Tia's Story
CHF and Elevated Ammonia Levels   

April 27, 2006:
Tia, my wife, age 31 calls me from home; she has left work because she doesn’t feel good.  I arrive home to a state of confusion.  Tia tells me she went to work at 10:00 am, only after her supervisor called at 9:00 am looking for her.  A call to work and I’m told Tia arrived at 11:45 am, then left at 12:00 pm.  Additionally, she had been oversleeping for a week.  This is highly unusual, Tia is extremely responsible and punctual.  Upon hearing this I decide we need to go to the Emergency Room.  Her response was, “no, I don’t want to go.”  This was not the response I expected.  Regardless, we were in the Emergency Room 30 minutes later.  Blood work, a CAT scan, and an MRI are done.  We are told Tia’s ammonia level is 76, normal being 30, with an abnormality in the frontal lobe of the brain.  We are advised to see a liver specialist and a Neurologist the next day.  Upon discharge, Tia is instructed to take Lactulose 3 times a day to keep her ammonia level down.  Surprisingly, the Neurologist tells us he could smell the ammonia at 76. 

The following day, the Neurologist is concerned, maybe the sleeping pill (Ambien) Tia has been taking caused her confusion and drowsiness, despite the fact that Tia has been taking it for a year without any problems.  He prescribed something else to help her sleep.  Not knowing what was wrong, it made sense to go this route.  Yet, Wednesday morning I have a difficult time waking Tia for the follow up Neurologist appointment.  Tia slept the 1 1/2 hour drive and when the receptionist asks Tia to sign a form, Tia only stares, then burst into tears.  She cannot comprehend what she is looking at or what to do with it; she is in a complete state of confusion!  The Neurologist thinks Tia is depressed, therefore increases the dosage.  Tia can barely verbalize to the doctor she is not depressed.   

The following week Tia seems to be better and I return to work.  Typically, Tia and I talk at noon.  Not hearing from Tia, I chalk it up to her needing sleep.  At 2:00 pm I decide to call.  The phone rings 7 times before it hits the floor.  Immediately I drive home and find Tia unresponsive, the phone emitting a busy signal.  Frantically I call 911.  Tia’s has only slurred responses.  The Emergency Room admits Tia with a 117 ammonia level.  Tuesday morning Tia questions why she is in the hospital.  She has no recollection of the paramedics or the ER visit!  That week her ammonia level spikes to 168.  One minute she is talking, the next she is having a seizure. 

Currently, Tia is listed for a dual transplant.  Initially it was for the liver only, but it soon became apparent her kidneys could not tolerate a liver transplant, therefore, she is currently listed for a dual transplant (kidney and liver).  During the evaluation process Tia underwent a stress test during which she went into a seizure.  Originally it was thought her seizures were a result of the ammonia seeping into her brain.  The seizures are still under investigation. 

Looking back, it is apparent there were warning signs.  Certain things didn’t add up.  In hindsight, symptoms started during Christmas season 2005, with most recent events occurring in April and May of 2006.   Tia had times which I’ll refer to as “spells”, where her short term memory was just not there.  She would argue with me that she did not watch a TV show the night before, when we discussed the show during the program.  There were instances where she would misspell her name or her handwriting was just completely different.  She would leave for work and forget to lock the front door.  She would lose coordination at times making it difficult to drive or type.  She underwent many personality changes and was not the same person I knew only months prior.  She lost 20 pounds in a matter of weeks and looked like she was fading away.  She was extremely exhausted although routinely administered Procrit.  I took her from doctor to doctor; they kept switching her medications, until she finally went unconscious and we found ourselves in an emergency situation.  That is when she was diagnosed with hepatic encephalopathy. 

It is extremely difficult to watch a loved one suffer and very frustrating to bring them to doctors and not be HEARD; I verbalized so many changes.  Families, you are the expert in your situation; you hold all the information the doctors need to hear.  They may get to a point where they can not think or speak appropriately.  I hope others can learn from our experience.  It is never too early to be preventative and prepared.  Don’t wait until you are at end stage to find out what is going on.  If you are not happy with a doctor’s answer, or a doctor can not answer you, keep pursuing and be persistent.  Much is not known about ARPKD and CHF as a whole, but we can help each other if we speak out and inform one another of experiences and gain knowledge.

Sincerely,

Sandy