Purpose of the
Hope for the Breath-less
How does a patient become breathless?
The main purpose of the lungs is to absorb oxygen by breathing in air from outside the body and then expressing it ridding the body of carbon dioxide. Another vital purpose of the intake of air is to supply cells with oxygen required by the body to survive. There are many small air sacs within the lung called “alveoli.” Each of the alveolus is “surrounded by a network of tiny tubular capillaries that bring hemoglobin-laden red blood cells in single file just opposite the air-washed side of each alveolus.” There are cells in the lungs whose main purpose is to be the immune line of defense. Anything that disrupts the delicate structure of the alveolus may impede the optimal transfer of oxygen and may lead to breathlessness. A group of diseases which can interrupt this exchange process are the Interstitial Lung Diseases (ILD). As these diseases “smolder”, they cause inflammation and scarring of the lung tissue specifically affecting the “interstitium” and little by little, the disease spreads and the lungs become incapable of producing the oxygen exchange process and unable to defend against infection.
Classifying ILD is very difficult because there are several categories – known and unknown causes. Further subdivision is “granulomatous and non-granulomatous.” A disease called sarcoidosis is a granulomatous disease where the cause is not known, which strikes younger adults, and is more severe in African Americans. Hypersensitivity pneumonitis is another granulomatous ILD of known cause related to environmental exposures. This happens when the subject develops an allergy in the lungs to one or more of any number of organic dusts found in the environment. Non-granulomatous ILD can also be triggered by systemic disorders like rheumatological diseases such as rheumatoid arthritis and lupus. The lungs can be affected in some, but not all of these patients.
Most ILDs can lead to pulmonary fibrosis which is characterized by excessive deposition of collagen in lung leading to pulmonary scarring or fibrosis. Thus, a diagnosis of ‘pulmonary fibrosis’ reflects the presence of scarring in the lung, but does not define the cause or ILD involved. One classic ILD known for pulmonary fibrosis is termed “Idiopathic Pulmonary Fibrosis” (IPF); idiopathic means “we don’t know what causes it.” This disease is the most common of the “non-granulomatous” ILD. IPF has been known for quite some time, but it wasn’t until 15-20 years ago that great attention has been placed on it. IPF affects the lungs through a scarring process which, over time, makes it hard to get oxygen from the lungs into the blood stream where it goes to the heart, brain, and all other vital organs. IPF is one of the lung diseases for which a lung transplant can be considered for qualifying candidates. In general, lung transplantation can extend life expectancy another 7 – 10 years.
Signs and Diagnosis
The first signs of ILD are shortness of breath, dry cough, and the sound coming from the lungs upon listening with a stethoscope which is likened to the sound you hear when you tear “Velcro” apart. This sound is called ‘crackle or rale’, and it often alerts physicians that there is scarring of the lungs After a thorough medical history is taken and a physical examination is performed, physicians might order a specialized chest imaging test called ‘high resolution computer tomogram or HRCT”. If the cause cannot be determined, sometimes (and depending on the condition of the patient) the next line of procedures is done which requires either fluid, cells or tissue extracted from the lung by a biopsy in order to confirm a diagnosis and determine the course of action required to help the patient.
Approximately 40,000 people die from IPF yearly, which is about the same number as those who die from breast cancer. As the disease progresses, the entire body suffers from the lack of oxygen, and the patient is forced to go on oxygen supplementation 24 hours a day. It is estimated that over 150,000 in the U.S. are afflicted with IPF; however, it is thought by many in the field, that many remain undiagnosed or misdiagnosed. The perceived low number makes it difficult to get funding because not enough research has been done, and the return on investment doesn’t meet funding agencies’ criteria, or companies don’t see enough financial return for them to invest in the research. In addition, it’s such a new awareness that the low numbers work against the cause. It’s the proverbial “catch 22!”
The best way to explain IPF is to think about the last time you ran a marathon, rode your bicycle full out or any other physical exertion which left you breathless, and that is what an IPF patient lives with minute to minute on a daily basis. Typically, after diagnosis, the average patient’s life expectancy is 3-5 years. The disease can progress very rapidly, as one very active patient reportedly was seen in September and by February was on a ventilator waiting for a lung transplant. That is why it is of the utmost urgency that funding be made available for research endeavors.
The Breath-less Charity was established with a three-fold purpose in mind:
First, to fundraise in order to make grants available to both clinical and basic research faculty whose main focus is: Developing more medicines that will help slow the progression of the devastating lung disease known as Idiopathic Pulmonary Fibrosis (IPF) until a cure can be found.
Second, the Charity will make grants available to help patients with the overwhelming financial costs that accompany this kind of disease. The medicines now available are very expensive. There is currently no cure for IPF. The only recourse a patient has when the disease progresses to the point at which they can no longer sufficiently breathe on their own is to receive a lung transplant, or they die.
The third purpose of the Breath-less Charity is to help educate the public about IPF, and what to look for regarding symptoms, what to ask the doctor, which specialist to seek out in order to find out if indeed the condition is IPF or some other lung malady. The first indication of IPF is a crackling sound, but it also mimics other lung diseases and can easily be misdiagnosed.