance, rude, hitting, throwing, kicking. This is not normal for Jonah. His appetite and sleep stayed normal.
Starting the school year, this odd behavior continuned and was even noticed by teachers.
So on 9-15-21, we contacted his endocrinologist to inquire if the higher dose was causing Jonah's irritability. This is listed as a side effect of too high of dose of levothyroxine. The doctor order TSH labs. His TSH was <.10. Normal range should be .5 to 4.50. This showed the doctor that his medicine dosage was too high and so she reduced him to 88 mcg.
For his 10-11-21 6mth checkup, his labs were .53 which means he is in normal range.
But the even better news is Jonah's behavior has evened out since going on the 88 mcg instead of 100 mcg.
11-16-21
Jonah had his 11 year well-child appointment.
I don't usually include well-child checks in this history, but after the year Jonah has had, I felt it was significant!
Both boys are under 10 percentile for weight and height when compared to typical kids. Both boys are in the 30-50 percentile in weight and height when compared to other kiddos with DS. So they are much smaller than their fellow 5th graders at school.
C day but a good day.
It felt good to take Jonah to the doctor because he’s healthy and not because there’s a problem. The past 12 months were rough for Jonah, but here’s to a smooth road going forward!
Sometime 2020/2021
I can't remember if I mentioned this yet, but this past year we noticed 2 orthopedic issues with Jonah:
1. His right knee would give out on him every once in a while, causing him great pain.
2. His feet (especially his LEFT) turn in when walking.
The orthopedic therapist who handles the boys' shoe orthotics suggested a knee brace and a compression shirt to help with core tightening. We also started Jonah on physical therapy 1x/wk to help him with core strengthening and proper foot placement when walking.
12-7-21
Today's ultrasound shows little improvement from the September one which showed little improvement from the April one.
However, the doctor is hopeful. He sees improvement in today's ultrasound that the radiologist didn't indicate on his report.
It's his opinion that Jonah's body is just going to take more time to reduce the size of the left kidney.
So we continue on a 2 hour voiding schedule, continue on the medication to help with voiding muscle control, and continue with the daily antibiotic- for another 6 months.
The doctor is not having us come back in 3 months because he feels that it doesn't pay to check the kidney that soon since he feels this is going to be a slow process.
If at 6 months, there is still little to no improvement, then he might pursue a renal test that would check kidney flow.
Late Dec 2021/Early Jan 2022
Jonah started to show signs of a UTI. Ran a culture and sure enough! Antibiotics for 7 days. Hopefully doesn't keep happening.
Winter/Spring 2022
Jonah continues physical therapy 1x/week to improve his core strength and his quad muscles. If these muscles are strong, that will help keep his knee cap in place.
Unfortunately, more frequently, Jonah is having his knee cap slide. It is happening on both knees, and seems to be triggered by jumping or running downhill.
Spring 2022
This spring brought 6 month check ups for both boys with endocrinology (thyroid).
Jonah's dose of levothyroxine stayed the same, but Ben's dose had to be increased.
Here are some interesting growth stats for the boys.
Jonah (DOB 9-14-10)
October 2021
Weight: 62 lbs
Height: 4'3.38"
April 2022
Weight: 71 lb 6.9 oz
Height: 4'4.64"
Ben (DOB 6-21-10)
November 2021
Weight: 64 lbs
Height: 4'5.15"
May 2022
Weight: 70 lbs 1.7 oz
Height: 4'6.51"
So both boys gained 6-10 lbs in the past 6 months.
This is the first time that Jonah has weighed more than Ben!
Doctor was not concerned about weight gain at this point. However, weight is something we will have to closely monitor because individuals with Down syndrome can have problems with being overweight which puts them at higher risk for other conditions like diabetes etc.
If you look at the boys the percentiles though, they are still pretty "little" compared to their typically developing peers. And compared to other DS kiddos, Jonah is close to average weight and height. Ben is actually taller and skinnier compared to other kids with DS.
Jonah
Weight: 17% typical, 29% Down syndrome
Height: 3.5% typical, 41% Down syndrome
Ben
Weight: 10% typical, 20% Down syndrome
Height: 8% typical, 80% Down syndrome
Interesting...
