The primary aim of medical and scientific research on cystic fibrosis is to understand, treat and cure cystic fibrosis. An inherited disease, cystic fibrosis is thought to affect about 30,000 Americans and is the most common, life-shortening genetic disease known. Cystic fibrosis is a life-threatening disease caused by a defective gene and affecting about 30,000 children in America.
Researchers from the Beth Israel Deaconess Medical Centre, the University of Massachusetts and Massachusetts General Hospital took tissue samples from 38 patients with cystic fibrosis. It was found they had extremely high levels of arachidonic acid (AA) and abnormally low levels of docosahexaenoic acid (DHA). People who did not have CF did not have the fatty acids imbalance.
Researchers believe that many of the symptoms of cystic fibrosis follow the same pattern: mutated gene produces a mutated glycoconjugate resulting in a defective cellular component. There are approximately ten million symptomless carriers of the defective cystic fibrosis gene in America.
They say too much of one acid and too little of another means patients' bodies are more prone to inflammation. In the New England Journal of Medicine, they suggest Omega-3 oils, found in fish, could help correct the imbalance.
Each week three young people in the UK die from the disease, which is caused by the faulty CFTR gene. CF causes an abnormally thick, sticky mucus to be produced in the body, causing chronic inflammation of the lungs leading to life-threatening infections. The average life expectancy for a person with CF is around 31.
To diagnose cystic fibrosis, the laboratory carries out a sweat test. When the lungs and airways are choked, the cystic fibrosis patient coughs and produces very thick sputum. The authors concluded that forced ionisation of the indoor air represents a natural and efficient treatment for respiratory diseases in patients with cystic fibrosis.
When the cystic fibrosisTR is not normal, the regulation of salt through the membranes becomes defective. In the respiratory system the thin mucus lining becomes thick and sticky. As the digestive juices do not reach the intestine, due to blocked ducts from the pancreas and liver, the fats and protein are not digested.
Dr Steven Freedman of the gastroenterology division at Beth Israel Deaconess Medical Center, who led the research, said: "Since 1989, we have known that the defective CFTR gene is responsible for CF. "But we didn't understand how this defective gene leads to the symptoms of the disease. "This new study sheds light on what may be happening and provides a link between CFTR function and fatty acid metabolism." He added: "It is known that high amounts of AA and low amounts of DHA would predispose to inflammation. "This discovery may help explain why there is an excessive inflammatory response among CF patients. "This is the basis for why Omega-3 fish oils, found in cold-water fish as well as supplements, reduce inflammation since they increase levels of DHA and suppress AA." 'No diet change'
Dr Adam Jaffe, head of the CF Research Group at London's Institute of Child Health, told BBC News Online the research was interesting but not conclusive. "Patients shouldn't change their diet based on spurious associations between fatty acids and inflammation. "But I would not be against them adding supplements to their diets."
About the Author
Tom O`Connor is an expert on the benefits of Omega-3 fatty acids. http://www.1st-Omega-3.Com.
Tuesday, May 08, 2007
Thursday, May 03, 2007
Liver Disease in Cystic Fibrosis
The median age of the population with cystic fibrosis (CF) has increased worldwide, which has led to the suggestion that the prevalence of liver disease would increase. The aim of this study was to evaluate the natural history of CF-associated liver disease over a 15-year period in a well-controlled population of patients with CF. During the years 1976 through 1993, 124 patients were followed up by yearly liver function tests (LFTs). Fifteen patients were followed up with liver biopsies throughout the whole study period.
More than 50% of the patients had pathological LFTs in infancy, later being normalized. Approximately 25% of children 4 years of age or older had biochemical markers of liver disease during the study period. In about 10% of the patients, cirrhosis or advanced fibrosis was confirmed at biopsy and 4% of patients had cirrhosis with clinical liver disease.
Severe liver disease developed mainly during prepuberty and puberty. Of the 15 patients prospectively followed up with liver biopsies, only 3 had progressive fibrosis. No specific risk factor was identified, but deficiency of essential fatty acids was found more often in patients with marked steatosis (P < .05).
