Neurology

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Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): Signs, Symptoms, and Treatment Options

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Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare autoimmune disorder affecting the peripheral nervous system (where your brain controls all of your muscles and nerves that allow you to move and feel). With CIDP, the body's immune system mistakenly attacks the protective covering of peripheral nerves (the myelin sheath), leading to nerve inflammation and damage.1,2

This makes it hard for nerves to work properly, causing weakness, numbness, and sensory disturbances. While it shares some similarities with Guillain-Barré Syndrome (GBS), CIDP is characterized by a more gradual onset and is therefore considered chronic.3 Here we’ll discuss CIDP signs, symptoms, treatment options, and helpful resources.

Signs and Symptoms

Though signs and symptoms of CIDP can vary widely among people, when someone has CIDP, they might start to feel weak in their muscles—especially in their legs and arms. It's like their body is running out of energy. They might also feel tingling or numbness in their arms and legs, as well as cramping. Sometimes, they might even have trouble keeping their balance or feel tired all the time. These feelings can be uncomfortable and also painful.4

Diagnosis

Finding out if someone has CIDP isn't always easy because it can look like other nerve conditions. A thorough medical history, neurological examination, and diagnostic tests such as nerve conduction studies and electromyography (EMG) are essential for an accurate diagnosis.5 Additionally, cerebrospinal fluid analysis and nerve biopsies may be performed to rule out other conditions.

Treatment Options

Although there is no cure for CIDP, various treatment options can help manage symptoms, slow disease progression, and help people keep doing the things they love. Common treatments include:6,7,8,9,10

  • Intravenous Immunoglobulin (IVIG) Therapy: Healthy antibodies from donors are given to patients through infusions to help suppress the overactive immune response and lower inflammation. This reduces symptoms for many patients.

  • Corticosteroids: High-dose corticosteroids such as prednisone may be prescribed to suppress inflammation and alleviate symptoms, particularly during flare-ups.

  • Plasma Exchange (Plasmapheresis): This procedure involves removing and replacing plasma to remove harmful antibodies and inflammatory factors from the bloodstream, providing temporary relief from CIDP symptoms.

  • Immunosuppressive Medications: Drugs like azathioprine, cyclophosphamide, or rituximab may be prescribed to dampen the immune system's activity and reduce inflammation, particularly in individuals who do not respond to other treatments.

  • Physical Therapy: Rehabilitation exercises and physical therapy play a crucial role in managing CIDP, helping to improve muscle strength, flexibility, and mobility and reducing the risk of complications such as falls and injuries.

Helpful Resources

The GBS/CIDP Foundation International provides support and resources for CIDP patients and their families. Some of these resources include: 

  • Local community groups and chapter meetings

Summary

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is a rare but debilitating autoimmune disorder affecting the peripheral nervous system. While there is no cure for CIDP, by knowing what to look for and getting the right treatment, people with CIDP can keep living their lives and doing the things they love. Through ongoing research and advancements in medical care, there is hope for better outcomes and increased understanding of this complex condition.

How BioMatrix Can Help

Though a CIDP diagnosis can feel overwhelming for you or a loved one, it’s important to know that you are not alone. Organizations like GBS/CIDP Foundation International mentioned above can provide a wealth of information and support as well as connect you to others in the community who have experienced the same diagnosis. In addition, your specialty pharmacy can offer individualized support to help manage treatment.

BioMatrix helps manage the individual needs of patients requiring IVIG therapy. Knowledgeable pharmacists and care coordination staff guide each patient through the potential medication side effects and, working with the prescribing physician, help manage treatment to reduce the prevalence and severity of relapses.

As a specialty infusion pharmacy, our clinical teams have extensive training and experience in rare diseases, infusion therapies, and complex medical conditions. Our nursing staff coordinates with pharmacists, healthcare providers, and other caregivers ensuring the best treatment outcomes possible.

Learn more about our individualized specialty pharmacy services for patients with CIDP and other neurological conditions.

Insurance Appeal Letter Sample & Template

Have you been denied insurance coverage for much needed treatment? Use this appeal letter template as a guide to help you or a loved one appeal insurance claim denials.

DISCLAIMER: THIS IS NOT MEDICAL OR LEGAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider or as legal advice. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

References

  1. National Institute of Neurological Disorders and Stroke. (2019). Chronic Inflammatory Demyelinating Polyneuropathy Information Page. https://www.ninds.nih.gov/Disorders/All-Disorders/Chronic-Inflammatory-Demyelinating-Polyneuropathy-Information-Page

  2. Dalakas, M. C. (2014). Advances in the diagnosis, pathogenesis and treatment of CIDP. Nature Reviews Neurology, 10(3), 160-171.

  3. Markey, K. A., et al. (2018). CIDP and variants as a disease continuum: Evidence from nerve histology. Neurology, 90(13), e1157-e1166.

  4. Gorson, K. C. (2014). An update on the management of chronic inflammatory demyelinating polyneuropathy. Therapeutic Advances in Neurological Disorders, 7(5), 227-237.

  5. Van den Bergh, P. Y., et al. (2010). European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society–first revision. European Journal of Neurology, 17(3), 356-363.

  6. Hughes, R. A., et al. (2006). Intravenous immunoglobulin (IVIg) for CIDP and related disorders. Cochrane Database of Systematic Reviews, 2, CD002062.

  7. Gorson, K. C. (2010). An update on the management of chronic inflammatory demyelinating polyneuropathy. Therapeutic Advances in Neurological Disorders, 3(5), 249-258.

  8. Léger, J. M., et al. (2011). Plasmapheresis in chronic inflammatory demyelinating polyneuropathy: A randomized, double-blind, sham-controlled study. Brain, 134(7), 1889-1899.

  9. Mahdi-Rogers, M., & Hughes, R. A. (2017). Immunosuppressive agents for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database of Systematic Reviews, 8, CD003557.

  10. Latov, N., et al. (2010). Peripheral neuropathy: Diagnosis and management. Cambridge University Press.

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Enhancing Transplant Patient Care: The Vital Role of Qualified Home Nursing Services in Home Infusion

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An older white woman sits next to a smiling Asian nurse.

Home infusion therapy has transformed healthcare by allowing patients to receive treatment in the comfort of their own homes. For individuals undergoing organ transplant procedures, this plays a pivotal role in their recovery and ongoing care. Here we discuss how the success of home infusion, particularly for transplant patients, hinges significantly on the expertise and support provided by qualified home nursing services.

The Significance of Home Infusion for Transplant Patients

Transplant patients benefit from home infusion both before and after transplant. Before transplant, patients are dealing with chronic complications that have caused them to need a transplant. Getting their immune systems prepared can require timed medication therapies such as desensitization. Post-transplant, patients are at risk of infection because of immune-suppressing anti-rejection drugs and/or because they develop chronic or long-term conditions compromising their immune systems.

Hospitals and infusion centers can take every precaution available, yet in these environments patients still have the potential for exposure to bacteria or viruses they would likely not encounter in their homes. Home infusion provides a safe and effective means to help transplant patients manage their prescribed therapy. Patients can receive their medication in the secure and familiar environment of their own homes, administered by a clinically-trained infusion nurse. Benefits include safety, convenience, cost-savings, and patient well being. 

Challenges and the Need for Qualified Home Nursing Services

The complexity of post-transplant care demands meticulous attention and specialized knowledge. This is where qualified home nursing services come into play. These professionals bring a wealth of expertise providing personalized care, monitoring patients' health, administering medications, and offering vital guidance to patients and their families.

