Top Health Tips on Living with Cystic Fibrosis

Cystic Fibrosis is a genetic disorder that is known to affect some of the vital organs of the body including the lungs, digestive system to name a few. For long it was believed that cystic fibrosis affects a person in their childhood. However, various research and studies suggest that adults are also susceptible to this inheritable disorder. In this article, we will discuss the cause and symptoms associated with cystic fibrosis.

Cause
The epithelial cells that line the stomach, nasal cavity and lungs, are responsible for the production of the digestive fluids, sweat, tears, mucus. The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene codes for a protein called the CFTR protein. The CFTR protein, a membrane protein plays a pivotal role in transporting the chloride ions of the epithelial cells inside and out of the cell. This regulation of the chloride ions goes a long way in ensuring that the mucus produced is free-flowing and thin.

In the case of Cystic Fibrosis, the CFTR gene that codes for the protein undergoes a mutation. With the CFTR protein unable to perform its function, sticky and thick mucus builds up and clogs some of the major systems of the body including the digestive system, reproductive, and respiratory system. There is also a higher salt content in the sweat of the affected person.

For a person to be affected by cystic fibrosis, he/she must inherit a copy of the mutated CFTR gene from both the parents ( Autosomal Recessive Disorder) at birth.

Symptoms
The symptoms associated with cystic fibrosis depends on the organs and systems affected.

The thick mucus formed can clog the digestive tract resulting in incomplete absorption of the essential nutrients from the food. This may have an impact on the overall growth and development of the body.
- There may be stunted growth or no weight gain.
- The stool may appear greasy and foul smelling. Some may also suffer from constipation.
- Newborns and infants may suffer from an intestinal blockage.
Cystic fibrosis, left unattended can lead to diabetes, intestinal blockage or obstruction.
- The mucus can also block the bile duct triggering a host of problems including liver disorders.
- At times, the obstruction or blockage can occur at the junction where the large and the small intestine meets (Distal intestinal obstruction syndrome).
The thick mucus can also affect the lungs and the respiratory system by blocking the tubes that act as a passage carrying the air inside and out of the lungs. As a result,
- A person may suffer from lung infections frequently.
- There may be difficulty in breathing, stuffy and blocked nose, or wheezing.
- Some people may also suffer from a chronic cough with a thick sputum.
Left untreated the complications can worsen resulting in

Nasal polyps, Chronic infections, Hemoptysis (there is blood while coughing), and Pneumothorax (air fills up in the space bifurcates the chest wall from the lungs).

In severe cases, there may be a respiratory failure.

Cystic fibrosis can also trigger fertility problems (both in males and females).

Cystic fibrosis is a genetic condition, which primarily affects the lungs and persistence of this condition might lead to severe breathing problems and loss of lung function. If you have inherited two copies of the defective gene from your parents, you are likely to suffer from this disease. Cystic Fibrosis causes a buildup of thick mucus in your lungs and affects other organs such as the kidneys, pancreas and the intestines in your body.

It isn't very uncommon as one out of fifty people in Asia is a carrier of this disease. The disease gets its name from the cysts formed in the pancreas of the sufferer. However, this disease can be detected by testing the quantity of salt present in your sweat. Prenatal testing for Fibrosis is another method to test if your newborn is likely to contract a lung infection.

Complications faced due to cystic fibrosis include:

Serious sinus infections
Serious damage in lungs due to inflammation and incessant infection
Malnutrition
Complication in breaking down protein
Respiratory tract contamination
If you observe one or more of the following symptoms, you might be suffering from Cystic Fibrosis:

Salty-tasting skin
Bowel obstruction caused due to meconium ileus (obstruction of the bowels)
Mucus amiss in your alveoli
Persistent coughing with phlegm discharge
Shortness of breath while engaging in a physical task
Stunted or measly growth despite healthy lifestyle habits
Infertility in males
If you're suffering from cystic fibrosis, there isn't any particular treatment which is recommended due to the varying degrees of it found in people. However, consultation with a medical practitioner and an individualised treatment plan can help you minimise the risks and lead a better life.

