Appointment Scheduled with Children's

We saw Nolan's primary care physician today and talked with her about the urethral blockage.

We didn't have to ask for a referral to Children's, because she recommended it. The likeliest cause of the problem is posterior urethral valves (PUV), so we will be consulting with the pediatric urologist to confirm the cause and to treat the problem before it becomes a real problem. PUV is the leading cause of renal failure in children. Finding the blockage accidentally may well be the luckiest thing that ever happened in Nolan's life. We may be able to prevent permanent damage to his kidneys and bladder by treating this early.

Many kids with PUV are diagnosed prenatally, but others (with milder valves) aren't found until they are older. Sometimes, the only symptom is failure to thrive. The definitive test is a VCUG, which we can't do with Nolan (already tried that one)! The next step will likely involve a scoping procedure to visualize the blockage, under sedation.

As a side note, Nolan still has his rash and low-grade fever (we're over three weeks with it now), but seems to be acting fine so we're all assuming it is a persistent little virus that doesn't want to go away. He's actually eating a little better than typical, so that is encouraging.

We have our consult with the pediatric urologist on April 30, so we can enjoy our trip to California knowing that our little guy is going to be taken care of!

Visit to the Oral Deaf School

There is an amazing oral deaf preschool in Buffalo (affiliated with BHSC) and we visited their parent-infant program today. We won't be able to go very often, because it is four hours in the car for a one hour program! I was impressed with the activities they did, and the individual focus on each child.

The class started with some free play, so that the children could settle in. The group of children was then assembled in a circle, with individual monkey mats to sit on. They sang a hello song, mentioning each child by name. We read "Wheels on the Bus" while singing the song with the finger plays, and then we played a dance game.

The teacher turned on music, and the music stopped at random intervals. When the music stopped, everyone had to freeze. The little guys loved this and it really reinforced listening skills. Then they moved over to the tables for a craft.

They were given diluted glue and paint brushes to paint over a picture of a bus. Nolan absolutely loved this, and Matt did, too. Then the children placed yellow squares of tissue paper onto the bus. We used words like "push," "paint," "sticky," "wet," "square," etc.

The children then had a goldfish cracker snack and we returned to circle time to finish the hour. We played a ball game with a song, where a ball was rolled to each child and the child would roll it back. Everyone had to listen for their name and wait for their turn. The final event was the goodbye song.

The only problem was that my children did not want to leave! They loved the classroom so much that I had to drag Matthew and Nolan out. Matt is not hard of hearing, of course, but he served as a good language model. Nolan was able to impress everyone by saying "I want ball" and other three word sentences. To say that I'm proud of the little guy would be an understatement.

I only wish we lived closer to utilize this resource on a more regular basis!

The Plan

After doing some of our own research via webmd and other online sources, we think we have come up with a plan for securing appropriate medical services for Nolan. The urologist (who only treats adults) gave us no information, other than mentioning the obstruction might be PUV. His plan is to simply monitor bladder volume and do no other diagnostic testing. The research I've done shows that:

• Most boys with PUV are diagnosed in utero, but mild cases may not be discovered until early childhood with a weak urine stream and a failure to obtain control over bladder function (failed toilet training).
• If untreated, PUV causes a thickening of the bladder wall. In addition, it can cause urine to flow into the kidneys and can cause renal failure.
• All cases of urethral obstruction (in boys) are assumed to be PUV until proven otherwise.

Because the effects of PUV are so severe, even in mild cases, we must see a pediatric urologist to verify this is the cause of Nolan's problem. The diagnostic standard is a VCUG, the same test Nolan was originally scheduled for. Unfortunately, they won't be able to do this test because his blockage is too severe. Another method exists, which involves a scope under sedation. If he does have PUV, there is a simple surgical procedure to ablate the valves and prevent later complications (such as urinary incontinence and kidney failure).

The radiologist at our local hospital asked us if we had noticed a normal, strong urine stream. Which made me think: I have never seen him pee! With my older child, we had gotten a wet surprise during more than one diaper change. That's never happened with Nolan. I thought I was just really good at diaper changes, but it turns out that may have been the first symptom that something was amiss. We'll find out later, I guess.

We have an appointment with his pediatrician on the 30th to get shots and to request the referral to the pediatric urologist. The questions I have written down for her include:

• Are we absolutely sure there really is a blockage?
• How can we be sure without an appropriate scan?
• How likely is this to cause future problems?
• What problems can this cause?
• Wouldn't it be better to correct it now, rather than waiting until he's older?
• How will this affect toilet training?