The expertise of qualified home nurses extends beyond administering medications. They serve as educators, ensuring that patients and their caregivers understand the treatment protocols, potential side effects, and the importance of adherence to prescribed regimens. Their vigilance in monitoring for any signs of complications or adverse reactions is crucial in preempting potential issues and ensuring timely interventions.

Extensive Vetting and Training

BioMatrix ensures a robust network of over 200 contracted nursing agencies nationwide, all meticulously vetted to align with Infusion Nurses Society guidelines. Similarly, individual home infusion nurses undergo a thorough assessment and preparation, including evaluations of their competency and detailed reviews of prescribed therapy. Prior to service, these nurses meet with BioMatrix nurse clinicians, ensuring a suitable match for the patient's needs. Should any nurse not meet their stringent competency standards, BioMatrix promptly reassigns the case to maintain their commitment to exceptional care.

Site-of-Care Coordination

We are well aware of how important it is for patients to avoid conflicts with work and other obligations. BioMatrix nurse clinicians aim to create plans that stay within the parameters of all protocols and instructions that their MD has ordered while causing the least possible disruption to patients' routines. When administering in-home with assistance from one of our home care nurses, our nurses work with patients and prescribers to make therapy administration as safe, convenient, and comfortable as possible. 

Safety Protocols and Clinical Interventions

All BioMatrix nurse professionals follow CDC guidelines for hygiene and germ reduction and help patients mitigate any issues to establish a safe environment for home infusion. When entering a patient’s home, the home infusion nurses follow all standard precautions and wear appropriate personal protective equipment. They also take the time to identify and review safety measures the patient can follow in the home both during and after infusion.

Following each home infusion, the home infusion nurse will submit a report to the BioMatrix clinical team to track response to therapy, monitor for adverse events, and help personalize and improve future care. By synthesizing clinical, social, and drug utilization information, our nursing team’s actionable interventions help improve health and save lives. Our interventions support patient adherence to therapy, reduce side effects, and help address both critical and every day issues related to life with a chronic health condition. 

Patient Education

We understand that starting a new therapy and navigating the treatment process can be challenging and confusing for a patient. Where appropriate, our nursing team provides self-administration training for injectable or infusible drugs, allowing patients to more independently manage their condition. Our nurses can also guide patients post administration to maintain therapy adherence, minimize or manage side effects, and answer questions that may arise throughout their treatment regimen.

The BioMatrix clinical team includes compassionate nurses who have extensive training and experience with rare diseases, infusion therapies, and complex medical conditions. 

Our nurses work together with patients, caregivers, pharmacists, and prescribers to coordinate the optimal site of care, conduct nursing interventions, and provide patient education.

Watch our video here to learn more about our home infusion services.

DISCLAIMER: THIS IS NOT MEDICAL OR LEGAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider or as legal advice. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.

Financial Resource Guide

Living with a chronic condition can create additional healthcare costs while also impeding one’s ability to work. Our financial resource guide can help. 

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

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Lambert-Eaton Myasthenic Syndrome (LEMS): Signs, Symptoms, and Treatment Options

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With an estimated 400 known cases in the U.S.1, Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare, autoimmune, neurological disorder in which the body's immune system attacks its own tissues. With LEMS, the body specifically attacks the neuromuscular junction—i.e. the connections between nerves and muscles. This causes weakness in the upper legs, hips, upper arms, and shoulders. Both walking and self-care can be difficult.

More than 50% of LEMS cases happen in middle aged or older people, with symptoms often occurring prior to the diagnosis of lung cancer.2 Because cancer cells share some of the same proteins as nerve endings, it’s thought that the body attacks nerve endings in an attempt to kill cancer cells. In the other half of LEMS cases, the cause is unknown and typically occurs around age 35.

Signs and Symptoms

LEMS is characterized by weakness starting in the legs and hips, progressing to the arms and shoulders. Onset of signs and symptoms is typically gradual. Additional signs and symptoms may include:

  • Muscle aches

  • Muscle weakness that gets worse with time

  • Fatigue

  • Difficulty walking and climbing stairs

  • Difficulty lifting objects and raising arms

  • Drooping eyelids

  • Dry eyes

  • Dry mouth

  • Blurred vision

  • Difficulty swallowing

  • Dizziness upon standing

  • Constipation

  • Erectile dysfunction

It’s important to note that because of similar symptoms of muscle weakness, LEMS is often misdiagnosed as Myasthenia gravis (MG). However key differences are in the severity and type of muscle weakness. For example, eye weakness tends to be milder in LEMS patients and unlike MG, is typically not the only symptom. Severe respiratory muscle weakness found in MG is also rare for people with LEMS.

Treatment Options

Although there is no cure for LEMS, treatment can help relieve and lessen symptoms. If LEMS is connected to a cancer diagnosis, treatment is first targeted at the cancer which may then greatly improve LEMS symptoms.

Medicines to help nerve signals reach the muscles as well as immunosuppressants (like steroids) can help relieve symptoms. If symptoms are advancing and other treatments have not helped, both plasmapheresis (plasma exchange) and intravenous immunoglobulin (IVIG) are used to treat LEMS. 

Plasmapheresis redirects blood through a machine which filters out the antibodies attacking the nerves. IVIG, which is immunoglobulin given intravenously or through a vein, suppresses the inflammatory response. Derived from thousands of healthy blood plasma donations, immunoglobulin therapy can help suppress an overactive immune system by preventing it from attacking healthy cells.

Helpful Resources

The Muscular Dystrophy Association provides support, education, care, and advocacy for patients with neuromuscular diseases, like LEMS, and their families and/or caretakers. Some of these resources include: 

Community resources like access to transportation, clinical trials finder tool, and equipment assistance

How BioMatrix Can Help

Though an LEMS diagnosis can feel overwhelming for you or a loved one, it’s important to know that you are not alone. Organizations like the Muscular Dystrophy Association mentioned above can provide a wealth of information and support as well as connect you to others in the community who have experienced the same diagnosis as well as others with neuromuscular conditions. In addition, your specialty pharmacy can offer individualized support to help manage treatment.

BioMatrix helps manage the individual needs of patients requiring IVIG therapy. Knowledgeable pharmacists and care coordination staff guide each patient through the potential medication side effects and, working with the prescribing physician, help manage treatment to reduce the prevalence and severity of symptoms.

The BioMatrix clinical team includes compassionate nurses who have extensive training and experience with rare diseases, infusion therapies, and complex medical conditions. Our nurses work together with patients, caregivers, pharmacists, and prescribers to coordinate the optimal site of care, conduct nursing interventions, and provide patient education.

Learn more about how our individualized specialty pharmacy services for patients with LEMS and other neurological and neuromuscular conditions.

