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Cystic fibrosis is an autosomal recessive disease that results in thick and sticky mucus to build up in the lungs and in the digestive tract. It is a common lung disease of children and young adults. It is rarely seen in the elderly as people die young from this disease.

The cause of cystic fibrosis is a defective, inherited gene that causes the body to produce abnormally thick, sticky mucus in the lungs, digestive tract and nasal mucosa. The pancreas produces thickened digestive juices so that absorption of food is impaired. It can result in life-threatening lung infections and problems digesting food. It can also affect the sweat glands and in men, the male reproductive system is affected.

The cystic fibrosis gene is defective and must involve inheritance from both parents to be considered symptomatic. About one in 29 Caucasians have the gene for cystic fibrosis. It is the most common inherited disorder among Caucasians in the US and is more common in those of Northern European or Central European descent. The diagnosis is almost always made by the age of two years but a small number are not diagnosed until they are older than eighteen years. The later the diagnosis, the less severe is the disease.

Symptoms of cystic fibrosis differ from person to person because there are a thousand mutations of the cystic fibrosis gene. Symptoms include stunted growth, failure to gain weight, salty skin and no bowel movements in the first twenty four or forty eight hours of life.

Bowel symptoms include pain in the abdomen from constipation, increased gas or bloating, nausea and a poor appetite, clay colored stools that have mucus in them (and that float) and loss of weight in childhood or failure to gain weight in childhood. When it comes to the sinuses and lungs, the most common side effects include increased thick mucus in the lungs and sinuses, fatigue, cough, nasal congestion, nasal polyps, recurrent episodes of pneumonia and sinus pain or pressure.

Examination of a person with cystic fibrosis will show the signs as noted above. Tests can look for the abnormal gene in the genetic profile. Immunoreactive trypsinogen or IRT test is a screening test for newborns for cystic fibrosis. A sweat chloride test evaluates the amount of salt in the sweat in the child's skin. Other tests that can be helpful include a fecal fat test, a chest x-ray or CT scan, lung function studies, secretin stimulation test, trypsin and chymotrypsin in the stool, pancreatic testing and an upper GI bowel series.

Treatment of cystic fibrosis includes managing the various symptoms. There are often specialized CF clinics in major metropolitan areas that can be helpful. Antibiotics are given to treat sinus infections or lung infections. This may be as part of a hospitalization, in which case IV drugs are used or can be used at home, orally. Inhalers are used to keep the airways as open as possible and DNAse enzyme replacement therapy can be used to thin the mucus and make it easier to expectorate mucus. Patients are given the pneumococcal vaccine and the flu shot on a regular basis. A lung transplant is used as a last resort because it doesn't really cure the disease. Patients with cystic fibrosis often need home oxygen therapy.

The bowel problems need to be treated, too. A high protein diet with extra calories is required for kids with the disease and pancreatic enzymes are given so that fat and protein are absorbed easily. Vitamins A, D, E and K are fat soluble vitamins that need to be given in excess so that enough can be absorbed into the system. Medications are given to treat constipation.

You need to keep the patient away from dirt, dust, smoke, fumes, mold, mildew and household chemicals. Chest percussion is used to loosen the thick mucus from the lungs. Patients need to drink plenty of fluids so the stool and mucus is as thin as possible. Exercise is a good choice to mobilize the bowels and the mucus from the lungs.

Most kids with CF are fairly healthy until the point at which they reach adulthood. Young adults can usually go to college and find employment. Unfortunately, the average lifespan of a person with cystic fibrosis is about 35 years.

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Medical Negligence Solicitors

Our personal injury solicitors operate a specialist medical negligence compensation service. Our Cystic Fibrosis solicitors deal with claims using a no win no fee arrangement which means that if you don't win then you don't pay them their professional costs. If you would like legal advice at no cost with no further obligation just complete the contact form or email our lawyers offices or use the helpline and a Cystic Fibrosis solicitor will review your medical negligence compensation claim and phone you immediately.

HELPLINE: ☎ 1800 633 634

The author of the substantive medical writing on this website is Dr. Christine Traxler MD whose biography can be read here