No patient developed clinical liver disease in adulthood and the histological changes in the liver biopsies were usually not progressive. Liver disease was no more frequent at the end of the study period although the median age of the patient population had increased.
Modern treatment might positively influence liver disease because it seemed less common, less progressive, and less serious than previously reported.
More than 50% of the patients had pathological LFTs in infancy, later being normalized. Approximately 25% of children 4 years of age or older had biochemical markers of liver disease during the study period. In about 10% of the patients, cirrhosis or advanced fibrosis was confirmed at biopsy and 4% of patients had cirrhosis with clinical liver disease.
Severe liver disease developed mainly during prepuberty and puberty. Of the 15 patients prospectively followed up with liver biopsies, only 3 had progressive fibrosis. No specific risk factor was identified, but deficiency of essential fatty acids was found more often in patients with marked steatosis (P < .05).
No patient developed clinical liver disease in adulthood and the histological changes in the liver biopsies were usually not progressive. Liver disease was no more frequent at the end of the study period although the median age of the patient population had increased.
Modern treatment might positively influence liver disease because it seemed less common, less progressive, and less serious than previously reported.
Thursday, April 19, 2007
Cystic Fibrosis With Vitamins And Over The Counter Products
Cystic fibrosis is a hereditary disease that affects the entire body, causing progressive disability and early death. Cystic fibrosis affects the entire body and impacts growth, breathing, digestion, and reproduction. Difficulty breathing and insufficient enzyme production in the pancreas are the most common symptoms.
The Pancreas of patients with Cystic Fibrosis fails to produce enough enzymes that are necessary to break down food. As a result the food eaten retains its fats and most of its nutrients as it passes through the body.
The bronchial tubes in the lungs also malfunction and produce a thick, sticky mucus. Germs multiply in this mucus and cause respiratory infection such as pneumonia, accompanied by a cough and high fever that is more severe than normal.
A multitude of other symptoms, including sinus infections, poor growth, diarrhea, and potential infertility (mostly in males) result from the effects of cystic fibrosis on other parts of the body. Patients with Cystic Fibrosis also sweat profusely and their perspirations also contains an unusually high percentage of salt.
Cystic fibrosis is the most common life-limiting recessive disease among people of European heritage. Two copies of the recessive mutated gene, one from each parent is needed by the human body to develop Cystic Fibrosis.
Because cystic fibrosis testing is expensive, testing is often performed on just one parent initially. If that parent is found to be a carrier of a CFTR gene mutation, the other parent is then tested to calculate the risk that their children will have cystic fibrosis. Cystic fibrosis can result from more than a thousand different mutations and, as of 2006, it is not possible to test for each one. Most commercially available tests look for 32 or fewer different mutations.
Couples who are at high risk for having a child with cystic fibrosis; i.e. cystic fibrosis has developed in family members, will often opt to perform further testing before or during pregnancy. After birth cystic fibrosis may be diagnosed in newborn with sweat testing, or genetic testing.
Most states and countries do not screen for cystic fibrosis routinely at birth. Children with cystic fibrosis typically do not gain weight or height at the same rate as their peers and occasionally are not diagnosed until investigation is initiated for poor growth. Males tend to have a longer life expectancy than females but the reason is unknown.
Use a mask nebulizer and other inhalations treatments are the most common forms of treatments for cystic fibrosis. The goal is the treating and limiting the amount of lung damage caused by thick mucus and infection. Albuterol and ipratropium bromide are inhaled to increase the size of the small airways by relaxing the surrounding muscles. As lung disease worsens, breathing support from machines may become necessary.
Most individuals with cystic fibrosis take additional amounts of vitamins A, D, E, and K and eat high calorie meals.
Common Vitamins and over the counter products can help with treating Cystic Fibrosis such as Vitamin E, Vitamin K, Vitamin A, Lactase Enzyme, Papaya, Protein Tablets, Vitamin B, Amino Acid and L-Carnitine.
Dr. Harry Schwachman reported "most patients with cystic fibrosis have low levels of vitamin E". Vitamin E protects lung tissue form inhaled pollutants and aids the functioning of the immune system.
