WAGR Wednesday~Double Your Fun: Why Our Kids May Need a Longer, Stronger Dose of Antibiotics

There seems to be a recurrent theme in our WAGR support group regarding the length of time and strength of antibiotics that our children need to be on, as well as their amazing ability to catch EVERYTHING that's going around. 

Knock on wood, it seems that our own little zebra outgrew this tendency to catch everything around age 6 or 7; but those first few years, especially the ones after chemo were rough.

Evie seemed to have at least an ear infection, urinary tract infection or pneumonia every month for a while there; and that meant a LOT of antibiotics.  She quickly built up a tolerance to the antibiotics, so it seemed we had to go with the big guns; like augmentin, in order to kill the bacteria causing the infections.  Often, with her ear infections and UTIs, we would re-test/re-check for them before completing the first round of antibiotics to see if the infection had even cleared. There had been one too many times that we would finish the round of medication only to have a flare up again a few days later. 

For that reason, the doctors had to start just prescribing the medicine for a longer period of time!  So, rather than being on antibiotics for 7 days, it might be 14 days for Evie. 

What about stomach upset from antibiotics?  My best advice I can give you is to get a probiotic quickly and give it to your child the whole time you are giving the antibiotics.  We normally use a form of acidophilus called Floragen. 

Because antibiotics kill bacteria, it also kills the "good" bacteria that lines the stomach.  When that bacteria dies and is expelled, it can cause a sensitive, and very upset stomach. 

Typically the probiotics did the trick for us and we rarely had to switch or quit antibiotics because of any upset tummy issues.

While Evie didn't complain of pain very much, I would realize she had a possible ear infection or UTI when she would become cranky and/or lethargic.  Neither were characteristic of her, and I started to recognize the signs.  Often, Evie would be unable to sleep at night, or just restless.  By morning she would have a fever.  I could take her in to see the doctor and her ears would look fine, but the next day, the symptoms wouldn't pass, and we'd go back in and get diagnosed with an ear infection.  It was almost as though her body would warn her ahead of time that something wasn't right.  

Another "scary" sign that an infection was on the way took place when we'd take her out of the bath tub.  She would begin to shiver and shake in an uncontrollable way.  We thought they were seizures at first.  But, after I shared the information on our wonderful WAGR listserve, I heard back from several parents that their child would do the same thing just before spiking a fever.  "I'll bet Evie is coming down with something and you'll find out that she has an ear infection or UTI in a couple days."  

Sure enough, those smart Gorilla moms and dads were right. 

Evie has also developed several staph infections.  She had one on her face that looked like chicken pox or acne, she has had one that developed after she was stung by a bee, and she had one that developed on her arm; and while we couldn't determine how it "got there," it was the week following us being at camp, so I think she may have been bit by a mosquito or scraped her arm a little and the staph infection developed around that opening in the skin.  Staph infections are nothing to mess around with.  They will be red/purple/white and blotchy, and often they are hot to the touch.  They are extremely painful for a "normal" person, but Evie never complained of pain with ANY of hers!  

Something to check out would be if the possible staph infection is growing and how quickly.  I did this with Evie's last infection because it was like it just popped up out of nowhere; so I took a marker and traced around the red blotch.  The next day, when I saw that the red area had grown beyond the border that I had traced, AND was raised up as though it were swollen, I was able to take Evie to urgent care having already documented that it was growing and changing.  That helped the doctor diagnose it and treat it more efficiently.

So, if you feel as though your child with WAGR is ALWAYS sick ...it may be something that will pass with time.  If your child seems to always have a relapse of an ear infection, UTI or pneumonia, ask your doctor to increase the amount of time your child is on an antibiotic from the start of treatment, OR ask the doctor if you can schedule an appointment after being ON the antibiotic for a while, to check and make sure the infection is cleared up before quitting the antibiotic.  And, finally, don't think you're being too paranoid.  Hopefully you have a supportive clinic like the one we do here in town where they have no problem getting us in to have ears checked or to take a quick urine sample to ensure we aren't overlooking something. 

That pretty much sums up what I have to say about how we "double our pleasure and double our fun by having longer and stronger doses of antibiotics".  

