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Archive for the ‘Cystic Fibrosis’ Category

Cystic fibrosis is a chronic condition wherein it affects various organs such as the liver, lungs, pancreas, and intestines.

Patients with chronic cystic fibrosis have disrupted salt levels in the body. There are little salts and water levels in the outside of the cells of the body which causes the thin layer of mucus that protects the lungs to become sticky and thick.

The mucus becomes difficult to cough out and clogs the airways which leads to infection and damaged lungs. This disease is considered to be a hereditary disease which leads to multiple organ failure as the condition worsens. The mucus caused by CF also affects digestion through the obstruction of pancreas and inhibiting natural enzymes from helping the body to break down and absorb the nutrients from the food eaten.

Since CF is a hereditary condition, it is mainly caused by a mutation in the genes. A certain gene called cystic fibrosis transmembrane conductance regulator works for an important function in the body. This gene is involved in creating sweat, mucus, and digestive juices. You only need one copy of this gene so that you can prevent the occurrence of CF, although most people have two copies. If a person lacks at least one unaltered version of this gene, it can result to CF.

Depending on the type of mutation that the gene has, the severity of CF is variable. Actually, there are almost 1,400 different mutations of the mentioned gene. However, the most common symptoms associated with this condition include wheezing, shortness of breath, little weight gain even with good appetite, poor growth, nasal polyps, skin that tastes very salty, persistent coughing often accompanied with phlegm or extra mucus, and greasy or bulky stools.

A genetic test is done in order to diagnose the presence of CF. Aside from that, there are also several other tests that can be ordered by the physician such as X-rays, CT scans, lung function tests, sputum examinations and blood tests.

In general, there is no cure for CF. Treatment is directed towards improving the quality of life of the patients with the condition. The main goal is to clear the airways and replace enzymes so that nutrients will be properly absorbed.

No matter what sickness or condition you may have, it is always important to keep your body healthy and engage in physical activities. You can contact personal trainer Sydney to get more information about trainings and workouts that are suitable for you.

Cystic fibrosis, which affects 30,000 American children and adults, is a multisystem disease caused by a defective gene. Presently only symptomatic management is possible, but there are very promising gene-therapy trials under way.

In the respiratory system the thin mucus lining becomes thick and sticky. In cystic fibrosis management, the primary treatment of the system is to thin or clear this mucus. Bronco dilators like albuterol are used to clear the clogged airways. Mucus thinning drugs delivered by aerosol, like pulmozyme, are helpful. The most effective way of clearing this mucus is by mechanically dislodging it. Clapping on the chest and back, with the head tilted on the edge of a table, is quite effective. There is an electrical clapper that does the job safely. There is an electrical inflatable vest that vibrates and dislodges the mucus. Infection is an ever-present risk with cystic fibrosis patients. Regular shots for pneumonia and influenza are very important. Bacterial infection is fought with newer antibiotics like TOBY, which delivers the medicine directly into airways with aerosols.

Because of the blocked ducts of the pancreas and liver, the enzymes and bile do not reach the intestine. Though the patient eats normally or even in excess, the fats and proteins are not digested. So the cystic fibrosis patient needs to have the enzymes supplemented with oral pancreatic enzymes. There is also the need to take vitamins, especially the fat-soluble ones. If lung function is completely damaged, the only alternative would be lung transplantation. This would involve many factors, like the availability of a donor and the patient’s present health, prior to undergoing major surgery.

The Cystic Fibrosis Foundation has 115 centers all over America, and provides guidelines and advice. They support many studies to find gene therapy to cure cystic fibrosis. The target is to add normal genes to the cells of the airways to prevent further damage. Biotech laboratories produce healthy genes, which need to be delivered to the appropriate cells. These vectors, or delivery systems, are the key areas of research to combat cystic fibrosis. One method that is actively pursued is to modify the common cold virus to carry the healthy genes into the correct cells. On another track, the DNA molecules are compacted to their minimum size and delivered directly to the relevant cells without the need for a carrier such as a virus. This technology is called PLAS min.

