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Ig Therapy Guide for CIDP

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Key Takeaways

  • CIDP is a chronic, neurological disorder that damages peripheral nerves and can cause muscle weakness, numbness, and difficulty walking.
  • Immunoglobulin therapy (Ig) therapy is an established treatment for CIDP. It helps regulate the immune response and may improve muscle strength, physical function, and other symptoms.
  • Both intravenous (IVIG) and subcutaneous (SCIG) Ig therapies may be treatment options for CIDP. 
  • Treatment plans are tailored to each patient. Providers consider symptom severity, treatment goals, medical history, and response to therapy when determining the most appropriate approach.

Neurological conditions are very common, with the World Health Organization (WHO) stating that they affect one in three people. Globally, they are currently the leading cause of illness and disability. Among these conditions, however, is one that is not so common— chronic inflammatory demyelinating polyneuropathy (CIDP). This rare neurological disease impacts only one to nine people per 100,000 worldwide. Although CIDP is rare, it is one of the most commonly treatable conditions with the first-line care being immunoglobulin (Ig) therapy. When started early after diagnosis, this type of infusion care can improve CIDP symptoms in as little as two to four weeks.

The team at AOM Infusion compiled this guide to support patients, caregivers, and providers seeking information about Ig therapy and CIDP. This resource aims to give readers more understanding about this condition and where Ig therapy fits in treating it.

What is CIDP?

In CIDP, the immune system mistakenly attacks the protective covering of the nerves, called myelin sheaths. The damaged myelin sheaths can cause the typical symptoms of CIDP, which include weakness, tingling, and numbness. Guillain-Barré syndrome (GBS) shares the same signs and symptoms as CIDP, but it differs in its onset and how long it lasts. GBS, considered to be an acute condition, happens suddenly and lasts over a period of days to weeks. Meanwhile, CIDP begins to occur over a longer period of time, from months to years, and lasts much longer than GBS.

Different Types of CIDP

CIDP usually affects both of the arms and legs; however, this does not always mean that all people with this condition experience the typical symptoms.

Classic/Typical CIDP

The most common type, classic/typical CIDP presents as muscle weakness, tingling, or numbness in both of the arms and legs.

Motor CIDP

Those with motor CIDP experience muscle weakness but no sensory symptoms, like tingling.

Sensory CIDP

Sensory CIDP exhibits symptoms of tingling and/or numbness, while not affecting motor functions.

Distal CIDP

This type of CIDP mostly affects the legs with muscle weakness and sensory loss.

Multifocal CIDP

Multifocal CIDP typically impacts the arms asymmetrically with muscle weakness and sensory loss.

Focal CIDP

Weakness and numbness or tingling happen in only one arm or one leg with focal CIDP.

How is CIDP Diagnosed?

Typically, physicians look for the following when diagnosing CIDP:

  • Muscle weakness
  • Sensory changes like tingling, numbness, or loss of balance
  • Slowed electrical signals in the nerves
  • Elevated protein levels in the spinal fluid

These signs and symptoms are usually identified through a combination of tests.

Blood and Urine Tests

Blood and urine tests are usually conducted to rule out other diagnoses that mimic CIDP. 

Electromyogram (EMG)

EMG tests measure the electrical activity muscles and the nerves that control them. These tests can show abnormalities in the nerves’ response, which can indicate CIDP.

Lumbar Puncture (Spinal Tap)

If blood, urine, or EMG tests are inconclusive, measuring spinal fluid is usually the next option. CIDP usually causes people to have a high amount of certain proteins in their spinal fluid, so a lumbar puncture could be used to help determine their presence.

Nerve Biopsy

Similar to the circumstances warranting a spinal tap, nerve biopsies are performed if other test results are inconclusive. A small piece of nerve tissue is removed from the body and is sent off to be analyzed in a lab.

Magnetic Resonance Imaging (MRI)

For difficult-to-diagnose cases, doctors will turn to MRIs. This machine scans the body with a magnetic field and radio waves to see the nerves in the body and if there is any damage to them.

What Causes CIDP?

The exact trigger of CIDP is not yet known; it is thought that infections, genetics, or other immune system factors may play a role, however. As mentioned earlier, the mechanism behind CIDP involves the immune system attacking the myelin sheaths that cover the peripheral nerves. This results in nerve inflammation and damage, leading to signal disruptions between the nerves and muscles.

How is CIDP Treated with Ig Therapy?

Although CIDP can be helped with corticosteroids and plasma exchange, Ig therapy remains one of the most effective treatment options. 

Immunoglobulin (Ig) Therapy

Immunoglobulin (Ig) therapy works by infusing plasma antibodies into the body; because the immune system attacks the nerves’ myelin sheaths in CIDP, the infused immunoglobulins help modulate the harmful antibodies and regulate the immune system. “Ig therapy helps reduce the immune system’s attack on the peripheral nerves that occurs in CIDP. By decreasing inflammation, it can improve strength, reduce symptoms such as weakness, numbness, and tingling, and help patients maintain or regain their functional abilities,” explains Mohamed Fouda, Regional Nurse Manager at AOM Infusion. 

