A Parent′s Guide to Cystic Fibrosisis a volume in a series addressing the needs not only of parents but also of physicians and persons concerned with the care of children with relatively common disorders. We used as a modelThe Child with Down′s Syndrome, written by David W. Smith, M.D., and Ann Asper Wilson, first published in 1973 by W. B. Saunders, Philadelphia. The book is very valuable because it makes the complex concepts of genetics and pediatrics understandable to parents. Such is the goal of our series.

InA Parent′s Guide to Cystic Fibrosis, it was the authors′...

A young couple decides to have a child. They are both healthy and, as far as they know, no major genetic disease has occurred in either family. After a smooth pregnancy, a baby boy is born. He sleeps well and has a superb appetite, but there is some delay in regaining his birth weight. After he has several bouts with colds and is not able to gain weight despite hearty eating, the baby′s doctor orders a sweat test.

″Your baby has cystic fibrosis,″ says the doctor, reading from a laboratory report. Gently, calmly, the doctor tells the parents about the...

The symptoms of cystic fibrosis and the severity of these symptoms vary considerably from patient to patient. Indeed, diagnosing cystic fibrosis may be difficult. Although most patients experience pulmonary and/or gastrointestinal problems early in life, their symptoms may be mild. A few may go undiagnosed for years or decades before their chronic symptoms are recognized as cystic fibrosis. Signs of cystic fibrosis in older patients may be male infertility or cirrhosis of the liver.

The characteristics of cystic fibrosis are sometimes mistakenly thought to be chronic bronchitis, pneumonia, asthma, or one of the other conditions associated with the body′s inability...

To understand lung problems in cystic fibrosis you first need to understand how the lungs work normally. All activities of living organisms are fueled by energy, and one of the ingredients needed to create this energy is oxygen. The process of converting oxygen into a biologically useful form is called oxidation.

Since only small amounts of oxygen can be stored in the body, an uninterrupted supply of oxygen to each cell is necessary to maintain metabolism. We use the term ″respiration″ to refer to those processes by which cells use oxygen, produce carbon dioxide, and convert energy to biologically useful...

All animals require carbohydrates, fats, proteins, vitamins, minerals, and water for cell synthesis and cell maintenance. After food and liquids are swallowed, enzymes break them down. The digestive process is like trying to pass pebbles through a strainer. A large pebble won′t go through unless it is pulverized into many small fragments. Digestive enzymes serve the same function as the pebble pulverizer—breaking down complex materials so they can pass through the walls of the digestive tract into the body for use by cells.

The digestive system—also called the gastrointestinal system—is basically a continuous tube or tract divided...

Cystic fibrosis affects nearly every organ of the body in some way. Although the effects of the disease are most prominent in the respiratory and digestive systems, other organs and systems may also be impaired.

The primary function of sweat glands is to help regulate our body temperature; 90% of heat loss occurs through our skin. The glands also contribute to the excretion of 5 to 10% of our metabolic wastes contained in the watery, salty, clear sweat. On a daily basis, we produce anywhere from half a liter (about a pint) to two or three liters. Evaporation of the...

Chronic disease in children can lead to a host of social and psychological problems for patients and their families. The burden on the family may cause marital conflicts, limit social life, distract attention from other children, and severely drain financial resources.

Cystic fibrosis takes such a toll. Parents quickly realize that time that would normally go toward one′s spouse or family fun activities may be consumed by daily treatments. But there′s a real reward for enduring the hardships—the chance of a quality life for your child.

Many of the psychosocial issues associated with cystic fibrosis are commonly found with...

How can we gauge our efforts at conquering cystic fibrosis?

One barometer of success is mean life span. CF is no longer solely a disease of children. Before the advent of antibiotics and digestive enzymes, the majority of children died in infancy. The most common causes of death were lung and heart failure, and an inability to digest food, which leads to starvation. In 1955 only 50% of children with cystic fibrosis lived to four years of age. The mean life span began to increase dramatically beginning in the early 1960s for several reasons: centralization of care at specialized cystic...

The last excerpt from the Family Diary ends this book where it began, with the involvement of family in treatment of cystic fibrosis.

‶I was getting sick and tired of the whole thing. Ben was having lung problems again, and it seemed like that′s all we did anymore . . . treatments, treatments, treatments. When I collapsed into bed late that night, I felt something under the pillow. I turned on the light and cried while I read this note:

‶He′s only eight; I didn′t know he even thought about the stuff I did.″...