Some therapies you can opt for are:

Airway clearance: This therapy helps sift the thick layer of mucus around your organs. Some people have also chosen to use the inflatable vest which vibrates around your chest, thereby, thinning and loosening the layer of mucus.
Inhaled medicines: Another breakthrough in the field of respiratory medicine, this method requires the patient to sniff a liquid consisting of mist, which is inhaled with the aid of a nebuliser. This method is known to be beneficial for opening airways.
Pancreatic enzyme supplements: Along with multivitamins, these specially designed supplements are known to cater to your pancreas with the much-needed nutrients and they need to be consumed with every meal.

Do you know about a condition called cystic fibrosis that makes your baby s skin taste very salty after birth? The condition changes over time, in which the body makes sweat and mucus, and also affects the digestive system and lung functioning. The condition generally occurs because of a flawed gene. This is a severe and life-threatening disease that affects different people in different ways.

Causes
The gene, called CTFR is present in all humans. It makes a protein that controls the amount of water and salt, which get moved in and out of your cells. In the case of cystic fibroids, the CTFR gene is broken. This happens when a person inherits two bad copies of this specific gene. One bad CFTR gene may cause your cells to make the wrong or imbalanced amount of water and salt. Because of this, your body becomes unable to get the supply of sufficient nutrients and oxygen that are lost through sweating.

Symptoms
There are several symptoms associated with cystic fibroids that include the following:

It is likely for you to cough up thin mucus, along with shortness of breath, and wheezing.
The development of polyps in your nose is indicated.
Sinus infections, pneumonia, or bronchitis may occur.
Your stool may be oily, bulky, and with a foul odor.
Liver diseases and gallstones may develop later in life because of cystic fibroids.
Treatment
There is no certain treatment for cystic fibroids. However, there are various treatment procedures that are used for providing relief to the symptoms of the condition. This allows an affected patient to breathe easily, feel better, and experience lesser stomach infections. You can opt for an airway clearance technique that helps you with your breathing. The procedure involves the clearance of mucus from your lungs. This procedure is carried out in several ways and special devices may be used during the proceedings.

Certain inhaled drugs like steroids, salt solutions, and antibiotics are used for thinning your mucus layers, and for clearing lung infections.
If your pancreas do not work properly, you may be prescribed enzyme pills for the digestion of fats and protein, and for the absorption of more nutrients from the food you consume.
You may also be prescribed vitamin A, D, and E supplements for getting these nutrients, which are not being absorbed by your intestine.
You must consult a doctor on experiencing any symptom of cystic fibrosis in your baby. This will allow the condition to be diagnosed at an early stage, thereby preventing further worsening.

Exocrine Pancreatic Insufficiency (EPI), also called pancreatic deficiency, is a disorder where the pancreas is unable to produce the sufficient amount of enzymes that are required to digest food. The pancreatic enzymes help to break down and absorb nutrients from the food in the small intestine. So, this disease causes nutritional deficiencies.

Causes of Exocrine Pancreatic Insufficiency

This condition is generally caused as a complication of other diseases because EPI develops only as a result of severe damage to the pancreas. The following reasons can cause EPI:

1. Inflammation of the pancreas
After surgery in the pancreas, stomach or the intestines, there may be inflammation in the pancreas as a post-surgical complication. A high content of triglyceride fat in the blood can also cause pancreatic inflammation and hinder the secretion of the enzymes.

2. Chronic Pancreatitis
In this disease, the pancreatic ducts are swollen and blocked and so the digestive enzymes cannot be passed into the small intestine. This condition is often caused by a heavy consumption of alcohol.

3. Cystic Fibrosis
The digestive fluids and enzymes become thick and sticky and block the passageways of the pancreas and other organs like the lungs and the kidneys. This can obstruct secretion and passage of enzymes afterwards.

4. Shwachman-Bodian-Diamond syndrome
This is an autosomal recessive genetic condition where enzyme-producing pancreatic cells are not formed properly. This rare disorder causes a number of associated disorders like bone marrow diseases, skeletal defects and exocrine pancreatic insufficiency.

