GBS, CIDP, and plasma

Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are rare neurological disorders and plasma-based therapies are used to treat them.

05/18/2023

Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are neurological disorders that impact the peripheral nervous system. Your peripheral nervous system (PNS) is one of two main parts of your body’s nervous system along with the central nervous system (CNS). Together the CNS and PNS transmit and process sensory information and coordinate bodily functions. The brain and the spinal cord make up the CNS and function as the control center that receives data from the sensory organs and nerves, process it, and sends back commands. The PNS works by carrying incoming and outgoing signals and information. It carries signals that allow you to move your muscles and perform unconscious actions, like beating your heart and breathing. It’s easy to see that any impact on the PNS could be life-threatening. 

For years, peer-reviewed clinical studies have shown that plasma-based therapies, such as immunoglobulin (Ig) replacement therapy and therapeutic plasma exchanges (TPE), are beneficial and effective therapies for both GBS and CIDP.

Guillain-Barré syndrome

GBS is a rare inflammatory disorder of the peripheral nerves outside of the brain and spinal cord and is characterized by the rapid onset of numbness, weakness, and often paralysis of the legs, arms, breathing muscles, and face. GBS ranges from mild cases of weakness to devastating paralysis that leaves individuals unable to breathe on their own. While the cause of GBS is unknown and can affect anyone, it is not contagious. It’s more frequently found in adults and it’s estimated that about one in 100,000 people are affected each year. 

According to The GBS|CIDP Foundation International, roughly 50% of all cases of GBS occur shortly after a microbial infection, which could be anything from the flu to food poisoning. Someone with GBS will begin to experience symptoms as their immune system begins to mistakenly attack healthy nerves. The damage of this attack can result in the nerves becoming unable to transmit signals efficiently to the brain and the muscles unable to respond to the brain’s commands. 

There is no known cure for GBS. However, there are therapies that can lessen the severity of the illness and shorten recovery time. Many diagnosed with GBS are hospitalized at the onset of their diagnosis to monitor breathing and other body functions until the disease is stabilized. High doses of immunoglobulin (Ig) replacement therapy, which is derived from plasma, are helpful in shortening the course of the acute phase of GBS, which can last anywhere from a few days to months. Most individuals move to the rehabilitative phase within four weeks.

Those with GBS also rely on therapeutic plasma exchange (TPE) or plasmapheresis. TPE is a process in which the plasma in an individual's body is exchanged for donated plasma. The process involves using an apheresis machine to remove the blood from an individual by IV, where an apheresis machine then separates plasma from other blood components (red and white blood cells and platelets). The individual’s plasma is discarded and the other blood components are mixed with donated plasma and returned to the body. 

A 2021 study shows that both Ig replacement therapy and TPE are significantly effective treatments for those with GBS. Treatment for those with GBS can greatly vary depending on their condition's severity, and the treatment method often is dependent on what works best for each individual, meaning some may have better results from IVIG than they would TPE. For those receiving Ig replacement therapy, treatments are administered as a single dose over five days and within two to four weeks of the symptoms' onset. Generally, no repeat treatments are necessary after receiving the complete dosage. However, some cases may need more treatment.

Chronic inflammatory demyelinating polyneuropathy

CIDP is a rare disorder of the peripheral nerves that is characterized by progressive weakness and sensory loss. Each year, the number of new cases of CIDP is around 1 in every 100,000 people. However, CIDP can be present for an extended period of time without a diagnosis. 

CIDP is caused by damage to the myelin sheath, which acts as a protective covering on the nerves. The myelin acts like insulation and allows electrical impulses to transmit quickly along the nerve cells. The damage to the myelin results in tingling and numbness in fingers and toes, weakness of the arms and legs, loss of deep tendon reflexes (which refers to the contraction of a muscle in response to passive stretching), fatigue, and unusual feelings in the body. While the exact cause isn’t known, the body’s attack on the myelin sheath shows that the immune system perceives it as foreign and attacks. 

Ig replacement therapy is one of the only U.S. Food and Drug Administration (FDA)FDA-approved drugs for treating CIDP and is a first-line treatment. For those with CIDP, an individualized plan of Ig replacement therapy, either intravenous (IVIG) or subcutaneous (SCIG), is administered and then assessed for further dosage or treatment. 

2020 study from the Journal of Neurological Sciences and a 2021 study from Frontiers in Neurology showed that both IVIG and SCIG have a positive impact on improving the symptoms of those living with CIDP. Much like with GBS, some with CIDP also rely on TPE or plasmapheresis and it has a history of being an effective treatment for them. The 2020 study shows that Ig replacement therapy is recommended for those with CIDP at the onset of the symptoms and as a maintenance therapy, meaning they'll receive the treatment regularly. The studies show that the treatment method, IVIG or SCIG, should be discussed with the primary physician to determine the patient's needs best. 

Plasma is not a product that can be synthetically produced, and those living with GBS and CIDP are reliant on donors to help provide them with lifesaving treatment. You can help make a difference by becoming a plasma donor. Read Plasma Hero Stories of those living with GBS & CIDP like Sara Vinson and Kiki Wagner.

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