Idiopathic pulmonary fibrosis (IPF) is a disease in which the tiny air sacs or “alveoli” that make up the lungs become inflamed and are gradually replaced by scar tissue (fibrosis). As the amount of scar tissue increases, the lungs stiffen and are unable to transfer oxygen from the lungs to the blood stream. This results in the brain and other organs becoming oxygen deprived.
As IPF progresses, day-to-day activities such as walking short distances, climbing stairs, dressing, or even talking on the phone become a problem because the person cannot catch their breath (dyspnea). The person feels as if they are suffocating and may require supplemental oxygen.
Advanced idiopathic pulmonary fibrosis makes people more susceptible to getting and fighting infections.
The term “ idiopathic” suggests that clinicians do not know what causes the disease. Lung inflammation may be triggered by infection with pathogens, airborne hazards, or certain types of medical treatments. Exposed to these types of challenges, the immune system boosts its inflammatory response to attack the pathogens and remove hazards or damaged tissues. In a vicious cycle, the uncontrolled inflammation results in greater lung damage.
Idiopathic pulmonary fibrosis may be considered an inflammatory autoimmune disease. Autoimmune (meaning against oneself) conditions result from the body’s overactive, defensive, inflammatory reactions to an immune challenge. The body’s own immune cells mistakenly attack and destroy previously healthy by-stander tissues or organs, very much like a forest fire damages healthy trees.
The body responds to injury by forming scar tissue, made mainly of the key protein collagen. Pulmonary fibrosis results in inflammation and scarring that occurs again and again. It is an imbalance between the build-up of scars, and the breakdown of collagen that is needed for tissue repair. In IPF, lungs with old scar tissue is found layered over old damage, while fresh scarring is seen over more recent damage.
Lung damage in IPF patients is due to imbalances between inflammatory and anti-inflammatory cytokines, immune messengers generated in response to substances or circumstances that initiated the lung damage in the first place. Imbalances of cytokines results in more and more fibrosis.
Individuals with IPF may find that if they are able to control the amount of inflammation produced by their immune systems, if they can stay in homeostasis, balance, their quality of life may change for the better.
Please contact Dr. Hellen if you wish her assistance in changing your quality of life. There is no fee for her services. She may be contacted by using this form or at: 302.265.3870 (ET, USA).
www.coalitionforpf.org/cytokine-functions/
www.nhlbi.nih.gov/health/health-topics/topics/ipf
www.ncbi.nlm.nih.gov/pubmed/26132817
www.immuneworks.com/autoimmune-lung-diseases/idiopathic-pulmonary-fibrosis-ipf-treatments
www.ncbi.nlm.nih.gov/pubmed/26150910
faculty.ksu.edu.sa/hadilalotair/Chests%20Library/IPF.pdf
