What is Guillain-Barré Syndrome?
Dallas Cowboys’ center Travis Frederick is battling a rare autoimmune disease called Guillain-Barré syndrome, named after Georges Guillain and Jean Alexandre Barré, the two French doctors who first described the the malady in 1916.
The condition is rare, with 1 in 100,000 people being diagnosed each year.
Luckily, Frederick’s case was caught early, which has shown to be integral to patients having a full and relatively quick recovery.
Dr. Farhan Siddiq, an endovascular neurosurgeon and the co-medical director of Texas Health Fort Worth’s Stroke and cerebrovascular disease program, helps us understand the nature of the uncommon disease and the fight Frederick had on his hands.
What is Guillain-Barré syndrome?
It’s called a syndrome because the symptoms can be a conglomeration of multiple different organs. No one knows exactly what happens, but most frequently it is triggered by an event and usually the event is an infection. There is a one particular type of bacteria, Campylobacter bacteria, which has been associated with this type of syndrome. But any infection can cause it. The Zika virus infections recently, especially in the Zika-hit countries, have shown an upsurge in Guillain-Barre.
What is happening to cause the disease?
For some odd reason, the virus triggers this phenomenon by presenting an antidote to the body which resembles myelin. The myelin [sheath] is a covering that covers all of the peripheral nerves in the body. For some reason, the bodies’ own immune system starts recognizing the myelin as an antibody or a foreign material. The body is triggered to start a response to any foreign material and in Gullain-Barre syndrome, the body starts recognizing its own structures as foreign material and myelin is perceived by the immune system as a foreign body. So the immune system becomes hyperactive all of a sudden and it starts attacking its own peripheral nerves.
How quickly will a patient start having symptoms?
Over the course of 10 days to two weeks when those peripheral nerves suffer damage from the bodies’ own immune system they start showing signs of peripheral nerve damage. It starts with longer nerves first and then the shorter nerves. So typically patients show lower extremity symptoms (their feet or legs) before they progress on to arms or sometimes the face.
Those symptoms would be?
Depending on the degree of the attack on the nerves, it could start with a tingling sensation, or numbness, and then progress on to motor skills weakness or even paralysis. There are times when people have paralysis and they can’t move their legs or arms. It can be really scary. They start sometimes with very minor symptoms with numbness or tingling in the feet and patients may think it’s nothing. But It can progress very quickly into a very severe illness and they may end up coming to the emergency room and then very quickly be in the ICU and sometimes even the breathing muscle can undergo failure and they have to be intubated.
How do you cure it?
The problem is there is no way for us to stop the progression of the disease. We can slow the progression and improve recovery if diagnosed quickly but everybody has a different presentation. Some patients may be very acute and some may be less acute and can linger on for a few days and then quickly progress to acute symptoms. Of course, it’s rare, so there are a lot of times when people may have mild symptoms and never progress on to motor [weakness] symptoms so we might not know exactly how many patients do actually have this syndrome. But the severe cases are rare so it becomes hard to diagnose quickly. And it requires a few tests that may take a few days to come back. For example, sometimes we have to do lumbar puncture and check the protein and cell count. a nerve conduction study can’t be done in an ER.
Much has been made of Frederick’s case being caught really early.
We can sometimes initiate treatment before a full diagnosis. Early treatment may help reduce the severity and also shorten the course of the disease.
So if you catch it early enough patients can totally beat it?
Well, that’s hard to say because we can’t predict what anyone’s symptoms are going to be. The theory is if we can initiate treatment sooner we might be able to, but there are times when we initiate treatment as soon as we can but patients still progress on to a very severe form of the disease, with paralysis. There’s no correlation that we’ve been able to scientifically associate with treatment and disease progression. The reason for that is the disease is very rare so there’s no way for us to do a randomized, controlled study in a scientific manner to say what impact treatment makes on the disease.
Has anyone ever died from this disease?
Yes, people can die. Mortality can occur if there is long-term paralysis. Those patients can develop complications of being paralyzed, which are infections or pneumonia, and those can sometimes be very severe and people can die from it. But it’s not common for it to be fatal. Seventy percent of people will recover and go on to gain complete, neurological function. But there are 30 percent of people who will be in a wide array of disability that could be minor or severe.
Does Frederick being in excellent shape make his prognosis better?
They caught it early, so with early treatment I think he probably lies in the 70 percent who will go on to regain full functional recovery.
Is that because they caught it early or because he was in such good health to begin with?
It’s a combination of both, but I do think his disease progression was not as severe compared to others. The severe ones, no matter what you do, they go on to progress and they have a very severe onset of the disease.
If Frederick was your patient how would you be treating him?
I would recommend he get four or five days of IVIG and then following that monitor him very closely. The goal is we don’t want to see any further progression of neurological symptoms. If there is a worsening, then we’d consider the second-line treatment, which is plasmapheresis. If there is no worsening of the symptoms, then we’d expect the disease to plateau its course over another week or 10 days and then symptom recovery would start.
Can a person be stricken twice?
Yes, they can, but it’s very rare because the disease itself is very rare. There are times when people can develop chronic, long term forms of the disease. About 5 to 10 percent continue to have lingering symptoms.