Symptoms of Exocrine Pancreatic Insufficiency

The symptoms appear in the middle stages of the disease when the process of absorption of nutrients has already been affected. The common warning signs are:

1. Digestive problems like gas, diarrhea and indigestion because the food remains mostly undigested.
2. Severe and frequent stomach pain in the lower abdominal region
3. Greasy stools due to the excretion of undigested fat
4. Rapid loss of weight and body mass due to mal-absorption of nutrients
5. Constantly feeling bloated and full even if you have not eaten anything
6. A general sense of fatigue and exhaustion
7. Excessive bleeding from small wounds because protein deficiencies hamper blood clotting
8. Pain in the muscles and bones
9. Increased susceptibility to infections of the body systems
10.Anemia
11.Joint pains
12.Abnormal swelling of the limbs or edema

Do you know about a condition called cystic fibrosis that makes your baby s skin taste very salty after birth? The condition changes over time, in which the body makes sweat and mucus, and also affects the digestive system and lung functioning. The condition generally occurs because of a flawed gene. This is a severe and life-threatening disease that affects different people in different ways.

Causes
The gene, called CTFR is present in all humans. It makes a protein that controls the amount of water and salt, which get moved in and out of your cells. In the case of cystic fibroids, the CTFR gene is broken. This happens when a person inherits two bad copies of this specific gene. One bad CFTR gene may cause your cells to make the wrong or imbalanced amount of water and salt. Because of this, your body becomes unable to get the supply of sufficient nutrients and oxygen that are lost through sweating.

Symptoms
There are several symptoms associated with cystic fibroids that include the following:

It is likely for you to cough up thin mucus, along with shortness of breath, and wheezing.
The development of polyps in your nose is indicated.
Sinus infections, pneumonia, or bronchitis may occur.
Your stool may be oily, bulky, and with a foul odor.
Liver diseases and gallstones may develop later in life because of cystic fibroids.
Treatment
There is no certain treatment for cystic fibroids. However, there are various treatment procedures that are used for providing relief to the symptoms of the condition. This allows an affected patient to breathe easily, feel better, and experience lesser stomach infections. You can opt for airway clearance technique that helps you with your breathing. The procedure involves the clearance of mucus from your lungs. This procedure is carried out in several ways and special devices may be used during the proceedings.

Certain inhaled drugs like steroids, salt solutions, and antibiotics are used for thinning your mucus layers, and for clearing lung infections.
If your pancreas do not work properly, you may be prescribed enzyme pills for the digestion of fats and protein, and for the absorption of more nutrients from the food you consume.
You may also be prescribed vitamin A, D, and E supplements for getting these nutrients, which are not being absorbed by your intestine.
You must consult a doctor on experiencing any symptom of cystic fibrosis in your baby. This will allow the condition to be diagnosed at an early stage, thereby preventing further worsening.

Exocrine Pancreatic Insufficiency (EPI), also called pancreatic deficiency, is a disorder where the pancreas is unable to produce the sufficient amount of enzymes that are required to digest food. The pancreatic enzymes help to break down and absorb nutrients from the food in the small intestine. So, this disease causes nutritional deficiencies.

Causes of Exocrine Pancreatic Insufficiency

This condition is generally caused as a complication of other diseases because EPI develops only as a result of severe damage to the pancreas. The following reasons can cause EPI:

1. Inflammation of the pancreas
After surgery in the pancreas, stomach or the intestines, there may be inflammation in the pancreas as a post-surgical complication. A high content of triglyceride fat in the blood can also cause pancreatic inflammation and hinder the secretion of the enzymes.

2. Chronic Pancreatitis
In this disease, the pancreatic ducts are swollen and blocked and so the digestive enzymes cannot be passed into the small intestine. This condition is often caused by a heavy consumption of alcohol.

3. Cystic Fibrosis
The digestive fluids and enzymes become thick and sticky and block the passageways of the pancreas and other organs like the lungs and the kidneys. This can obstruct secretion and passage of enzymes afterwards.

4. Shwachman-Bodian-Diamond syndrome
This is an autosomal recessive genetic condition where enzyme-producing pancreatic cells are not formed properly. This rare disorder causes a number of associated disorders like bone marrow diseases, skeletal defects and exocrine pancreatic insufficiency.

Symptoms of Exocrine Pancreatic Insufficiency

The symptoms appear in the middle stages of the disease when the process of absorption of nutrients has already been affected. The common warning signs are:

1. Digestive problems like gas, diarrhea and indigestion because the food remains mostly undigested.
2. Severe and frequent stomach pain in the lower abdominal region
3. Greasy stools due to the excretion of undigested fat
4. Rapid loss of weight and body mass due to mal-absorption of nutrients
5. Constantly feeling bloated and full even if you have not eaten anything
6. A general sense of fatigue and exhaustion
7. Excessive bleeding from small wounds because protein deficiencies hamper blood clotting
8. Pain in the muscles and bones
9. Increased susceptibility to infections of the body systems
10. Anemia
11. Joint pains
12. Abnormal swelling of the limbs or edema

Cystic fibrosis is a genetic condition, which primarily affects the lungs and persistence of this condition might lead to severe breathing problems and loss of lung function. If you have inherited two copies of the defective gene from your parents, you are likely to suffer from this disease. Cystic Fibrosis causes a buildup of thick mucus in your lungs and affects other organs such as the kidneys, pancreas and the intestines in your body.

