The Ideal Cystic Fibrosis Diet Plan – What Patients Should Follow

Cystic fibrosis is a genetic condition that affects the digestive and respiratory systems. Under this condition, a mutation in the CFTR gene triggers the over-production of mucus in the body, which in turn blocks the digestive enzymes secreted by the pancreas from passing into the small intestine. Though the disease has no known cure, it can be managed through other cystic fibrosis treatment measures, along with the right cystic fibrosis diet plan.

cystic fibrosis diet

Increasing your intake of salt is recommended as part of the cystic fibrosis diet, as this condition causes your body to lose excess of salt.

Why Do You Need A Cystic Fibrosis Diet Plan?

As the condition blocks the digestive enzymes from reaching the small intestine, nutrients like proteins and fats are not normally absorbed, resulting in vitamin/mineral deficiency and malnutrition. This causes weakness, which is one of the definitive cystic fibrosis symptoms.

This is why people who suffer from cystic fibrosis have to consume more calories when compared to normal individuals – about 2900 to 4500 calories per day.

However, the amount of calories required might differ depending on the severity of the disease. By following a nutritional diet that consists of extra fatty foods and enzyme supplements, individuals with cystic fibrosis can lead a healthy life. Once cystic fibrosis has been diagnosed, a specific diet plan has to be made by consulting a cystic fibrosis dietician.

The Complete Cystic Fibrosis Diet For Patients

A cystic fibrosis diet plan mainly covers the amount of calories required per day. Some of the essential nutrients that should be included in such a diet have been given below.

  • Essential Fatty Acids

    Plasma fatty acid levels are generally low in individuals with cystic fibrosis. Plant oils like safflower oil and soybean oil are rich in plasma fatty acids.

    Using such oil to prepare food would help build the cell membrane of epithelial cells, which in turn would aid in the normal functioning of the lungs. You should also add food items that are rich in omega 3 fatty acids, like flax seeds, salmon, and walnuts.

  • Fat-Soluble Vitamins (Vitamins A, D, E, And K)

    Fats are not properly digested or absorbed in the body of an individual with cystic fibrosis. This contributes to the low levels of fat-soluble vitamins, which in turn affects the immune system. In such cases, dietary supplements that are rich in vitamin A, D, E, and K should be incorporated into the diet.

  • Proteins

    The body stops absorbing protein and this has to be supplemented in the diet. Protein-rich foods that you can eat are soya, nuts, beans, eggs, fish, and meat.

  • Calcium

    Patients with cystic fibrosis face a risk of developing conditions like osteoporosis, which are caused by brittle and weak bones that result due to the inadequate absorption of calcium from the food. The right cystic fibrosis diet should include dairy foods that are good sources of calcium and fat.

  • Iron

    In most cases, individuals with cystic fibrosis suffer from anemia because of an iron deficiency, and this makes them susceptible to infections. The cystic fibrosis diet should also include fortified cereal, dried fruits, green vegetables, and meat to supplement the lack of iron in the body.

  • Salt

    The loss of excess salt from the body through sweat is one of the main concerns associated with cystic fibrosis. This brings about weakness, low blood pressure, high heart rate, dehydration, and heat strokes. For this reason, it is necessary to add more salty foods in the diet on a daily basis to compensate for the loss of salt from the body.

  • Zinc

    One of the important components to be incorporated in the diet is zinc. This helps in fighting against infections, and helps in growth as well as healing. Foods that are rich in zinc are eggs, meat, liver, and sea food.

  • Enzyme Supplements

    The enzymes made by the pancreas are blocked from reaching the small intestine, where they are required for the normal absorption of nutrients. Because of this, it is necessary to include enzyme supplements on a daily basis so that the nutrients from the consumed food are easily absorbed. A certified doctor will be able to recommend such supplements.

With the right cystic fibrosis diet, the condition can be effectively managed. Consult your doctor for further guidance on getting the diet plan right.

Cystic Fibrosis Diagnosis – Prenatal And Genetic Testing Measures

Cystic fibrosis is a chronic lung disease that occurs due to specific genetic mutations. The symptoms of cystic fibrosis worsen with age and become hard to ignore, so the condition usually gets detected much before the individual reaches adulthood. One of three types of tests is conducted as part of cystic fibrosis diagnosis, to check if the individual is carrying the mutated gene responsible for this condition.

cystic fibrosis diagnosis

Tests for cystic fibrosis diagnosis should be carried out if your child has frequent coughing, chest infections, and shortness of breath.

