Children’s Interstitial Lung Disease (chiLD) is a group of rare diseases found in infants, children and adolescents. There are a large number of interstitial and diffuse diseases that fall under the chiLD umbrella.
The main feature of chiLD is that oxygen has problems passing into the bloodstream via the lungs, and there are variable degrees of lung scarring.
The severity of disease and long term outcomes will vary considerably from person to person. Generally some children may grow out of their condition, others will need lung transplantation and sadly others may die.
Many chiLD children need additional care and attention. For those children who require oxygen or feeding supplementation, traveling can be a challenge. From visiting friends or family, a quick trip to the shops, all of these require planning..
In a nutshell ABCA3 is when the lungs do not produce the surfactant in the lungs to transport the oxygen into the blood stream correctly.
It also causes the lungs to stick together if they were to close. Therefore the use of the bipap machine to keep the lungs inflated especially at night when Tiana is asleep giving her lungs and heart a rest from working so hard.
The ABCA3 gene provides instructions for making a protein involved in surfactant production. Surfactant is a mixture of certain fats (called phospholipids) and proteins that lines the lung tissue and makes breathing easy. Without normal surfactant, the tissue surrounding the air sacs in the lungs (the alveoli) sticks together after exhalation (because of a force called surface tension), causing the alveoli to collapse. As a result, filling the lungs with air on each breath becomes very difficult, and delivery of oxygen to the body is impaired.
Pulmonary hypertension (PH), is a complex and often misunderstood disease. The term PH means high blood pressure in the lungs. In “regular” hypertension (also known as high blood pressure or “systemic hypertension) the pressure in the arteries throughout the body is higher than it should be. This can be measured with a blood pressure cuff. In PH, the blood vessels specifically in the lungs are affected. They can become stiff, damaged or narrow, and the right side of the heart must work harder to pump blood through.