As this gene therapy experiments are at an advanced stage, the cystic fibrosis sufferers can hope for a permanent cure in the near future.

Omega 3 fatty acids particularly DHA and EPA are good for the treatment of inflammatory diseases including cystic fibrosis. Because of this reason omega 3 and cystic fibrosis are very much related to each other.

Omega 3 fats obtained from fish oil are required for the prevention of many diseases. The most prominent of these diseases is cardiovascular disease. These fats reduce inflammation throughout the body and also control high cholesterol levels in the body. They are excellent for the cure of high blood pressure, arthritis, prostatitis, and gallstones.

The connection between omega 3 and cystic fibrosis can be established by the fact that omega 3 fatty acids provide relief in nearly all kinds of inflammatory diseases. Omega 3 long-chain fatty acid known by the name of ALA is of particular interest in this context since it is primarily obtained from flax seed oil which is good for people of all ages.

The other rich source of omega 3 fats is fish oil. Since fish oil in its raw form is not easy to intake because of its bad taste, fish oil capsules are normally used by people who want to receive optimal levels of these fats on a daily basis.

It has been found that people who eat fish or seafood regularly are at reduced risk of developing coronary heart disease, breast and prostate cancer, diabetes and Alzheimer’s disease. Polyunsaturated fats obtained from fish also prevent and cure prolonged depression and anxiety.

It is advisable to consume at least 300 mg of DHA and EPA on a daily basis. A single fish oil capsule provides up to 180 mg of EPA and 120 mg of DHA. You can consume 2 to 3 capsules every day, one after each meal to receive your daily required amount of these fats.

For maximum absorption of these nutrients by the bloodstream, try to take capsules after a gap of 4 to 5 hours. This will ensure proper absorption and will also keep a check on the balance of omega 3 and omega 6 fats in the body. The recommended ratio of these fats is three parts of omega 6 to one part of omega 3 fats.

Plant oils particularly hemp oil and olive oil provide excellent concentration of both types of fats. However, if you can digest fish oil then it is the best and most healthful source of omega 3 fats known to date.

Cystic fibrosis is a potentially fatal genetic disorder that affects large numbers of people worldwide. The condition involves the failure of a particular gene to be expressed, known as the cystic fibrosis transmembrane conductance regulator. The failure to express this gene results in the failure of the normal secretion processes, and affects the lungs and pancreas in particular.

Effects of Pancreatic Problems

The pancreas is connected to the digestive tract by means of a duct, and it produces important digestive enzymes that travel through that passage into the duodenum. These pancreatic enzymes are essential for the proper digestion and absorption of nutrients. With cystic fibrosis, however, the pancreatic ducts are blocked, preventing the digestive enzymes from reaching the small intestine.

Also, the thickened secretions harm the pancreas itself, often causing irreparable damage. Beyond a certain extent, the organ will fail entirely, resulting in organ failure and followed soon by death if proper treatment is not forthcoming.

The blocking of the passage leading from the pancreas to the digestive tract leads to malabsorption. This is due to the inability of the digestive tract to break down and assimilate certain important nutrients and minerals without the digestive enzymes produced by the pancreas. Protein and fat in particular can’t be broken down and are not absorbed into the body. Malabsorption is followed by malnutrition, where the body does not get all of the minerals, nutrients, and other compounds that it requires in order to function properly. Younger patients usually suffer from normal growth rates and adults look often thin.

In addition, the disorder may also cause irreparable damage to the islets of Langerhans, which are glands responsible for the production of insulin. Once damaged, the lack of insulin leads to diabetes in patients.

Treatment Options

As mentioned, the only treatment for blocked enzyme release consists of taking pancreatic enzymes with food, especially fat and protein rich food.

Treatment of diabetes caused by cystic fibrosis usually involves injections of insulin, which is nothing more than an attempt to manage the symptoms of the condition. In patients where the genetic disorder has damaged too much of the pancreas, the only treatment option available to patients is an organ transplant in order for the body to continue functioning.

