Cystic Fibrosis: A Brief History of the Disease in the United States

Cystic Fibrosis (CF) is a progressive, often fatal, genetic disease that affects thousands. CF affects mucous production in the body, causing the CF sufferer to produce thicker mucous which clogs lungs, creating a great place for bacteria to build and thus resulting in lung infections, as well as clogging the pancreas and often causing digestive and absorption problems resulting in low weight gain and vitamin deficiencies. However, with the thanks of many great scientific breakthroughs, a disease which often left infants dead in its wake is now faced with a median life expectancy of nearly 40 years old, some living well into their fifties, sixties, and even seventies.

At one time, there was no diagnosis of Cystic Fibrosis. Until the 1930s, infant mortality was high around the world and babies often inexplicably died. It is said that there was an old adage that if a baby tasted salty when kissed on the forehead that the baby would soon die. Cystic Fibrosis patients often taste salty due to an abundance of sodium chloride in their systems.

In the 1930s and 40s, advancements were made in the diagnosis of Cystic Fibrosis, then called Cystic Fibrosis of the Pancreas. Though infants were still dying from this disease, the symptoms were being documented, recorded, and studied, so that by the 1950s, with the advent of antibiotics like penicillin, diagnoses were made and infants were living longer: though still, CF was a fatal childhood disease. One mother recalls the birth of her child with CF in the late 1940s. The doctors were unsure of what was wrong with the baby, and by the time they were able to diagnose CF, the baby was too ill to recover. The baby died after only five weeks of life. This same mother went on to have another baby with CF a few years later. That child lives on into his fifties. The difference in the medical advancements of just a few years made all the difference between these two children.

In order for CF to be passed on to an infant, both parents must carry the CF gene. If both parents carry the gene, there is a 25% chance their child will have CF, a 50% chance the child will simply carry the CF gene but be unaffected by the disease, and a 25% chance that the child will be free of both CF and carrying the CF gene.

The 1960s continued in advancements with the treatment of antibiotics, new developments in understanding the nutritional problems in CF as well advancements of CF centers being set up through the country to treat children with CF. At this time in history, some CF children were made to sleep in mist tents to help with the mucous build up in their lungs. Physical therapy was introduced as a treatment and parents were taught how to pound on the backs and chests of their CF children to help loosen their mucous and free their airways. Nutritional supplements were introduced to aid in the digestion and absorption of food.

The 1970s and 80s found new hope for CF. Doctors began to recognize that, though still progressive and fatal, CF was treatable for a time with good nutrition, physical clearance of the airways, exercise, and antibiotics. CF children were now living into their teens. In 1989, the gene that causes CF was discovered and the idea of a cure came on the horizon.

The 1990s saw advancements with inhaled antibiotics as well as devices to help aid in airways clearance such as a flutter device and the Vest. The Vest is a machine that vibrates the chest of the CF patient and helps to loosen the mucous without the need to parents to perform PT any longer. Nutritional enzymes and antibiotics continued to improve and the life expectancy rose yet again.

The twenty-first century has proven the most promising in CF care. Established centers through the country serve patients, and these centers now also focus on adult care, and even have begun talks of servicing seniors with the disease. Women with CF are now often healthy enough to have children, and men, most once thought of as sterile due to a commonplace missing vas deferens, are able to look to in-vitro fertilization techniques should they want to father children of their own. New machines are able to electronically nebulize medications for CF patients to inhale in a short amount of time. IV antibiotics can be done at home and science keeps progressing. With stem cell and other genetic research there is new hope for the CF patient. Though many CF patients are reluctant to ever hope for a cure, the idea that science may make CF a disease that is livable if treated rather inevitably fatal is hope enough for the people touched by this disease.