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Let me try and explain…

April 24, 2011

There is a lot going on with Lucy and I am going to do my best to try to summarize things.

Lucy’s body is complicated due to the fact that she has mitochondrial disease, a disease that can affect everything in the body down to the cellular level, as a result she has a lot going on with her systemically.  A person with mitochondrial disease is at “highest risk  for neurological and organ damage during and for the two weeks following an illness.  Therefore even a simple flu or cold virus can have devastating effects on the patient, even death." (UMDF) 

Lucy’s body is stressed…how’s that for stating it simply.  The flu virus has proven to be too much for our little one’s body and is reeking havoc on many of her major organ systems.  Pick an organ, any major organ, and I could tell you something that is going wrong with it at the present time in Lucy’s body.  Our biggest issues at the moment are being caused by the fact that Lucy has macrophage activation syndrome (MAS), an inflammatory response of her immune system.  This syndrome is extremely complex to try and explain, even the doctors who are treating Lucy are having a difficult time understanding and explaining it.  Dr. R told us that you will not find a lot of literature out there that explains MAS and mito, saying that he has only found three case studies in I believe Alberta, Canada that describe MAS and mito.  All three patients had failing bowels, just like the two patients that Dr. R has ever seen this happen to.  Lucy’s immune system had a hypersensitive response to the influenza virus causing her body to send out uncontrolled activation and proliferation of macrophages.

Lucy’s liver,spleen, and gallbladder are taking up a majority of space in her abdomen, as well as her bladder before we straight cathed her and then put in a Foley catheter.  Our PICU doctor (who by the way credits himself with saving Lucy’s life in October when she was in need of life saving central access, he likes to remind Dr. R of that often) told us that her liver and spleen are down in her groin area.  You can feel it, Lucy’s belly looks and feels like she swallowed a basketball.  Her liver is trapping all kinds of red blood cells and platelets making it challenging to get Lucy’s blood counts up.  We had to transfuse Lucy with some more packed red blood cells today because her counts went up only minimally with the first transfusion.   

Lucy is in respiratory distress because her lungs are so compressed from the extreme swelling of her liver, and having a  pneumonia doesn’t help the issue.  Her respiratory rates are up as high as 97 breaths per minute.  Her breathing is being assisted by using Vapotherm, she is currently at 12 liters of O2 at 45% oxygen to help maintain her oxygen saturations.  The high doses of ketamine and morphine that she is on are suppressing her respiratory function as well.

Lucy’s bladder was the size of a small pool on ultrasound, the girl can hold her urine.  After voiding close to 200mls of urine, we straight cathed her to see how much residual was left in her bladder.  Boy were we surprised when another 250mls of urine came flooding out.  We have suspected for some time that Lucy is not emptying her bladder all the way, we are suspicions that she has a neurogenic bladder.  All of the narcotics she has been on can also be contributing to this issue.  Lucy has a Foley catheter in place which is helping to empty her bladder for her, she is peeing like a champ with the Foley.  We are going to follow up with urology sometime in the next week or so about this issue.

Lucy’s gallbladder is inflamed and has increased mucus secretions which is blocking a biliary duct.  Surgery was consulted on this issue as well as GI and for the time being we all believe that Lucy’s primary issue is the inflammatory issues related to the MAS which is causing her gallbladder issues.  Lucy is on an antibiotic to treat her pneumonia which should cover her gallbladder if it just so happens that there is an infection that is causing the inflammation. 

Unfortunately, there is nothing we can do to counteract the MAS, instead we are supporting Lucy’s needs by providing pain control and respiratory support.  These types of support come with their own set of problems that I will save for another post as I believe that I have bombarded this post with enough information for one night.

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kristi said...

Praying for healing for Lucy.

Heidi said...

I don't know what to say, Its just awful knowing she is struggling with so much right now. I'm checking often for updates hoping and praying shes made a turn around :( I'm continuing to pray...hugs hugs hugs.