Cystic fibrosis

Cystic fibrosis is a common inherited disease. It affects over 8,500 children and young adults in the UK, where five babies are born with the condition every week.

Cystic fibrosis affects the internal organs, especially the lungs and digestive system. It causes them to become clogged with thick, sticky mucus. It is caused by a faulty gene that controls the movement of salt and water in and out of cells in the body. When cystic fibrosis occurs, too much salt and not enough water pass into the cells and turn the body’s secretions, which normally act as a lubricant, into a thick mucus. This mucus clogs up many of the body’s tubes, ducts and passageways so they cannot work properly. In the lungs, this leads to frequent and severe infections.

The faulty gene that causes cystic fibrosis is recessive. This means that you need two faulty genes (one from each parent) to inherit the disease. Around 1 in 25 people in the UK are carriers of the cystic fibrosis gene. Carriers do not have the disease but their children may inherit it.

Cystic fibrosis causes recurrent chest infections, poor growth and related health problems, such as diabetes and infertility.

There is no cure for cystic fibrosis, but many treatments and therapies can make cystic fibrosis easier to live with. It is difficult to predict life expectancy for people with cystic fibrosis as the condition affects everyone differently. According to statistics, around half of all people with cystic fibrosis can expect to live beyond the age of 38.

Symptoms of cystic fibrosis

Symptoms

When a child is born with cystic fibrosis, symptoms usually appear in the first year of life, although occasionally they can develop later.

The thick sticky mucus in the body affects a number of organs, particularly the lungs and digestive system.

The symptoms and related problems of cystic fibrosis can vary in severity from person to person. The main symptoms and problems are detailed below.

Lungs

It is common for people with cystic fibrosis to have difficulties such as:

Cough and wheeze. The body tries to shift the thick mucus in the lungs by coughing it up
Recurring chest and lung infections. Infections are caused by the continual build-up of mucus in the lungs, which provides an ideal breeding ground for bacteria

Cross infection

People with cystic fibrosis are vulnerable to harmful lung infections caused by certain strains of bacteria (these bacteria are rarely harmful to people without cystic fibrosis).

Two strains of bacteria that commonly infect people with cystic fibrosis are Pseudomonas aeruginosa and Burkholderia cepacia complex. They multiply in the thick mucus inside the lungs and may cause serious health problems, such as repeated chest infections. The danger is that a person with cystic fibrosis who has such an infection can easily pass it on to another person with cystic fibrosis through close personal contact or by coughing near them. This is known as cross-infection. As more and more people with cystic fibrosis become infected with these bacteria, the bacteria may become resistant to antibiotic treatment, which is why cross-infection is such a problem.

There is a concern that people with cystic fibrosis are more likely to pick up strains from each other than from the environment. For this reason, it is recommended that people with cystic fibrosis do not come into close contact with each other. Patients infected with these dangerous bacteria may be treated in separate clinics to those without the bacteria, to avoid cross-infection.

Digestive system

Cystic fibrosis can also cause mucus to block the ducts in the pancreas. The pancreas produces essential food-digesting enzymes. When it is blocked, not enough of the enzymes reach the intestines (bowel) to help break down food, which can cause:

Large, smelly stools. If the digestive enzymes are not being produced, food is not adequately digested and excess fat is lost in the stools, making them bulky, oily, smelly and difficult to flush away.
Malnutrition. Because the body cannot digest essential nutrients in food (particularly fat), it is often difficult to gain weight and infants may struggle to put on weight and grow. Adults with cystic fibrosis often find it difficult to gain and maintain weight. In children with cystic fibrosis, this can result in delayed puberty if they are severely underweight.
Diabetes. In older people with cystic fibrosis, the pancreas can become more damaged. Diabetes can develop if the pancreas does not produce enough insulin, a hormone that controls the level of sugar in the blood. Diabetes in people with cystic fibrosis is different from diabetes in people without cystic fibrosis. Usually, the symptoms of diabetes include feeling constantly thirsty, frequently needing to pass urine and feeling extremely tired. These rarely occur in patients with cystic fibrosis. People with cystic fibrosis who develop diabetes may find it difficult to gain weight or may lose weight and see a decline in their lung function. Cystic fibrosis-related diabetes is usually controlled by regular injections of insulin. Diabetes rarely occurs in children with cystic fibrosis.

Ears, nose and sinuses

People with cystic fibrosis can be prone to sinusitis and hay fever, which may need to be treated with nasal sprays or antibiotics. Some older children and adults develop nasal polyps, which are fleshy swellings that grow from the lining of the nose or sinuses. If they become troublesome, they may need to be removed.

Bones and joints

Some older children with cystic fibrosis develop a form of arthritis (swelling and pain of the joints), usually in one or two large joints such as the knee. In most cases, symptoms improve with time and treatment.

Older children and adults may also be prone to osteoporosis (thin brittle bones) for many reasons, including repeated infection, poor growth or weight, lack of physical activity and lack of vitamins and minerals due to digestive problems. People with cystic fibrosis are more at risk of developing osteoporosis if they are taking steroids to help with lung infections.

Osteoporosis as a result of cystic fibrosis may cause joint pain and bones may fracture (break) more easily. Some people need to take drugs called bisphosphonates to help maintain their bone density.

Infertility

Both men and women with cystic fibrosis can have problems conceiving children. In virtually all men with cystic fibrosis, the tubes that carry sperm do not develop correctly, making them infertile. Women with cystic fibrosis may find that their menstrual cycle becomes absent or irregular if they are underweight. There is also an increased thickness of cervical mucus, which may reduce fertility. However, most women with cystic fibrosis can become pregnant without any difficulty.

Liver

In a few people with cystic fibrosis, the tiny bile ducts in the liver can become blocked by mucus. This can be serious as the disease progresses, and in some cases it may be necessary to have a liver transplant.

Incontinence

People with cystic fibrosis, especially females, are more likely to have incontinence. Urine usually leaks during coughing fits. It is important that people with cystic fibrosis are asked about this and encouraged to talk to a member of their cystic fibrosis team as soon as possible so they can get help.

Related Articles

You have permission to reprint what you just read. The only requirement is including the following footer with it… ”Article by allvitalpoints.com, visit http://www.allvitalpoints.com for more original content like this. Reprint permission granted with this footer included.”

All Vital Points: Health and Fitness Issues

Reliable source for information on health conditions and treatments