Cystic Fibrosis Diagnosis – A History Of The Disease

Cystic fibrosis was first diagnosed in the year 1938 by Dr. Dorothy Andersen, in New York Babies Hospital. In 1989, a team of geneticists headed by Dr. Tsui Lap-chee from the Hospital for Sick Children, Toronto found the cause of this condition to be a mutation in the gene CFTR (Cystic Fibrosis Transmembrane Regulator).

So far, more than 900 mutations have been found in the CFTR gene.

The Three Tests Used In Cystic Fibrosis Diagnosis

Several diagnostic tests have been developed to detect cystic fibrosis even before its symptoms start to appear. Cystic fibrosis diagnosis can be made at three different stages of life: before birth (prenatal diagnosis), after birth (postnatal diagnosis), and during early childhood.

  1. Prenatal Testing

    This test is usually done to check if the unborn child has cystic fibrosis, when either of the parents or both of them are known to be carriers of the defective CFTR gene. In this test, the CFTR gene is screened for mutations that are associated with cystic fibrosis.

    If the test reveals that the unborn child has cystic fibrosis, it helps the parents decide if they want to continue with the pregnancy or not. If they decide to continue with it, then they have the advantage of receiving early and timely treatment for their child. There are two common types of prenatal tests:

    • Amniocentesis: This test is usually carried out between the 15th and the 18th week of the pregnancy. A sample of the amniotic fluid that surrounds the unborn child in the uterus is taken using a fine needle, for testing.The DNA is isolated from this sample and the CFTR gene is screened for mutations. By analyzing the mutations, the doctors can determine if the unborn child has cystic fibrosis or not.The fetal enzymes in the sample are also tested to aid the diagnosis. If the level of enzymes appears decreased, it indicates that the child has cystic fibrosis.
    • Chorionic Villus Sampling: This test is usually carried out when the pregnancy is between 11 and 14 weeks. A small amount of tissue sample is taken from the chorionic villus region of the placenta, using a small needle. Again, the DNA is isolated from the tissue sample and screened for mutations in the CFTR gene to determine if the unborn child has cystic fibrosis or not.

    Both these tests provide 99% precision in determining whether the unborn child has this lung disease or not. However, there is a 1-2% chance of a miscarriage after the tests are carried out because of probable infections caused during the sample collection.

  2. Postnatal Screening Or Newborn Screening

    A test known as Immunoreactive Trypsinogen Test (IRT) is carried out about 2 to 3 days after the birth of the child. This test measures the level of the protein trypsinogen. High levels of this protein usually indicate cystic fibrosis.

  3. Early Childhood Test (Sweat Test)

    The Sweat Test is the most common test widely used for the diagnosis of cystic fibrosis in children and young adults. This test is a rapid and painless procedure in which the levels of sodium and chloride are measured in the sweat excreted from the individual’s body.

    As part of this test, a chemical called pilocarpine is applied to the forearm of the subject and a mild electric current is applied to the area where sweating is experienced. Higher than usual levels of sodium and chloride usually indicate cystic fibrosis.

Other tests like lung function tests, chest X-rays, sputum cultures, and stool examinations are also commonly used as part of cystic fibrosis diagnosis. It is important to test for the condition and start cystic fibrosis treatment as soon as possible to manage the condition better.

Interesting Cystic Fibrosis Facts – An Introduction To The Disorder

Cystic fibrosis is a genetic disorder that was first identified in 1938 by a pathologist named Dr. Dorothy Andersen from New York Babies Hospital. However, the first mutation in the CFTR gene that causes the disease was identified only in 1989, by a team of geneticists headed by Dr. Tsui Lap-chee, in the Hospital for Sick Children, Toronto. Here are some more cystic fibrosis facts that will help you understand the disease better.

cystic fibrosis facts

Want some cystic fibrosis facts? Here’s one: Coughing and respiratory infections are two of the earliest signs of this condition.

Cystic Fibrosis Facts You Should Know

  • Cystic Fibrosis Affects Bodily Systems

    This is probably the most defining one among cystic fibrosis facts – the disease is known to affect the digestive and reproductive systems in the body.

  • It Is An Inherited Disease

    Cystic fibrosis is inherited in an autosomal recessive manner. The condition rises if both parents are carriers of the mutation in the CFTR gene and pass the defective gene to their child. However, if the child inherits only one copy of the defective gene, then the child would become a carrier of cystic fibrosis. In such cases, the symptoms of the disease will not be present.

