Neuromuscular Disorder
IVIG is a safe and effective treatment for neuromuscular disorders such as CIDP, GBS, MG, and MMN. While steroids, plasmapheresis, and immunosuppressants may work initially, they often cause side effects or lose effectiveness. IVIG is generally preferred because it is safer, less invasive, and effective in 70–90% of cases, though long-term periodic infusions are often needed. It is usually well tolerated, with mild side effects like headache or fatigue, and rare serious reactions. IVIG helps reduce inflammation, improve strength, mobility, and recovery, making it a key therapy for these chronic and acute conditions.
Frequently Asked Questions
CIDP is a neurological disorder characterized by gradually (over a time period of months or years) increasing weakness of the legs and arms. It is caused by damage to the protective covering of the nerves, called myelin. Symptoms are variable and may be mild to debilitating. CIDP is treatable with IVIG home infusion therapy.
A patient with CIDP typically presents with difficulty walking which progressively worsens over months to years. Weakness, tingling or other abnormal sensations may also be experienced, and usually begin in the fingers or toes (on both sides of the body). Physical examination will usually show loss of reflexes, such as the knee and ankle jerk. Evaluation by a neurologist will often include an electrical test, a nerve conduction velocity-electromyography study. Your doctor may obtain blood and urine tests, including analysis of proteins, to look for causes of CIDP. CIDP is usually a chronic condition, which means that it may require long-term treatment.
Although the exact cause is unknown, it is believed that the immune system, which is normally protective, perceives myelin as foreign and attacks it. Just what starts this process is not clear. Some patients are found to have abnormal proteins in their blood, and these may facilitate damage. Over time, the destruction of myelin leads to weakness, numbness and tingling in the arms and legs.
Several treatment options are available. These include steroids, plasmapheresis and intravenous immune globulin (IVIG). The goals of treatment are to stop further damage to the myelin, prevent damage to the nerve fibers (axons), alleviate symptoms and prevent relapse, and if possible, create an environment that allows the myelin to regenerate. In patients with CIDP, IVIG has been shown to reduce disability, prevent relapse and even improve quality of life.
CIDP can be treated with steroids, plasmapheresis (PP) and immunosuppressive drugs. Many patients initially respond to these treatments, but develop resistance to the therapy or experience side effects causing the treatments to be stopped. Researchers believe that intravenous immunoglobulin (IVIG) is longer lasting and provides patients with CIDP a safer, more effective alternative to standard therapies for the disease. IVIG is a drug that has been used successfully to treat other immune-related diseases of the nervous system. IVIG and PP seem to be equally effective. IVIG is generally preferred to PP because it is safer, more accessible and less invasive. Long-term treatment with steroids can have serious side-effects. IVIG is effective in 70% to 90% of cases; however, most patients with initial improvement need long-term periodic doses of IVIG to maintain clinical stability[1]. At least five small randomized controlled studies have demonstrated the benefit of IVIG in the majority of patients with CIDP[2-7]. The ICE study is the largest and most recent trial of IVIG to treat CIDP[7]. The study not only confirmed the short-term efficacy of IVIG, but also demonstrated that a maintenance therapy can sustain improvement, increase quality of life over 12 months, and prevent further axonal degeneration[7-10]. The ICE study advocates IVIG as a first-line therapy, and has led to FDA approval of one brand of IVIG. The study has also shown that repeated therapy is usually needed to improve symptoms and then to maintain that improvement. Typical doses recommended repeat every 1 to 6 weeks. Interestingly, a large part of the IVIG treatment responsive patients were able to wean off of therapy after 24 weeks without showing a relapse before the study period ended. So, the amount of medication and the frequency of the need for repeated doses can vary widely from patient to patient and is usually determined by the patient’s response to the drug and their physician’s experience with other similar patients in the past.
IVIG is usually given initially at a dose of 0.4 g/kg per day for 5 days, followed by 1.0 g/kg or less as a single infusion in monthly or bimonthly intervals. The response is assessed after 1 to 2 months[11]. A weekly dosing schedule may also be useful for maintenance therapy in select cases[12].
If you have been diagnosed with CIDP and your physician recommends IVIG therapy, you will receive IVIG therapy on a regular basis. The infusion is usually given intravenously, which means through a needle directly into a vein at a doctor’s office, hospital, or infusion center. You may also be able to arrange to have your infusion at home.
Tolerability of IVIG is usually very good and adverse reactions are usually minor. The most common side effects are headache, nausea, chills, flushing, myalgia, hypotension, hypertension, chest discomfort, and fatigue. Infrequent adverse reactions include thromboembolic events, skin reactions, aseptic meningitis, renal tubular necrosis, and severe anaphylactic reaction.