• What has your experience been with your child?  
• Do the doctors ever resist your requests for such things?  
• Have you ever been made to feel like the paranoid parent?  

Share your experiences here if you are comfortable, and be sure to watch for next week's WAGR Wednesday~Mirror, Mirror.

Et Cetera~Part III

Here we are, we've made it halfway through the letters of WAGR, but there will be even more to come once we get through that.  I've told you about the "W" for Wilms Tumor, and the "A" for aniridia; today I'll tackle the "G", a commonly over-looked, misunderstood, but often quite complicated part of WAGR Syndrome, the genitourinary abnormalities.

The "G" manifests itself in different ways.  First though, I'll help you understand what "genitourinary" means.  According to the Children's Hospital of Pennsylvania , genitourinary "refers to the urinary and genital organs".  Since I don't have a boy, I'll just insert here that the ways the boys might be afflicted by the genitourinary abnormalities typically include undescended testicles, and hypospadias.  Both of these conditions usually require some sort of intervention from a medical professional, and that may include surgery.  Helpful links regarding these two conditions can be found at the International WAGR Syndrome Association's website. 

As far as girls with WAGR are concerned, here is what is documented on the IWSA website:

In a report of 7 girls with WAGR syndrome, it was noted that although each was genetically female (had two X chromosomes) and all had normal-appearing external genitals, they also had various abnormalities of the uterus, vagina, and/or ovaries.   5 of the girls had absent, small, or abnormal ovaries, and one girl had gonadoblastoma (a type of cancer that is more common in abnormal ovaries). 

I asked Evie's surgeon responsible for removing her Wilms Tumor to take a peek at her ovaries "while he was in there"...I mean, hey, let's take advantage of  the fact that we had her belly opened up, right?  He said Evie's ovaries appeared normal, of course she was only 16 months at the time, but that was still good news to hear.

Our biggest struggle in the "G" category would be with recurrent urinary tract infections (UTI).  Evie started getting them when she was on chemotherapy, and they just kept coming.  More often than not, we didn't even know she had a UTI.  Evie has a really high pain tolerance, and processes pain differently, so she would never complain of any discomfort.  That combined with the fact that she has a cool thermostat inside (her average temp is usually 96.8 rather than 98.6), she wouldn't even run a typical fever despite the infection.  The way we "caught" the majority of the infections was by us taking her in to the clinic for a bad cold, or for just a routine "pee in the cup" type of appointment (once every 3 months for quite a few years; now more like once ever 6 months).  If, however, the UTI came back after Evie had just finished a round of antibiotics, it would come back with vengeance.  That's when she would become lethargic and feverish.  There was actually a quarter at school, when Evie was in kindergarten, that she missed nearly as many days of school as she was present. 

Finally, after so many struggles and so much missed school, we asked that Evie be put on a prophylactic antibiotic.  That did the trick.  I think she has gotten 2 or 3 UTIs since going on the antibiotic so many years ago.  Prophylactic antibiotics are a lower dosage than a treatment strength, but help ward off those recurrent infections.  We had taken her off the prophylactic antibiotic for about a 6 month period to see how she did without it, and she got 2 UTIs within that time-frame, so we put her back on it.

Evie and Jenny with their canes at WAGR Weekend 2013

So, that's the "G" in WAGR.  Genitourinary problems, usually more easily diagnosed in the boys with WAGR, strike in several different ways.  It's another part of the et ceteras (all the rest) that come with genetic deletion on that 11p chromosome in the DNA of the body.  

Tomorrow I'll tell you about "R".  When WAGR originally showed up medical textbooks, maybe only 20-30 years ago now, the "R" stood for a term that isn't usually used in society anymore.  Since the movement to end the "R" word, we have come to refer to the "R" in WAGR as a Range of Developmental Delays that people with WAGR show.  

For those who haven't heard much about WAGR before, and for those who have but didn't understand,  thank you for reading these posts.   WAGR is so rare that there are only a few hundred cases WORLDWIDE.  I think it's so important to raise awareness of WAGR so we can ensure earlier diagnosis, preventative monitoring as necessary, and effective treatment.  The future for our loved ones with WAGR is so bright...