At the Asembia Specialty Pharmacy Summit 2019, a panel of experts educated audience members about chronic inflammatory demyelinating polyneuropathy.
Chronic inflammatory demyelinating polyneuropathy (CIDP) starts insidiously and presents as either a slowly progressive disease or as a relapsing one, with partial or complete recovery between recurrences. Its symptoms—limb weakness, sensory symptoms such as tingling and numbness, and motor symptoms—are disabling and frustrating for patients and challenging for clinicians.
On Monday at the Asembia Specialty Pharmacy Summit 2019, a panel of experts educated audience members about this condition. Tuan H. Vu, MD, presented a disease overview starting with a case study that highlighted that CIDP can occur at any age but most often presents between ages 40 and 60 years.
“CIDP has many phenotypic variants. For this reason, we think that CIDP may be a spectrum of discrete albeit related conditions in which immunogenetic variations drive individual phenotypic differences,” Vu said.
Vu also covered diagnostic criteria for CIDP, demonstrating how various guidelines differ but are all based on the same pertinent features. He explained that numerous diagnostic pitfalls exist and have led to a number of patients being treated for years for a disease they do not have. To avoid misdiagnoses, clinicians must adhere strictly to clinical and electrophysiological criteria.
The bottom line is that treatment should have 3 goals: to increase strength, to improve functionality, and to enhance quality of life. Therapeutic options include corticosteroids, immunosuppressants, plasmapheresis, and immunoglobulin.
When immunoglobulins are used, approximately two-thirds of patients with CIDP will improve, but the adverse effects are daunting and may include anything from headache and fever to aseptic meningitis, heart failure, myocardial infarction, and renal failure.
Subcutaneous immunoglobulins afford patients more independence and are an option for those with poor venous access, cardiovascular risks, or adverse effects related specifically to intravenous immunoglobulins (IVIGs). Because subcutaneous immunoglobulins are administered once or twice weekly, patients are less likely to experience a wearing-off effect. Investigators have not yet determined whether subcutaneous immunoglobulin is as effective as IVIG. Cost differentials have not been elucidated either.
“Choosing a treatment is a balancing act. Clinicians need to consider disease severity, patient age, general health, and potential contraindications. Adverse effects are a consideration too,” Vu said.
He reminded the audience that IVIG is the first choice in patients with moderate-to-severe disability. He also noted that the dose can be reduced by more than 20% in almost half of patients once they are stable.
Amy Clarke, RN, IgCN, discussed patient management for CIDP. She emphasized that patient preference is a consideration when selecting therapy and urged for increased pharmacist and nursing education. She noted that the clinical team must determine whether a caregiver needs to accompany patients to appointments and to also consider lost time from work or school.
“Many clinicians think that if a medication is administered at home, it reduces burden,” Clarke said. “But scheduling infusion time in the home may also create burden of productivity loss. If patients are capable of self-administering medication or have a caregiver who can do so, that’s a viable option.”
Clarke discussed pharmacist-driven risk assessment to determine the appropriate treatment for the individual patient and the best site of care. Pharmacists should examine comorbidities; risk factors including renal status, cardiac status, clotting risks, and age; the likelihood that the patient will be adherent; previous experiences with immunoglobulins; the patient’s ability to self-administer immunoglobulin; and the need for pre-medication.
When selecting a treatment, clinicians must be aware of some unusual considerations, such as sodium content and osmolarity, and pay special attention to the patient’s comorbidities, as immunoglobulins can contain sodium and sugar.
Clarke reminded participants that nursing should be included for in-home or infusion suite administration if the immunoglobulin is administered intravenously. Nurses should be present for the entire infusion and, during that time, can provide comprehensive education to patients. Nurses also monitor for intolerance, anaphylaxis, and phlogistic reactions in the presence of an active infection (more problematic for patients with primary immunodeficiency).
Clarke underscored the need for a workable emergency plan. Everyone involved in medication administration needs to be able to differentiate between symptoms and questions that can be answered at the pharmacy, those who need to be referred to the prescriber, and those who require activation of emergency medical response.