Cystic Fibrosis (CF)
Cystic Fibrosis is a chronic disease that affects the lungs, digestive system and other body parts. It is a life threatening genetic disease affecting the young children and adults.

Causes and Risk factor of Cystic Fibrosis
The disease is caused due to defect in the gene which produces a protein called CFTR which lines the digestive tract, respiratory tract, urinary tract and sweat glands.
Due the defective gene the cells that produce mucus, sweat and digestive juices are affected. The secretions from the sweat gland or other secretions becomes thick and sticky. Hence these secretions blocks the ducts of pancreas or lungs. This sticky mucus can be life threatening lung infection if they stick in the lungs and cause breathing difficulty or may cause severe digestive illness as the food will not be able to pass smoothly through the gut .
Risk Factors - 1) It is a genetic disorder so it runs in the families. If any of the parents are suffering from the illness then there is high chance of passing those defective gene to the child and thus the cycle goes on.
2) Commonly seen in Americans and Europeans ( white people)
3) Younger kids are commonly affected.

Clinical Manifestations with complications
The symptoms vary from person to person and shows the symptoms depending on the area affected.
1) In babies (infants) - The mother notices that the skin of baby tastes salty whenever she kisses her. It happens because CF affects the epithelial cells in sweat glands. Also baby will not pass the stool immediately after the birth. It is because the mucus becomes so thick that it blocks the glands and intestines.

2) Respiratory system - Recurrent lung infection due to blockage of airways. Recurrent cough with thick bloody sputum, pneumonia and bronchitis develop over the time. Wheezing sound present during breathing. Recurrent sinus infection ( Sinusitis) occurs.
Serious complications like pneumothorax may develop where the child is unable to breath due to accumulation of excess air in the lungs and unable to expel that air.

3) Digestive System - The sticky mucus can block the pancreatic duct so that the digestive enzymes cannot pass through pancreas to the intestines. This can lead to abdomen distention, constipation, foul smelling stools, loss of weight or no weight gain. Some children also suffer from diarrhoea.
Complications likely to occur are inflammation of pancreas leading to severe abdominal pain.
Liver disease and Gall stones due to blockage of bile ducts present in the liver.

4) General - Vitamin deficiencies occur as intestines cannot absorb fats and other mineral required for the nutrition.
Excess loss of salt in the sweat can lead to dehydration, weakness, increased or irregular heartbeats, decrease in blood pressure and sometimes death.
CF also affects the bone density which causes thinning of bones and finally lead to osteoporosis.

Diagnosis
1) Sweat Test - It helps to confirm the diagnosis of Cystic Fibrosis (CF). In this test a chemical called pilocarpine is applied to small portion of the skin and a painless electric current is passed through it to stimulate the sweat glands in that area which produces sweat in 30 to 60 minutes. The sweat is collected and tested for chloride. If the sweat chloride has value more than 60 in two tests then it is the confirm diagnosis of cystic fibrosis.

2) Gene (DNA) Test - DNA samples are collected from the blood or saliva and tested for the specific gene of cystic fibrosis. The newborns are screened for cystic fibrosis in many countries. This screening shows whether the child has faulty CFTR gene or not.

3) X-rays - Chest x-ray is performed to detect the abnormalities in lungs and heart. It helps to understand whether the air is trapped into the lungs or whether the lungs are functioning properly or not. Sinus x-ray is also performed to see the level of sinus affection due to the infection.

4) Prenatal screening test - It is the genetic testing of fetus done in pregnant women to find out the defective CFTR gene.

Treatment and Management
There is no cure for cystic fibrosis but treatment can improve the lifestyle and reduce the suffering of the kids. The treatment depends on the affection of system.

A) Treatment for Lung Infections -
1) Breathing techniques - The parents or kids are taught the breathing techniques like deep breathing, relax breathing and short breaths. This technique helps to reduce or dislodge the mucus present in the respiratory tract and opens the airways.
2) Chest Physiotherapy - It is a therapy given by physiotherapist. It includes tapping over chest and back with hands to loosen the mucus in the airways.
3) Medicines- Medicines like antibiotics to prevent lung infection, mucus thinning medicine and bronchodilators which help to relax the airway are prescribed. If the lung disease is too advanced where oxygen levels are too low then oxygen is provided through nose.
4) Physical Exercise - Exercise like aerobics helps to maintain the physical condition balanced by improving the airways without any breathing difficulty.

B) Treatment for Digestive diseases -
1) Nutritional Supplements - As the intestines cannot absorb fats and other nutrition from the body , nutritional supplement are prescribed to gain more weight and maintain the body nutrition. They also helps to resist the recurrent lung and sinus infections.
Along with vitamin supplements the child also require proper nutritious food which is high in calories and vitamin.
2) High salt intake - Diet must include high salt as excess of salt is lost during the exercise and sweat.
3) Pancreatic enzymes - The oral medicines of pancreatic enzymes are given as pancreas get blocked by the mucus and the required enzymes are not transferred to the intestines.
4) Surgery - If the intestines are blocked completely surgery is the option to remove the block.

C) Treatment for the complications and other diseases -
1) Diabetes - Diabetes is the common complication of cystic fibrosis. So depending on the blood sugar levels the medications and diet is prescribed by the doctors.
2) Respiratory complications - Serious life threatening respiratory complications are managed at the hospitals. The child require to be admitted to the emergency room where all critical cases are handled under close observation.
3) Osteoporosis - The doctors prescribe medications like vitamin D and calcium to prevent bone thinning and finally osteoporosis.

D) Gene Therapy - Gene therapy is latest tretament and various clinical trial are going all over US. In this therapy the defective gene and protein is corrected into normal gene CFTR.

E) Other Measures and Advice -
1) Take regular checkup appointment with the doctors so that treatment is not delayed.
2) Maintain the proper lifestyle like no smoking, eat proper nutritious food, drink plenty of water, regular physical exercise and maintain personal hygeine.
3) Avoid emotional stress and anxiety by involving in the activities that you like. Yoga and meditation is good for mental disturbances and improves the breathing.