In the 1970’s, much notoriety was given to the “The Boy in the Bubble”. The youngster, David Vetter, lived alone for 12 years in a germ free plastic tent. His mother never held him in her arms. No one was allowed to touch him with their bare hands. The toll on his psyche and the heartache of his parents are immeasurable.
David was extremely susceptible to germs and foreign substances. His valiant battle against Severe Combined Immune Deficiency (SCID) made national and international news. He succumbed to the disease when a bone marrow transplant went awry.
Recurrent infections are not uncommon in otherwise healthy children. These infections, over time, occur less frequently and without any lasting harmful consequences.
Some children have a predisposition to upper respiratory tract infections (URIs)., ear infections sinusitis and other infections because of:
- Allergies
- Anatomical defects of the upper or lower airway
- Gastroesophageal reflux disease
- Passive exposure to cigarette smoke
On the other hand, some children can experience a dangerously high susceptibility to infections because their immune system breaks down. Although the immune system works hard 24/7 to identify and destroy germs, it does not always work perfectly.
Immunodeficiency means that part of the body’s defense system is defective and is losing the battle against foreign invaders (e.g. viruses, bacteria, fungi). In these instances, the body lacks sufficient firepower (white blood cells) to fight and defeat infections and foreign substances.
Primary immune deficiency diseases are caused by genetic flaws in the immune system (passed down from one or both parents). Secondary or acquired immune deficiency diseases are caused by infectious, viral, chemical or radiological agents. HIV/AIDS is an acquired immune deficiency disease. There are warning signs of immune deficiency.
10 Warning Signs of Primary Immunodeficiency
- A family history of primary immunodeficiency
- Delayed growth and development of an infant
- Four or more new ear infections within one year (especially after 4 years of age)
- Three or more serious, bacterial sinus infections within one year
- Pneumonia twice or more within one year
- Inflammation and infection of internal organs
- Antibiotics
- Need for preventive antibiotics (to decrease the number of infections)
- Need for intravenous antibiotics (to clear infections)
- Two or more months on antibiotics with little effect
- Autoimmune disorders (rheumatoid arthritis, multiple sclerosis, type1 diabetes)
- Persistent thrush in mouth or fungal infection on skin
- The development of unusually severe infections that started out as common bacterial infections.
Two or more of the above signs suggest a primary immune deficiency and further evaluation by your physician.
The immune system is a complex network of many components which are intricately linked together. Each part has a specific responsibility to keep us safe and healthy. If one link in the chain is missing or broken, it can have devastating consequences.
In David’s case, SCID was the consequence. Because of his bravery and the support of his family, the world empathized with his condition. Also, through his sacrifice, the scientific community was galvanized to better understand, better treat and hopefully, discover a cure for immune deficiencies. Because of David and his parents, children are no longer isolated or treated in plastic bubbles.
References
Ballow M; Approach to the Patient with Recurrent Infections. Clin Rev AllergImmunol. 2008; 34(2):129-140.
Bonilla FA, Bernstein IL, Khan DA, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. Ann Allergy Astham Immun. 2005;94(5 suppl 1):S1-S63.
Reust, Carin E; Evaluation of Primary Immunodeficiency in Children; Am Fam Physician; June 1 2013
Anticaglia, Joseph R; A Snapshot of the Immune System Our Bodies 24/7 Defense System; HC Smart, 2017
Jeffrey Model Foundation; CDC, Warning Signs of Primary Immunodeficiency
Mayo Clinic; Primary Immunodeficiency; Jan 20, 2015
This article is intended solely as a learning experience. Please consult your physician for diagnostic and treatment options.