Vitamin K helps the blood to clot after and injury.
A number of studies have suggested that taking antioxidants such a Vitamin A reduces the risk of bronchoconstriction. Vitamin A is stored in the liver and fat cells of the human body and can reach toxic levels. DO NOT take more than the recommended dosage of Vitamin A.
Lactase Enzyme makes milk products more readily digestible.
Papaya contains lipase, which assists in Fat and Cellulose digestion.
Protein tablets increase protein intake.
All of the different Vitamin Bs taken together as B-Complex work together as a team to perform vital biological processes, such as energy production and efficient metabolic function. Vitamin B boost energy levels and help fight fatigue.
Amino Acid help regulate growth, digestion and maintaining the body's immune system.
L-Carnitine is essential for the body's ability to turn food into energy. L-Carnation increases energy at the cell level by increased fat burning, increases the body's ability to remove toxic disease-causing compounds and helps cells live longer.
Always consult your doctor before using this information.
This Article is nutritional in nature and not to be construed as medical advice.
About the Author
David Cowley has created over 50 articles about the relationship between diseases and vitamins. For other Articles on Diseases and Vitamin Needs feel free to visit my Web Site at http://www.dfcinvestment-team.com/
The Pancreas of patients with Cystic Fibrosis fails to produce enough enzymes that are necessary to break down food. As a result the food eaten retains its fats and most of its nutrients as it passes through the body.
The bronchial tubes in the lungs also malfunction and produce a thick, sticky mucus. Germs multiply in this mucus and cause respiratory infection such as pneumonia, accompanied by a cough and high fever that is more severe than normal.
A multitude of other symptoms, including sinus infections, poor growth, diarrhea, and potential infertility (mostly in males) result from the effects of cystic fibrosis on other parts of the body. Patients with Cystic Fibrosis also sweat profusely and their perspirations also contains an unusually high percentage of salt.
Cystic fibrosis is the most common life-limiting recessive disease among people of European heritage. Two copies of the recessive mutated gene, one from each parent is needed by the human body to develop Cystic Fibrosis.
Because cystic fibrosis testing is expensive, testing is often performed on just one parent initially. If that parent is found to be a carrier of a CFTR gene mutation, the other parent is then tested to calculate the risk that their children will have cystic fibrosis. Cystic fibrosis can result from more than a thousand different mutations and, as of 2006, it is not possible to test for each one. Most commercially available tests look for 32 or fewer different mutations.
Couples who are at high risk for having a child with cystic fibrosis; i.e. cystic fibrosis has developed in family members, will often opt to perform further testing before or during pregnancy. After birth cystic fibrosis may be diagnosed in newborn with sweat testing, or genetic testing.
Most states and countries do not screen for cystic fibrosis routinely at birth. Children with cystic fibrosis typically do not gain weight or height at the same rate as their peers and occasionally are not diagnosed until investigation is initiated for poor growth. Males tend to have a longer life expectancy than females but the reason is unknown.
Use a mask nebulizer and other inhalations treatments are the most common forms of treatments for cystic fibrosis. The goal is the treating and limiting the amount of lung damage caused by thick mucus and infection. Albuterol and ipratropium bromide are inhaled to increase the size of the small airways by relaxing the surrounding muscles. As lung disease worsens, breathing support from machines may become necessary.
Most individuals with cystic fibrosis take additional amounts of vitamins A, D, E, and K and eat high calorie meals.
Common Vitamins and over the counter products can help with treating Cystic Fibrosis such as Vitamin E, Vitamin K, Vitamin A, Lactase Enzyme, Papaya, Protein Tablets, Vitamin B, Amino Acid and L-Carnitine.
Dr. Harry Schwachman reported "most patients with cystic fibrosis have low levels of vitamin E". Vitamin E protects lung tissue form inhaled pollutants and aids the functioning of the immune system.
Vitamin K helps the blood to clot after and injury.
A number of studies have suggested that taking antioxidants such a Vitamin A reduces the risk of bronchoconstriction. Vitamin A is stored in the liver and fat cells of the human body and can reach toxic levels. DO NOT take more than the recommended dosage of Vitamin A.