The one treatment method that shows potential to actually cure cystic fibrosis is known as gene therapy. This method of treatment actually involves replacing incorrect genes with correct genes, thereby resolving the problem. Gene therapy has yet to be fully tested, however, and scientists still struggle to find an effective method of inserting the correct copies of genes into the affected cells. Until gene therapy becomes a viable solution, treatment of pancreatic problems simply involves holding off the effects of the disease and keeping the patient healthy for as long as possible, with no hope of curing it.

Medications for cystic fibrosis are formulated to help keep lungs healthier, help contain and lower mucus formation in the lungs, and help replace necessary digestive enzymes. It is very important to get proper consultation with appropriate health experts. Self medication is not, and should not, work among sufferers of the condition just because of the simple reason that there are medications for cystic fibrosis that works in some patients but not in others.

If a certain medication works for some sufferers of cystic fibrosis, there is no assurance that the same medicine would work in all cases. It could take significant time before you finally find medications that could treat your or your child’s condition. If you aim to know every significant information about cystic fibrosis, it would be best if you would know that there are available medication choices you could take.

The primary and most common medicines are those that treat infections, or antibiotics, which are best for treating sinus and lung infections. Such medicines could be in pills, intravenous, and inhalable forms. Many medications for cystic fibrosis are aimed at clearing thickening mucus, especially those that clog breathing tubes within the lungs. The disease is characterized by building up of very thick mucus that grows in the airways. In interesting occasions, a therapist may be able to clear such secretions through manually clapping his hands on the back of the patient. This physical or manual lung-clearing technique could also be taught to parents of children sufferers.

There may be inflammation of air passages where mucus accumulates. That is why many medications for cystic fibrosis also include medicines that specifically help lower or eliminate general inflammation like corticosteroids and membrane stabilizers.

Aside from medicines that target the lungs and the breathing passages, there are also medicines treating the disease that are functioning to replace digestive enzymes. Cystic fibrosis may also affect the pancreas and its functions. That is why enzyme replacement therapies are also more common.

Take note that experts warn patients and families to be watchful over administration or application of medications. Logically, most of the medicines are prescribed. Self-medication is not advisable to patients suffering from this genetic disease. Doing so could very much put the life of any patient at greater danger and risk.

The life expectation for people with cystic fibrosis has been steadily increasing over the past 40 years. Some people with CF have serious lung and digestive problems. Other people have more mild disease that doesn’t show up until they are adolescents or young adults. CF is caused by a mutation in a gene called the cystic fibrosis transmembrane conductance regulator (CFTR). Respiratory failure is the most dangerous consequence of cystic fibrosis. Also, the secretions block pancreatic enzymes that help digest fats and proteins, and they prevent your body from absorbing key vitamins. Treatments for cystic fibrosis are aimed at relieving symptoms and complications.

Causes of Cystic Fibrosis

The common Causes of Cystic Fibrosis:

Cystic fibrosis affects the mucus and sweat glands of the body and is caused by a defective gene.

Other bacteria may be involved.

Radiation, eg after radiotherapy to other organs in the pelvis.

The main cause of cystitis fibrosis (and other urinary tract infections) is bacteria known as coliform bacteria, which are a common occupant of the bowel.

Symptoms of Cystic Fibrosis

Some Symptoms of Cystic Fibrosis:

Weight loss, or failure to gain weight normally in childhood.

Coughing or wheezing.

Fatigue.

Infants may have salty-tasting skin.

Recurrent respiratory infections, such as pneumonia or sinusitis.

Stools that are pale or clay colored , foul smelling, or that float.

Diarrhea.

Delayed growth.

Treatment of Cystic Fibrosis

Antibiotics for respiratory infections.

Vitamin supplements, especially vitamins A, D, E, and K.

DNAse enzyme replacement therapy. The medication dornase (Pulmozyme) contains an enzyme that thins the mucus and makes it easier to cough up.

Postural drainage and chest percussion.

Lung transplant may be considered in some cases.

Pancreatic enzymes to replace those that are missing.

Research has shown that the pain reliever ibuprofen may slow lung deterioration in some children with cystic fibrosis. The results were most dramatic in children ages 5 to 13.