While I've looked up the answers to many of these questions, I still want her input. And since we were not referred to the appropriate testing facility (the children's hospital), we have no scans which show the location and cause of the blockage.

In the meantime, we are reassuring ourselves with the fact that his kidneys are thus far unaffected.

What Do We Do?

Note to doctors: when a parent asks you "what should we do?" the correct answer is not "that is a philosophical question."

So, here's the rundown:

• Nolan's renal ultrasound is normal. His kidneys are healthy.

That's the good news. The bad news is:

• Experienced nurses could not get a catheter into his bladder.
• The radiologist couldn't get any imaging done.
• He has a urethral obstruction.

We were sent to a urologist (not a pediatric urologist) because of the blockage. We asked him what was going on, and he told us, "well, he has a blockage." Duh.

The doctor asked about how far in the blockage was. I told him about an inch, but we don't have any imaging to show the location or source of the problem. I asked him what could cause it, and he said that some boys have posterior urethral valve obstruction. We don't know if this is the cause of Nolan's issues or not.

We totally found this by accident, since we were screening for ureteral reflux because of my history and Nolan's low weight. Of course, we won't know if he has ureteral reflux because we can't test for it due to the obstruction. On the other hand, his kidneys don't show any signs of hydronephrosis yet, so we are just going to watch and wait.

The blockage is very real, but we don't know the severity. He still has wet diapers, so that is a good thing. On the other hand, they had no problem catheterizing him at the age of 8 weeks for a routine urinalysis (part of the standard congenital hearing loss work-up). So it is possible that the blockage has gotten worse as he's gotten older.

Our conversation with the urologist went as follows:

Us: What should we do?

Him: That's a philosophical question. What do you want to do?

Us: We don't know. What should we do?

Him: Well, you can do everything. Or nothing.

Us: If we do nothing, what can happen?

Him: He could get end stage renal disease.

Us: How likely is that?

Him: I don't know. What do you want to do?

Us: What do you recommend?

Him: That depends on what you want to do.

ARGHHHHH! I just want a concrete plan of action. I don't want to do needless tests, but I also don't want Nolan's health to be in jeopardy.

We settled on doing monthly bladder scans to make sure his bladder is emptying enough to prevent hydronephrosis and kidney damage. His bladder scan number today was 24. Anything above 50 is considered a problem. We also have to keep a sharp eye on him- if he stops wetting his diapers it is a medical emergency.

For the long term, I just don't know what to do. Do we request a referral to a pediatric urologist? Do we do a scan to find the cause, severity, and location of the problem (this is highly invasive under sedation)? I just wish the answers were more clear-cut. Mr. adult doctor wouldn't really give us his opinion.

We are going to talk to his pediatrician again, and I want to write down some questions to ask her. If you have any question suggestions, I'd be grateful. I wasn't expecting to find this problem, but now we have it and have to make decisions. And we just don't know what to do!

Adding Another Specialist to the Mix

Urology, here we come...

I'll update more later.

Relief

The second urine culture (taken in the doctor's office) has come back negative. This means a unrinary tract infection is not the source of Nolan's fever. We're on Day 11, this morning's temp was 100.1 again. He still has the funny rash, confined to his stomach and he's still not eating much. I really, really hope this fever disappears soon!

Nolan and the Terrible, No Good, Very Bad Day

We had to go back to the pediatrician for a catheterization to get a "clean" urine sample since the previous one was contaminated and showed positive growth.

Let's just say that it sucked. Two tries with two different catheters, and they couldn't get it inserted to collect the urine. They finally resorted to putting another bag on him. Hopefully this time the sample won't be contaminated and we'll get negative growth. Unfortunately, there was some trauma and the little guy is in some pain every time he needs to wet his diaper. Hopefully that will pass soon. It is not a good day today.

We also got his chem panel back today. The good news is that his BUN and Creatinine are normal. The bad news is that his liver enzyme (AST) and alkaline phosphatase are elevated (ALP). The pediatrician isn't quite sure of what this means, but will re-draw in a few months to see if they are still elevated or are at normal levels.

It's always something!

Positive Urine Culture

Poor little kiddo. His "bag" urine sample is positive for growth, so now we have to go for a more sterile method of collection at the pediatrician's office.