Lactase Enzyme makes milk products more readily digestible.
Papaya contains lipase, which assists in Fat and Cellulose digestion.
Protein tablets increase protein intake.
All of the different Vitamin Bs taken together as B-Complex work together as a team to perform vital biological processes, such as energy production and efficient metabolic function. Vitamin B boost energy levels and help fight fatigue.
Amino Acid help regulate growth, digestion and maintaining the body's immune system.
L-Carnitine is essential for the body's ability to turn food into energy. L-Carnation increases energy at the cell level by increased fat burning, increases the body's ability to remove toxic disease-causing compounds and helps cells live longer.
Always consult your doctor before using this information.
This Article is nutritional in nature and not to be construed as medical advice.
About the Author
David Cowley has created over 50 articles about the relationship between diseases and vitamins. For other Articles on Diseases and Vitamin Needs feel free to visit my Web Site at http://www.dfcinvestment-team.com/
Tuesday, November 28, 2006
Supplements Cystic Fibrosis
Supplements Important to Cystic Fibrosis Patients by Steven Godlewski
Cystic Fibrosis or CF is an inherited disease that affects the normal movement of salt (sodium chloride) into and out of certain cells, including those that line the lungs and pancreas. This results in thick, sticky mucus and other secretions. The mucus clogs the lungs, causing breathing problems. It also provides a breeding ground for bacteria to grow. This leads to frequent lung infections, which eventually damage the lungs and contribute to early death.
Thick digestive fluids also may clog ducts leading from the pancreas to the small intestine. This prevents the fluids from reaching the small intestine, where they are needed to digest food. This can cause digestive problems and slow growth. Some CF patients also suffer from poor liver function which may eventually become chronic liver disease.
Malabsorption of fat soluble vitamins is likely in most patients with cystic fibrosis, especially for those who are pancreatic insufficient. Vitamins A, D and E have been found to be deficient in early diagnosed infants as well as older patients. Different levels of these vitamins are prescribed depending on the age of the patient, whether the patient is pancreatic insufficient or sufficient and other health factors that can be affected by cystic fibrosis. Vitamin K may also be prescribed under certain conditions.
The recommended daily supplements which usually achieve normal plasma levels in infancy are vitamin A 4000 iu (120 mcg), vitamin D 400 iu (10 mcg) and vitamin E 37 - 75 iu (25 - 50 mg). The recommended doses for children over 1 year of age are vitamin A 8000 iu, vitamin D 800 iu and vitamin E 100 - 200 mg.
These doses are considerably higher than the usual dietary intake and generally are adjusted to meet the needs of the patient as they get older. Vitamin K is generally prescribed for those cystic fibrosis patients who suffer from liver disease or who are going to undergo a surgical procedure, or whose blood tests do not me certain standards.
* Vitamin A: Vitamin A deficiency may cause night blindness in older patients and can progress to severe xerophthalmia if not checked.
* Vitamin D: Vitamin D deficiency may cause rickets which is very rare and osteomalacia. Although in the past there is little clinical evidence of vitamin D deficiency is rare in cystic fibrosis new research and studies osteoporosis, osteopenia and low levels of vitamin D metabolites are being increasingly recognized in children and adults with cystic fibrosis.
* Vitamin E: Vitamin E deficiency may cause neurological problems in older CF individuals. Correction of vitamin E deficiency improves hemoglobin levels. Vitamin E is an antioxidant and protects cell membranes from oxidative damage. Because of this role vitamin E may be important in controlling the progression of lung disease in cystic fibrosis.
Recent studies have suggested that cystic fibrosis patients have inadequate antioxidants defenses to cope with certain stresses on their system. Pulmonary dysfunction in cystic fibrosis is associated with oxidative stress and higher levels of supplementation may be required.
* Vitamin K: Regular vitamin K supplements are not given unless there is chronic liver disease, a prolonged prothrombin time or an upcoming surgical procedure. Given any of these conditions an oral daily supplement of 5-10mg is given for a week prior to the procedure. Vitamin K is required for the formation of osteocalcin which is involved in bone metabolism.
