By Linda Harvan, FAUSA
Every day we are exposed to viruses, bacteria, parasites and fungi, but our immune system keeps us healthy by fighting most pathogens. A healthy immune system will not prevent all illnesses, but it will help reduce the severity and duration of symptoms when we are ill. People with healthy immune systems usually recover from a cold or the flu in a few days to a week without requiring any visits to the doctor or medicines other than those that help reduce symptoms.
For people with primary immunodeficiencies (PI), the situation is different. Children and adults with PI can have frequent and unusual infections, often affecting the upper and lower respiratory tracts, such as in the ears, sinuses and lungs. These infections can be hard to clear and sometimes require more than one round of antibiotics or even IV antibiotics. Infections can be located anywhere in the body, including the skin and digestive system.
My husband Tom has Common Variable Immune Deficiency, or CVID, which was not diagnosed until he was in his late 30s/early 40s. Prior to his diagnosis, he had many sinus infections, pneumonia, bronchitis and giardia.
He first began to get sinus infections when our daughters started preschool and were exposed to other children. He also got sinus infections every time he traveled by airplane. More than once, a mild illness quickly turned into pneumonia or bronchitis, and I had to take him to the emergency room because he had a high fever. Often he had recurrent sinus infections with symptoms starting again a week or two after he finished the first antibiotic prescription. He has learned that certain antibiotics will lessen his symptoms but will not completely clear his sinus infections. Doctors who treat him regularly know which antibiotics work best, but he is often prescribed antibiotics that are not as effective when he has seen a new doctor or gone to urgent care while traveling.
After beginning treatment with immunoglobulin replacement therapy, the number of sinus infections he has each year has been reduced dramatically. However, even while receiving monthly IV immunoglobin treatments, he developed cellulitis after his first total knee replacement surgery. After initially using oral antibiotics, he had to be admitted to the hospital to receive IV antibiotics. After this first case of cellulitis, he developed it again a few more times in the same leg.
Tom currently has an abscessed tooth, which is a deep tissue infection. He has been prescribed antibiotics three times in six weeks. Because his surgery is not for another two weeks, he will have to watch closely to make sure the infection does not return.
CVID is the most diagnosed primary immunodeficiency and is usually diagnosed in adults. There are more than 400 primary immunodeficiencies recognized by the International Union of Immunological Societies, each with its own symptoms. It is estimated that 70 to 90 percent of primary immunodeficiencies worldwide are undiagnosed. Early diagnosis and treatment are essential to improving the health and lives of patients with PI. The general warning signs of PI are different for adults and children, but in all cases, signs include multiple ear or sinus infections, pneumonia, recurrent deep abscesses of the skin or internal organs, persistent thrush or fungal infections, need for IV antibiotics, and a family history of PI. Parents should also watch for the failure of an infant to gain weight or grow normally. Adults may also have chronic diarrhea with weight loss or recurrent viral infections such as colds, herpes and warts. Generally, if you or your child is affected by two or more of these warning signs, you should speak to your primary care physician (PCP) about the possibility of a primary immune deficiency. Patients will often be referred to an immunologist if their PCP suspects a PI.
Immunodeficiency Canada produced this video with the help of children and adults relating their symptoms and experiences to the warning signs of Primary Immunodeficiency: 10 Warning Signs That Every Parent Should Know – YouTube.
The Jeffrey Modell Foundation created two posters listing the 10 Warning Signs of Primary Immunodeficiency. One is a generic list and the second is a list specific to adults.
These warning signs were developed by the Jeffrey Modell Foundation Medical Advisory Board. Consultation with Primary Immunodeficiency experts is strongly suggested.
The next step for patients whose doctor suspects a primary immunodeficiency is diagnosis. This will be the topic of the next article in the series.
Part 1 in the series. Tom’s CVID Journey
Part 2 in the series. The Warrior Immune System
Sources:
Home - JMF (info4pi.org) (Jeffrey Modell Foundation)
Immune Deficiency Foundation | (primaryimmune.org)
Bringing about change together - World PI Week
IPOPI (International Patient Organization for Primary Immunodeficiencies)
Photo: Canva