“While every patient’s response is different, many patients experience improved mobility, independence, and overall quality of life with consistent treatment,” says Fouda. “At AOM, our mission is to provide an exceptional patient experience that improves health and enhances quality of life, and Ig therapy can play a significant role in helping CIDP patients achieve those goals.”

AOM Infusion provides both IVIG and SCIG therapy for appropriate CIDP patients. “We coordinate all aspects of care, including insurance approval support, medication delivery, nursing services, patient education, and ongoing clinical monitoring,” states Fouda. “Our goal is to make therapy as seamless as possible while ensuring patients receive safe, high-quality care in the setting that best fits their clinical needs.”

Intravenous Immunoglobulin (IVIG) Therapy

Approved for treating CIDP in 2008 by the FDA, Intravenous Immunoglobulin (IVIG) therapy has historically been the most commonly used form of Ig therapy for the condition. With this therapy, a nurse inserts a catheter IV into a patient’s vein. Depending on the severity of the CIDP symptoms, the IVIG delivers immunoglobulin into the body for several hours, typically every few weeks.

Subcutaneous Immunoglobulin (SCIG) Therapy

Rather than delivering antibodies intravenously, Subcutaneous Immunoglobulin (SCIG) therapy involves infusion via injection into the locations of the body with fatty tissues, such as the lower belly, thighs, or upper arms. This type of therapy requires shorter, more frequent treatments compared to IVIG.

Is IVIG or SCIG Therapy Better for CIDP?

Both IVIG and SCIG can be effective treatment options for CIDP, but the best choice depends on the individual patient. IVIG is often used when initiating therapy or when larger doses are needed at longer intervals. SCIG may be a good option for stable patients who prefer greater flexibility and independence with more frequent, smaller doses.

“I don’t view one option as universally better than the other,” says Fouda. “The decision should be based on the patient’s clinical condition, treatment goals, lifestyle, and the provider’s recommendation.”

What Should Patients Know Before Starting Ig Therapy?

“Before starting Ig therapy, patients should understand that treatment is intended to help manage symptoms and slow disease progression, but it is not necessarily a cure,” states Fouda. “It is important to discuss their medical history, current medications, and any previous infusion experiences with their healthcare team.”

What Happens During Ig Therapy for CIDP?

At the start, the nurse delivering the Ig therapy might administer pain medication like acetaminophen to help with light headaches or an antihistamine for any allergic reaction. The care team may also recommend having something to drink, as keeping hydrated before and during Ig therapy can help prevent complications.

What happens next depends on the environment in which the Ig therapy is delivered.

Infusion Suites for CIDP Treatment

Infusion suites are a private, low-cost alternative to clinics or hospitals. With these specialty infusion locations, patients are given a room to receive their Ig therapy administered by a nurse. 

After preparing for the infusion, the nurse will deliver the Ig therapy intravenously or subcutaneously. Infusion suites typically offer amenities for patients receiving the therapy. Some of these may include wi-fi so that patients can browse the internet on their devices, televisions they can watch while the infusion is delivered, or snacks they can enjoy for the duration of the treatment. For IVIG, patients may choose to rest in their private treatment room or walk through the infusion suite with their IV stand during their infusion therapy. 

After the patient completes Ig therapy, a nurse will check vital signs like blood pressure, pulse, and temperature to ensure that everything is stable. For the first few treatments, it is recommended to have someone drive you home from the infusion clinic until it is determined how your body reacts to the therapy. 

At-Home Infusion for CIDP Treatment

Patients receiving Ig therapy may also opt for at-home infusion for CIDP. The care team will coordinate with the pharmacy to have the infusion medications delivered to the patient’s home, with instructions on how to store them properly.

Once the nurse arrives at the patient’s home, they complete an initial health assessment, review medications, and discuss any potential side effects. After the treatment begins, the nurse will either stay for the duration or will give you guidance on how to self-infuse. Similar as infusion suites, at-home IVIG therapy allows for patients to move around their homes with their IV stand.

Fouda explains, “Many CIDP patients are excellent candidates for home infusion therapy, and home treatment can offer significant convenience and comfort. Receiving therapy at home can reduce travel burden and allow patients to remain in a familiar environment during treatment.”

“That said,” Fouda continues, “the appropriate site of care should always be determined based on the patient’s clinical situation, infusion history, safety considerations, and provider preference. Some patients may begin treatment in a monitored setting before transitioning to home infusion.”

What Are Other Treatments Available for CIDP?

If traditional Ig therapy isn’t working, Vyvgart is a new option that works in a completely different way. Instead of putting new, healthy donor antibodies into the body like IVIG does, Vyvgart clears out the harmful antibodies. It does this by blocking a specific recycling protein in the body, forcing the immune cells that attack the nerves to be safely broken down and washed away. 