The good news is that his fever is gone (98.8 degrees) this morning. Hopefully the original sample was contaminated (likely) and we'll be free and clear after getting the results of the cath. sample.

Update: the fever isn't really gone. Our ear thermometer just sucks. He's still at 99.9 degrees. I'll update more later.

Doctor #3 Says...

Well, Drew's Mom came the closest with Nolan's funny rash and fever! She was closer than doctor #1 (who thought it was teething, possibly) and WAYYY closer than doctor #2 who thought it was bug bites and that 100 degrees wasn't a fever. It isn't Roseola, though- the fever was never high enough for that to be in the running. Doctor #3 believes that he has some virus (not sure which) and the rash is definitely viral. She couldn't believe that doctor #2 thought that this type of rash could possibly be due to insect bites (no inflammation, and only on the torso- apparently bug bites are more common on the extremities).

This makes me feel a WHOLE lot better, because a viral rash and fever makes sense. Random bug bites (about 50 in total) and an unrelated fever that won't go away do NOT make sense!

We are holding off on his vaccinations, though, until the fever resolves (we're on day 8) and until some additional lab work comes back clean. Somehow, my history of vesicoureteral reflux (VUR) didn't make it on to Nolan's medical history. Matt had a kidney ultrasound when he was born to screen for it, but Nolan has never had a kidney ultrasound.

Add in that he's below the 3% for weight (now a whopping 20 pounds, 13 ounces- the size of your average 9 month old) and has the hearing loss to boot... there are a few things that ring the "worry meter."

She drew another CBC today and the results indicate that there is something viral going on (normal WBC but elevated lymphocytes). In addition to that, she drew a chem panel to check his electrolytes, BUN, and creatinine levels. We expect these to be normal, but it doesn't hurt to be sure.

We're also doing some urine collection for a urinalysis. If it comes back clean, then that means no urinary tract infection. If it comes back positive, then we have to do a straight cath to be 100% sure of the source of infection. We should have the results by Friday (I'm taking the sample in tomorrow).

She also ordered a renal ultrasound, of the same type Matt received when he was a newborn. This should have been done on Nolan as well, but was missed from his work-up. Kidney ultrasounds are not that specific for VUR, however, so she wants to do a voiding cystourethrogram (VCUG).

I wouldn't want to go that far ordinarily (it's not a fun test), but it turns out that VUR is thought to be a dominant trait. Which means my kids have about a 50% chance of inheriting it. It has variable penetrance, though, so some kids show more symptoms than others. If Nolan's VCOG comes back positive for reflux, then Matt will have to have one, too. Hopefully it will be negative.

Other than his low weight, unrelentant fever, hearing loss, and funny rash, though- he looks great! He's in the 25% for height (31.5 inches) and is doing everything else he is supposed to be doing. When they asked if he had 4-12 words, I said, "Pshaw! Try around 50! And some 3 word sentences!"

He might be a little thing, but he's got a lot to say!

Fever: Day 8

He's running 100.1 this morning. Not high enough to be considered a real "fever," but nonetheless not normal. The good news: the rash on his belly is starting to disappear and hasn't spread. Doctor #2 might have been right about one thing- the rash and the fever may not have been related. If not, then what is causing the fever? For EIGHT days?

I'm probably a tad more stressed than I ought to be, but something similar happened to me when I was a kid. My mom was sent away from the pediatrician's office many times for an unspecified fever. She was finally taken seriously when I was in kindergarten and fell asleep. And wouldn't wake up. I was born with severe vesicoureteral reflux in both kidneys, which was surgically corrected when it was finally found. I'm not saying that I think Nolan has this (though it can be hereditary), but fevers with no known cause CAN be indicative of something more serious.

It's probably just a virus. But I'm taking a stand today. No well-baby shots until the fever/non-fever goes away or we find the cause. Wish me luck with doctor #3. Sigh...

So Totally Beyond Frustrated

I managed to get into the pediatrician's office today. To see doctor #2 regarding Nolan's unending fever, and the new funny rash.

We got to wait in the parking lot for our turn, because they wanted to keep us quarantined in case it was Chicken Pox. We were called in through a back entrance and put into an exam room. The nurse took a quick look and said it didn't look like Chicken Pox. She took his temp and his low-grade fever is still there. We're on Day Six of a 99-100 degree fever.

