A Bit of a Break

I took the boys on a road trip to Buffalo Children's yesterday, for Nolan's annual renal ultrasound. They've always been normal in the past, so we were happy to see another set of normal, healthy kidneys. After the ultrasound, there was a bit of a wait to get into the clinic at 1:00 to discuss the results and future plan for Nolan. Killing time in a children's hospital that lacks playing areas for outpatient testing is not an easy task - I managed to sneak back into the radiology waiting room and let the boys watch TV for about an hour, then we headed to the cafeteria for lunch.

WCHOB is a great hospital for inpatient stays or for day surgery patients, but it lacks amenities for kids undergoing outpatient testing. For the longest time, they didn't even have changing tables in the restrooms (thank goodness, they seem to have gotten a clue and added a few of them). In our early days there, I would have to change Nolan on the dirty tile floor of the bathroom.

The cafeteria, for instance, only offers 0.5 liter bottles of soda and juice: there is no juice box or milk carton to be seen. There aren't really any meal choices for kids, either, outside of pizza/fried chicken strips/onion rings. I sat the kids down with their lunches (definitely not reflux friendly) and vowed to BRING our lunches next time. Nolan, of course, immediately spilled his Hawaiian Punch all over the table and himself. Neither boy ate very much, and it was difficult to kill time with a wet and sticky three year old.

We headed up to the clinic a little early, since I have experience with this system. Our appointment was at 1:00pm, and I knew that everyone had an appointment for 1:00pm. Basically, they open up the window and it is a mad dash to sign your name to the list - the child is called based on the position they hold on the sign-in sheet. We joined two other families that were long-term patients: we were all in the know with regard to the madhouse that the urology clinic can be. We heard the shutter roll up, and we made our mad dash to sign in on the sheet. I felt a *little* guilty for not informing the new parents of this crazy system, but I wasn't waiting 3 hours to get in like last year.

We were the second family called in (I could hear the other parents in the waiting room beginning to murmur "wait, your appointment is at one o'clock? So is ours!"). Dr. W. brought in a resident and explained Nolan's case - he's apparently pretty famous since posterior urethral valves are fairly rare (1/8000 births). He's also famous because we caught his valves before they did any damage: since it was an "accidental" finding, we spared his bladder and kidneys. Usually, late-presenting boys are found when they go into kidney failure.

I was hoping we'd be discharged from urology this year, but apparently Nolan will be a lifelong patient. He probably won't have the negative consequences of having PUV's, but there is no guarantee that we won't see problems emerge as he grows. Since he is doing so amazingly well, however, we don't have to come back for two full years. Yippee! I am glad to catch a break with this system.

I also talked to the urologist about Matt's situation. Matt has been on the DDAVP for about 3-4 weeks, and we haven't seen much of an improvement. For about 2-3 days, we thought we had a dry Matt during the day, but he is just as wet as ever. The urologist explained that the DDAVP is not supposed to be used for daytime wetting. Since it is taken at night, the drug is entirely out of the system by the time the child wakes in the morning (it only lasts about 8 hours). We'll finish out this week on the drug, then request an appointment with Dr. W. for Matthew to find out why he can't achieve daytime continence. Fortunately, the investigation consists of an X-Ray and an ultrasound, which are easy, non-invasive tests.

A Busy Week Ahead

Nolan's preschool field trip to the Fire Station

This week is a little busier than most:

Monday: Matt's preschool, Nolan's speech therapy, and Matt's second preschool.

Tuesday: Drive to Buffalo for a hearing test, and to adjust Nolan's FM system. Matt's first soccer practice.

Wednesday: Matt's preschool, then drive to Erie for an ophthalmologist appointment (we get to find out if Nolan gets to rock the fashion world by sporting a patch and going pirate-style). Then Nolan has a soccer practice, and Matt has swimming lessons.

Thursday: Nolan's last day of preschool, and his "birthday" during class. I have a mothers of preschoolers steering meeting, and Matt has preschool.

Friday: Matt has an end-of-year picnic for pre-K at a local amusement park, so we will be spending the day having a ridiculous amount of fun in the sun!

I love the summer months, but June does get a little insane with the end-of-school parties/events, and then we have a bazillion doctor appointments for Nolan. He sees urology on the 16th (this is routine, and we are hopeful he will be discharged from this specialist). The sleep neurologist is scheduled for the 21st, and the upper GI is scheduled for the 27th. At least one trip a week to Buffalo Children's is on tap for the remainder of June: if only the drive were shorter!

The good news is that preschool ends on the 24th of June (for Matt), so July should ease up a bit with the schedule. I plan on spending a lot of time at the local parks, municipal swimming pools, and on "field trips" to the beach!

Another Trip to Buffalo

Nolan, panning for "gold"

Nolan's post-sleep study ENT appointment is today, and I have been on pins and needles regarding the outcome of the study. I really, really wish the pediatrician had released the report to me, so that we could review the results prior to the appointment. On the one hand, if he has passed the sleep study, then we could have been relieved of stress over a week ago. If he has failed it, then we could prepare appropriate questions prior to considering our options. Doctors have a lovely tendency to spring the results on parents, then shove surgical papers at them before the information has been fully digested.