It isn't very uncommon as one out of fifty people in Asia is a carrier of this disease. The disease gets its name from the cysts formed in the pancreas of the sufferer. However, this disease can be detected by testing the quantity of salt present in your sweat. Prenatal testing for Fibrosis is another method to test if your newborn is likely to contract a lung infection.

Complications faced due to cystic fibrosis include:

Serious sinus infections
Serious damage in lungs due to inflammation and incessant infection
Malnutrition
Complication in breaking down protein
Respiratory tract contamination
If you observe one or more of the following symptoms, you might be suffering from Cystic Fibrosis:

Salty-tasting skin
Bowel obstruction caused due to meconium ileus (obstruction of the bowels)
Persistent coughing with phlegm discharge
Shortness of breath while engaging in a physical task
Stunted or measly growth despite healthy lifestyle habits
Infertility in males
If you're suffering from cystic fibrosis, there isn't any particular treatment which is recommended due to the varying degrees of it found in people. However, consultation with a medical practitioner and an individualised treatment plan can help you minimise the risks and lead a better life.

Diagnosis:

Diagnosis of Cystic fibrosis can be made by measuring the quality of salt i.e. the amount of chloride in your sweat (sweat chloride test). Genetic testing for CFTR gene mutation detection can also be done at specialized centres.
Some therapies you can opt for are:

Airway clearance: This therapy helps sift the thick layer of mucus around your organs. Some people have also chosen to use the inflatable vest which vibrates around your chest, thereby, thinning and loosening the layer of mucus.
Inhaled medicines: Another breakthrough in the field of respiratory medicine, this method requires the patient to sniff a liquid consisting of mist, which is inhaled with the aid of a nebuliser. This method is known to be beneficial for opening airways.
Pancreatic enzyme supplements: Along with multivitamins, these specially designed supplements are known to cater to your pancreas with the much-needed nutrients and they need to be consumed with every meal.

Influenza is more commonly known as the flu. It is an infection that affects the respiratory system and is caused by the influenza virus. This injection is recommended for kids who are under the age of 6 months. It ensures that the baby is safeguarded from the virus during an influenza outbreak. Since the nature of virus changes year on year, the immunity doesn t last for more than 1 year. This vaccination is ideally taken during the month of October and November. The effect of the vaccination typically lasts for 6-8 months.

Ideally, kids who have never had a flu vaccination should take 2 vaccinations within 4 weeks. Kids who have received a shot of flu should undertake this vaccination once in a year. This arrangement is ideal up to 9 years of age. Flu shot is extremely important for a kid due to the following reasons:

It saves a kid from chronic lung and heart disorders such as asthma and cystic fibrosis
Kids suffering from immune deficiency, cancer, and HIV should take this vaccination to avoid further complication of the existing disease
Kids suffering from diabetes, kidney diseases, and metabolic disorders should go for this vaccination without a second thought
Kids suffering from a neurological disorder, haemoglobin deficiency, and anaemia should take this vaccination to refrain from the risk of aggravation of the present disease
Pregnant women should ideally take it for the safeguarding the fetus
Flu shot should not be taken be taken if:
1. A baby is under the age of 6 months.
2. The baby has thimerosal allergy.

Is flu shot safe?
The flu shot is considered to be very safe. It does not result in flu. Few side effects include:
a. Soreness of the place where the needle is injected. This doesn t last for more than a couple of day.
b. Body ache and mild fever for the first couple of days after the vaccination is done.
c. There is little to no pain in the place where the injection is injected by the doctor.

What is nasal flu vaccine?
This is an influenza dose that is not injected into the body but given in the form of nasal spray. Healthy kids above the age of 2 years are fit to get this injection. For those kids who have a history of chronic illness should check with their doctor to go ahead with nasal spray. Each dose is given in each nostril. Like the influenza vaccine, nasal flu vaccine should be undertaken once for those who have already taken this dose once. Kids who have not undergone this vaccination are eligible for 2 dosages each 4 weeks apart from each other.