DISCLAIMER: THIS IS NOT MEDICAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

References

  1. (2019). Lambert-Eaton Myasthenic Syndrome. National Organization for Rare Disorders (NORD). https://rarediseases.org/rare-diseases/lambert-eaton-myasthenic-syndrome/

  2. (2023). Jayarangaiah A, Theetha Kariyanna P. Lambert-Eaton Myasthenic Syndrome. National Library of Medicine (NIH). https://www.ncbi.nlm.nih.gov/books/NBK507891/

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Guillain-Barré syndrome (GBS): Signs, Symptoms, and Treatment Options

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With an estimated 3,000 to 6,000 people in the U.S. developing Guillain-Barré syndrome (GBS) every year1, GBS is a rare neurological disorder in which the body's immune system attacks its own nerves causing muscle weakness and sometimes complete paralysis. It’s most common between ages 30-50, though onset can happen at any age.2

Though the cause of GBS is not fully understood, two-thirds of patients report a viral or bacterial infection in the weeks prior to GBS symptoms—typically gastrointestinal or respiratory. Evidence suggests that this infection triggers the immune response which damages the myelin sheath that protects the nerves, leading to numbness and weakness. Most people recover fully from GBS and are able to walk within 6 months to two years of when symptoms began. However, some have lasting effects, and for some, the disease can be fatal.3

Signs and Symptoms

Early signs of GBS often include a rapid onset of tingling, weakness, and numbness that start in the feet and legs and spread to the upper body. Some people notice these symptoms first in their arms or face. While initial weakness may be mild, symptoms can progress rapidly over just a few days. Additional signs and symptoms may include:

  • Difficulty raising a foot or walking without assistance

  • Paralysis or the loss of ability to move one’s legs, arms, breathing muscles, and face

  • Paralysis that travels up the limbs from fingers and toes towards the torso

  • Loss of reflexes such as the knee jerk

  • Pain in the muscles

  • Blurred vision

  • Difficulty swallowing or chewing

  • Difficulty speaking

  • Shortness of breath or difficulty breathing

  • Low or high blood pressure

  • Difficulty with bladder control or bowel function

  • Rapid heart rate

It’s important to note that not all patients with GBS experience paralysis. Some with mild cases may just experience tingling and weakness throughout their body.

Treatment Options

For some with mild symptoms, no treatment is necessary. For most new cases of GBS, however, patients need to be hospitalized. To monitor breathing and other body functions, patients are typically admitted to the ICU (Intensive Care) until the condition is stabilized. Although there is no cure for GBS, immune-suppressing treatments can prevent breathing problems and relieve symptoms by reducing the inflammation caused by the immune system’s response to the disease. Both plasmapheresis (plasma exchange) and intravenous immunoglobulin (IVIG) are used to treat GBS, and IVIG suppresses the inflammatory response.

Derived from thousands of healthy blood plasma donations, immunoglobulin therapy can help suppress an overactive immune system by preventing it from attacking healthy cells. Immunoglobulin given intravenously, or through a vein, is called intravenous immunoglobulin (IVIG).

Helpful Resources

The GBS/CIDP Foundation International provides support and resources for GBS patients and their families. Some of these resources include:

  • Local community groups and chapter meetings

  • Community forums

  • GPS educational webinars and conferences

  • Rehabilitation guide for caregivers

How BioMatrix Can Help

Though a GBS diagnosis can feel overwhelming for you or a loved one, it’s important to know that you are not alone. Organizations like GBS/CIDP Foundation International mentioned above can provide a wealth of information and support as well as connect you to others in the community who have experienced the same diagnosis. In addition, your specialty pharmacy can offer individualized support to help manage treatment.

BioMatrix helps manage the individual needs of patients requiring IVIG therapy. Knowledgeable pharmacists and care coordination staff guide each patient through the potential medication side effects and, working with the prescribing physician, help manage treatment to reduce the prevalence and severity of relapses.

The BioMatrix clinical team includes compassionate nurses who have extensive training and experience with rare diseases, infusion therapies, and complex medical conditions. Our nurses work together with patients, caregivers, pharmacists, and prescribers to coordinate the optimal site of care, conduct nursing interventions, and provide patient education.

Learn more about our individualized specialty pharmacy services for patients with GPS and other neurological conditions.

Insurance Appeal Letter Sample & Template

Have you been denied insurance coverage for much needed treatment? Use this appeal letter template as a guide to help you or a loved one appeal insurance claim denials.

DISCLAIMER: THIS IS NOT MEDICAL OR LEGAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider or as legal advice. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

References

  1. (2023). Guillain-Barré Syndrome and Vaccines. CDC. https://www.cdc.gov/vaccinesafety/concerns/guillain-barre-syndrome.html

  2. Guillain-Barré Syndrome. Mount Sinai Today Blog. https://www.mountsinai.org/health-library/diseases-conditions/guillain-barr-syndrome

  3. (2022). Guillain-Barré Syndrome. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/guillain-barre-syndrome/symptoms-causes/syc-20362793

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Myasthenia Gravis (MG): Signs, Symptoms, Positive Self-Management, and Treatment Options

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Myasthenia gravis (MG) is a rare, autoimmune neuromuscular disease that’s characterized by weakness in the voluntary muscles (muscles you can control) such as the eyes, face, jaw, neck, arms, and legs. With MG, the immune system attacks the communication between the nerve signals and the muscles, causing weakness and loss of control of these muscles.

Though MG can occur at any age, the onset of symptoms most commonly happens in women under 40 and men over 60.1

Signs and Symptoms

Ocular weakness is typically one of the first signs of MG. This is when muscles that control eye and eyelid movement cause a partial paralysis of eye movements, double vision, and droopy eyelids. Roughly half of those who first experience these symptoms will go on to develop muscle weakness and/or fatigue in the rest of their body within two years—typically in their neck and arms first, then their legs.2 Lifting one’s arms over their head, standing up from a seated position, walking long distances, and climbing stairs may become more and more difficult. MG symptoms can appear suddenly with rapid fatigue and loss of muscle control.

Positive Self Management

The Myasthenia Gravis Foundation provides a wealth of resources for MG patients and their families including disease education, research, community events, and wellness strategies. In addition to a patient’s medical/treatment protocol, wellness strategies and positive self management can improve day-to-day quality of life for those living with MG. According to the Myasthenia Gravis Foundation, some of those strategies include:

  • Sharing facts about MG with family, friends, and coworkers as you feel comfortable

  • Learning ways to cope

  • Conserving energy

  • Staying cool

  • Accepting help

  • Potentially recovering some strength through low impact movement and exercise

  • Proper nutrition and diet modification to help with chewing and swallowing

  • Learning your rights in the workplace

You can learn more and dive deeper into these wellness strategies here.

Treatment Options

Although there is no cure for MG, treatment can help relieve and lessen symptoms. The type of treatment depends on age, disease severity, and how fast it’s progressing. Medications like cholinesterase inhibitors can improve muscle contraction and muscle strength in some people. Corticosteroids, like prednisone, can lessen the immune system attacks. Immunosuppressants can help alter the immune system. All of these medications, however, can cause serious side effects with prolonged use.3

Infusion treatments such as IVIG and other infused medications are typically used to treat worsening MG symptoms. Because medication is infused directly into the bloodstream, a healthcare professional should be present to administer the infusion and monitor the patient for side effects.

How BioMatrix Can Help

BioMatrix helps manage the individual needs of patients requiring infused medications by providing options for administration site of care, education, and support to help improve quality of life. Knowledgeable pharmacists and care coordination staff guide each patient through the potential medication side effects and, working with the prescribing physician, help manage treatment to reduce the prevalence and severity of relapses.

The BioMatrix clinical team includes compassionate nurses who have extensive training and experience with rare diseases, infusion therapies, and complex medical conditions. Our nurses work together with patients, caregivers, pharmacists, and prescribers to coordinate the optimal site of care (including home infusion), conduct nursing interventions, and provide patient education.

Learn more about our individualized specialty pharmacy services for patients with MG and other neurological disorders.

