What Does THAT Mean? ~ WAGR Wednesday

Image Courtesy of Zirconicusso/freedigitalphotos.net

Another Gorilla Mom (a mom of a child with WAGR Syndrome) and I were discussing the baffling or confusing terms that doctors may use when describing our children or a diagnosis concerning our children.  It led me to consider writing about my top 5 confusing terms that our healthcare providers may use concerning the health of our children or a particular diagnosis for them.  

1. His/Her vision will be (severely) compromised.
If your child is a non-communicative baby and you hear something like this, you have no clue what to expect.  However, from my experience with Evie, I see how well she functions even with 20/200 vision (legally blind).  She has adapted well to her surroundings and uses her vision, as it is, so I will often forget that she IS legally blind until I see her bump into something, or skip over something she may be trying to read.  
 
2.  The scan/ultra sound show the kidneys are unremarkable.
Is that good?  Is that bad?  I don't get it.  When we get a clean kidney scan I want to hear, "Everything looked great."  I want to spike the ball in the end zone and do a touchdown dance.  But, unremarkable is good; that means there is nothing to be seen that needs further investigation.  I will often just follow up with the doctor and say, "That's good?  Everything is clear?" just to calm my anxiety.  
 
3.  90th percentile.
Woo-hoo!  With my academic background, 90% was always AWESOME!  Not so much with the blood pressures or the BMI (Body Mass Indicator), though.  
Because of the increased risk of kidney failure in persons with WAGR/11p Deletion, we need to keep blood pressure and weight LOWER than might often be "acceptable."  Evie was once in the 99th percentile for BMI, and her BP was also in the 90th percentile.  
With the BMI, bumping over 99th percentile would have significantly increased her risk for developing diabetes; we had to take action to help her lose weight.  And, concerning the elevated blood pressure,  we needed to reduce the strain on the kidneys, so, according to our doctor at the NIH, we wanted to get her blood pressure to within the 50th percentile. 

4.  Kidney function is adequate/sufficient.  
Pardon me?  That does NOT sound good.  However, because Evie had part of her kidney removed, she would have less than 100% function anyway.  A person who has only one kidney will be at 50% kidney function, so it doesn't necessarily mean that kidney isn't working.   20% kidney function will start affecting the persons health, and 10-15% kidney function requires dialysis or transplant.  (WebMD)

5.  Elevated white blood cell counts.
I knew that when Evie was on chemo for Wilms Tumor, her white blood cell counts could drop and that would put her at risk for contracting illnesses because it would mean her immune system was suppressed.   However, the first time we went in to the clinic because of a fever while she was on chemo, they reported that her white blood cell count was elevated.  I was RELIEVED because I thought it meant that she still had a high immunity despite her chemo treatment.  I was wrong.  The elevated white blood cell count indicated that her body was FIGHTING an infection.  It turned out that, at that time, Evie had pneumonia, so her white blood cells were rallying and trying to fight it off. 

These are just five of the common (or most memorable for me) "ambiguous" terms that can affect a person with WAGR.  I'm sure there have been other terms that have confused me, but these just stand out as the ones that really caught me off guard.  

What ambiguous or confusing terms have you come across in your medical journey?

WAGR Wednesday ~ Why we have the BEST toys for playing Doctor!

As a family of a child with WAGR Syndrome, we seriously have the COOLEST toys for playing pretend doctor at our house.  Why are they so cool?  Because some of the equipment is actually real!  

We have two blood pressure monitors.  The first  is the kind you see in hospital rooms; big...really big.  That one stopped working about 6 months ago and we replaced it with a much more portable smaller machine.  We regularly are taking Evie's blood pressure and e-mailing the numbers to the nephrology (kidney) department at the Children's Hospital.

We have been dealing with Evie's high blood pressure since she was an infant.  She had part of her kidney removed when she was about 17 months old because of Wilms Tumor, which about 50% of the kids with WAGR will develop.  Whether it was the chemotherapy, the partial nephrectomy, or "just one of those things", we really don't know; but Evie's blood pressure sky-rocketed.

