Friday, July 29, 2016

Week 2

Last night, very, very late, we got home from two long days at the University of Utah hospital with Cardiology/Pulmonology.

15 hours in clinic
4 CT Scans
6 Pulmonary Function Tests
7 IV's
3 VO2 Stress Tests
3 Echocardiograms
3 Electrocardiograms

...half a dozen or so little melt downs of frayed nerves.
These hospitals are beautiful...I'm glad they are at least nice places to spend two days. 


We got some good news but we mostly got the news that at least two of the kids are advancing in their lung problems.

It's so very complicated.  I suppose the reality came slapping back in our faces that the best doctors in this country have never treated such a rare disease so all they have to base their exam on, is the normal, healthy-ish public or even the yardstick of the common diseases they deal with on a daily basis.

Let's start one by one...

Shelbie- There has definitely been an increase in growth of her granulomas in her lungs and Pulmonary Arterior Venous Malformations that are scattered about.  Sadly, there are many AVM's that are too small to fix but big enough to cause problems.  Still no word on the pulmonary embolism.  There was an error in the ordering of the CT scans and the first round was done without contrast so they couldn't get a good look.  Shelbie did a second CT scan late on Wednesday afternoon, but as of yesterday afternoon, the results still weren't back.

Her echo showed that darn hole in her heart.  With every beat, blood is crossing over where it shouldn't be.  The current trending studies, do not recommend fixing it because of complications but after two 'events' they will consider it.  Our doctor felt that the mini stroke Shelbie had last November was in fact a blood clot that had managed to pass through that hole and likely came from her lungs.

She did okay on her stress test that measures how the heart and lungs exchange gases like oxygen and carbon dioxide.  Her heart and lungs are only functioning at 44% of someone her age.  They expected her heart rate to jump to 192 during the hardest part of the test but it only got up to 166.  The doctor said that was a surprise and borderline low but still better than where he could get her heart with a pace maker.  So...she will have to continue to live with it.  Since this is the kids first V02 test, this will serve as the baseline but we don't know if it is lower than it would have been if we had done it last year or stable.

Our plan for Shelbie is try some inhalers and get her as much relief as we can but ultimately, they aren't sure what to do.  Her symptoms are so much worse than the numbers are and they don't feel that they can completely correlate the granulomas and AVM's in her lungs to her difficulty in breathing and functioning.  I have an opinion or two about this I will share later.

Spencer- Spencer has a great heart and great lungs!  No holes in either.  His pulmonary function tests came back nigh on perfect...but...he has severe asthma.  He can't even get through the pulmonary function tests so it makes no sense that he is having as many issues as he is but the tests don't pick up on it.   With Spencer, we have actual physical abnormalities that prove he isn't processing oxygen correctly because of the clubbing in his fingers, purple fingers and some other things so nothing adds up. It's confusing and perplexing and extremely frustrating.  Even our doctor feels lost as to what to do.

When addressing Spencer's passing out spells he has had for most of his life off and on, the doctor suspects that the problem lies in his brain, like the autonomic nervous system or in an electrical problem of his heart that wouldn't show up on any imaging.  Spencer gets to wear a heart monitor for 30 days.  I'm not holding my breath that any helpful information will come from this.

Spencer's heart response in the stress test performed just shy of perfect; at 93% what is predicted for a 21 year old.  He did show some stress and issues with his lungs even though they didn't catch that on the Pulmonary Function testing.  His lungs only performed at 78-88% of what was predicted. We will probably follow up with a pulmonologist.


Samuel- Bad new for Sam.  It looks like he is trailing down the same path as Shelbie.  A nodule showed up in his left lung.  It is likely a granuloma or it could be a larger AVM but I think they are more convinced it is a granuloma because of what the Echo showed.  Sam has many, tiny AVM's in his lungs as well, but again, too small to fix but big enough to cause issues.  His echo did confirm the AVM's are creating some shunting but it doesn't seem to account for the fact that Sam can hardly do active, physical things anymore.

I have watched him go from extreme activity and training to not so much.  He can't even make it up the stairs in our house without being winded.  When he was in the middle of his training, his resting heart rate was 55bpm...very low.  Sometimes, we saw it dip into the high 40's.  This week, his resting heart rate is 35.  That is crazy low!  An advanced athlete in his age group rarely gets below 45. An elite athlete like Lance Armstrong is in the 35 range.   Sam is not an elite athlete so this is worrisome.  When activity wanes, the resting heart rate naturally goes up, not down.  The doctor wasn't sure what to think of this but it isn't normal or good really.

Despite all this going on with his heart and lungs, Sam nailed the V02 testing and by 'nailed' it, I mean, he performed 118% over what was predicted for his age group.  Granted, this was an 8 min. bike ride and my kids excel at pushing through anything, especially 8 min. So, I wonder how much we can really invest in this and like the doctor mentioned, we don't know how much any of the kids have dropped off the charts because this was the first test we've done to measure any of this.



It's just so frustrating to have reports that contradict what the kids can do.  The technicians running these tests even make notes of how much the kids were struggling to do any of these tests so I know it's not just in my head!  It's an unbelievable thing to see.

I asked around on the DC support group if our experiences sounded familiar and sure enough, it was a familiar routine to many parents...the tests seem okay but the kids are suffering and some have even passed away, with tests that showed they were fine.  It's crazy and emotionally draining.

I have some theories that I am checking out with the National Institute of Health but they are just theories.

I wanted to make this trip a little bit fun but it really wasn't.  We were tired and frustrated and our one plan to have fun fell through and I hadn't taken into account that we would have to spend an extra day at the hospital.

As a single mom, all I ever get is the homework, the illness, the chores...their dad gets the weekends of fun.  I spend the weekends trying to get work done that I couldn't do during the week because of sickness or medical trips.  I want my kids to experience one normal thing like a family vacation that doesn't include doctors! Or a vacation that I don't have to limit what we do because money is so horribly short in supply.  I can really get so resentful and bugged at times.  This was one of those weeks. Still...we try to laugh and have as much fun as possible.  And, someone left an envelope of cash on my door late Tuesday night.  It was such a blessing and helped immensely with hotel costs, gas and food.

Sam and Shelbie finished their V02 tests an hour before Spencer so we went to find them some food since it had to be done fasting.  There was a sculpture outside of 4 little kids (you can see it through the window) so Sam, Shelbie and Abby (Spencer's girlfriend) pretended to be the statues.  It was fun!  Abby is a great sport to join in our craziness! 
 We did make a run to Park City because they have tube rides done the ski jumps at Olympic Park.  I thought that would be a fun thing to do but they had to cut the rides short because of other events going on so we missed the opportunity.  We did sit and watch them ski jump into a huge swimming pool.  That was pretty interesting.
The free trolley ride. 
 We wandered downtown and spent some time in art galleries and the Family History place downtown.  They took our picture and had a little visit with some missionaries. It was so blazing hot in Salt Lake, unbearable for my little troop!
Park City after the testing was all done.  There is a restaurant called Fletcher's so the kids wanted me to stand under it...since my maiden name is Fletcher...but you can't see the sign very well. 

I wore my favorite t-shirt to the hospital yesterday...you can't see if very good in this picture but it says..."I literally cannot."  Truer words have never been spoken!



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