Special Saturday Post. Oh, wait, by the time I post this, it’ll be Sunday. One of my classes just went through respiratory system, and another will go through that in a couple weeks. It makes a great time to talk about something in the news.
Sarah Murnaghan, a 10-year-old Pennsylvania girl with cystic fibrosis, came into national attention in May when her parents tried to get her onto the transplant list for adult lungs. Until then, kids were on the “kids lungs list,” separate from the list for adult lungs. It is rare for kids lungs to be available for transplant – adult lungs are much more commonly available. So after waiting a long time for no lungs to show up, they sought adult lungs, which can be trimmed to fit into a child’s chest.
They tried to get the United States Secretary of Health and Human Services, Kathleen Sebelius, to change the rules, but it wasn’t until a court order for her and another child that Sarah got on the list for adult lungs.
After a court order in early June, Sarah got adult lungs. It was revealed this week those first lungs failed, so she underwent a second transplant a couple days later of another set of adult lungs. I’ve posted a few articles below if you want to know more about Sarah and transplants. Let’s talk cystic fibrosis.
What is Cystic Fibrosis?
Cystic Fibrosis (CF) is an inherited disease that causes thick, sticky mucus to form in the lungs, pancreas and other organs. In the lungs, this mucus blocks the airways, causing lung damage and making it hard to breathe. In the pancreas, it clogs the pathways leading to the digestive system, interfering with proper digestion.
Bacteria grows in the mucus, leading to life-threatening lung infections that can damage the lungs. The mucus also clogs the pancreas, which prevents normal digestion and leads to malnutrition. People with CF are also at increased risk of diabetes and osteoporosis.
People with CF have a shorter-than-normal life expectancy. The good news is that as treatments for CF improve, the life expectancy for people with the disease is rising. Fifty years ago, children with CF often died before attending elementary school. Today many people with the disease live into their 30s, 40s and beyond. Getting early treatment for CF can improve your quality of life and your lifespan.
A person is born with CF – he or she must inherit 2 defective genes on chromosome #7, one from each parent. So (those studying genetics) this is an autosomal recessive disorder.
Some common symptoms are…
- Very salty-tasting skin
- Persistent coughing, wheezing or shortness of breath
- Excessive appetite but poor weight gain
- Greasy, bulky stools
Treatment for CF depends on the stage of the disease and the organs involved. It may include:
Airway clearance techniques to clear mucus from the lungs
One technique is called postural drainage and percussion. The person with CF sits, stands or lies in a position that helps free up mucus. The chest and back are pounded and clapped to loosen the mucus. A person with CF may use a mechanical vest or blow into a device that shakes the mucus loose.
These may be taken with a nebulizer, a machine that changes liquid medicine into a fine mist which makes it easier to inhale deep into the lungs. Some medicines can also be breathed in through a metered dose inhaler (MDI).
Other treatments for CF
- Azithromycin, an antibiotic that fights bacteria in the lungs
- Anti-inflammatory medicines such as ibuprofen to help reduce swelling in the airways
Lung transplantation may be an option in some severe cases of CF.
References on Sarah Murnaghan: