Guillain-Barré Syndrome (GBS) is an inflammatory disorder of the peripheral nerves outside the brain and spinal cord. No one knows for sure what causes Guillain-Barre, but it is rare – about one person in 100,000 have GBS.
According to the GBS and the CIDP Foundation International, 50 percent of Guillain-Barré cases occur after a viral or bacterial infection, such as the flu or food poisoning, and GBS has also been associated with the influenza vaccination.
The Centers for Disease Control and Prevention (CDC) report that each year an estimated 3,000 to 6,000 people develop Guillain-Barré Syndrome (GBS), whether or not they received a vaccine.
To hear first hand what it is like to have GBS, we will hear from Stephanie, a mother to two young boys, one of whom developed GBS after receiving his flu shot.
Symptoms of GBS
The first symptoms of GBS start in the legs with a tingling sensation and sometimes weakness – this then spreads to the arms and the upper body. These symptoms increase with intensity until the patient cannot move his or her muscles at all. When this occurs, it is a medical emergency, since breathing and heart muscles can be affected as well.
Patients reach the point of greatest weakness or paralysis days or even weeks after the first symptoms appear. Symptoms then begin to stabilize and can last for days, weeks, or sometimes months, reports the National Institute of Neurological Disorders and Stroke.
One Mother’s Story
Stephanie, a critical care nurse, took both her boys to the doctor for a check up on December 5, 2011. While at the doctor they also received their flu shots and had no adverse reactions at the time. From December 12 – 16, 2011, the youngest son, three-year old Zachary began to run a low-grade fever which went away. However on the night of Christmas, things changed drastically.
Stephanie tells Decoded Science,
“I was awakened to Zachary screaming, “Mommy I’m scared!” I found him bent over the toilet holding himself up with his arms; underneath, his legs looked like noodles unable to bear any weight. He told me he had gotten up to use the bathroom and his legs began hurting so badly that he couldn’t stand. I scooped him up and carried him to my bed, gave him some ibuprofen, and he was soon fast asleep. My nursing education kicked in and wondered, “Could this be Guillain-Barre’?” I quickly waved that away; as the risk was minuscule. “
As the days went on, like most patients with GBS, Zachary’s gait became more and more unstable. Stephanie described her son’s condition, “Zach was attempting to walk to me, he was extremely pale and looked like a spaghetti noodle trying to ambulate. At this stage, Zach was exhausted all of the time and barely played. I carried him everywhere we went; the pain was so intense when he attempted to straighten his legs.”
Since GBS is so rare, doctors may not think to test for GBS; this was the case for Zachary. Multiple trips to the pediatrician and the emergency room yielded no diagnosis. Finally, after a long battle, the head of the Neurology Department conducted a spinal tap to determine if Zachary had GBS.
A spinal tap, also known as a lumbar puncture, is a procedure that uses a needle to retrieve cerebrospinal fluid from between two vertebrae. Zachary’s cerebrospinal fluid was eight times higher than what is should be; he was diagnosed with GBS, and treatment began.
There are a variety of treatments available for GBS patients. One therapy, called plasmapheresis, also known as plasma exchange, is a method in which the whole blood is taken out of the body and processed so that the red and white blood cells separate from the plasma.
The red and white blood cells are then returned to the body without the plasma, and the body quickly replaces the missing plasma. This therapy seems to reduce the severity and duration of GBS.
Another form of treatment that doctors may use is administering a high dose immunoglobulin therapy. Immunoglobulin is the plasma proteins that plasma cells secrete. Intravenous immunoglobulin (IVIG) is a blood product that is prepared from 10,000 to 20,000 donations of plasma.
Multiple purification steps are taken to remove pathogens that could be transmitted to the patient. During high dose immunoglobulin therapy, doctors give intravenous injections in small quantities; research has proven this therapy lessens the immune attack on the nervous system. This is the treatment that little Zachary had; however, it carries a risk of an anaphylactic reaction, so doctors give an antihistamine before the IVIG.
In Zachary’s case, the therapy caused vomiting and hives, but the treatment continued in smaller doses, allowing the child to tolerate the treatment without reactions. He was then released from the hospital to a rehabilitation facility, where he stayed for one week and received occupational and physical therapy. Unfortunately, in Zachary’s case, a second round of treatment was necessary, but then he made significant strides, and eventually a full recovery.
Patients with GBS develop symptoms quickly; however, recovery takes a long road. Recovery can take anywhere from a few weeks to a few years. According to the National Institute of Neurological Disorders and Stroke, 30 percent of patients have residual weakness after three years. About three percent of patients may experience of relapse many years after the first GBS attack.
As for Zachary, Stephanie will have to watch him closely for any signs of re-occurrence.
Mazen M. Dimachkie M.D, Richard J. Barohn M.D. Guillain-Barré Syndrome. (2013). Current Treatment Options in Neurology. Accessed July 10, 2013.
Centers for Disease Control and Prevention. Guillain-Barre Syndrome. (2012). Accessed July 10, 2013.
GBS and CIDP Foundation. All About GBS. Accessed July 10, 2013.
National Institute of Neurological Disorders and Stroke. Guillain-Barre Syndrome Fact Sheet. (2011). Accessed July 10, 2013.