DISCLAIMER: THIS IS NOT MEDICAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

References

  1. (2023). Myasthenia Gravis. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/health-information/disorders/myasthenia-gravis

  2. Myasthenia Gravis (MG) Signs and Symptoms. Muscular Dystrophy Association. https://www.mda.org/disease/myasthenia-gravis/signs-and-symptoms

  3. (2021). Myasthenia Gravis Diagnosis & Treatment. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/myasthenia-gravis/diagnosis-treatment/drc-20352040

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Polymyositis and Dermatomyositis: Signs, Symptoms, and Treatment Options

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More than 50,000 people in the U.S. are living with myositis, and roughly 7,000 people are diagnosed every year.1 Myositis is a neurological, autoimmune disease that causes chronic inflammation of the skeletal muscles due to cell damage. This inflammation and swelling can cause muscle weakness and pain as well as skin conditions.

There are different types of myositis. While polymyositis affects just muscles, dermatomyositis affects both muscles and skin. We’ll examine both polymyositis and dermatomyositis more below.

Polymyositis Signs and Symptoms

Polymyositis is characterized by muscle weakness and pain particularly in the abdomen, shoulders, upper arms, and hips. Muscles in the center of the body tend to be affected the most, though any skeletal muscles can be affected. The skeletal muscles are connected to your bones, allowing you to control movements. When these muscles are inflamed and damaged, it can affect the ability to walk, run, sit, and stand. This inflammation can also cause challenges for eating and breathing. 

Polymyositis signs and symptoms include:

  • Muscle pain

  • Muscle stiffness

  • Muscle weakness

  • Joint pain and stiffness

  • Trouble breathing

  • Difficulty swallowing

  • Inflammation of the heart causing irregular heart rhythms

Dermatomyositis Signs and Symptoms

In addition to affecting the muscles, dermatomyositis also affects the skin. In addition to signs and symptoms of polymyositis involving muscle weakness and pain, symptoms of dermatomyositis include:2

  • Painful, itchy red or purple rash on sun exposed areas

  • Red or purple swelling of upper eyelids

  • Red or purple spots on knuckles, elbows, knees, and toes

  • Joint pain in cold conditions that lessons in warmer conditions

  • Scaly, dry skin that can cause hair thinning

  • Surrounding areas of fingernails that get swollen and red

  • Hard lumps under the skin caused by calcium deposits

Polymyositis and Dermatomyositis Treatment Options

Though there is no cure for polymyositis or dermatomyositis, symptoms can be managed. Treatment may include anti-inflammatory medicines like steroids or corticosteroids and/or immunosuppressive medicines that suppress the body’s immune system. Rituxan, an infusion therapy, is typically prescribed if anti-inflammatory medicines don’t provide adequate control of symptoms. Immune Globulin (IG) therapy may also be prescribed if there’s no response to other treatments. Administered via an infusion, IG therapy consists of donated blood products that help boost your body's immune system. Antihistamine drugs or anti-inflammatory steroid creams may also be prescribed for skin rashes.

In addition to treatment, physical therapy, heat therapy, rest, and/or special devices can help support muscles and movement. Avoiding sun exposure and wearing sunscreen can also help prevent skin rashes.

How BioMatrix Can Help

The treatment of neurological disorders using infusion therapies has become the standard of care in many neurological practices throughout the United States. BioMatrix helps manage the individual needs of patients needing infusions, providing education and support to help improve quality of life. Knowledgeable pharmacists and care coordination staff guide each patient through the potential medication side effects and, working with the prescribing physician, help manage treatment to reduce the prevalence and severity of relapses.

The BioMatrix clinical team includes compassionate nurses who have extensive training and experience with rare diseases, infusion therapies, and complex medical conditions. Our nurses work together with patients, caregivers, pharmacists, and prescribers to coordinate the optimal site of care (including home infusion), conduct nursing interventions, and provide patient education.

Learn more about our individualized specialty pharmacy services for patients with neurological disorders.

DISCLAIMER: THIS IS NOT MEDICAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

References

  1. (2017). Facts About Myositis Infographic. Hospital for Special Surgery (HSS). https://www.hss.edu/conditions_infographic-facts-about-myositis.asp#

  2. Dermatomyositis. John Hopkins Medicine. https://www.hopkinsmedicine.org/health/conditions-and-diseases

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Multiple Sclerosis (MS): Signs, Symptoms, Positive Self-Management, and Treatment Options

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Multiple sclerosis (MS) is a disease of the central nervous system (CNS)—i.e. the brain, spinal cord, and optic nerves. The CNS, which controls the entire body, is attacked by the body’s own immune system, damaging the protective layer, or myelin, that insulates the wire-like nerve fibers.

This nerve damage disrupts signals to and from the brain. MS is an immune-mediated disease, which is when the body’s immune system overreacts and attacks itself. Though similar, an immune-mediated disease is different from an autoimmune disease. Both involve the immune system attacking and damaging the body’s own healthy cells via proteins (or autoantigens—antigens produced by one’s own body). However with an autoimmune disease, the proteins/autoantigens which attack the cells have been identified. With an immune-mediated disease, these proteins/autoantigens have not been identified.1

Signs and Symptoms

Though everyone’s experience with MS is different, there are some common signs and symptoms to look out for. These include:3

  • Numbness or weakness in one or more limbs, typically occurring on one side of the body at a time

  • Tingling

  • Electric-shock sensations that occur with certain neck movements, especially when bending the neck forward (Lhermitte sign)

  • Lack of coordination

  • Unsteady gait (having trouble with balance) or inability to walk

  • Partial or complete loss of vision, usually in one eye at a time with pain during eye movement

  • Prolonged double vision

  • Blurry vision

  • Vertigo

  • Problems with sexual, bowel, and bladder function

  • Fatigue

  • Slurred speech

  • Cognitive problems

  • Mood disturbances

Positive Self Management

In addition to a patient’s medical/treatment protocol, positive self management can improve day-to-day quality of life for those living with MS. Patients who self-manage their condition have the skillset to accept and communicate with others their need to move at their own pace. Research has shown that those who have developed self-management skills have more confidence to better communicate their needs to others, therefore receiving improved support.4 Found to be an empowering strategy to improve health for many people living with chronic conditions, self management is a philosophy that acknowledges living with a condition like MS is an ongoing experience.

The National Multiple Sclerosis Society and Multiple Sclerosis Foundation both give resources for developing positive self management skills. 

The National Multiple Sclerosis Society has a section on their website dedicated to “Living Well with MS”. They provide guidance and resources for diet, exercise, and healthy behaviors; emotional well-being (i.e. managing stress and coping skills); spiritual well-being (i.e. building on values and beliefs that provide purpose); and cognitive health (i.e. keeping your mind engaged and challenged). Learn more about these resources here. 

The Multiple Sclerosis Foundation is helping MS patients get connected to support groups and grants. Some of their grants and programs include help with rent or utilities, homecare assistance, and transportation. They also host multiple events for the MS community to join together and learn more about managing their condition. To learn more about these resources and events, click here.

Treatment Options

For some with mild symptoms, no treatment is necessary. For others, treatment to help ease MS symptoms may include corticosteroids or plasma exchange. Although there is no cure for MS, treatment can also help slow disease progression by reducing the amount of damage and scarring to the myelin—the layer surrounding the nerves. This reduction in damage can help MS patients have fewer and less severe relapses. Treatment to slow progression may include injectable, oral, and infusion medications. 

Infusion treatments may help slow the progression of MS and lessen flare-ups for those with aggressive or advanced MS. Because medication is infused directly into the bloodstream, a healthcare professional should be present to administer the infusion and monitor the patient for side effects.