As you can see vitamin supplementation is essential for Cystic Fibrosis patients. It is crucial for you to discuss any changes to your current vitamin regimen with your physician prior to adding, removing or changing the doses of any vitamins. The balance of vitamins in your system can be very delicate and should be closely monitored by your physician.
About the Author
Steven Godlewski is currently working with the staff at PillFreeVitamins.com He has an extensive background in nutrition as well as other health related fields. For more health-related articles see their website at: http://www.pillfreevitamins.com
Cystic Fibrosis or CF is an inherited disease that affects the normal movement of salt (sodium chloride) into and out of certain cells, including those that line the lungs and pancreas. This results in thick, sticky mucus and other secretions. The mucus clogs the lungs, causing breathing problems. It also provides a breeding ground for bacteria to grow. This leads to frequent lung infections, which eventually damage the lungs and contribute to early death.
Thick digestive fluids also may clog ducts leading from the pancreas to the small intestine. This prevents the fluids from reaching the small intestine, where they are needed to digest food. This can cause digestive problems and slow growth. Some CF patients also suffer from poor liver function which may eventually become chronic liver disease.
Malabsorption of fat soluble vitamins is likely in most patients with cystic fibrosis, especially for those who are pancreatic insufficient. Vitamins A, D and E have been found to be deficient in early diagnosed infants as well as older patients. Different levels of these vitamins are prescribed depending on the age of the patient, whether the patient is pancreatic insufficient or sufficient and other health factors that can be affected by cystic fibrosis. Vitamin K may also be prescribed under certain conditions.
The recommended daily supplements which usually achieve normal plasma levels in infancy are vitamin A 4000 iu (120 mcg), vitamin D 400 iu (10 mcg) and vitamin E 37 - 75 iu (25 - 50 mg). The recommended doses for children over 1 year of age are vitamin A 8000 iu, vitamin D 800 iu and vitamin E 100 - 200 mg.
These doses are considerably higher than the usual dietary intake and generally are adjusted to meet the needs of the patient as they get older. Vitamin K is generally prescribed for those cystic fibrosis patients who suffer from liver disease or who are going to undergo a surgical procedure, or whose blood tests do not me certain standards.
* Vitamin A: Vitamin A deficiency may cause night blindness in older patients and can progress to severe xerophthalmia if not checked.
* Vitamin D: Vitamin D deficiency may cause rickets which is very rare and osteomalacia. Although in the past there is little clinical evidence of vitamin D deficiency is rare in cystic fibrosis new research and studies osteoporosis, osteopenia and low levels of vitamin D metabolites are being increasingly recognized in children and adults with cystic fibrosis.
* Vitamin E: Vitamin E deficiency may cause neurological problems in older CF individuals. Correction of vitamin E deficiency improves hemoglobin levels. Vitamin E is an antioxidant and protects cell membranes from oxidative damage. Because of this role vitamin E may be important in controlling the progression of lung disease in cystic fibrosis.
Recent studies have suggested that cystic fibrosis patients have inadequate antioxidants defenses to cope with certain stresses on their system. Pulmonary dysfunction in cystic fibrosis is associated with oxidative stress and higher levels of supplementation may be required.
* Vitamin K: Regular vitamin K supplements are not given unless there is chronic liver disease, a prolonged prothrombin time or an upcoming surgical procedure. Given any of these conditions an oral daily supplement of 5-10mg is given for a week prior to the procedure. Vitamin K is required for the formation of osteocalcin which is involved in bone metabolism.
As you can see vitamin supplementation is essential for Cystic Fibrosis patients. It is crucial for you to discuss any changes to your current vitamin regimen with your physician prior to adding, removing or changing the doses of any vitamins. The balance of vitamins in your system can be very delicate and should be closely monitored by your physician.
About the Author
Steven Godlewski is currently working with the staff at PillFreeVitamins.com He has an extensive background in nutrition as well as other health related fields. For more health-related articles see their website at: http://www.pillfreevitamins.com
Saturday, June 03, 2006
Cystic Fibrosis Symptoms
Cystic Fibrosis Symptoms by Kent Pinkerton
Cystic fibrosis is a very serious disorder that is fatal if not treated properly. A defective gene causes thin body secretions, such as lung mucus, digestive juices, sweat and reproductive secretions, to become thick and sticky. Serious and life-threatening problems may arise due to this thickening. There is no permanent cure for cystic fibrosis yet, but symptomatic treatment is given.