June 2022
Today was Jonah's 6 month urology check up.
His last kidney/bladder ultrasound was in December 2021. At that time, the left kidney was slowing decreasing in size. The doctor was quite hopeful that if we waited for 6 months, we would see more decrease in that left kidney size.
Unfortunately, that is not what we saw in today's ultrasound.
Left kidney was 13.9 cm in Dec 2021. Today it was 15.6 cm. At it's largest back in Aug 2020 when all this trouble started, Jonah's left kidney was 17.3 cm.
Today the right kidney was also slightly larger from Dec 2021, 9.6 from 9.4. But the more concerning thing was the doctor could see some slight dialation in the right kidney which had been normal in December.
Jonah's bladder was also very distended. Normally someone Jonah's weight would be able to hold about 250 ccs in his bladder. Jonah's bladder today had over 500 ccs of urine.
Once again, Jonah is presenting a puzzle to the urologist.
What he thinks is happening is that Jonah doesn't adequately empty his bladder. This is happening because of one or both of these reasons:
1. Jonah doesn't know, doesn't try hard enough, or loses focuses and doesn't pee completely when he goes to the bathroom.
AND/OR
2. When a bladder is stretched out or distended, it can start to not empty efficiently. So Jonah may be doing things correctly, but his bladder may not be letting the urine out the way it should be because of being so big and loose.
Next thing, because Jonah is not adequately emptying his bladder, this causes pressure to rest against the ureter openings (right and left) up to the kidneys which is causing the kidneys to not be able to adequately drain down to the bladder. Jonah's left kidney is more susceptible to this pressure blockage because of that ureter is the one that needed to be reconnected when Jonah was 12 months old due to a congenital defect. There may be scar tissue from that surgery that is making this side "thicker" and more easily blocked.
If this were another child that the doctor was seeing this in today, he would probably recommend immediate surgery to go in and try to poke through and stretch that ureter to bladder connection. He would also insert a temporary stent until things could drain and get back to normal size. A suprapubic catheter might also be another option to add to the solution.
However, Jonah doesn't handle stents well. He is very sensitive to how it feels and tends to hold his urine even more when he has a stent placed. Both at 12 months old and then back in Fall 2020, he had several severe kidney infections because of holding urine because of the stent.
Jonah also had a very hard time weaning off the suprapubic catheter. What should have been a 2-4 week placement back in September 2020 ended up being 9 months!
So the doctor would really like to avoid a stent or catheter.
For now, he would like to have us double void Jonah every 2 hours. This means have him go, and then a few minutes later have him go again. We had been doing double voiding morning, noon, and bedtime. Now we have to do it every bathroom time.
This sounds a lot easier than it actually is. Jonah gets very defiant when you ask him to go again. We are opening ourselves up to many power struggles for the next two months. But if a "simple" bathroom technique can prevent surgery, we will do the best we can!
Another ultrasound on 8-8-22. If things are improved, then we are probably looking at long-term double voiding and long-term pull ups since there is on way we can ask him to try and keep underwear dry- that would be way too confusing for him when we are also needing him to pee!
If things are the same or worse, then it looks like some kind of surgical intervention is in the picture.
I would think at some point if things don't improve, another renal function test will have to be done because the doctor said he is concerned that this severe dialation on the left kidney will start to decrease it's function.
So as is typical for Jonah, he's not typical. He doesn't follow the textbook description or the normal trajectory of anything. Hence the reason for the puzzle joke in the title. However, nothing about today seemed very funny except the fact that Jonah is an awfully cute and sweet little fellow. And the good news he is feeling good, no kidney infections despite this severe dialation. Probably the daily antibiotic (yes, he's been on a daily antibiotic since November 2021) is helping him with that.
6-22-22
Jonah continues to have physical therapy 1x/week to improve his core and quad strength. This is to prevent his kneecaps from sliding out of place. The PT also works on teaching Jonah to walk with his feet pointing straight rather than inward. And how to jump properly so he lands with equal weight on both feet.
The PT advised us to keep a close eye on Jonah when he is jumping or running downhill because this is when the kneecaps tend to slip. She said we could probably stop with the knee brace because she feels like Jonah is getting stronger in his core/quads. Plus the kneecap slips have been less frequent.