  • The Disease Is Rare In Asia

    Worldwide, the incidence of cystic fibrosis varies. In the United States of America, 1 child in every 3500 live births is affected with cystic fibrosis. Cystic fibrosis facts published after research state that a majority of Americans are carriers of the mutation, but however, they remain ignorant about the condition. In Europe, 1 child in every 2000-3000 live births is affected by cystic fibrosis. However, cystic fibrosis facts also state that it is relatively rare in Asia, though it may also be largely under-diagnosed.

  • The Symptoms Might Vary

    The most common symptoms of cystic fibrosis are coughing, frequent respiratory infections, salty skin, weight loss, and greasy stools. The organs in the body that are mainly affected are the sinuses, lungs, liver, intestines, pancreas, and reproductive organs. However, the symptoms might vary from mild to severe, and between individuals.

  • Excess Mucus Causes Most Complications

    The effects of cystic fibrosis are many. In individuals with cystic fibrosis, the sensory glands (mucus and sweat glands) are the most affected. The mucus is sticky and thick and accumulates in the lungs, and this in turn blocks the airways. Such blockage causes repeated lung infections, eventually injuring the lungs and causing severe breathing difficulty. The mucus also blocks the ducts in the pancreas that make the digestive enzymes, and because of this, the digestive system is affected. The body doesn’t absorb nutrients like proteins and fats effectively, resulting in malnutrition and deficiency. The sweat glands also produce salty sweat, leading to weakness, a drop in the blood pressure, increased heart rate, dehydration, heat stroke and in some cases, death.

  • The Condition Can Be Easily Diagnosed

    Cystic fibrosis diagnosis can be done by measuring the amount of salt present in a skin sample. Blood tests that measure the protein trypsinogen and genetic tests where the CFTR gene is screened for mutations are other common diagnostic tests.

  • There Is No Cure For Cystic Fibrosis

    So far, there is no known cure for cystic fibrosis. However, an early diagnosis can aid in managing the disease well. Through proper cystic fibrosis treatment and care, you can definitely improve your lifespan and counter the disease over time.

It is essential to know such basic cystic fibrosis facts, as these will help you identify any symptoms at the earliest possible opportunity, among your friends or even family members.

Cystic Fibrosis Effects – How It Affects The Body

In people who are affected by cystic fibrosis, the function of the CFTR gene is disrupted. This, in turn, leads to developments that adversely affect the body. One of the main cystic fibrosis effects is the disturbance of water/salt flow along the cells.

cystic fibrosis effects

Excessive sweating is one of the common cystic fibrosis effects. It often leads to dehydration.

As a result of this, the mucus that is normally secreted becomes thick and sticky, in turn blocking the organs and disrupting their normal function. The organs in the body that are most prone to cystic fibrosis effects are the lungs, pancreas, liver, gall bladder, intestine, bones, sweat glands and reproductive organs.

Cystic Fibrosis Effects – How Every Organ Is Affected

  • Lungs

    The thick and sticky mucus, over a period of time, accumulates and blocks the airways, causing breathing difficulty.

    The mucus also facilitates the growth of bacteria, which in turn infects and causes swelling in the lungs, nose, and sinuses. Nasal polyps develop in some cases. These have to be surgically removed.

  • Pancreas

    The function of the pancreas is to produce digestive enzymes that are passed into the intestine, where these enzymes help the body absorb the nutrients from food items. This organ also produces insulin, which is necessary to control the sugar level in the blood.

    Due to cystic fibrosis effects, the pancreatic ducts are blocked, preventing the enzymes from travelling to the intestine. The intestine fails to absorb nutrients like fats, protein, and vitamins from the food, leading to further complications.

    The sugar level in the blood is not regulated as the insulin produced by the pancreas is blocked by the mucus. This ultimately causes damage to the cells in the pancreas that produce insulin and enzymes. In this way, cystic fibrosis effects contribute to insulin dependent diabetes, due to the increase of sugar level in the body. This problem affects close to 15% of the individuals with cystic fibrosis. Malnutrition and vitamin deficiency, which affect the overall growth and development of the individual, are secondary effects.

  • Liver And Gallbladder

    The mucus formed due to cystic fibrosis blocks the bile ducts in the liver and the gall bladder, in turn preventing them from functioning normally.

  • Intestines

    Similar to the previous cases, the mucus blocks the intestine – a development that could be life threatening in some cases. This condition is called Meconium Ileus and is common in young children.

  • Bones

    In individuals with cystic fibrosis, the absorption of calcium is also affected. This mineral is essential for bone health, and the lack of it causes the bones to become weak and brittle. The condition is commonly called Osteoporosis or Osteopenia, and it is one of the primary cystic fibrosis symptoms.