Living with CIDP and Ig Therapy Treatment

Being diagnosed with CIDP and navigating treatment options can be overwhelming. Though some troubling symptoms may come from CIDP, living with it and Ig therapy is manageable.

Handling Common Ig Therapy Side Effects

Although nurses take measures to prevent mild side effects of Ig therapy through pain medication or antihistamines, they still could happen.

IVIG Therapy Side Effects

Some mild side effects for IVIG therapy include:

  • Nausea
  • Headache
  • Fever
  • Pain

A couple of ways to help with these side effects, besides supplementing treatment with pain medication or antihistamines, is to slow the infusion rate and/or add IV fluids.

SCIG Therapy Side Effects

SCIG therapy shares several mild side effects with IVIG therapy:

  • Redness, itching, or swelling at injection site
  • Headache
  • Fatigue
  • Nausea

Staying well hydrated, slowing the infusion rate, and placing a cold compress on the injection site can all help with SCIG side effects.

Adapting to Life Situations with CIDP and Ig Therapy

Living with CIDP can be difficult; however, many find ways to keep enjoying the things that make life fulfilling. To help manage muscle weakness, some people with CIDP perform low-impact activities, such as walking or swimming, or work with a physical therapist to help improve muscle strength. Assistive tools like walkers or braces can also help improve one’s quality of life. Since Ig therapy can be flexible with portable (if stored correctly) medication, it’s easy for those with CIDP to travel.

AOM Infusion Suites or In-Home Infusion for CIDP

Although it is a rare condition, CIDP nonetheless affects thousands of people across the world. Thankfully, it is treatable and manageable. At AOM Infusion, we care for numerous patients across the nation with chronic conditions like CIDP. We’re able to provide our patients options, whether they receive infusion therapy at one of our infusion suites or would rather have their medication administered at home. 

“What sets AOM apart is our commitment to providing a highly coordinated and patient-centered experience,” states Fouda. “We understand the complexities of immunoglobulin therapy and work closely with patients, providers, pharmacists, and nurses to ensure care is delivered safely and efficiently.”

For over 30 years, we’ve proudly provided exceptional care for over 80 complex conditions. With pharmacy licensure in all 50 states, a growing number of infusion suite locations, and an Ig distinction accreditation, we are committed to providing exceptional care with clinical excellence at a higher standard than others.

Our team focuses on timely therapy initiation, experienced infusion nursing support, proactive communication, and individualized patient care. We strive to remove barriers to treatment so patients can focus on their health, and providers can feel confident their patients are well supported.

Want to learn more about our infusion therapy services? Contact our team of experts today!

FAQs

What is chronic inflammatory demyelinating polyneuropathy (CIDP)?

Chronic inflammatory demyelinating polyneuropathy, otherwise known as CIDP, is a rare neurological condition that affects the myelin sheaths on nerves in the human body. With CIDP, the immune system attacks the myelin sheaths, which act as insulators for the electrical currents traveling within the nervous system. When these are damaged, the electrical currents sent to and from the brain are affected. This can cause gradual weakness of the muscles in the body, which is a symptom of CIDP.

What is the difference between CIDP and Guillain-Barré syndrome (GBS)?

The signs and symptoms of CIDP and GBS are very similar. The main differentiator between the two is how quickly the condition onsets and how long it lasts. In CIDP, the myelin sheath damage is gradual over a period of several weeks and can last a long time, which requires ongoing therapy. GBS onset is more sudden; it can progress over hours or days, but is usually an acute event and gets better over the next weeks to months.

What causes CIDP?

Currently, it is not known what causes CIDP or why the immune system attacks the myelin sheaths on nerves. It does not appear to be genetic, and it is not triggered by infections, unlike GBS. More research needs to be performed on what induces the immune response to damage the nerves.

What are some signs and symptoms of CIDP?

Some signs and symptoms of CIDP include:

  • Tingliness or numbness in arms and legs
  • Fatigue
  • Gradual weakening of the arms and legs
  • Loss of balance and difficulty walking
Is CIDP an autoimmune disease?

Yes, CIDP is a rare autoimmune disease due to the immune system mistakenly attacking the myelin sheaths on the body’s nerves.

What is Ig therapy?

Immunoglobulin (Ig) therapy is a treatment consisting of infusing antibodies from healthy plasma into the patient’s body via injection.

What’s the difference between IVIG and SCIG?

Though both IVIG and SCIG are types of Ig therapies, they are different. IVIG stands for intravenous immunoglobulin therapy, in which the antibodies are distributed through the body from an IV injection. SCIG is subcutaneous immunoglobulin therapy, and the antibodies are injected slowly right underneath the skin into fatty tissue.

How does Ig therapy help treat CIDP?

Infused healthy antibodies modulate the patient’s antibodies that are attacking the nerve’s myelin sheaths. This means that they neutralize the attacking antibodies, stopping the damage and allowing for the myelin sheaths to heal.

How quickly does Ig therapy work for CIDP?

Ig therapy typically provides relief to patients typically within days or weeks. To get back to 100% usually requires months of therapy.