Anyhow, Dennis and I are both going to this appointment, just in case he did fail the test. If he has apnea, we will have to make a decision about doing a revision supraglottoplasty. In addition, if his laryngomalacia has returned, then we know his reflux isn't under control with the medication.

I'm trying not to think about it too much, since we simply don't know the results yet - if he passed the sleep study, then all is well and we can relax. In fact, if he passes this one, I think I will throw a party. Everyone is invited!

Matt is still on the DDAVP, without any success at the 0.2mg/day dosage. We increase the dosage to two pills/night (0.4mg/day) this week. I am hopeful that we will see some improvement, so that we can avoid a trip to the pediatric urologist. Buffalo is a great city, but I'd like to go there for a reason other than doctor appointments, thank you very much.

We did get some nice, sunny weather this weekend and the boys were invited to a birthday party in Dry Gulch - a pretend mining town in our area. They had a blast, digging up gems and seashells. Matt won an agate slice during a game, which made his day. I think we have two budding geologists on our hands!

Post- Op Appointment

Mr. Nolan on the deck

We had a nice, uncomplicated visit with the urologist today, concluding our little encounter with posterior urethral valves. He confirmed that the VCUG was completely normal, and I reported that the post-operative period has been fairly uneventful. Except for a low-grade fever of about 100 degrees, but Nolan seems to almost always have that. We don't have to see the urologist again for an entire year, and when we do it will be for a bladder ultrasound. No big deal, and we'll just monitor the little guy over time to make sure his bladder doesn't show evidence of thickening (something that can happen with PUV boys). Nolan's in a really low risk category since he has no apparent kidney or bladder damage, and normal creatinine levels.

The only complicated thing is the travel. When Nolan was an infant, trips to Buffalo meant a screaming baby and a frustrated toddler. Then things got easy for a little while- Matt was old enough to entertain himself and Nolan was older and less fussy.

With a toilet trained 3 1/2 year old and an almost-2 year old, things have gotten "complicated" again. Just past Fredonia (and long past any bathrooms), Matthew announces he has to go pee pee. While I am thrilled that he is completely toilet trained, I wasn’t thrilled at the location of his urgent request. There is NOTHING between Fredonia and Cassadaga. A few minutes later. Matthew-with-the-yellow-eyes announces that perhaps he could go in the woods. I was tempted. The shoulders were narrow, the traffic fairly busy, and there was NO bathroom in sight. I told him to hold it “a little bit longer.”

We make it to Fredonia and the most blessed Sunoco on earth. I rush inside with Nolan (who has conveniently lost his shoes in the car) and a scissor-legged Matt. We got the key to the bathroom, and Matt walks inside to see a… URINAL. Wide eyed, he says (de sotto voice): “I want to pee in that.” Unfortunately, Nolan also spied it and managed to get his hands into it before I could stop him. ACK! I pull Nolan away and hold Matt up so that he can use the thing. Nolan thinks the “fountain” is funny and tries to stick his hands in the stream- EGADS! He has no ears on, of course, because I left them in the car in my haste. I don’t know what the Quickie Mart patrons could have been thinking, with me yelling, “Nolan! Don’t touch the PEE!” I get Matt cleaned up, wash Nolan’s hands in the dirty gas-station sink, and rush both boys back out to the car. Luckily I had some wet wipes in there.

I think I am going to invest in a little porta-potty. No one ever tells you about gas station bathrooms and toddlers before you have kids. Blech.

Hitting Little Snags

Nolan was doing so well the day after surgery and yesterday, that I thought we wouldn't have any of the expected complications from surgery. We haven't had anything major, but he is a little warm (99.6 degrees) and there is some bleeding and pain today. He isn't too bad, so we're going to attempt to get outside and play a little.

I do have to slather the kid in about 8,000 gallons of sunscreen, because his antibiotic (sulfamethoxazole with trimethoprim) causes sun sensitivity. It can also cause kidney stones, so he gets to drink as much diluted juice as he wants. Luckily we get to discontinue the antibiotics after this weekend.

I still don't have the results of the scintiscan, but I know the ENT's office has them. I called and inquired about them, and they told me that they would call me back with the results. No news is usually good news, though I wish the receptionist would just say, "the scan was normal."

Back to Normal

The Good News: Nolan is back to normal!!

The "Bad" News: Nolan is back to normal.

Next on the schedule: room clean up and reassembly time!

Medical Update

Nolan, the day before surgery

Nolan is sleeping off the morphine, so I have a minute to post a short update. He had his scintiscan on Friday for gastric reflux, and I don't know the results of that test yet. To my untrained eye, there was no gastric reflux, but we don't have the radiologist's report yet. I'm not sure where the rest of the failure-to-thrive investigation will lead, but I'm loathe to do any more testing. I'm really working hard on the "fatten up Nolan" campaign!

