Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery. But in people with CF, they're thick and sticky. Instead of acting as lubricants, these secretions plug up the tubes, ducts and airways in your body.
CF mucus is often described as more solid and having a “flake” form. In addition, it has a higher concentration of proteins called mucins. Mucins make up the main component of mucus and are responsible for the gel-consistency of mucus.
Brown Phlegm
Really dark brown, tenacious phlegm is seen in patients who have cystic fibrosis or bronchiectasis, which is a chronic lung disease. The phlegm is brown because of blood and the intense chronic inflammation that comes with the chronic disease state.
CF causes thick mucus to build up and clog certain parts of the body such as the lung. The buildup is caused by an abnormal gene called CFTR (cystic fibrosis transmembrane regulator). CFTR controls the flow of water and salt in and out of the body's cells. Changes cause mucus to become thickened and sticky.
The cystic fibrosis (CF) disease is characterized by abnormally thick and sticky mucus. Normal airway mucus has bulk viscosity (macroviscosity) in the range of 12–15×103 cP increasing to 14–110×103 cP for CF patients.
Mucus thinners (such as mucolytics) help thin and then move the mucus out of the airways so it can be coughed out. These medications can be taken through a nebulizer during ACTs. Inhaled antibiotics should be taken after ACTs are finished and the lungs are as clear of mucus as possible.
The average steady-shear viscosity is found to vary for frequencies between 10−4 and 102 Hz, spanning viscosity values as high as 103 Pa-s and as low as 10−2 Pa-s. In general, at low shear rates, the viscosity of human mucus is as high as 104−106 times that of water [6].
The thick and sticky mucus associated with cystic fibrosis clogs the tubes that carry air in and out of your lungs. This can cause signs and symptoms such as: A persistent cough that produces thick mucus (sputum) Wheezing.
Atypical CF is a milder form of the CF disorder, which is associated with mutations of the cystic fibrosis transmembrane receptor gene. Instead of having classic symptoms, individuals with atypical CF might only have mild dysfunction in 1 organ system and might or might not have elevated sweat chloride levels.
Typical symptoms include abdominal bloating, loose stools, and poor weight gain as well as coughing, wheezing, and frequent respiratory tract infections throughout life. The diagnosis is based on sweat test results and/or genetic testing. About half of the people with this disease in the United States are adults.
Symptoms tend to start in early childhood, but sometimes they can develop soon after birth and very occasionally they may not be obvious until adulthood. Nowadays, cystic fibrosis is usually diagnosed before symptoms appear, through screening tests carried out soon after birth.
Symptoms of CF
Frequent lung infections including pneumonia or bronchitis. Wheezing or shortness of breath. Poor growth or weight gain in spite of a good appetite. Frequent greasy, bulky stools or difficulty with bowel movements.
People with cystic fibrosis (CF) often look healthy, but it's a life-limiting condition that affects those living with it in many ways. The condition is caused by a genetic mutation that means cells in the human body are unable to move salt and water around effectively.
PCD is easy to confuse with cystic fibrosis because the two genetic diseases have similar effects on the lungs. In both disorders, mucus accumulates in patients' lungs, causing frequent infections and severe congestion.
Median age at diagnosis of cystic fibrosis is 6-8 months; two thirds of patients are diagnosed by 1 year of age. The age at diagnosis varies widely, however, as do the clinical presentation, severity of symptoms, and rate of disease progression in the organs involved.
Rhonchi. Rhonchi sounds have a continuous snoring, gurgling, or rattle-like quality. Rhonchi occur in the bronchi as air moves through tracheal-bronchial passages coated with mucus or respiratory secretions. This is often heard in pneumonia, chronic bronchitis, or cystic fibrosis.
Sweat and cystic fibrosis
As CF is caused by a faulty gene that controls the movement of salt and water into and out of cells, people with CF often sweat more than people without the condition, and this sweat contains high levels of salt, which can crystalise visibly on the skin.
Common sleep complaints in cystic fibrosis include :
frequent awakening. nocturnal cough. snoring.
An infection can make mucus thicker and stickier. Infections also lead to inflammation in the mucous membranes that line the nose and the rest of your airway. This can cause certain airway glands to make more mucus. That mucus can get thick with bacteria and cells that arrive to fight the infection.
Thin and clear mucus is normal and healthy. White. Thicker white mucus goes along with feelings of congestion and may be a sign that an infection is starting.
Many people with CF can use an inhaler rather than a nebuliser for their bronchodilator. Examples of bronchodilators are Salbutamol (Ventolin®), Terbutaline Sulphate (Bricanyl®) and Ipratropium Bromide (Atrovent®). Antibiotics are used to treat, prevent and control infection.