How BioMatrix Can Help

BioMatrix helps manage the individual needs of patients requiring infused medications by providing options for administration site of care, education, and support to help improve quality of life. Knowledgeable pharmacists and care coordination staff guide each patient through the potential medication side effects and, working with the prescribing physician, help manage treatment to reduce the prevalence and severity of relapses.

The BioMatrix clinical team includes compassionate nurses who have extensive training and experience with rare diseases, infusion therapies, and complex medical conditions. Our nurses work together with patients, caregivers, pharmacists, and prescribers to coordinate the optimal site of care (including home infusion), conduct nursing interventions, and provide patient education.

Learn more about how our individualized specialty pharmacy services for MS patients.

Financial Resource Guide

Living with a chronic condition can create additional healthcare costs while also impeding one’s ability to work. Our financial resource guide can help. 

DISCLAIMER: THIS IS NOT MEDICAL OR LEGAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider or as legal advice. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

References

  1. What is an immune-mediated disease? The National Multiple Sclerosis Society. https://www.nationalmssociety.org/What-is-MS/Definition-of-MS/Immune-mediated-disease

  2. About Multiple Sclerosis (MS). Penn Medicine. https://www.pennmedicine.org/for-patients-and-visitors/patient-information/conditions-treated-a-to-z/multiple-sclerosis-ms

  3. (2022). Multiple Sclerosis. Mayo Foundation for Medical Education and Research (MFMER). https://www.mayoclinic.org/diseases-conditions/multiple-sclerosis/symptoms-causes/syc-20350269

  4. (2010). Wazenkewitz, J., McMullen, K., Ehde, D. Self-Management: Keys to Taking Charge of Your MS. Multiple Sclerosis Foundation.

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Neurological Disorders and Intravenous Immunoglobulin (IVIG) Therapy

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Intravenous immunoglobulin (IVIG) is a type of immunotherapy that has been used for nearly 30 years to treat neurological disorders. IVIG was first approved by US regulatory agencies to treat chronic inflammatory demyelinating polyneuropathy (CIDP), Guillain-Barré syndrome (GBS), and multifocal motor neuropathy (MMN) in 2008. In 2021, IVIG was approved to treat dermatomyositis.1

Neurological conditions for which IVIG currently has indicated use includes CIDP, severe dermatomyositis/polymyositis, Kawasaki syndrome, and multifocal motor neuropathy. IVIG has off-label use for Guillain-Barré syndrome and myasthenia gravis.2 While just as effective as plasmapheresis and steroids, unlike plasmapheresis and steroids, IVIG is found to be safe and effective long term.3

What is intravenous immunoglobulin (IVIG)?

Immunoglobulin (IG) given intravenously, or through a vein, is called intravenous immunoglobulin (IVIG). Immunoglobulin is a human blood product containing proteins that likely link themselves with antibodies or other substances directed at the nerve.4 It’s made from the donated antibodies of between 1,000-15,000 human donors per batch.5 The human body has different antibodies to fight different infections, like how there are different keys for different locks. If the body does not have enough antibodies or has damaged antibodies, IVIG can help replace them.

Where can a patient receive IVIG therapy?

A patient can typically receive IVIG therapy in their home or physician’s office. At BioMatrix, our nursing team coordinates the best site of care for scheduled infusions. For many patients needing regular infusions, choosing home as their site of care is a convenient and comfortable option. Research indicates that home infusion is a safe method of receiving IVIG therapy for neurological conditions.6,7,8

Additional benefits to home infusion include:

  • Less risk of infection. Hospitals and infusion centers can take every precaution available, yet in these environments patients still have the potential for exposure to bacteria or viruses they would likely not encounter in their homes.
  • Cost savings. Home infusion reduces the burden on costlier sites of care, saving money for both patients and payers.9
  • Patient wellbeing. Patients report significantly better physical and mental wellbeing with less disruption to daily activities, overwhelmingly preferring home infusion.10

Summary

The treatment of Neurological disorders using IVIG has become the standard of care in many neurological practices throughout the United States. BioMatrix provides site-of-care options for IVIG that offer convenience for patients and cost-savings to insurance providers. 

As a national provider of IG, BioMatrix also has broad access to brands and inventory supply. We purchase IG directly from manufacturers or manufacturers’ approved distributors, ensuring distribution channel integrity, proper handling, and quality control. 

To learn more about our IVIG capabilities to treat neurological conditions, visit:

DISCLAIMER: THIS IS NOT MEDICAL OR LEGAL ADVICE. All information, content, and material is for informational purposes only and is not intended to serve as a substitute for the consultation, diagnosis, and/or medical treatment of a qualified physician or healthcare provider or as legal advice. Please consult a physician or other health care professional for your specific health care and/or medical needs or concerns and never disregard professional medical advice or delay in seeking it because of something you have read here or on our website.

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

By giving us your contact information and signing up to receive this content, you'll also be receiving marketing materials by email. You can unsubscribe at any time. We value your privacy. Our mailing list is private and will never be sold or shared with a third party. Review our Privacy Policy here.

References

  1. Dalakas M. C. (2021). Update on Intravenous Immunoglobulin in Neurology: Modulating Neuro-autoimmunity, Evolving Factors on Efficacy and Dosing and Challenges on Stopping Chronic IVIg Therapy. Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics, 18(4), 2397–2418. https://doi.org/10.1007/s13311-021-01108-4. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8585501/#:~:text=IVIg%20inhibits%20the%20differentiation%20and,IVIg%2Dresponding%20autoimmune%20neurological%20diseases.

  2. (2023). Indications. Lexicomp. https://online.lexi.com/lco/action/doc/retrieve/docid/fc_dfc/6647276?cesid=1cTY1wl5C8I&searchUrl=%2Flco%2Faction%2Fsearch%3Fq%3DIVIG%26t%3Dname%26acs%3Dfalse%26acq%3DIVIG%26nq%3Dtrue

  3. Zandman-Goddard, G., Krauthammer, A., Levy, Y., Langevitz, P., & Shoenfeld, Y. (2012). Long-term therapy with intravenous immunoglobulin is beneficial in patients with autoimmune diseases. Clinical reviews in allergy & immunology, 42(2), 247–255. https://doi.org/10.1007/s12016-011-8278-7. https://pubmed.ncbi.nlm.nih.gov/21732045/

  4. Shehata N. (2023) Patient education: Intravenous immune globulin (IVIG) (Beyond the Basics). UpToDate. https://www.uptodate.com/contents/intravenous-immune-globulin-ivig-beyond-the-basics#:~:text=WHAT%20IS%20IVIG%3F,to%20help%20you%20fight%20infection

  5. Jolles S, Sewell WA, Misbah SA. Clinical uses of intravenous immunoglobulin. Clin Exp Immunol. 2005 Oct;142(1):1-11. doi: 10.1111/j.1365-2249.2005.02834.x. PMID: 16178850; PMCID: PMC1809480.

  6. Le Masson G, Solé G, Desnuelle C, et al. (2018). Home versus hospital immunoglobulin treatment for autoimmune neuropathies: a cost minimization analysis. Brain Behav. 2018;8(2):e00923. doi: 10.1002/brb3.923

  7. Luthra R, Quimbo R, Iyer R, Luo M. (2014). An analysis of intravenous immunoglobin site of care: home versus outpatient hospital. Am J Pharm Benefits. 2014;6(2):e41-e49.