The cystoscopy was this morning. The urologist said he didn't expect to find any posterior urethral valves (PUVs), because they are so very rare (something like 1/8000 births). Not only are they rare, but they usually have very severe side effects early in development. Many babies don't survive the neonatal period with this particular problem.

The procedure (for no valves) was supposed to take 10 minutes. 45 minutes later.... the urologist came out. He took us into the conference room and explaned that Nolan had posterior urethral valves and they did have to remove them. The bladder and kidneys don't appear to be affected at all- we're extremely lucky. The VCUG also showed no urinary reflux, which means that his kidneys aren't in jeopardy from that particular problem.

We are dealing with some amount of pain- the morphine has mostly worn off and he's just on Tylenol from here on out. We're trying to get him to drink a lot to keep things dilute. Hopefully the worst of it is over, and he will be up and running around by tomorrow morning!

I hate this medical stuff. I'm so glad we're past this and it looks like we have the PUV's resolved before they could cause any damage to his bladder and kidneys. Here's to the end of medical appointments!

ENT Visit

We headed up to Buffalo for a routine ENT check-up. Kids with hearing loss see an ENT on a regular basis to make sure the middle ear is as healthy as it can be, since fluid or ear infections can make a sensorineural hearing loss worse.

The left ear has a patent tube and looks great- no fluid, no wax, no infection.She took a look in Nolan's ear and confirmed the right PE tube was out, and that there was a lot of wax in that ear. She did a tympanogram on the right ear and the eardrum is a little stiff. After further investigation, the stiffness is not caused by fluid. I'm guessing the bad infection/perforation and two sets of PE tubes may have caused some scar tissue- hopefully the "stiffness" isn't enough to affect the hearing in that ear. Still, I'm thrilled because no fluid means no third tube and no adenoidectomy.

I did mention the possible PUV's and scheduled cystoscopy to the doctor and nurse, and that I was still concerned about his lack of appreciable weight gain. She entered the information into her computer and did the usual "mm-hmm" response.

Then she went to clean out his right ear (this involves a papoose board and all sorts of unpleasant metal funnels in the ear). After doing this, she checked his records again and saw that the ENT office hasn't taken an official weight since October 2007. She told the nurse to get a weight on him, then set us up for a 4 month appointment.

The nurse took his weight, furrowed her brows, then sent us back to the exam room. The ENT came back in, took one look at his growth chart, then proceeded to freak out. In a very professional, medical manner, of course. Apparently, seeing a drop from the 60th% to the 3rd% line makes more of an impact than the mom telling the doctor that he's only 21 pounds at 21 months of age.

A yummy cookie. If only he'd actually eat the whole thing!

After asking us if the pediatrician had started up a fail-to-thrive evaluation (um, nope!) and having me fill out another symptomology sheet, she set us up for a feeding evaluation at children's and a scintiscan to check for gastric reflux. Apparently, pulling food out of your mouth, refusing food after the first two bites, spitting up occasionally at the age of 21 months, and being very texture sensitive are indicative of a feeding disorder.

They're sort of treating it as an emergency and have us in for the feeding evaluation on Tuesday. There is a part of me that finds this rather ironic- he's been technically in the "failure to thrive" camp for EIGHT months. I have mentioned the weight issues like, um, Every. Single. Time.

The ENT is a tad concerned that this bizarre constellation of symptoms might be indicative of a syndrome. We've been there, done that before. It's more likely just a bizarre constellation of symptoms. .

For now, we have a bazillion appointments at Children's for the reflux evaluation, feeding evaluation, and cystoscopy. Hopefully it is just reflux and a little Zantac will help him put on the weight.

Urology Consult

We visited the urologist yesterday and he told us that the blockage could be one of three things:

1. there is no blockage and the pediatrician and staff at our local hospital just couldn't place the catheter because they're not as experienced at it.
2. There is a congenital stricture (very rare)
3. He has PUV's.

Because options 2 and 3 are rather serious, we need to investigate. He also wants to complete a VCUG (which we weren't able to do before) becuase of the family history of urinary reflux.

He didn't try to place a catheter today because Nolan's been through so much already. He gave us two options for investigating the problem:

1. Do a VCUG while he is awake, and if they can't place the catheter then they'll call down urology. Then if urology can't place the catheter, we'll have to come back another day to do a procedure while he's put under anaesthesia.

2. Do a cytoscopy under sedation to view any possible problem. If a problem is found, they'll fix it right then. If no problem is found, they'll place a catheter under sedation and then wait for him to wake up, then do a VCUG.

We chose option 2, since we've tried option 1 at our local hospital and it didn't work out. He agreed this was probably the best course of action, and we will be scheduling surgery soon. Hopefully they won't find a problem, but even if they do they will release him the same day. So at least there is no overnight hospital stay. If they do find PUV's and ablate them, he'll have a catheter placed overnight and that will have to be removed the following day, but he'll still be allowed to come home. The nurse will call me next week to schedule the procedure.

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!

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.