Friday, April 5, 2013

Fact-Buzz Friday: PVS

What is PVS? 

Persistent Vegetative State is defined as "a clinical condition of unawareness of self and environment, in which the patient breathes spontaneously, has a stable circulation, and shows cycles of eye closure and opening [that] may simulate sleep and waking."  reference

Doctors generally leave out the details on hydranencephaly, such as which areas of the brain are affected or what the affected areas of the brain mean for the child. That is good and bad. Bad because it turns out that even the formal diagnosis of hydranencephaly is avoided, simply stated as:

"Your child does not have a brain. They are surviving on only their brainstem which controls involuntary functions such as breathing, blinking, and some reflexive-like movements. They will live for a little while in a persistent vegetative state." 

(that's usually followed up by some unethical opinion of what the parents should now do for their child, which is generally not much)

Good because when doctors leave out those details, they are not placing inaccurate expectations for the child's life (or more likely, adding to that long list of impossibilities that come along with diagnosis). 

You may have heard of PVS during the case of Terri Schiavo, the woman who experienced a heart attack-like episode in 1990 and went in to a coma which later was deemed a persistent vegetative state. There was a battle between her family & her husband whether to remove necessary equipment such as her feeding tube. The government was involved and eventually she passed away due to dehydration after more than 13 days without nutrition or hydration in 2005. This case happened years before I myself embarked on this journey with hydranencephaly, but it had a huge impact on me. You can learn more and support the foundation, which is working to change the misconceptions that surround a diagnosis of PVS, in her name here: Terri Shiavo Life & Hope Network

The truth is, our kids living with hydranencephaly are not in this state of awareness. Some defined symptoms of PVS include:

  • ~unresponsiveness to external stimuli
  • ~varying levels of consciousness
  • ~eyes remain is relatively fixed position, sometimes able to track moving objects but not consistently
  • ~may experience inconsistent sleep-wake cycles, or pursue a state of chronic wakefulness
  • ~exhibit some involuntary behaviors that exhibit signs of partial consciousness such as: teeth grinding, swallowing, smiling, shedding tears, grunting and other vocal sounds without language
  • ~not dependent upon life-sustaining equipment other than a possible feeding tube when the brainstem is intact (some children with hydranencephaly do not have the brainstem)

and if you have a little one with hydranencephaly in your life, you know that these symptoms are not accurate descriptions of the child. 

  • ~The children are responsive to stimuli, which is encouraged and expanded upon with therapies. 
  • ~They are conscious of their surroundings and the people in them. While they may not recognize the minute details, they respond to negative situations, strangers, people they love, and show preference for specific activities and people. 
  • ~sleep-wake cycles are generally inconsistent in most children in infancy. While sleep often does continue to be a challenge for children with hydranencephaly, this is more likely due to damage to the hypothalus which regulates the body's circadian rhythym... not PVS.
  • ~Children with hydranencephaly instead exhibit MANY signs of purposeful movement and even language skills. Their abilities, like their responses to stimuli, can be encouraged to grow with therapy. Most of the children smile a characteristic "elvis-grin" which is pure joy! Tears are shed when they're sad or mad, they're crying, not just spontaneously but in response to negative stimuli. Teeth grinding is common, but can also be attributed to seizures or the areas of damage within the brain.
  • ~life-sustaining equipment is a rarity... though some would argue that equipment our children DO use is considered extreme life-saving measures, but I instead believe them to be life quality-enhancing measures: feeding tube, tracheotomy, brain shunt placement, and oxygen use.

This is quite the controversial topic, and while I have my personal views on the diagnosis itself, it is a difficult fog of a condition to navigate. Misdiagnosis is not uncommon since it is difficult to evaluate activity within the brain, even with advances in imaging technology such as functional MRI and PET scans. And the determination of level of consciousness, along with brain function and response, is next to impossible. 

However, it is without a doubt that the phrase persistent vegetative state is not one that should be used to define children with hydranencephaly. 




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