When the lungs and airways are choked, the cystic fibrosis patient coughs and produces very thick sputum. He is short of breath and develops wheezing. Polyps may grow in the nasal passages. The frequency of sinus, chest infections, pneumonia and bronchitis increase.
As the digestive juices do not reach the intestine, due to blocked ducts from the pancreas and liver, the fats and protein are not digested. The stool is bulky, greasy and foul smelling. The cystic fibrosis patient may have excessive appetite but is undernourished and underweight, as the food is not digested properly for absorption. A trypsin test may indicate whether sufficient enzymes from the pancreas are available for digestion.
The sweat may taste salty. When we kiss a child with cystic fibrosis we can taste this excessive salt taste. To diagnose cystic fibrosis positively we measure this saltiness in sweat. Technicians coat an odorless chemical on a small area of the skin in the arm, and stimulate that area with electric current to produce copious sweat. This sweat should contain about 40 m mol/l in a normal person. Above 60 m mol/l of salt in a sweat test will confirm the diagnosis of cystic fibrosis. With cystic fibrosis patients the duct that connects the testes and vas deferens may get blocked. Most cystic fibrosis patients are sterile.
Treatment is mainly to ease the symptoms and lead a normal life in spite of the malady. Bronchodilators and mechanical vibrators keep the lungs and airways clear. Enzyme and vitamin supplements keep the digestive system near normal. With correct lifestyle management Americans are living to their 30 s and 40s with cystic fibrosis.
About the Author Cystic Fibrosis provides detailed information on Cystic Fibrosis, Cystic Fibrosis Symptoms, Causes Of Cystic Fibrosis, Cystic Fibrosis Treatments and more. Cystic Fibrosis is affliated with Living With COPD .
Cystic fibrosis is a very serious disorder that is fatal if not treated properly. A defective gene causes thin body secretions, such as lung mucus, digestive juices, sweat and reproductive secretions, to become thick and sticky. Serious and life-threatening problems may arise due to this thickening. There is no permanent cure for cystic fibrosis yet, but symptomatic treatment is given.
When the lungs and airways are choked, the cystic fibrosis patient coughs and produces very thick sputum. He is short of breath and develops wheezing. Polyps may grow in the nasal passages. The frequency of sinus, chest infections, pneumonia and bronchitis increase.
As the digestive juices do not reach the intestine, due to blocked ducts from the pancreas and liver, the fats and protein are not digested. The stool is bulky, greasy and foul smelling. The cystic fibrosis patient may have excessive appetite but is undernourished and underweight, as the food is not digested properly for absorption. A trypsin test may indicate whether sufficient enzymes from the pancreas are available for digestion.
The sweat may taste salty. When we kiss a child with cystic fibrosis we can taste this excessive salt taste. To diagnose cystic fibrosis positively we measure this saltiness in sweat. Technicians coat an odorless chemical on a small area of the skin in the arm, and stimulate that area with electric current to produce copious sweat. This sweat should contain about 40 m mol/l in a normal person. Above 60 m mol/l of salt in a sweat test will confirm the diagnosis of cystic fibrosis. With cystic fibrosis patients the duct that connects the testes and vas deferens may get blocked. Most cystic fibrosis patients are sterile.
Treatment is mainly to ease the symptoms and lead a normal life in spite of the malady. Bronchodilators and mechanical vibrators keep the lungs and airways clear. Enzyme and vitamin supplements keep the digestive system near normal. With correct lifestyle management Americans are living to their 30 s and 40s with cystic fibrosis.
About the Author Cystic Fibrosis provides detailed information on Cystic Fibrosis, Cystic Fibrosis Symptoms, Causes Of Cystic Fibrosis, Cystic Fibrosis Treatments and more. Cystic Fibrosis is affliated with Living With COPD .
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