  8. Schmidt R. (2012). Home Infusion Therapy: Safety, Efficacy, and Cost-Savings. PSQH. https://www.psqh.com/analysis/home-infusion-therapy-safety-efficacy-and-cost-savings/

  9. Home Infusion Creates Savings for Patients, Taxpayers. NHIA. https://nhia.org/wp-content/uploads/2020/03/Home_Infusion_Creates_Savings_for_Patients_Taxpayers.pdf

  10. Polinski J, Kowal M, Gagnon M, Brennan T, Shrank W. (2016). Home infusion: Safe, clinically effective, patient preferred, and cost saving. NIH. https://pubmed.ncbi.nlm.nih.gov/28668202/

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Support Resources for Patients Taking Austedo® (Deutetrabenazine)

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At BioMatrix, we provide individualized specialty pharmacy services and support for patients requiring specialty and infused medications. We empower patients to live each day to its fullest potential.

Here we provide support resources for patients taking Austedo. This information is intended for educational purposes only and should not replace directives from your physician or pharmacist. Refer to Austedo’s Prescribing Information1, Medication Guide2, and website3 for more information. Never make any adjustments to your treatment plan without speaking to your physician or pharmacist first.

Drug Profile and Indicated Use

According to the Austedo Prescribing Information1 and its website3, Austedo is a vesicular monoamine transporter 2 (VMAT2) inhibitor indicated in adults for the treatment of the involuntary movements (chorea) of Huntington’s disease and movements in the face, tongue, or other body parts that cannot be controlled (tardive dyskinesia).

It is not known if Austedo is safe and effective for children.

Support Resources for Patients Taking Austedo

The Austedo website claims that roughly 90% of patients pay $10 or less per month for Austedo.4 Their financial assistance programs include:

  • Austedo Copay Card: As little as $0 copay per month for eligible, commercially insured patients*

  • Austedo Free Trial Voucher: Offer available for patients new to Austedo (includes sampled patients)

  • Low-income subsidy (LIS): Medicare Part D patients who qualify for and utilize the LIS may pay as little as $9.85 per month for Austedo

To enroll for the free trial voucher or the copay card, click here.

To learn more about Austedo financial support: 

  • Patients click here or call 1-800-887-8100

  • Providers click here or call 1-800-887-8100

*See Terms and Conditions

Our Commitment to Every Patient…  

To provide individualized specialty pharmacy and infusion services that improve health and empower patients to live each day to its fullest.

Learn more about the therapies we serve.

Stay informed on the latest trends in healthcare and specialty pharmacy.

Sign up for our monthly e-newsletter, BioMatrix Abstract.

We value your privacy. Review our Privacy Policy here.

References

  1. Teva Pharmaceuticals USA, Inc. (2020). Prescribing Information. https://www.austedo.com/globalassets/austedo/prescribing-information.pdf

  2. Teva Pharmaceuticals USA, Inc. (2020). MEDICATION GUIDE AUSTEDO® (aw-STED-oh) (deutetrabenazine) Tablets, for Oral Use. https://www.austedo.com/globalassets/austedo/medication-guide.pdf

  3. Teva Neuroscience, Inc. Austedo® (deutetrabenazine). https://www.austedo.com/

  4. Teva Neuroscience, Inc. Coverage and support. https://www.austedohcp.com/tardive-dyskinesia/cost-and-coverage-support?gclid=Cj0KCQjwjN-SBhCkARIsACsrBz4KG-4UqibPr40GXU0dz3FYmUVpab5yDtTA_K1yU6DtJsKsar4iuoUaAtbIEALw_wcB&gclsrc=aw.ds

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An Overview of Intravenous and Subcutaneous Immunoglobulin (IVIG/SCIG) In Neurology

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The utility of intravenous and subcutaneous immunoglobulin has evolved significantly from being a therapy narrowly used in primary immunodeficiencies, to becoming an option for patients in a wide array of clinical categories, including neurology.1

The exact role of IVIG in neurologic disorders varies based on the amount of clinical research for its use in each indication and can be categorized as being FDA approved (highest level of evidence), used off-label per guideline recommendations, or used with clinical caution in indications with more limited data.

Indications with FDA approval for IVIG

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Indications with off-label use for IVIG

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Indications with limited clinical support for IVIG

  • Stiff person syndrome12,13
  • Multiple sclerosis14,15,16
  • Childhood encephalitis17

IVIG is reserved as a last line treatment option in these indications if other treatments fail or cannot be tolerated by the patient. Dosing guidelines have yet to be clinically decided, and dosing is typically determined by the clinical judgement of the prescriber and individual, institutional protocols.

Monitoring

Monitoring for IVIG patients typically includes:

  • Blood pressure before, during and after the infusion3
    • - IVIG may cause hypotension or hypertension
  • Renal function3
    • - Monitor before initiating therapy, and then at appropriate intervals.
    - IVIG has a US boxed warning for renal dysfunction.
  • IgG concentrations3,18,19
    • - Monitor before initiating therapy, and then every 4-6 months. Measure every 2-3 months if switching from IV to SC IG.
    - IgG reference level: 700-1600 mg/dL
    - Goal trough level: >600 mg/dL OR at least as high as the previous trough level from the previous IVIG dose.
  • IgA concentrations3,18
    • - Low levels of IgA may lead to the formation of IgA antibodies.
    - Patients with IgA antibodies are at a greater risk of infusion reactions from IVIG.
    - Use low IgA IVIG for patients with low IgA and monitor closely for infusion reactions.
    - IgA reference level: 70-400 mg/dL
  • Signs of thrombosis3
    • - IVIG has box warning for increased risk of thrombosis.
    - Patients who have difficulties ambulating may be at a greater risk of thrombosis events.
  • Clinical response

The role of subcutaneous immunoglobulin (SCIG)

SCIG offers several benefits for patients who receive immunoglobulin therapy. The primary benefit is convenience, as the patient may be trained to self-administer SCIG or be able to have a caregiver trained to administer at home. Patients who have a busy schedule, live far away from infusion centers, or who have transportation barriers may find great freedom in switching from IVIG to SCIG. SCIG produces a less dramatic spike in serum IgG levels which is beneficial in reducing systemic side effects. Additionally, more frequent dosing of SCIG has been shown to produce higher trough levels, and patients may experience less variations in clinical response.20

The main limitations of SCIG are increased infusion-site reactions, increased dosing frequency, and less opportunity for healthcare monitoring during infusions. For these reasons, SCIG should be reserved for patients whose disease states are well controlled. If patients experience a decrease in therapeutic response while on SCIG, they may need to be switched back to IVIG therapy.

SCIG therapy, Hizentra, is currently FDA approved for CIDP.3 Refer to each product’s prescribing information leaflet for dosing guidance.

Final considerations

The use of IVIG and SCIG in immunology is an evolving topic with new research conducted on a regular basis. Consult your institution’s clinical databases to stay up to date with the latest information regarding IVIG in specific indications.

BioMatrix is proud to make a difference in the communities we serve, one patient at a time.

Our clinicians and support staff offer a tailored approach to every therapeutic category, improving quality of life for patients and producing positive outcomes along the healthcare continuum. Learn more.

References

  1. Hartung HP, Mouthon L, Ahmed R, Jordan S, Laupland KB, Jolles S. Clinical applications of intravenous immunoglobulins (IVIg)--beyond immunodeficiencies and neurology. Clin Exp Immunol. 2009 Dec;158 Suppl 1(Suppl 1):23-33. doi: 10.1111/j.1365-2249.2009.04024.x. PMID: 19883421; PMCID: PMC2801038.

  2. Hughes RA, Donofrio P, Bril V, et al., for the ICE Study Group. Intravenous immune globulin (10 percent caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomized, placebo-controlled trial. Lancet Neurol 2008 Feb;7(2):136–44.

