Saturday, January 4, 2014

Et Cetera...Part I

We see and hear the term so often, ETC..., et cetera....  It means "and other things".  In our life, in our home, we have a LOT of et ceteras.  Rare disease will do that. You see, the 'WAGR' in  WAGR Syndrome (www.wagr.org) is pretty misleading.  Each of the letters stands for one of several "afflictions" that are common among people with WAGR.  

  • Wilms Tumor 
  • Aniridia
  • Genitourinary Abnormalities
  • Range of Developmental Delays

What "they" {they being whoever names diseases and syndromes) really need to do in the medical world is add the proverbial "ETC" onto the end of WAGR Syndrome...

I'm going to take the next week and write daily about the "Et Ceteras" of WAGR Syndrome.  I'll start with the actual letters first and then move into the "Et Ceteras" that many doctors don't recognize yet, but that the families with WAGR have identified through our networking with one another. 

First, "W"...Wilms Tumor.  At least 50% of people with WAGR Syndrome will develop Wilms Tumor (childhood kidney cancer.)  The cancer can occur quite early, so Evie started having ultra sounds at the age of 6 months.  She was diagnosed with Wilms Tumor already at age 15 months.   This shows why early diagnosis of WAGR is quite important; because early detection of Wilms is better for treatment. 

I'm glad, now, that Evie had Wilms so young.  I don't' want to ever have to tell her that she has cancer.  I don't ever want to try to explain to her how bad things like cancer are just "something" that she has to deal with.  But, it was also not easy having an infant with cancer.  An infant who stopped drinking after the second dose of chemo, and we didn't know why.  An infant who would cry in the middle of the night and we didn't know if it was constipation or just intense pain from the chemo. 

We were lucky, though.  Evie's Wilms was only on the left kidney.  But for some of the children it can occur on both kidneys, that's called Bilateral Wilms.  At first Evie's doctors wanted to remove the entire kidney, but there's also an increased risk for kidney failure in people with WAGR, so sparing as much kidney as possible is important.  Evie's doctors decided to do chemo first and then just remove the "tumors" and the cancer cells rather than remove the entire kidney.  Evie had 18 weeks of chemotherapy.  That's a lot less than other kids have to endure if the cancer is discovered in a more advanced stage; it's also much less than the children diagnosed 20 years ago had to endure.

The other tricky thing about these WAGR kidneys is that they develop nephrogenic rests.  These are things that look like tumors, but may not be cancerous.  HOWEVER, they can turn into cancer; so many doctors, when they see the nephrogenic rests will also treat the rests in a similar way to the actual tumors; with chemotherapy.  It's hard to mentally justify seeing your child go through chemotherapy when there's 'technically' not a cancer diagnosis; but that's a WAGR Et Cetera that comes with the territory. 

So, we have a lot of Wilms Champions in our support group.  Our kids always astound us with their strength, their resilience and their attitudes.  They usually handle Wilms Tumor better than many of us would handle a cold.  Their grace and perseverance keeps us going on even the most difficult days. 

And, that's the "W", in a nut shell.  If you'd like to read more from me about our personal journey through cancer, I had a Caringbridge site that I would update regularly when Evie was going through chemotherapy.  The web address for that site is www.caringbridge.org/visit/eviejo

Tomorrow I'll write a bit (keep in mind that I'm not a doctor or scientist, just a mom writing from my own basic understanding) about the "A" in WAGR...aniridia...or the absence of an iris in the eye. 


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