Cystic Fibrosis: Defined

Overview

Cystic fibrosis is a disease of the secretory glands, which create mucus and sweat. It mainly affects the lungs, pancreas, liver, intestines, sinuses and sex organs.

cystic fibrosis lung drawing with healthy airway and blocked, due to CFMucus lines multiple organs inside the human body, preventing them from drying out or becoming infected. If you have cystic fibrosis, your mucus is too thick and sticky and can build up in the lungs, which can block your airways.

In addition to blocking airways, the thick mucus can block tubes in your pancreas — which prevents the digestive enzymes the pancreas creates — from reaching the small intestine. Without these digestive enzymes working properly, the body cannot absorb the necessary fats and proteins that come from food.

Mucus buildup from cystic fibrosis can provide an environment that allows bacteria to grow. This can lead to lung infections that may damage them over time.

Because of this imbalance with the secretory glands, individuals with Cystic Fibrosis typically have salty sweat thereby losing high amounts of the necessary salt content from the body. Dehydration, increased heart rate, fatigue, weakness, decreased blood pressure, heat stroke and sometimes even death could come as a result.

Having Cystic Fibrosis also puts an individual at increased risk for diabetes and osteoporosis, which causes the bones throughout the body to thin. In men, Cystic Fibrosis causes infertility and for women, may make getting pregnant more difficult.

Symptoms

Symptoms vary drastically from individual to individual with Cystic Fibrosis as does the time of onset of symptoms as well as their severity.

Typically, lung function begins to decline in early childhood with continued damage to lungs causing serious breathing problems as one ages. Respiratory failure is the most common cause of death to those with Cystic Fibrosis.

Digestive problems are also common for those with Cystic Fibrosis. As mucus blocks the pancreatic enzymes needed for proper food digestion, people with Cystic Fibrosis may suffer regularly from diarrhea, malnutrition, weight loss and poor growth.

Additionally, with mucus blocking the airways in the lungs, coughing and wheezing affect those suffering from Cystic Fibrosis.

Diagnosis

Since Cystic Fibrosis is a generic inherited condition, one cannot contract it later in their lifetime. Both parents of a child born with Cystic Fibrosis carry one copy of the mutated gene but usually do not have the disease themselves.

While there is no treatment or full cure for Cystic Fibrosis, the disease can be regulated and managed. It is important to see your doctor and discuss the possibility of treatment and regulation with a Cystic Fibrosis specialist. A Cystic Fibrosis specialist will often work with their own team of nurses, physical therapists, dietitians and social workers and are typically located in major medical centers.

There are more than 100 Cystic Fibrosis Centers across the United States. Each center has specialized teams with expert training in Cystic Fibrosis care. Many centers have both pediatric and adult clinics or programs for specialized treatment.

Treatments

While there is no treatment or cure for Cystic Fibrosis, there are steps that can be taken to regulate the disease. As treatments for Cystic Fibrosis continue to improve, life expectancy for those with the disease continue to go up. Today, some with Cystic Fibrosis are living into their forties and fifties.

CysticFibrosis_Child getting oxygenEarly treatment includes nutritional and respiratory therapies, medicines, specialized exercise and other varying treatments. Speak with your doctor about pulmonary rehabilitation (PR). PR is a broad program that helps to improve the livelihood of those living with chronic and ongoing breathing problems.

Chest physical therapy (CPT) can be used to loosen mucus trapped in the lungs and other breathing techniques can be utilized to release additional pressure.

Your doctor may prescribe antibiotics, anti-inflammatory medication, bronchodilators, or mucus-thinning medicine to treat and prevent lung infection, swelling, and open up airways.

Oxygen therapy can also be used to help with advanced lung disease.

Nutritional therapy and a specialized diet are also essential to those with Cystic Fibrosis and can improve growth and development in children. Keeping up a diet rich in calories, fat and protein can make you strong enough to resist certain lung infections.

Vitamin supplements, oral pancreatic enzymes, high calorie shakes, a high-salt diet and a feeding tube can also be prescribed by your doctor.

Causes

cystic fibrosis family treeCystic Fibrosis is inherited in an autosomal recessive pattern, meaning both copies of the gene in each cell has mutations.

Therefore, the parents of a child with Cystic Fibrosis most likely do not have the disease themselves but both carry a gene that they pass on through reproduction.

Living With

Living with Cystic Fibrosis takes effort and vigilance as the upkeep of the body and a dedicated diet, medication and exercise regimen are essential to staying healthy.

Make sure to see a specialist regularly, who will provide you with personalized lifestyle choices and habits that will make your particular case of Cystic Fibrosis easier for you to maintain.

Related Information

What is clapping? And how does it help those with Cystic Fibrosis?

  • Clapping or percussion is another term for Chest Physical Therapy, a regulatory measure to aid in removing mucus from the lungs so those with Cystic Fibrosis are able to breathe easier. It involves pounding on the chest and back to shake mucus from the lungs making it easier to cough up. Sitting down and lying on your stomach helps as the extra gravity and force assist in draining the mucus.

What complications can I expect if I have Cystic Fibrosis?

  • Complications differ from person to person but two of the most common complications of Cystic Fibrosis are diabetes and osteoporosis or thinning of the bones. See a health care professional to learn further preventative measures and medications that can assist in regulating and preventing these complications.

Besides a balanced diet, what should I eat to stay healthy if I have Cystic Fibrosis?

  • Supplements of vitamins A, D, E, and K are important to remember as they help in replacing the fat-soluble vitamins that your intestines cannot absorb. A high salt diet as well as oral pancreatic enzymes which aid in the digestion of fats and proteins are also essential. Because those with Cystic Fibrosis sweat out a large portion of their body’s salt content, it is vital to incorporate salt into your diet or take salt supplements, especially before exercising. A feeding tube at night is another addition to consider. Threaded through the nose and throat, it feeds you while you sleep, providing nutrients during the night hours when you cannot regulate your ingestion.

Getting Help

Living with Cystic Fibrosis, it is essential to get regulatory assistance from your doctor and a Cystic Fibrosis specialist who can help you in regulating the disease and giving you lifestyle choices that make maintaining your health easier.

Cystic Fibrosis Care Centers are also helpful as they have entire teams on staff to aid you in your treatment process. If you or a loved one has severe onset of Cystic Fibrosis, it may be easier to live within close range of a specialist or a Cystic Fibrosis Care Center to ensure proper regulation and nearby treatment.