  3. Lexi-Drugs. [cited 2020 June 23] In Lexicomp Online [Internet]. Hudson, Ohio: Wolters Kluwer Clinical Drug Information, Inc. Available from:Available from: http://online.lexi.com.

  4. Elovaara I, Apostolski S, van Doorn P, et al; EFNS. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS Task Force on the Use of Intravenous Immunoglobulin in Treatment of Neurological Diseases [published correction appears in Eur J Neurol. 2009;16(4):547]. Eur J Neurol. 2008;15(9):893-908.[PubMed 18796075]

  5. Patwa HS, Chaudhry V, Katzberg H, Rae-Grant AD, So YT. Evidence-based guideline: intravenous immunoglobulin in the treatment of neuromuscular disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2012;78(13):1009-1015.[PubMed 22454268]

  6. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Lancet. 1997;349(9047):225-230.[PubMed 9014908]

  7. Raphael JC, Chevret S, Harboun M, Jars-Guincestre MC; French Guillain-Barré Syndrome Cooperative Group. Intravenous immune globulins in patients with Guillain-Barré syndrome and contraindications to plasma exchange: 3 days versus 6 days. J Neurol Neurosurg Psychiatry. 2001;71(2):235-238.[PubMed 11459901]

  8. Bain PG, Motomura M, Newsom-Davis J, et al. Effects of Intravenous Immunoglobulin on Muscle Weakness and Calcium-Channel Autoantibodies in the Lambert-Eaton Myasthenic Syndrome. Neurology. 1996;47(3):678-683.[PubMed 8797464]

  9. Barth D, Nabavi Nouri M, Ng E, Nwe P, Bril V. Comparison of IVIg and PLEX in patients with myasthenia gravis. Neurology. 2011;76(23):2017-2023.[PubMed 21562253]

  10. Gajdos P, Tranchant C, Clair B, et al; Myasthenia Gravis Clinical Study Group. Treatment of myasthenia gravis exacerbation with intravenous immunoglobulin: a randomized double-blind clinical trial. Arch Neurol. 2005;62(11):1689-1693.[PubMed 16286541]

  11. Zinman L, Ng E, Bril V. IV immunoglobulin in patients with myasthenia gravis: a randomized controlled trial. Neurology. 2007;68(11):837-841.[PubMed 17353471]

  12. Dalakas MC, Fujii M, Li M, Lutfi B, Kyhos J, McElroy B. High-dose intravenous immune globulin for stiff-person syndrome. N Engl J Med. 2001.

  13. Dalakas MC, Fujii M, Li M, McElroy B. The clinical spectrum of anti-GAD antibody-positive patients with stiff-person syndrome. Neurology. 2000. 

  14. Fazekas F, Deisenhammer F, Strasser-Fuchs S, Nahler G, Mamoli B. Randomised placebo-controlled trial of monthly intravenous immunoglobulin therapy in relapsing-remitting multiple sclerosis. Austrian Immunoglobulin in Multiple Sclerosis Study Group. Lancet. 1997.

  15. Achiron A, Kishner I, Dolev M, et al. Effect of intravenous immunoglobulin treatment on pregnancy and postpartum-related relapses in multiple sclerosis. J Neurol. 2004.

  16. Sorensen PS, Haas J, Sellebjerg F, Olsson T, Ravnborg M, Group TS. IV immunoglobulins as add-on treatment to methylprednisolone for acute relapses in MS. Neurology. 2004.

  17. Iro MA, Martin NG, Absoud M, Pollard AJ. Intravenous immunoglobulin for the treatment of childhood encephalitis. Cochrane Database Syst Rev. 2017;10(10):CD011367. Published 2017 Oct 2. doi:10.1002/14651858.CD011367.pub2

  18. Dati F, Schumann G, Thomas L, et al. Consensus of a group of professional societies and diagnostic companies on guidelines for interim reference ranges for 14 proteins in serum based on the standardization against the IFCC/BCR/CAP Reference Material (CRM 470) Eur J Clin Chem Clin Biochem. 1996;34:517–20. International Federation of Clinical Chemistry, Community Bureau of Reference of the Commission of the European Communities, College of American Pathologists.

  19. Agarwal S, Cunningham-Rundles C. Assessment and clinical interpretation of reduced IgG values. Ann Allergy Asthma Immunol. 2007;99(3):281–283

  20. Shrestha P, Karmacharya P, Wang Z, Donato A, Joshi AY. Impact of IVIG vs. SCIG on IgG trough level and infection incidence in primary immunodeficiency diseases: A systematic review and meta-analysis of clinical studies. World Allergy Organ J. 2019 Oct 9;12(10):100068. doi: 10.1016/j.waojou.2019.100068. PMID: 31641401; PMCID: PMC6796775.

Our entire team is committed to maintaining the health and wellbeing of those we are privileged to serve.

For updated information regarding our response to developments related to COVID-19, a letter from our CEO, and early refill requests, please click here.

Stay healthy, and be well.

We are with you and will get through this together.

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What to Expect from Intravenous Immunoglobulin (IVIG) Therapy

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What is intravenous immunoglobulin (IVIG)?

IVIG is a blood product made from the donated antibodies of between 1,000-15,000 human donors per batch.1 Antibodies are proteins in your body that help fight infection. Your body has different antibodies to fight different infections, like how you have different keys for different locks. If your body does not have enough antibodies or has damaged antibodies, IVIG can help replace them.

Why would I need IVIG?

IVIG may be prescribed for different reasons. The most common reasons include:

  • Immunodeficiency disorders
    • - Primary immunodeficiencies (PID)
    - Common variable immunodeficiency (CVID)
  • Autoimmune disorders
    • - Immune thrombocytopenia (ITP)
    - Autoimmune hemolytic anemia (AIHA)
    - Guillain-Barre syndrome (GBS)
  • Neurologic disorders
    • - Chronic inflammatory demyelinating polyneuropathy (CIDP)
    - Multifocal motor neuropathy (MMN)
    - Myasthenia Gravis
    - Multiple sclerosis (MS)
    - Stiff person syndrome (SPS)
  • Organ transplant
    • - Desensitization
    - Post-transplant maintenance
    - BK virus

What can I expect before starting therapy?

Before starting IVIG therapy your doctor will perform the necessary tests to determine whether IVIG therapy would be a good fit for you. Once you and your doctor make the decision to start IVIG, you will then decide where you will receive your IVIG infusions. Options include a doctor’s office, infusion centers and even your own home.

Before your first infusion, your pharmacist will call you to review your medical history, infusion date and other information for the success of your infusion.

What can I expect the day of infusion?

The day of infusion, you will have a healthcare professional administer your IVIG. IVIG is given through a vein which may be accessed from your arm, a port or PICC line. Your doctor may prescribe pre-medications such as acetaminophen or diphenhydramine to prevent side effects such as headache and infusion reactions.

During your infusion, your nurse or doctor will likely monitor your blood pressure. It is also important to stay hydrated during the infusion, and you may be given IV fluids or be encouraged to drink fluids by mouth. In total your infusion will take between 2 to 4 hours. Remember to have a cell phone, book, or other activity that you can use to relax during the infusion.2

What are the side effects of IVIG?

