- Infertility in both males and females.
- Growth malfunctioning
- Bloody cough
- Nasal polyps, as well as sinusitis.
- Chronic respiratory failure
- Intestinal blockage, gallstones, and rectal prolapse.
- Biliary cirrhosis, pancreatitis, and liver failure.
- Immunoreactive Trypsinogen (IRT) level test for infants.
- Sweat test.
- Genetic test.
- Preventing and controlling infections in the lungs.
- Removing mucus from the lungs.
- Treating and preventing blockage in the intestine.
- Providing adequate nutrition.
- Medications that target gene mutation and treat the most common genetic mutation causing cystic fibrosis.
- Antibiotics to treat and prevent lung infection.
- Anti-inflammatory drugs to lessen swelling in the airways.
- Mucus-thinning drugs to help cough out the mucus such as hypertonic saline.
- Inhalers, called “bronchodilators” to help keep the airways open by relaxing the bronchial muscles.
- Acid-reducing medications.
- Oral pancreatic enzymes to help absorb nutrients.
- Stool softeners to prevent constipation.
- Specific drugs for diabetes or liver diseases.
- In cases of gene mutations, CFTR modulators may be recommended.
- For 6 months and above; Kalydeco, which contains ivacaftor.
- For 6 years and above; Symdeko, which is a combination of tezacaftor and ivacaftor.
- For 12 years and above; Trikaftor, a new combination medication of elexacaftor, tezacaftor, and ivacaftor.
- Airways Clearance Techniques
- Vest Therapy or Cystic Fibrosis Physical Therapy (CPT)
- Oxygen and Non-invasive Ventilation Therapy
- Pulmonary Rehabilitation
- Surgical Procedure
- Feeding tube, inserted permanently into the bowels to feed meals directly into to stomach to prevent choking.
- Nasal and sinus surgery.
- Bowel surgery
- Lung transplant.
- Liver transplant.
The majority of CF cases are identified shortly after the delivery of a newborn baby. Signs and symptoms vary depending on the severity of the disease. Such signs are:
- A constant cough that produces thick mucus
- Sweat appears very salty
- Poor growth
- Weight gain
- Greasy, smelly stools that are bulky and pale-colored.
- Exercise intolerance
- Recurrent lung infections
- Inflamed nasal passages
- Stuffy nose
- Sinusitis; a condition in which the cavities around the nasal passage becomes inflamed, either triggered by a cold or allergies and may resolve on its own.
What Causes Cystic Fibrosis?
The rare genetic disorder is caused by the mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, which provides instruction to make the important cystic fibrosis transmembrane conductance regulator (CFTR) protein.
Thus, the protein is made with errors or not made at all and this leads to the build-up of thick sticky mucus in and around different organs. This causes persistent lung infections and limits the ability to breathe in the cause of time.
As an inheritance, this defect is transferred from one or both parents, who is a recessive carrier of the mutated gene, to the newborn baby. In the child, the genetic mutation becomes active and causes the condition.
Is it possible to have mild cystic fibrosis?
Atypical CF is a mild form of cystic fibrosis disorder and those suffering from this type of CF, unlike the classic CF patients, may only have a mild dysfunction in one organ system and may or may not have high sweat chloride levels.
How does cystic fibrosis affect the body?
Cystic fibrosis causes the secretion of unusually thick, dehydrated, and sticky mucus blocking the airways, pancreatic ducts, sweat ducts, sinuses, and bowels, resulting in lung infections, inflamed nasal passages, stuffy nose, and breathing difficulties. Other complications may follow.
What does cystic fibrosis poop look like?
Large greasy stools.
Do they test for CF at birth?
Yes. Newborn Screening (NBS) for cystic fibrosis is done in the first few days after birth. Early diagnosis helps parents to learn ways to keep their infected child healthy and to give treatments that will enable them to live better lives.
Is Cystic Fibrosis permanent?
CF is an inherited, life-long disease. It has no cure, but can be adequately managed. These infections cause chronic coughing, wheezing, and inflammation, resulting in permanent lung damage including the formation of scar tissue (fibrosis) and cysts in the lungs.