At that point, we had never met with nephrology; only oncology (cancer department) and urology.  However,one of the doctors who was supposed to sedate Evie for an MRI saw that her blood pressure was averaging around 140/120 and he refused to sedate her until someone from nephrology came to consult with us.  Especially considering that infants blood pressure should be lower than adults, and this isn't the blood pressure you want to see in an adult, we were pretty fortunate that the sedation doctor put his foot down.

Since then, I have had to kind of "put my cranky pants on" a couple of times with nephrology.  They have never really treated Evie's high blood pressure with the aggressiveness that I'd like to see.  People with WAGR have an increased risk for kidney failure down the road.  The higher a person's blood pressure, the greater strain is put on the kidneys.  It only seems logical to me that we would want to keep Evie's blood pressure at a controlled, and healthy range.

We've been through lots of medications, and seen or experienced a lot of side effects.  The four types of medications used to lower high blood pressure are:

• Calcium blockers
• Beta blockers
• ACE inhibitors 
• ARBs

The most visible side effect Evie had was to a calcium-blocker she was on as an infant.  The two side effects she experienced were her gum tissue growing over her teeth (you could barely see them coming in when she cut her baby teeth), and dry mouth.  If you've ever experienced dry mouth, you know it is intensely uncomfortable.  Dry mouth also can cause or make one susceptible to sores and ulcers in the mouth.  Evie's "skin" in her mouth would be so dry that she'd often have bleeding gums.  After several weeks of this (we didn't notice it right away because she was such a young baby) I asked to have Evie taken off of the calcium blocker.

The "non" visible  side effects include a raised creatinine level, too much protein in the blood, too much potassium in the blood (hyperkalemia), and too much calcium (hypercaldemia) in the blood.  At one point in time or another, we have seen these effects in Evie and have had to switch medications in order to eliminate the problem. 

Evie's also been on beta-blockers, ACE inhibitors and ARBs.  Actually, I think with the addition of her most recent medication, she's on all three of those right now.  (Grrrr...wish we didn't have to keep adding to her medication regimen.)

Blood pressure is such a frustration for me.  A year ago, the nephrologist had Evie wear a 24 hour monitoring cuff to see if we could identify any root-cause of the high blood pressure, but the results didn't yield any great "aha" information.  

We've added medication, taken away medications and switched medications; we've tried music therapy (which seemed to help for a while)...but we just can't get her blood pressure as low as we would hope to keep it in order to place as little strain on her kidneys as we can.

Low sodium diet?  Oh, don't worry...we are on that day and night.  We took her off school lunches and pack her lunch every day.  I don't bother buying many things that say, "just add water" because "just add water" usually means "here's your entire day's worth of sodium."  

A recommended sodium amount for an adult who needs to reduce his or her intake is 2 grams...an example of what that equates to would be two bowls of chicken noodle soup.  Here are some other things we've done to try to limit Evie's sodium intake:

• I have begun to shop for foods that have fewer than 100 mg. of sodium per serving.  
• I never add salt to anything, even if a recipe calls for it.
• I buy many things organic, like peanut butter, because I've found that much of the organic foods have lower sodium.
• I don't let labels fool me!  A label may say, "Low Sodium" or "Half the Sodium," but I still want to check the amount of sodium because I don't know to what they are "comparing" their measurement.

Just last night I was looking at Evie's blood pressure readings.  We had added a medication back in December because her blood pressure had crept back up, and the frustrating thing is that we are seeing the systolic number (the number on top) has only changed a little. 

Evie's blood pressure is a daily reminder to me that I am not in control, and that medicine can only do so much.  I try to remember to give it to the Lord who already knows the plans for Evie's life.

In the meantime, we'll continue to monitor, add whatever we need to in attempts to keep her blood pressure lower, and, of course, enjoy having some of the coolest "doctor" equipment right here in the comfort of our own home.