  • Sweat Glands

    Cystic fibrosis brings about the excessive loss of salt from the body through sweat. This causes weakness, decreased blood pressure, increased heart rate, dehydration, heat stroke, and in some cases, death.

  • Reproductive Organs

    Cystic fibrosis effects target both males and females differently. Males are rendered infertile, as the thick mucus blocks the ducts and prevents the passage of sperm into the testes. Women face decreased fertility as the cervix is blocked, with the mucus preventing the entry of sperm.

Such cystic fibrosis effects can be countered through proper cystic fibrosis diagnosis and treatment measures, as recommended by a physician.

A Look At What Causes Cystic Fibrosis – The Genetic Factors Involved

Cystic fibrosis is a genetic disorder that’s caused by mutations in the CFTR (Cystic Fibrosis Transmembrane Regulator) gene. A quick look at the function and purpose of the CFTR gene can help you understand what causes cystic fibrosis.

what causes cystic fibrosis

A mutation of the CFTR gene is what causes cystic fibrosis, and it leads to degrading lung function early in childhood.

What Causes Cystic Fibrosis – About The CFTR Gene

The CFTR gene is located on chromosome 7. The normal function of this gene is to produce the necessary proteins that are found in the epithelial cells, which line the internal parts of the lungs, pancreas, genitor urinary tract, and colon. The protein helps move the negatively charged chloride ions in and out of the cell through the cell membrane.

In individuals with cystic fibrosis, the movement of chloride ions is disrupted, and this causes the cells to produce an abnormal amount of mucus, affecting the supply of water.

Because of the lack of water supply to the cells, the excessive mucus produced is not washed away. This makes the mucus thick and sticky, in turn blocking and inflaming the sensory glands. All these developments finally injure the tissues of the body, causing the symptoms of cystic fibrosis.

A Deeper Look At What Causes Cystic Fibrosis

In about 70-80% of individuals with cystic fibrosis, the main cause is the production of an abnormal protein called deltaF508 CFTR, which results due to the deletion of 3 nucleotides in the CFTR gene. In the remaining 20-30% of the cases, cystic fibrosis might be caused by other mutations in the CFTR gene that make abnormal protein. However, the severity of the disease depends on the mutation in the gene and the type of protein produced by the defective CFTR gene.

This genetic disorder is inherited in an autosomal recessive manner. If both the parents are carriers of the mutation in the CFTR gene, the child has a 25% chance of inheriting both defective genes and 50% chance of inheriting the mutated gene that would bring about the complications of cystic fibrosis.

Other Causes Of Cystic Fibrosis

Environmental factors contribute to the severity of cystic fibrosis, as most genetic disorders are caused due to interactions between the genotype and environmental factors. For example, if an individual doesn’t consume enough calories, the prognosis of the disease is likely to be affected. These interactions might explain the difference in the disease’s manifestation in individuals with cystic fibrosis, although they might carry the same mutation in the CFTR gene.

By understanding what causes cystic fibrosis, you’ll be able to look for a family history of the condition and subsequently opt for Cystic Fibrosis treatment measures.

Cystic Fibrosis Symptoms – Signs That You Should Look Out For

Cystic fibrosis symptoms vary from mild to severe and can present differently between individuals. In some cases, the symptoms starts showing as early as infancy, however in other cases, individuals might not show symptoms until they are adults.

cystic fibrosis symptoms

Blockages in the lungs and respiratory problems are some of the early cystic fibrosis symptoms.

The first sign is usually the salty skin that’s often noted in affected children. In individuals with cystic fibrosis, the organs in the body that are affected include the sinuses, lungs, liver, intestine, pancreas, and reproductive organs.

The Common Cystic Fibrosis Symptoms

The effects of cystic fibrosis can be noted across multiple organs, so the symptoms can be divided on this basis.

  • Respiratory Cystic Fibrosis Symptoms

    In individuals with cystic fibrosis, the mucus becomes thick and sticky and accumulates in the lungs. This blocks the airways and prevents the flow of air in and out of the lungs, providing an ideal condition for bacteria to infect and grow.

    Respiratory complications that ensue can include cough that produces excessive sputum, breathing difficulties, wheezing, and enlarged nasal passages. Affected individuals are also likely to suffer from bronchitis and pneumonia, and in some cases, the nasal polyps that develop will have to be removed surgically.

  • Digestive Cystic Fibrosis Symptoms

    The sticky thick mucus blocks the ducts in the pancreas that make the digestive enzymes. This prevents the enzymes from moving to the small intestine, rendering the intestine unable to absorb nutrients like proteins and fats.

