Primary Immunodeficiency or PI is also called primary immunodeficiency disease or PIDD. The term defines a series of conditions and is an umbrella term for more than 300 diseases of the body’s natural self-defense or immune system.
There is no one specific PI disease. All of the diseases included in this category are considered rare and chronic by the Immune Deficiency Foundation. The good news is that none of the diseases are contagious. The bad news is that all of the disease are inherited diseases and could be passed on to children of PI patients.
Symptoms vary depending on which of the 300 + PI diseases a patient has. The most common symptom of all is that patients get infections more often and get infections which do not go away. People who need more than two kinds of antibiotics, including IV antibiotics, to stop an infection may have PI.
Other common symptoms include sudden loss of weight; children that grow more slowly than normal; recurrent bouts of ear infections; recurrent bouts of pneumonia; enlarged spleen; constantly swollen lymph nodes; abscesses in the skin or in the internal organs.
Most primary immunodeficiency disorders are inherited, meaning they are passed down from a parent to a child. One does not contract these disorders the same way as cases of flu and colds. Children born with any of these primary immunodeficiency disorders have a mutated gene that is linked to the immune system’s functionality.
Although any of the immune related disorders sometimes run in families, they can also happen on their own. DNA, the genetic code acting as a blueprint for cell generation, sometimes faces problems, thus causing system shortcomings.
Children affected by primary immunodeficiency disorders lack particular types of white blood cells responsible for fighting infections. Such children are susceptible to infections that wouldn’t otherwise take a toll on children with healthy immunity.
Primary immunodeficiency diseases are not curable and stay with the patient all throughout his or her life. However, many of the symptoms can be treatable so that patients can lead mostly normal lives, fulfilling commitments to family, friends and employers. Treatment depends on the symptoms, what parts of the immune system are affected and health history of the patient. One treatment plan that works for one patient may not work for another. Treatment plans can range from prescription allergy medications or injections to stem cell therapy.
One treatment that works for some patients is called immunoglobulin therapy. Blood plasma has many different kinds of antibodies or immunoglobulins. Plasma from tens of thousands of volunteers is given intravenously to the patient. The theory is that the immunoglobulins from the donors help give the patient a more normal immune system.
Families with these disorders need counseling on the risk of the disorder recurring in future children and any possibilities of family planning.
Since primary immunodeficiency disorders are typically gene related, there is no known way of preventing them. However, parents can implement risk management steps to reduce chances of infection from other diseases. If any of the disorders are identified during pregnancies, infection prevention measures and plans for therapeutic solutions begin after birth.
Other preventative measures for helping a child with immunodeficiency avoid illness: