Heather is suffering from a disease called Constrictive Bronchiolitis. This is a rare disease that affects the smallest airways in the lungs and restricts the body’s ability to oxygenate the blood.
As an analogy, imagine the lungs airways as the root structure of a large tree. They are large at the top and get smaller and smaller the deeper they reach into the earth. The tiny roots, some as thin as a human hair, bring the needed water and nutrients to the tree.
The Lungs act in a similar fashion.
1. When we breathe,our Lungs fill up with air.
2. At the same time the Right side of the Heart is pumping blood to the Lungs.
3. As the oxygen reaches the tiny airways of the Lungs, it enters into the blood, removing the carbon dioxide.
4. We then exhale the carbon dioxide(waste) our blood had absorbed on its journey through our body.
5. The clean(oxygenated)blood is then pumped to the Left side of the Heart which sends it back to the body.
In Heather’s case, these very small airways are becoming restricted and squeezed from her disease. This limits the amount of oxygen entering into her bloodstream. At rest Heather seems to be fairly stable.
Her oxygen levels stay in the mid 90% range. Keep in mind that a normal persons oxygen levels will always stay in the high 90’s to 100%. As we all know, with activity,we begin to breathe harder and our Heart pumps faster. With any activity, even walking, Heather’s Lungs can’t oxygenate her blood fast enough on their own. This requires her to be on concentrated oxygen to recover.
Just walking from one room to another we have seen Heather’s levels drop to the high 70’s with a heart rate of 120 and above. This would feel like suffocation to any normal person.
As this disease progresses the tiny airways become what looks like scar tissue and no longer function at all. As more of these airways get destroyed Heather will will become more dependent on supplemental
The secondary problem caused by this disease affects the Heart. If the blood does’t get the oxygen it needs from the Lungs, the blood in turn doesn’t get rid of the carbon dioxide. The blood is still sent back out to the body which sends an emergency signal to the Brain. The Brain tells the Right Heart to send more and more blood to the Lungs because the body isn’t getting enough clean blood. The Right Heart thinks it needs to work harder even though it is the Lungs that are dropping the ball.
This added pressure on the Pulmonary Artery leads to Pulmonary Hypertension and more. Just like any muscle you focus on at the gym, the Right Heart begins to get larger. Unlike other muscles, with the Heart, larger doesn’t mean stronger. An enlarged, overworked Right Heart can lead to cardiac arrest.
Thanks to the team at UW, they are not only watching the disease but working hard to keep all the side effects under control as well. -John Rima