Understanding Cystic Fibrosis: A Life-Limiting Condition

Cystic Fibrosis is a chronic non-gender biased illness which affects the digestive system and the lungs. This condition also results in the buildup of mucus, which clogs in the respiratory system as well as the pancreas. Cystic Fibrosis occurs because a defective gene causes the body to excrete excessive sticky and thick mucus that clogs the lungs leading to a life-limiting lung infections. When these thick secretions obstruct the pancreas, they prevent the digestive enzymes from reaching the intestines to aid in breaking down as well as absorbing food. However, if Cystic Fibrosis are not treated, it can be fatal as there is no cure. Research shows that each day one person dies from Cystic Fibrosis. As such, this is the most deadly condition

For a baby to have Cystic Fibrosis, he/she has to have precisely two defective cystic fibrosis genes from each of the parents. Both parents must be carriers of this condition. The parents will also not show any signs of having this condition. It is a recessive condition which explains why a majority of the carriers never have a clue when they are carriers. The problem lies on chromosome seven which has the defect in those people living with this condition. Typically, people have 46 chromosomes, which pair up forming 22 pairs in every nucleus in the human body (Wicks, 2007). The remaining two chromosomes are the sex chromosomes hence the seventh chromosome is the one that gets mutated causing Cystic Fibrosis. Some of the common symptoms of Cystic Fibrosis include persistent coughing, salty-tasting skin, wheezing and shortness of breath. Additionally, there is very poor weight gain despite excessive appetite, fleshy growths in the nose and bulky, greasy

Pancreatic enzyme supplements, vitamins, salts are required to balance the absorption of nutrients. Since CF can cause impaired growth, a high fat, high-calorie diet is key for normal growth and development in kids with this condition. These diets can aid in maintaining optimal growth. Additionally, CF can cause increased lung infections, thus good nutrition is imperative to maintain a great defense against these infections. Other methods of managing this condition include implanted devices which allow long-term access to the bloodstream for the common administration of drugs (Cohen-Cymberknoh, Shoseyov & Kerem, 2011). With this method, management of this condition is less intrusive and more efficient. The treatment that targets the defective CF-causing gene is CF transmembrane conductance regulator modulators (CFTR). This medication facilitates effective flow of fluids and salts on the lung surface, thus thinning the thick mucus, which people with CF have built in their lungs. The commonly used modulator brands are Orkambi and Kalydeco which are prescribed for kids with ten various mutations of the gene causing the condition (Wicks, 2007). Future treatments are also focusing on gene therapy such as CF transmembrane conductance regulator