Like any medication, IVIG is associated with certain side effects. Every patient will experience IVIG differently. You may have no side effects at all, or you may have several side effects. The most common side effects include:

  • Headache (mild-severe)
  • Flu-like symptoms
  • Injection site reactions
  • Feeling tired
  • Blood pressure changes

Seek medical attention immediately if you experience or think you might be experiencing any of the following rare but serious side effects:

  • Renal dysfunction
  • Allergic reaction (hives, swelling of the lips, tongue, or face)
  • Thrombosis, or clotting
  • Aseptic meningitis (severe headache, confusion, stiff neck, fever, fatigue)

Your doctor and pharmacist are there to help make IVIG as safe and comfortable as possible. If any side effect is troubling you, start a conversation. They may be able to slow the infusion rate to help reduce side effects or offer ways to help side effects such as over the counter pain relievers or cold/warm packs for infusion site reactions.3

How should I store IVIG?

IVIG should be stored in the refrigerator. The best place is in the middle of the fridge. Avoid the back of the fridge where it may be colder and the door, where it may be warmer. Do NOT heat, freeze, or shake your IVIG medication.3

Can I get immunizations while on IVIG?

Immunizations may not work as well while on IVIG. If possible, it is recommended to receive your vaccines prior to beginning your IVIG therapy. Talk to your doctor about the best vaccine schedule for you.2

More questions?

Contact your doctor or pharmacist! This article and others are not a replacement for the direct advice of your doctor or pharmacist. It is important for you to feel confident and comfortable with your infusion. Your health care team is there to help.

BioMatrix is proud to make a difference in the communities we serve, one patient at a time.

Our clinicians and support staff offer a tailored approach to every therapeutic category, improving quality of life for patients and producing positive outcomes along the healthcare continuum. Learn more.

References

  1. Jolles S, Sewell WA, Misbah SA. Clinical uses of intravenous immunoglobulin. Clin Exp Immunol. 2005 Oct;142(1):1-11. doi: 10.1111/j.1365-2249.2005.02834.x. PMID: 16178850; PMCID: PMC1809480.

  2. Silvergleid A. Intravenous immune globulin ivig beyond-the-basics [Internet]. UpToDate. 2020 [cited 2020Jun23]. Available from: https://www.uptodate.com/contents/intravenous-immune-globulin-ivig-beyond-the-basics

  3. Lexi-Drugs. [cited 2020 June 23] In Lexicomp Online [Internet]. Hudson, Ohio: Wolters Kluwer Clinical Drug Information, Inc. Available from:Available from: http://online.lexi.com.

Stay informed on the latest trends in healthcare and specialty pharmacy.

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Three Simple Ways Medical Providers Can Improve Therapy Adherence

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Filled prescriptions that lay wasting in pill boxes and bottles do nothing for patients while costing payers $ billions each year. No one can ‘make’ people stick to their treatment plans. The answer to “are you taking your medications?” is usually “yes.” But the statistics tell us that “yes” often means “sometimes” or “no”. And while it’s common to embellish lifestyle choices to our healthcare provider (i.e. when 1-3 drinks per week really means 5-7), not adhering to a prescribed treatment plan is a big deal. And can be very dangerous.

Data shows that in 2018, over 3.7 billion retail and mail order prescription drugs were filled at pharmacies by payers costing them over $510 billion.(1) And only 30% to 50% of drugs were being taken as prescribed. In other words, roughly $250 billion worth of drugs had 0 impact and were thrown away. The scariest part is that roughly two-thirds of those (many times life-saving) prescription drugs were intended to treat chronic, many times deadly, disease. (2) 

Used to treat chronic, more complex conditions, specialty drugs are especially susceptible to non-adherence and its consequences. While treatment for chronic disease can be cumbersome, time-intensive, and bring about more side-effects, there’s a lot more at stake when, for example, a cancer patient misses their critical dose. While adherence technology (e.g. reminders built into pill boxes and medication sensors) will likely be an important component in the future, nothing replaces personal interactions with patients. Here are three tips for patient interaction to help improve medication adherence. 

Listen and Respond with Empathy

In the face of non-adherence, an obvious question to ask patients is ‘why’. And while that may seem like the first logical question to ask, it can also feel accusatory. 

A recent Pharmacy Times article gives some good advice on how to better spark the adherence topic. This is inline with the “motivational interviewing” method that focuses on decisional balance and internal motivation. For example, instead of starting the conversation with “Why aren’t you taking your medications?” you can rephrase the question as, “Many people have trouble taking their medications on a regular basis. Do you find that this is the case for any of your medications?”3 The phrasing of the second question removes the blame and shame by lending the perspective that many people struggle with taking their medications, it’s not just ‘you’.

When continuing the conversation, it’s easy to instantly get into advice-giving mode before gaining a little more understanding of the patient, especially when they are clearly not following their treatment plan. However, if the patient feels that you’re listening in a non-judgmental way and have some empathy first, they’re more likely to be honest, come to their own conclusions, and be more open to what you have to say. Examples of some questions to guide the conversation include: 

  • Sometimes we get busy and we do not take all the doses of our medicines.

    • How often do you have difficulty remembering to take all your medicines?

  • Taking medication every day is a real inconvenience for some people.

    • Do you ever feel hassled about sticking to your regimen?

    • What gets in the way of taking your medication as prescribed?(3)

Provide Multiple Means of Education for the Patient

To the prescriber and pharmacist, the correlation between following one’s treatment plan and positive outcomes is a given. To the patient who may not be seeing positive results right away (and on the flip side is seeing only the negative—i.e. side effects, high drug costs, and time intensive routines), it may not be so obvious. Especially with specialty drugs, educating the patient beyond just asking “are there any questions with your medication?” encourages adherence. Some ways to do this include:

  • Offer both in-person and online training for self-infusible and injectable medications

  • Provide programs that discuss the importance of therapy adherence and positive self-management

  • Encourage community outreach and support through events and online platforms

Check In Frequently

Similar to New Years Resolutions, we all have intentions of sticking to our plans. But without consistent and frequent accountability, intentions and will-power run dry. An online article published in U.S. Pharmacist, presents multiple studies where increased in-person pharmacist interaction led to increased adherence rates.4 While in-person interactions are typically the most effective, it’s unrealistic that all patients requiring specialty pharmacy drugs are within reasonable range of their physical specialty pharmacy location and/or are even able to get there on a regular basis. The good news is that text messaging has also shown to be effective. The same article cites a study where text messaging doubled the odds of medication adherence and improved overall adherence rates by 17.8%. There are several platform options available for sending automated texts at desired frequencies, encouraging a response and further dialogue. 

Medication adherence takes a joint effort in open communication between the patient, prescriber, and pharmacist. Allowing patients to feel heard in a non-judgmental environment, providing patient education, and frequently checking in with the patient all contribute to the patient taking more responsibility for their health. This leads to higher adherence rates, ultimately improving therapeutic outcomes.

BioMatrix Specialty Pharmacy is committed to provide an individualized clinical approach to specialty pharmacy and infusion services that improve health outcomes and empower patients to live each day to its fullest potential. Learn more. https://www.biomatrixsprx.com/about-us

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1. KFF website. https://www.kff.org/health-costs/state-indicator/total-retail-rx-drugs/

2. World Health Organization. Adherence to long term therapies: evidence for action. Geneva: WHO; 2003.

3. Marden B, Martineau C. Emphasize Medication Adherence to Patients. Pharmacy Times website. https://www.pharmacytimes.com/publications/health-system-edition/2019/September2019/emphasize-medication-adherence-to-patients

4. Medication Adherence: The Elephant in the Room. U.S. Pharmacist website. https://www.uspharmacist.com/article/medication-adherence-the-elephant-in-the-room

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