Tuesday, May 14, 2019

Unfortunately we have a reason to blog again, plastic bronchitis

Around Halloween 2018 Olivia began having an incessant cough.  We started off blaming it on kids at school passing around a good cough.  Then we blamed it on Olivia was just sick and couldn't get rid of it. 

After a month we were getting her doctor involved more.  The cough was blamed on a virus, pnumonia, digestion issues, adenoids etc.  The aero-digestive clinic at Ochsner (you see a ENT, pulmonologist, and gastroenterologist) said she needed to start asthma inhalers, tried some zantac, and she needed her adenoids removed.  We finally hinged her cough on seriously infected adenoids which was leaking puss constantly.  At the end of February we finally had a procedure done at Ochsner under anesthesia.  It was a huge step for us, but the doctors at Ochsner were awesome.  They are building a great cardiac program, and it was apparent with how well their cardiac anesthesia team was.  They also decided to put in her 4th set of tubes, this time putting in super-duper T-Tubes so they would last longer.  The plumonologist couldn't make it so the ENT did the bronch for him and he said there was a lot of white puss in her lungs so he flushed it out.  He also took out the adenoids and inserted the tubes.  Little did we or he know what all that puss was from. 

Even after the adenoids came out and she healed up, she still had the cough.  Until one sad day a bronchial cast was launched out of her.  When you cough out a bronchial cast your cough becomes harder and more constant.  You have trouble breathing as you constantly cough and eventually the bronchial cast dislodges and comes flying out. 

The first cast might have happened at school and she swallowed it.  Then a week later Melanie saw one fly out but Olivia was freaking out so much Melanie looked at it real quick and pushed it down the sink.  I think we were more in denial and did not want to put her cough onto her cardiac issues.  It wasn't until two weeks later when we had another crazy coughing bout.  She just so happened to be sleeping over at her aunt BB's house when she had them worried she just got everybody sick.  No one was getting sick because after Olivia got home she was coughing so much, gasping for air, until she finally coughed out a nice big bronchial cast.We had her going to school with a specimen cup just in case, but I ran and grabbed the cup and put this nasty, slimy, stringy "octopus" into the cup.  We also took a picture of it.   


There was no more denying it, we finally got one of the bigger complications a HLHS fontan girl can get, plastic bronchitis.  Plastic bronchitis, and PLE (protein losing entropy) are two of the scary monsters in the fontan closet.  We are lucky to only have one, and we are hoping to keep it that way!  Once we collected this specimen we spoke with our cardiologist, Dr. Crittendon, and he conferred with our CHOP cardiologist to get things in motion to take care of it.  Everyone was in agreement that we had a new diagnosis to add to our list of medical conditions.  

Plastic bronchitis is a scary diagnosis because they really don't know what causes it, and in the past few years they have found a few ways to combat it.  The end all be all fix is to get a heart transplant, with other possible fixes including undoing her fenestration closure or the Fontan and return to a Glenn.  

It is also scary because it's hard to describe as well.  A lot of the problem can be attributed to the pressures that exist in the circulatory system, lungs, and lymphatic system.  When Olivia had her Fontan they left a fenestration that would act as a pop-off valve if the pressures within her fontan circulation got to high.  In December 2017 Olivia needed more energy, and was satting lower then normal.  It was decided at that time to close the fenestration, in some kids it closes by itself but Olivia's would not.  We went to CHOP and through a heart catheterization they inserted a device which closed her fenestration allowing her to have a more consistent flow through her Fontan to maintain her energy and stamina.  With extra pressure comes the possibility of more pressure existing in the lungs then more pressure in the lymphatic system.  If you recall after Olivia's Glenn procedure she had a chylous leak which caused her to get on a special diet so the lymphatic system could heal up and the leaks could stop.  Dr. Natarajan thinks Olivia has a predisposition to lymphatic leaks, as do several people who are heart healthy, but that predisposition does not mix well with Fontan kids.  In Olivia's case her lymphatic system is leaking because the pressure is too high.  Imagine a pipe leaking at a certain spot and the leak is pooling fluid in the easiest place to go, which is her lungs.  The lymph fluid pools in her longs and congeals to form these molds of her bronchial trees.  You can heart her "casting," as these things develop in there by her every now and then cough.  The cast starts releasing and her cough gets worse and worse until she can clear it.  She is currently coughing out a cast a week from what we can tell.  She sometimes swallows them at school because she doesn't want to spit out an octopus and be embarrassed in front of her friends.  This condition only gets worse, until she is coughing out a few casts everyday.  It can cause permanent lung damage, low saturations, and just complicates everything.  Hopefully, we are getting a jump on this before she has any serious damage.  

Lucky for Olivia, one of the newer ways of treating PB is being pioneered at CHOP.  Dr. Dori, an interventional cardiologist at CHOP, has come up with a way to map the lymphatic system, find the leaks and glue them shut.  They have been doing this procedure for a few years and have had a few hundred successful outcomes.  If we can stop the leaks and eventual casts Olivia can keep her fenestration closed, enjoy the benefits and extra energy she got from that.  It will help get her Fontan circulation back on track to working good for her and keeping us away from the rabbit hole of possibilities.  So that is what we are going to do.

Are you still confused?  So am I, luckily I married an amazing medical professional who can explain it even though I go cross-eyed most of the time.

3 comments:

  1. Erin Ellis DavisMay 15, 2019 at 8:51 AM

    Olivia and her family are so brave and strong as they continue on this journey toward better health! There are many people out here who are thinking and praying for you all. I am thankful for the medical staff who are helping guide her and the family through this journey. Know that there are many of us thinking about Olivia and her family with love and concern.

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  2. Lots of love and prayers coming your way. Love to you too, Michelle.

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  3. Damn Brett. Hope they can glue the sucker. Thinking about you guys.

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Olivia loves to hear her mommy/daddy read your comments!