Immune Deficiency Isn’t Rare—Learn the Facts and How Compounding Pharmacists Can Help

Immune Deficiency Isn’t Rare—Learn the Facts and How Compounding Pharmacists Can Help

immune deficiencyPrimary immunodeficiency diseases are often categorized as rare, but compounding pharmacists who think PIDs are too rare to warrant attention could miss a significant opportunity. Current estimates place prevalence at 1 in 2000 people, a rate that doesn’t even include secondary immunodeficiency diseases. More importantly, many of these patients are frequent visitors to local pharmacies long before they’re diagnosed. If you stay alert for signs of immunodeficiency in the undiagnosed—and regularly counsel those being treated for PIDs—you can improve the overall health and longevity of patients with immune deficiency.

Complex Nature of Primary Immunodeficiency Diseases

With more than 230 different primary immunodeficiency diseases—presenting with diverse symptoms depending on the immune system dysfunction—it’s easy to see why diagnosis is a challenge. Key factors for compounding pharmacists to keep in mind include:

  • Lag in diagnosis: The average time from symptom onset to diagnosis is 12.4 years.1
  • Patient profile: 26 percent of all patients are under the age of 18, 30 percent fall between 18 to 44, and 35 percent are 45 to 64 years old.
  • Common symptom: While presentation varies, the one common symptom is frequent, recurring infections, especially in the form of chronic sinusitis and lung disease.
  • Adult-onset immunodeficiency: Common variable immunodeficiency, or hypogammaglobulinemia, is the most common form in adults.
  • Newborn screening: In 2010, the CDC added one form of PID—severe combined immunodeficiency—to the Recommended Uniform Screening Panel for newborns, but the test isn’t required in all states.

Antibacterial and Antifungal Treatments

First-line treatment consists of IV or subcutaneous injections of immunoglobulin concentrates. The injections boost the immune system and lower the risk of infection, which is crucial since infectious diseases impact morbidity and mortality in patients with PIDs. The most common infections occur in the respiratory, pulmonary and gastrointestinal system or present as abscesses. Immune deficiency patients often follow a long-term prophylactic regimen of antibacterial and antifungal treatments.

  • Patients with chronic sinusitis or lung disease are often prescribed tetracycline, cephalosporin, ciprofloxacin, and trimethoprim.
  • The top recommended antifungals are itraconazole, fluconazole, cotrimoxazole, and sulfamethoxazole. Fluconazole is used in a newborn’s first four weeks.2

Compounding pharmacists can improve treatment compliance by combining multiple medications into a single dose and making them more palatable for children. They can also collaborate with primary care physicians or immune specialists about delivery options, as well as individualized treatments to relieve respiratory and lung symptoms.

Supporting from Compounding Pharmacists

As pharmacists focus on patient-centered care, they’re faced with the challenge of fitting more responsibilities into a schedule that’s already packed with medication management. When finding a balance seems impossible, remember that time spent with patients makes a difference. For example, research shows that patients with HIV achieve faster virus suppression when they receive care from pharmacists rather than primary care providers.3

Screening: Experts say that early diagnosis and treatment are imperative to reduce morbidity and mortality, yet statistics say that the average patient isn’t diagnosed for about 12 years. During that time, patients suffer from recurring infections, so they’ll seek OTC products or come to you with a prescription. Pharmacists can facilitate earlier diagnoses with screening and counseling.

  • Watch for patients with recurring infections using your medication management system. Patients on antibiotics for two months or more without results may be at risk for an immune deficiency.
  • Use the Jeffrey Modell Foundation’s 10 warning signs of immune deficiency to quickly screen for recurring pneumonias and infections.4
  • Patients with PID should keep records to ensure treatment continuity. Remind patients that you’re a source of medical records. Also recommend the free IDF ePHR mobile app, which patients use to track info about PID treatments.5

Complementary Treatment: Patients receiving prophylactic treatment with antibacterial medications may benefit from complementary treatments to offset side effects. This is an opportunity to discuss the use of probiotics to fight the overgrowth of Helicobacter pylori and other bacteria.

Immunizations: Live bacterial and viral vaccines are dangerous, especially if patients have immune deficiencies of T cell, B cell, or phagocytic cell origin.6 While inactivated vaccines are safe for most PID patients, they’re likely to be ineffective because a compromised immune system can’t generate protective antibodies. The type of vaccine that’s effective and safe, versus contraindicated, depends on the severity and cause of the immune deficiency. If you offer immunizations in your pharmacy, it’s crucial to screen for PID.

Targeting PID Patients Improves Their Outcome

Diagnoses of primary immunodeficiency diseases are more common than the “rare” label that pervades the literature.7 As a compounding pharmacist, you can effectively improve the quality of life—and lengthen the lifespan—of people with PID, especially when you’re able to identify potential patients living in the limbo before being diagnosed with immune deficiency.

Pharmaceutica North America provides bulk APIs including the antifungal medications preferred for prophylactic treatment of primary immunodeficiency diseases. Please contact us today to learn more about how we can support your compounding pharmacy.

Show 7 footnotes

  1. “Primary Immunodeficiency Diseases in America: 2007,” May 2009,
  2. “Prevention of Infections During Primary Immunodeficiency,” November 2014,
  3. “Outcomes of Pharmacist-Assisted Management of Antiretroviral Therapy in Patients with HIV Infection: A Risk-Adjusted Analysis,” September 2015,
  4. “Primary Immunodeficiency,” November 2011,
  5. “IDF ePHR and PI Connect,” July 2015,
  6. “Recommendations for Live Viral and Bacterial Vaccines in Immunodeficient Patients and Their Close Contacts,” April 2015,
  7. “Prevalence and Morbidity of Primary Immunodeficiency Diseases, United States 2001-2007, November 2014,

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