Cystic Fibrosis(CF) is a condition that interferes with your body's ability to produce mucus, which keeps your systems and your organs functioning.
When you have Cystic Fibrosis, your mucus becomes like a glue and very thick instead of being thin and slick like it should be. This obstructs ducts and tubes all over your body.
This heavy mucus accumulates inside your airways with time. Breathing is challenging as a result. Infections are caused by the mucus, which collects microorganisms. Additionally, it can result in serious lung damage such as scar tissue (fibrosis) and cysts( sacs that are filled with fluid). CF gained its name in this manner.
Cystic fibrosis gives the following symptoms:
Constipation issues or frequently occurring, oily stools.
Breathing difficulties or wheezing
Lung infections that are common.
Reproductive issues, particularly in men.
Difficulty in gaining weight or developing.
Skin with a strong salty taste.
Cystic fibrosis is brought on by a mutation in the CFTR gene (cystic fibrosis transmembrane conductance regulator). This gene regulates the movement of fluids and salt into and out of the cells in your body. When the CFTR gene isn't functioning properly, your body produces a buildup of sticky mucus.
You must inherit the gene with the mutation from your two parents in order to develop CF.
If you have just one copy, you won't show any signs of the illness, but you will be a carrier anyway. Therefore, there is a possibility that you will pass it on to your offspring.
There is a 25% probability that the child of two CF carriers will be born with the disease.
A blood test
This examination measures the amount of immunoreactive trypsinogen (IRT). Blood levels of it are higher in those with Cystic Fibrosis
DNA analysis
This checks for CFTR gene mutations.
A sweat test
Your sweat's salt content is measured. Results that are higher than usual point to Cystic Fibrosis.
Some infants who weren't screened for CF aren't given the diagnosis until they are adults. If you exhibit symptoms of the disease, your doctor could do DNA or sweat testing on you.
Cystic fibrosis has no known cure, however there are treatments that can lessen symptoms.
Your doctor may prescribe medications to make the mucus thin, make your airways open, stop infections, and improve your body's absorption of nutrients from food. These consist of:
Antibiotics: They improve lung function and can be used to treat or prevent lung infections. They may be prescribed to you as tablets, an inhaler, or an injection.
Medications that fight inflammation: Ibuprofen and corticosteroids are a couple of them.
Bronchodilators: These are delivered via inhaler. Your airways will be made more open and relaxed.
Mucus thinners: They'll assist you in clearing the debris from your airways. They are delivered by an inhaler.
Modulators for CFTR: These facilitate proper CFTR operation. They may improve the functionality of your lungs and aid in weight gain.
These can aid in clearing mucus. You could try:
Percussion or chest therapy: To remove mucus from your lungs, do this by tapping or smacking your chest or back. Another individual can do this for you.
Oscillating technology: You inhale into a unique machine that vibrates or oscillates your airways. Mucus is loosened as a result, making coughing easier. A chest vest that oscillates is an alternative.
This involves breathing techniques that force air through thick mucus layers and against your chest wall. They facilitate clearing obstructed airways and make it simpler to cough up debris. Common exercises consist of:
Self drainage: To accomplish this, you huff or exhale forcefully. This facilitates the passage of mucus by moving it away from the smaller to the central airways.
Active cycle of breathing: This regulates breathing and eases the shoulders and upper chest, which can aid in clearing mucus and preventing airway blockages. You take a deep breath, hold, then puff for varying periods of time.
Apart from the lungs, CF also damages other portions of the body. The following organs are also affected by cystic fibrosis:
Pancreas: Your pancreas' ducts are blocked by the thick mucus brought on by CF. This prevents digestive enzymes, which are proteins that help break down food, from getting to your intestine. Your body thus struggles to obtain the nutrition it requires. With time, one may develop diabetes because of this.
Liver: Your liver becomes inflamed if the channels used to drain bile get blocked. This may result in cirrhosis, a severe scarring condition.
Small intestine: The intestinal lining can deteriorate because high-acid foods from your stomach can be challenging to digest.
Large intestine: Your stomach's heavy fluid may cause your faeces to be bulky and challenging to pass. Blockages may result from this. Your intestine may occasionally begin to fold into itself such that it looks like an accordion, a condition known as intussusception.
Bladder: Your bladder muscles become weakened by persistent or chronic coughing. Nearly 65 percent of CF-afflicted females experience stress incontinence. This means that if you laugh, sneeze, cough, or raise something, a small amount of urine leaks out. Men can also get it, though women seem to have it more frequently.
Kidneys: Kidney stones can happen to some CF patients. These tiny, hard mineral pieces can make you feel sick, make you throw up, and hurt. You can develop a kidney infection if you don't get them treated.
Reproduction systems: Both male and female fertility is impacted by excess mucus. Most CF sufferers have issues with the vasa deferentia, which transport sperm. Due to their extremely thick cervical mucus, women with CF may have trouble getting sperm to fertilize eggs.
various other body components.
Additionally, osteoporosis and muscle weakness are side effects of CF. Low blood pressure, weariness, a rapid heartbeat, and a general sensation of weakness can also result from it because it throws off the mineral balance in your blood.
Although cystic fibrosis (CF) is a serious ailment that requires daily care, there are various methods of treatment, and those therapies have greatly improved over time. CF patients nowadays can anticipate living a significantly longer life than patients in the past.