Immunodeficiency

Primary Immunodeficiency

Primary immunodeficiency, or PIDD, is an inherited genetic mutation that usually develops in men before the age of 20. PIDD is a group of more than 100 immune system disorders that prevents the immune system from functioning properly. With the immune system now working properly, PIDD weakens the immune system and allows infections and other health issues to occur easily. Some forms of PIDD are mild and go undetected for years, others forms are so severe that they are diagnosed shortly after birth.

Symptoms of Primary Immunodeficiency

The first signs of PIDD are having repeated or unusual infections that are difficult to treat. Other signs may include:

 

  • Frequent and recurrent upper respiratory infections, such as pneumonia, bronchitis, sinus infections, ear infections, meningitis, skin infections
  • An enlarged spleen or swollen lymph nodes
  • Delayed growth
  • Weight loss
  • Inflammation and infection of internal organs
  • Blood disorders, such as low platelet counts, or anemia
  • A family history of primary immunodeficiency
  • Autoimmune disorders, such as lupus, rheumatoid arthritis, or Type 1 diabetes
  • Problems after receiving a live vaccine
  • A need for multiple courses of antibiotics to fight infections
  • Digestive disorders, such as loss of appetite, cramping, nausea, diarrhea

Diagnosis of Primary Immunodeficiency

PIDD is diagnosed based on the patient’s personal and family medical history, a physical examination, and laboratory testing that may include:
  • Blood tests
  • Genetic testing
  • Prenatal testing

Treatment of Primary Immunodeficiency

The treatment of PIDD is focused on the prevention and treatment of infections with rapid and aggressive treatment of infections with antibiotics. Treatment is also focused on boosting the immune system with immunoglobulin therapy or interferon-gamma therapy and infusions or injections for a period of time. A stem cell transplant to replace defective or absent immune system components can provide a permanent cure for some of the forms of PIDD as well a means of addressing the cause of the immune systems problem. Patients with PIDD should try to avoid getting an infection. Steps include:
  • Washing hands regularly with soap and water
  • Avoiding crowds
  • Avoiding people who are ill
  • Getting enough rest
  • Eating a sensible diet
Dr. Ogden can create a treatment plan for patients with primary immunodeficiency. This includes steps to prevent infections.