Unfortunately, tonight Jonah took a bad fall. It happened while running UP a ramp to his LEFT kneecap. Remember, originally, we were seeing this problem on the right knee. Now it's happening pretty evenly on both sides.
This was the worst it's ever been for poor Jonah. Usually it takes him about 15 minutes to recover from the pain this slip causes. Tonight, he couldn't get over it. He didn't want to get back up and walk on the left leg. He ended up whimpering most the night during his sleep whenever he would reposition in bed. Then on Thursday morning, his left knee was very swollen. He would not walk at all on Thursday.
Fortunately, we were camping with friends who are physical therapists. The dad assessed Jonah for us and said it felt like the knee cap was in place so that is good news. He advised to let him rest and give him Advil to help with the swelling.
By Friday, he was limping around. Today he is walking less than limping so these are all good signs. The knee is still quite swollen though. He has physical therapy on Monday so we will have his PT assess it then.
We are thinking, however, that perhaps Jonah needs to see a orthopedic doctor at Children's because this is obviously something that is getting worse not better even with the strengthening work being done with him. We will see what the PT thinks and/or check with our pediatrician for his thoughts.
UPDATE ON JONAH'S KNEE:
Even though Jonah was walking better today than yesterday, his knee gave out on him once this morning and once this afternoon. This caused him great pain. Plus the swelling has not went down.
We decided that maybe we were doing more harm than good by letting him move around without knowing exactly what was going on with that knee. And we felt the knee needed to be wrapped or braced to prevent it from slipping out again. So we took Jonah to urgent care thru Children's Hospital.
Xray showed no fracture which is good. It did show the patella is to the side when it should be in the middle (subluxation). In order to know if anything is torn, Jonah would need an MRI. Urgent care consulted with ortho, and it was decided that Jonah would use a knee immobilizer and walker for the next few days as well as regular icing and advil. Hopefully this will help the swelling go down. Then next week we will check in with our pediatrician who will decide if ortho should be seen to evaluate for a tear and to discuss how to prevent this from happening again and again.
Update since 6-22-22 left knee injury:
Swelling has went down. However, his left knee has slid out of place several times in this past month. Also, his right knee also slid out of place just the other day too.
So this past month, we have been having Jonah wear almost full-time the Target-bought knee braces on both knees for whatever help they can provide. We are hopeful the orthopedic doctor will prescribe better braces for Jonah's knees.
On Sunday when Jonah's left knee slipped out, swelling came back.
Very glad we finally have our orthopedic appointment today!
My notes from our appointment today with orthopedic doctor:
8-8-22
His last ultrasound in June was not good. The doctor indicated if today's ultrasound was the same or worse, Jonah would most likely need surgery. So a lot was riding on today's results.
They were great! Jonah's right kidney is almost back to normal size (10 cm instead of the normal 9), and his left kidney went from 15.6 cm in June to 14.1 cm today. The doctor was very pleased with that reduction. Jonah is still considered to have severe hydronephrosis, but the doctor is very hopeful that Jonah's left kidney will continue to reduce in size. We return in 6 months for another renal ultrasound.
8-9-22
Jonah's MRI of his left knee came back looking good- no tears! That is great news.
The concern going forward is how to keep Jonah's kneecaps (both knees) from continuing to slip out of place. And of course, there is always the risk that if there is a dislocation, it will cause a tear.
So for now, here is the plan:
1. Wear knee-braces full-time.
2. Physical therapy with the hope of eventually strengthening Jonah's core and leg muscles to act like the knee brace and prevent dislocations.
However, given Jonah's loose muscle tone and shallow grooves for both knee caps, he might not be able to get strong enough to prevent dislocations without wearing braces.
12-20-22
Jonah has a double "blessing" of having extreme laxity on both knee caps plus he has shallow grooves on both knees. This makes him very prone to both knee caps sliding out of place which is very painful for him.
This summer, after his major fall in June, the orthopedic doctor suggested we really work on strengthening Jonah's core and leg muscles. The hope being this would keep his knee caps from slipping without the need of the braces.