Then the doctor came in. He took a look and informed me that the rash was not due to a virus. He thinks it might be flea bites. Except they don't look like flea bites. But he's pretty sure they're not viral. Or bug bites. He just doesn't know.

I told him that sounded like a strange diagnosis, because they've been cropping up on Nolan's belly over the weekend and spreading beyond his torso. Oh, yeah- and he has a fever. THAT WON'T GO AWAY.

The doctor then informed me that 99-100 degrees is NOT a fever.

Really? Because doctor number one thought it was enough of a fever to do a WBC on the kid. I'm pretty sure there's a reason a LOW GRADE FEVER is called a fever.

I am so frustrated, and worried for my little guy. There are just so many unknowns that keep me awake at night lately.

Failure to grow, for no known reason

Hearing loss, for no known reason

And then you add in:

Fever, for no known reason

Rash, for no known reason

Yeah, I'm a little stressed out!

I just don't know what to do. Nolan is due for his immunizations on Wednesday, and if he still has this non-fever I don't want him to get the shots.

Could It Be?

Well, this might explain our unknown origin of the fever... (you might need to enlarge the photo go get a gander at the spots).

I'm not sure what kind of rash this is, but could it be Chicken Pox? Nolan was vaccinated against that disease at 12 months of age.

Now I just need an infectious disease expert to add to our portfolio. Sigh...

FUO

Well, the good news is that Nolan's ears are perfectly fine. And so are his throat, lungs, and nose. There is absolutely nothing wrong with him, except for a fever that will not go away. The official diagnosis? FUO- Fever of unknown origin. It sounds more like an alien disk descending from space than a medical issue, but there you go. So now we watch and wait, and hopefully the fever will disappear eventually. They did do a finger stick to see if his white blood cell count was up, and it's not. He's due for shots next Wednesday (well baby appointment) so hopefully the fever will be gone by then.

He's also up to 20 pounds, 10oz! We're almost at the 21 pound mark, so at least he's still gaining weight. This child seems to subsist on a diet of milk and three cheerios per day, but at least he's still gaining some weight.

Fever, Day Three

Nolan has had a low-grade fever since Tuesday evening. I managed to get into the pediatrician today- I hope it isn't another ear infection!

He has no other symptoms (other than not eating, but that's nothing new).

I want spring to hurry and get here so that we can get past the nasty season of infectious diseases!

Survey Says...

Jodi is fervently working to improve parental support for children who have a hearing loss detected through newborn screening programs. She has posted a short survey for parents of children with hearing loss. Please take a few minutes to help these wonderful advocates out!

http://www.surveymonkey.com/s.aspx?sm=5eHe_2bpFgBxwGqaf_2fisWQog_3d_3d

Earless

Notice anything missing? Nolan has been leaving his hearing aids in for no more than 15 minutes at a time. If I keep him extremely occupied, I might get an hour of hearing aid wearing. How frustrating is this behavior?

Imagine, if you will, hunting all over the grocery store/house/preschool/parking lot for two very tiny pieces of ear candy worth $3000. Now imagine doing this EVERY DAY. We do have a retention lanyard, but Nolan can disassemble the entire aid faster than I can blink, and the aids are removed from the string and thrown or hidden quickly.

At least he can't hide them well yet.

I keep telling myself it is just a phase. But it is a frustrating phase! I deal with things better when I have a plan-of-attack, so here's what I'm planning on doing:

• This will not become a power struggle. I dont' want his hearing aids viewed as a negative or as a source of tension. When he takes them out, I will wait a few minutes and then replace them without a word.


• I am taking his aids in for some maintenance because the right one sounds a tad "rough." I don't have a better description for the sound, but it is every-so-slightly coarse. There might be nothing wrong with it, but it won't hurt to have it checked out.


• We get new earmolds on the 13th, which might help with keeping his aids in.

I don't think we've had a change in hearing, since Nolan can still say all the Ling sounds back to me (with the exception of ooo and mmm, which he can't discriminate). He is still adding new words daily, though his articulation does give me some pause (daddy has turned into da-yee, coffee is boppy, diaper is bahpoo, cup is bup, etc). Because he has lost hearing since birth, the possibility of losing more is always in the back of my mind. If all else fails, we'll schedule another hearing test.

Fun at "School"

Just a few random pictures from speech therapy today. Speech "therapy" is all play- the work is hidden within all the fun!

Why I honestly believe our SLP should be paid more- she has to handle two at once!