Submitted by Lauren Kondev, M.D.,
New England Medical Center, Pediatric Neurology
Editorís Note: On September 1, 2001, Faith suffered one of the most significant episodes in her illness, a seizure-like episode that impaired her ability to communicate with us. We were frightened beyond belief and concerned that she was gone from us (in mind, if not in body) never to be returned. We were not ready for death or anything like it. One of the doctorís concerns was how to correctly medicate her. Had she really experienced a seizure? Scheduling an EEG would have taken too long. Instead he arranged to have someone from Neurology evaluate Faith. Our visit from Lauren Kondev turned out to be far more significant than simply deciding on a medication.
I met Faith in early September 2001. Little did I know what an impact she would have on me. At the time her disease process was quite advanced and her parents were concerned that she was losing touch with her surroundings. She had right-sided weakness since Spring 2001, had become non-verbal since Summer 2001, and shortly thereafter developed near-paralysis of her eye movements. Up until the day prior to my seeing her, she was able to point to a communication board with her left hand, but she lost this ability. Was Faith trapped inside her body, unable to communicate?
Because of the location of Faith's advanced pontine glioma, she developed "locked-in syndrome." She could absorb input, but over time lost the ability to communicate in return. Because of Faith's severe impairments it was quite a challenge to find a means for her to communicate. She was unable to speak, point, or even look in various directions to indicate her wants. The key was then to find what she could do. This is what took time, effort, and determination from all involved: Faith, her parents, and me. Ultimately, we found that Faith could open and close her eyes, although only slightly because she had bilateral ptosis (drooping of her eyelids), and incomplete closure of her left eye. Also, she could flex the fingers on her left hand slightly, giving the impression of a "thumbs up." After finding Faithís abilities, the next step was to test Faith in an objective manner, to see if she had the cognitive and physical abilities to communicate by these limited means. She was given a set of rules, such as closing your eyes means "yes" and opening your eyes means "no," and thumbs up or closing the left hand means "yes" and not doing so means "no." She was then asked several objective yes-or-no questions. She passed the test and was able to communicate by these means for a short period of time. As her disease progressed she lost these skills, but while she had them she was able to connect with her family. Also, her family confirmed what they had suspected; Faith could understand them and knew when they were with her, talking to her, or telling her that they loved her.
The lessons I learned from Faith and her family are multiple and priceless. Do not give up on even the most impaired patients. Don't assume that a patient has no cognitive ability just because they can't communicate. Be determined in trying to find an individualized means for the patient to communicate. Even if you are unsuccessful, always treat your non-communicative patients with respect and compassion. Even when Faithís parents were uncertain as to whether or not she could understand them, they constantly talked to her, read to her, and expressed their love to her. It is of utmost importance to encourage families with impaired children to show the kind of love and support that Faith's parents showed to their child. They were, and continue to be, her advocates.
There is a book titled The Diving Bell and the Butterfly: A Memoir of Life in Death by Jean-Dominique Bauby. I encourage you all to read this book. It was "written" by a man who had a stroke, leaving him with locked-in syndrome. His only means to communicate was by blinking one eye. The transcriber would say each letter of the alphabet and the author would blink when she said the letter he wanted. In this manner, he spelled out each word of this powerful book. It provides a rare glimpse into the mind of someone with locked-in syndrome.
Editorís postscript: In late September 2001, Faithís condition declined further during a three week stay in the hospital; she became less and less responsive. Lauren was not on rotation in the hospital at that time, but at our request she came to reevaluate Faith. Repeating the tests performed previously, it became clear that Faith was no longer able to respond to us. While this confirmed what we suspected, we could not have handled this news had it come from anyone else. But Laurenís commitment to Faith enabled us to accept this new sign of decline.