What we have noticed over the past 5 months of this targeted PT is Jonah is very strong in his leg muscles and somewhat strong in his core. However, we are continuing to see him fall from his knee giving out, close to 1x week. His PT suggested we contact orthopedics to get an assessment for where we are at with Jonah given that PT is rather impressed with Jonah's strength at this point.
Interestingly enough, Jonah's knee hasn't given out at school even though I know the knee braces tend to slide down during the day. My only thought is Jonah doesn't move quite as much or as impulsively at school as he does at home where he is running and jumping and twisting and turning constantly :)
Our appointment yesterday was with an orthopedic surgeon to see what options there are for Jonah. Surgery at this point could be an option, but it doesn't hold the best results because Jonah also has the shallow grooves which often make surgery results unsuccessful. Also, even with the surgery, the doctor would still require Jonah to wear the braces full-time so that really doesn't get us ahead either. Plus the recovery for 1 knee surgery is 6mths, Jonah would need both knees. Plus, there is a chance, he would need more surgery once his growth plates close up in his later teens. At this point, we are choosing not to go the surgical route right now. Too hard on Jonah and not the greatest outlook for results. If Jonah's falls increase or get worse, we will reconsider.
So going forward, we know the knee braces are our best bet. I think the only take away for school regarding this issue is to make sure the braces are in place, especially when he goes off to recess or gym class. Not sure if braces are considered an IEP issue?
For PT going forward, our original goal was to help with Jonah walking with not walking with his feet pointing in. Then we added the core/leg strength to help prevent the knee caps slipping. Not sure where we are at with goals going forward or what we have to meet for BadgerCare/Forward Health to continue covering?
One thing the doctor pointed out yesterday is that the braces may not be a lifetime thing for Jonah because often as individuals with DS get older, their activity level goes down which puts less stress on the knee caps. Right now I have a hard time imagining Jonah not being active- haha! Plus it's kind of a double edged sword- less activity might be good for his knees, but high activity level is good for reducing the other health risks for DS like obesity and diabetes etc.
Oh well, as with everything with these boys, it's in God's hands. He'll give us the strength and wisdom to deal with whatever situation they present to us. I have to remind myself of that quite often because they can present some interesting challenges:)
Final take away from yesterday's appointment: the doctor doesn't think the compression vest is necessary at this point if PT is having good success with Jonah. So one less thing for Jonah to deal with wearing each day!
1-13-23
Today, finally, after countless ultrasounds, much medication, a 9 mth suprapubic catheter, several surgeries, and much pain, discomfort, and hard work for Jonah, there is finally no paw print in Jonah's left kidney.
Praise the Lord!
This means we do not need to be seen for a year by the urologist unless we suspect something is going wrong again. This also means we are going to try and stop the daily antibiotic that Jonah has been on since November 2020. Since his left kidney is back to normal and Jonah has been voiding very efficiently, the doctor feels it is safe to try and stop the antibiotic.
The only difficult piece of news today is that the doctor suspects Jonah will never be fully trained.
2-18-23
Jonah stopped his daily antibiotic for his kidney on 1-14-23. On that day, he started with a bad cold.
Between him and Ben, they have both struggled with cold symptoms for the past month. However, neither have not had a fever over the course these past 4 weeks.
On Saturday, though, Jonah started to complain of a very sore throat. Jonah has a high pain tolerance so when he says something hurts, that usually means it really hurts. We also noticed a white spot in the back of his throat. So off to urgent care we went where he was diagnosed with strep and a sinus infection. We decided to have Ben checked too, and it ends up he also has strep.
9-20-23
Jonah had strep throat in February 2023 as explained above. He also had strep throat in June 2023. Now he just finishing up a 10-day regimen of antibiotics after testing for strep on 9-20-23. The standard of care is after 3 "strikes" in short period of time, you get referred to ENT. Our ENT has since retired since last seeing him in May 2020. Jonah was dismissed from his care when his ear infections stopped when Jonah was home quite a bit because of Covid shut downs and then Jonah's kidney/bladder issues. So in December we see a new ENT.
October 2023
Jonah graduated from PT for his walking issues (turned in feet) and knee issues. Plan going forward is to encourage Jonah in the placement of his feet when walking, wearing orthotics, and wearing the knee brace.
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