This is Claire's Story

Another “Allergy” Turns Into
- The Bumpy Road of Kidney Disease

By Heather Welzant

As I read through the personal stories on the NephCure Foundation web site, I draw an eerie sense of comfort from knowing others understand and have experienced what we are dealing with.  Our precious Claire presented so similarly to the other kids in these stories.  After starting her kindergarten year , Claire had a cough that would not go away .  Additionally, some mornings she would wake up with "puffy eyes."  The Navy Clinic pediatrician in Newport , RI diagnosed it as allergies and she went on Albuterol and inhalers .

While dealing with this we failed to notice the gradual weight gain and just before Memorial weekend in 2002 Claire's tummy seemed to "pop" out.  We thought perhaps she was going through a growth sprit and she would get taller to make the tummy disappear.

Then one day her puffy eyes did not go away and the Pediatrician had photographs taken and got Claire a referral for an allergist.  That turned out to be the least of our worries.

That evening Claire refused to sit down for dinner and my husband and I grew impatient with her ( which we regret now).  When we pressed her , Claire said it hurt to bend her legs.  Claire is very stoic about pain , so we were alarmed.  When I lifted up her pants to look at her legs , I was shocked.  She had an acute onset of edema that was painful to look at, so I can't even imagine how it must have felt.  Still, she barely complained!

I told my husband “this is it -- we’re going to the ER! ” Thank heavens for a terrific ER doctor who quickly did a urine test and found that Claire had a significant amount of protein in her urine.  Because he had a doctor friend who had Nephrotic Syndrome (Minimal Change Disease) as a child , he diagnosed it right away.  We were sent home to follow-up the next day with the Chief of Pediatric Nephrology (Dr. Brem) from Brown University , who was fantastic .  He prescribed Prednisone and Lasix  because our poor little girl had 12 extra pounds on her , which she peed it out in 4 days . After that she seemed to be back to normal, although she was a little gaunt -looking for a few weeks!

Just after her diagnosis, we had to move (we are a military family) to the Balt imore/Wash ington Area and we were able to get a referral to a top nephrologist at Johns Hopkins University in Baltimore , a world famous hospital.  We felt blessed.  Claire's case seemed to go well.  We did a very slow taper off the steroids and she stayed in remission a long time.  Her first year was a bit bumpy but manageable.  The second year was a breeze.  She may have relapsed twice as far as I can remember and she always responded within days to the steroids.

Year Three has been the worst!  We were lead to believe that in Minimal Change Disease each year gets better until the child eventually grows out of the disease.  Not so in Claire's case , which took us back. Claire became a more complicated case of Nephrotic Syndrome and she now is classified as a steroid dependent/frequent relapser with Minimal Change Disease.

It got so unmanageable she spent two full days in the ER with a low albumin and high BUN and all other labs out of whack.  They did not admit right away, but a few days later a repeat blood test at the doctor's office confirmed her albumin dropped significantly.  She ended up with a level of 1.1 (3.5 is normal ), her fluids were abnormal and her body would not absorb the oral steroids.  The result: Easter week 2005 in the hospital, where she had to have two albumin infusions as well as Lasix and IV steroids .   

It was not long after she was out of the hospital and on a taper schedule that she relapsed again.  It was time for a kidney biopsy.  My poor Claire was all prepped and just after I had to leave the OR she had a serious reaction to Ketamine.  Her heart rate soared and her blood pressure doubled.  They decided it was unsafe to do the procedure .  She was so confused and we felt so very sad for her!  Still, she did not cry and she did not complain -- she just had a lot of questions!  Who could blame her!  We had a lot of questions too!

So, now we sit and wait.  We wait for her to relapse again (and she will) and we will attempt a biopsy again.  The nephrologist assures us that it is so rare for kids such as Claire who are steroid dependent to have FSGS, but we will not be convinced until we have the biopsy results.

I want so much to do and learn more and be involved, but I feel so drained from the day to day uncertainty of what is happening.  We are sitting back and expecting our precious 8 year old to develop steroid toxicity, which means we must try other medication ( none of which seems very promising).  I've read other stories at this site to try and get some creative ideas.  I've listened to a few other parents and I've begun giving Claire the Aloe Vera Juice too.  I figure, it can't hurt.  It's too soon to tell if it is having a positive impact or not.  Yet, I pray that it will!

Our poor girl has gained a lot of weight from the prolonged use of steroids, she has acne and a "moon face."  She simply does not even look like herself.  She refuses to give in to it and we do notice a definite decrease in her activity level and energy.  None of this seems to bother her.  She is a straight "A" student despite missing a lot of school.  Claire simply shines from the inside out!  In fact, her God given name means "bright or illustrious" and she is!  She is a lover of Irish Dancing and plays the violin gracefully!  She is such a special gift from God with all kinds of unique quirks.  She has a head "tic" too, since birth.  It's actually more like a sway (picture Stevie Wonder).  All of this makes her so special and unique .  There is simply no one else on earth like her nor would we want there to be.

I am not sure what to expect or what we are waiting for, but that is the nature of the Nephrotic Syndrome beast.  That is what I call it now -- "NSB" for short, as it is truly a beast!  The kicker is that we chose for my husband to go back to a ship in the Coast Guard because Claire was doing so well (that was year Two).  He is currently deployed for 9 weeks.  Ironically, he was assigned the Coast Guard Cutter Harriet Lane and Claire is seen at the Harriet Lane Kidney Center at Johns Hopkins in Baltimore .  I think it must be prophetic!  


Heather Welzant - phwelzant@comcast.net.