Cystic fibrosis is a genetic condition, which primarily affects the lungs and persistence of this condition might lead to severe breathing problems and loss of lung function. If you have inherited two copies of the defective gene from your parents, you are likely to suffer from this disease. Cystic Fibrosis causes a buildup of thick mucus in your lungs and affects other organs such as the kidneys, pancreas and the intestines in your body.

It isn't very uncommon as one out of fifty people in Asia is a carrier of this disease. The disease gets its name from the cysts formed in the pancreas of the sufferer. However, this disease can be detected by testing the quantity of salt present in your sweat. Prenatal testing for Fibrosis is another method to test if your newborn is likely to contract a lung infection.

Complications faced due to cystic fibrosis include:

Serious sinus infections
Serious damage in lungs due to inflammation and incessant infection
Malnutrition
Complication in breaking down protein
Respiratory tract contamination
If you observe one or more of the following symptoms, you might be suffering from Cystic Fibrosis:

Salty-tasting skin
Bowel obstruction caused due to meconium ileus (obstruction of the bowels)
Mucus amiss in your alveoli
Persistent coughing with phlegm discharge
Shortness of breath while engaging in a physical task
Stunted or measly growth despite healthy lifestyle habits
Infertility in males
If you're suffering from cystic fibrosis, there isn't any particular treatment which is recommended due to the varying degrees of it found in people. However, consultation with a medical practitioner and an individualised treatment plan can help you minimise the risks and lead a better life.

Some therapies you can opt for are:

Airway clearance: This therapy helps sift the thick layer of mucus around your organs. Some people have also chosen to use the inflatable vest which vibrates around your chest, thereby, thinning and loosening the layer of mucus.
Inhaled medicines: Another breakthrough in the field of respiratory medicine, this method requires the patient to sniff a liquid consisting of mist, which is inhaled with the aid of a nebuliser. This method is known to be beneficial for opening airways.
Pancreatic enzyme supplements: Along with multivitamins, these specially designed supplements are known to cater to your pancreas with the much-needed nutrients and they need to be consumed with every meal.

Cystic fibrosis is a genetic condition, which primarily affects the lungs and persistence of this condition might lead to severe breathing problems and loss of lung function. If you have inherited two copies of the defective gene from your parents, you are likely to suffer from this disease. Cystic Fibrosis causes a buildup of thick mucus in your lungs and affects other organs such as the kidneys, pancreas and the intestines in your body.

It isn't very uncommon as one out of fifty people in Asia is a carrier of this disease. The disease gets its name from the cysts formed in the pancreas of the sufferer. However, this disease can be detected by testing the quantity of salt present in your sweat. Prenatal testing for Fibrosis is another method to test if your newborn is likely to contract a lung infection.

Complications faced due to cystic fibrosis include:

Serious sinus infections
Serious damage in lungs due to inflammation and incessant infection
Malnutrition
Complication in breaking down protein
Respiratory tract contamination
If you observe one or more of the following symptoms, you might be suffering from Cystic Fibrosis:

Salty-tasting skin
Bowel obstruction caused due to meconium ileus (obstruction of the bowels)
Mucus amiss in your alveoli
Persistent coughing with phlegm discharge
Shortness of breath while engaging in a physical task
Stunted or measly growth despite healthy lifestyle habits
Infertility in males
If you're suffering from cystic fibrosis, there isn't any particular treatment which is recommended due to the varying degrees of it found in people. However, consultation with a medical practitioner and an individualised treatment plan can help you minimise the risks and lead a better life.

Some therapies you can opt for are:

Airway clearance: This therapy helps sift the thick layer of mucus around your organs. Some people have also chosen to use the inflatable vest which vibrates around your chest, thereby, thinning and loosening the layer of mucus.
Inhaled medicines: Another breakthrough in the field of respiratory medicine, this method requires the patient to sniff a liquid consisting of mist, which is inhaled with the aid of a nebuliser. This method is known to be beneficial for opening airways.
Pancreatic enzyme supplements: Along with multivitamins, these specially designed supplements are known to cater to your pancreas with the much-needed nutrients and they need to be consumed with every meal.