Types of Immune Deficiencies - Ask The Pharmacist

Aug 21, 2014
Experts from the Immune Deficiency Therapeutic Resource Center® provide counsel to patients to ensure better management of a variety of conditions.
Tags
  • Immune Deficiency

Immune disorders are a group of conditions affecting patients whose immune systems are either malfunctioning or not functioning at all.

The deficiency is often treated with intravenous or subcutaneous infusions of immune globulins that replace some of the immune cells that the body lacks.

Value of Specialized Care

The specially trained clinicians in Accredo’s Immune Deficiency Therapeutic Resource Center® (TRC) are experienced in managing a range of immune deficiency disorders.

In addition, they also treat patients with other rare diseases such as lysosomal storage disorders or hereditary angioedema. These conditions can have a challenging or severe impact on patients’ lifestyles by making them more susceptible to develop an infection.

Those with severe immune deficiency may even have to avoid crowds altogether or not be able to visit a sick loved one.

Types of Immune Deficiency Conditions

  • Primary Immune Deficiency: Patients with PID have low levels of specific immune globulins – a substance in human plasma that is rich in antibodies – which makes patients more vulnerable to frequent infections, in some cases this can be life-threatening. These patients are treated with immune globulin (IG) therapy which helps to replace decreased or deficient levels of IG in the body.
  • Chronic Inflammatory Demyelinating Polyneuropathy: CIDP is a neuropathic disorder in which the patient’s immune system attacks and destroys the protective covering of the nerve fibers. IG therapy provides CIDP patients healthy antibodies that block the immune and inflammatory process, decreasing the devastating results from nerve damage.

IG therapy is also approved to treat other immune deficiency (ID) conditions such as idiopathic thrombocytopenic purpura, chronic lymphocytic leukemia, multifocal motor neuropathy and others.

One of the important roles of TRC clinicians is to monitor use for appropriate, supportable indications.

When we speak with patients who have been diagnosed with an ID-related condition, we share tips such as the following:

  • Avoid contact with anyone showing active signs of illness or infection.
  • Tell all of your medical providers about your condition. For example, a patient with an ID may not be able to receive live vaccines.
  • Document how you feel on a regular basis, especially during and immediately following an infusion. Reporting the timing and severity of any adverse events may help treat or prevent them.

Leverage Expertise With Other Conditions

Specialist pharmacists in the Immune Deficiency TRC also have training in other disease states and care for patients with other types of treatment needs. Some of these conditions cause growth retardation, stunt mental development, cause organ damage or with certain diagnoses can lead to death. Fortunately, our experienced clinicians can help manage these special populations, including:

  • Alpha-1 Antitrypsin Deficiency: a protein deficiency where patients lack the protective protein alpha-1-antitrypsin (AAT), which is made primarily in the liver. Normal white blood cells in the lung produce an enzyme called neutrophil elastase that destroys invading germs and digests damaged or aging cells. In most people, the alpha-1 protein neutralizes the enzyme after a short time. In Alphas, there isn’t enough alpha-1 protein in the lungs; the enzyme then keeps on working, attacking and destroying normal lung tissue. As this damage continues over years, lung disease such as COPD can develop.
  • Hereditary Angioedema: a rare genetic disorder that causes episodic swelling in the hands, feet, face and airway, which can be life threatening. 
  • Lysosomal Storage Disorders: a group of approximately 50 genetic metabolic disorders that can lead to a number of disabilities and health issues resulting from severe organ damage and dysfunction due to accumulation of metabolic byproducts. 
comments powered by Disqus