    Over time, inadequate nutrition can result in malnutrition and deficiency. Weight loss and affected growth development are other characteristics. The primary digestive cystic fibrosis complications include severe constipation and intestinal blockage (more common in infants). The stools are released with bad odor, and individuals might also suffer from rectal prolapse, which occurs when the end of large intestine protrudes outside the anus when straining repeatedly to pass stool.

    In severe cases, affected individuals might suffer from pancreatitis (enlargement of the pancreas), liver disease (when bile ducts are blocked by the accumulation of mucus), diabetes (when the function of the pancreas is affected), and gallstones.

  • Reproductive Cystic Fibrosis Symptoms

    The Vas Deferens, which transports sperm from the testes to the penis, is absent in male individuals suffering from cystic fibrosis, and for this reason they are generally infertile.

    Women who are suffering from cystic fibrosis symptoms might be unable to conceive easily because of the blockage caused in the cervix by the mucus, and also due to other associated conditions.

  • Other Cystic Fibrosis Symptoms

    Cystic fibrosis affects the mineral concentration in the body. Excessive salt loss from the skin in the form of sweat is one of the common cystic fibrosis symptoms. This in turn causes weakness, low blood pressure, increased heart rate, dehydration, heat strokes, and in some cases, might prove to be fatal.

    In many cases, bone density is reduced and this mainly occurs late in life among cystic fibrosis patients. The gradual reduction in bone density leads to thin bones, a condition that’s otherwise called osteoporosis. In individuals with cystic fibrosis, especially females, bladder control is usually lost. As a result of this, urine might leak out of the bladder while coughing.

If such cystic fibrosis symptoms are noted right during their early stages, steps for cystic fibrosis treatment can be taken once the physician recommends them.

Cystic Fibrosis Treatment – Measures For Managing The Condition

Cystic fibrosis is a genetic condition that does not have a documented cure till date. However, with timely cystic fibrosis diagnosis and proper treatment, the disease can be managed efficiently. The cystic fibrosis treatment or management plan is formulated based on the severity of the disease.

cystic fibrosis treatment

Chest physical therapy is commonly used as part of cystic fibrosis treatment.

The various treatment options can also differ based on the part of the body that’s affected, and these have been mentioned below in detail.

Cystic Fibrosis Treatment For Lung Problems

The most common organ affected in cystic fibrosis patients is the lung. The treatment options available for specific cystic fibrosis effects are chest physical therapy, medications, and exercise.

  • Chest Physical Therapy (CPT)

    The lungs are blocked by mucus and this has to be loosened in order to facilitate breathing. CPT is a specialized physical therapy where the chest and the back are pounded with the help of a specific device so that the mucus comes loose and is coughed out. This would in turn clear the airways and help in easy breathing.

  • Medicines

    Individuals suffering from repeated lung infections can be treated with medicines like antibiotics that prevent bacterial infections. Anti inflammatory drugs can be used to reduce the swelling in the airways, which is mainly caused due to infections. Also, mucus-thinning medicines can be used to clear any blocked airways and bronchodilators can also be used for this purpose.

  • Exercise

    Breathing exercises help in loosening the mucus, so much so that it can be coughed out. Through regular exercise, the overall condition can be improved.

How Advanced Lung Disease Can Be Treated

If the disease in the lung has reached an advanced stage, oxygen therapy is advised. Oxygen is supplied through a nasal mask or prongs in order to facilitate breathing. If this does not help, a lung transplant is the only option in such cases.

Pulmonary Rehabilitation Treatment

This treatment plan concentrates on the overall well being of the individual with cystic fibrosis. The plan lays out specific training routines for exercising, a nutritional guide, and proper education to help patients manage the lung disease.

Cystic Fibrosis Treatment For Digestive Problems

The second most common bodily system to be affected by cystic fibrosis is the digestive system. This, in turn, leads to improper growth due to the lack of proper nutrition. A well-balanced diet that is rich in calories and contains more salt is usually recommended for coping with cystic fibrosis.

Oral supplements like pancreatic enzymes, vitamins A, D, E, and K and medications for thinning the mucus are also advised for people with cystic fibrosis.

The Need For Surgery In Cystic Fibrosis

In severe cases, surgery might be required to remove the nasal polyps that develop in the nose to facilitate breathing. Endoscopy and lavage is also performed to remove mucus from blocked airways in severe cases.

To facilitate digestion, a feeding tube is inserted into the stomach or implanted into the abdomen in order to continuously pass nutrients from an attached bag. In many cases, intestinal surgery might be required as a cystic fibrosis treatment measure if there is any blockage.