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Cystic Fibrosis
Respiratory System
Abigail Kluck and Lindsey Faraon
Cystic fibrosis (commonly known as CF) is an inherited disorder that causes severe damage to the lungs, digestive system, and other organ system in the body.
CF causes malfunction in the electrolyte transport system causing cells to absorb too much sodium and water. This causes the normal thin secretions in the lungs to become very thick and hard to move around which leads to these thick secretions increasing the risk for frequent respiratory infections. Recurring respiratory infections will lead to extensive damage of the lungs and eventually the death of cells in the lungs.
There are more than 30,000 children and adults in the US suffering with cystic fibrosis. Due to improved medical treatments and research many patients diagnosed with CF can expect to live well into their 30’s, 40’s and beyond. According to the Cystic Fibrosis Foundation’s patient registry data supports people living longer, healthier lives with CF. However, the median age of death is still only 32 years old. Those living with the disease still face many challenges including hospitalization, complications, and even depression.
CF is a genetic disease and the only way to prevent or cure it would be with gene therapy at an early age. Ideally, gene therapy could repair or replace the defective gene. Another option for treatment would be to give a person with CF the active form of the protein product that is scarce or missing. For patients whose disease is very advanced, lung transplantation may be an option.
Hannah has always had cystic fibrosis her entire life. In July of 2019, Hannah came down with the flu that quickly worsen to pneumonia. The doctors decided since the medication wasn't working the only option left was to do a double lung transplant. It took about a week to find a set but when they did, they took her into surgery late July 25 and finished up on the 26th. According to the doctor everything went well but wanted to wait to take her off the vent to give her body time to heal. About after a week of getting off the vents, she started therapy to strength her body and eventually was able to leave the hospital about a month after the transplant.