For some people with PI who have a history of bacterial infections, doctors may prescribe prophylactic antibiotics. Unlike antibiotics used to treat an infection, prophylactic antibiotics are given to help prevent an infection. They are usually given in low doses.
IgG treatment is used to temporarily replace some of the antibodies (immunoglobulins) that are missing or not working properly in people with PI. The goal of IgG treatment is to use IgG obtained from normal donor plasma to keep enough antibodies in the blood of patients with PI to fight off bacteria and viruses.
IgG treatment can be given in two ways (referred to as “routes of administration”):
Doctors work together with their patients to determine which route of administration is best, taking into consideration health needs and lifestyle.
Hematopoietic stem cells (HSCs) form the different types of blood and immune cells. For the most severe types of PI, hematopoietic (blood forming) stem cell transplantation (HSCT) can be an effective treatment option. This is when one person's hematopoietic stem cells are transfused into the person with PI. It is not surgery. The process is more like a blood transfusion.
The traditional way to perform HSCT is by bone marrow transplantation, thereby utilizing the HSCs from another person's bone marrow and transfusing it into the person with PI. But now, HSCs can also be taken from cord blood (the blood removed from the placenta after childbirth). Cord blood can be a HSC source for immune and blood systems.
Most forms of PI are caused by errors (mutations) in specific genes. Gene therapy uses a person's own HSCs that have been modified by addition of a normal copy of the gene in order to fix the genetic mutation. This may be an option for individuals with more severe types of PI who cannot be matched with appropriate stem cell donors. Currently, gene therapy remains experimental and is not yet used routinely.
While your doctor knows which treatment is best for your PI, you should talk to your doctor to make sure you understand your treatment and address any concerns you may have.