CF Awareness

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whateverlife.com What is Cystic Fibrosis?
Cystic Fibrosis is a life threatening inherited diesease, that causes muscus to build up and clog some of the organs in the body. Majorly in the lungs and pancreas.
When the mucus clogs the lungs it makes breathing very difficult. The thick mucus also causes bacteria/germs to get trapped in the airways, which causes inflammations and infections that damage the lungs.
The mucus can also block the digestive tract and pancreas. It stops the enzymes from getting into the intestines. The body needs enzymes to break down food, which provides important nutrients to help us grow and stay healthy. People with cystic fibrosis often need to replace these enzymes with capsules they take with their meals and snacks to help digest the food and get the proper nutrition.
Cystic Fibrosis IS NOT contagious!
How do people get Cystic Fibrosis?
Cystic Fibrosis is a genetic diesease. Which means people inherit it from their parents through genes (or DNA), which also determine a lot of other characteristics including height, hair color and eye color. Genes, found in the nucleus of all the body's cells, control cell function by serving as the blueprint for the production of proteins.
The defective gene that is responsible for causing cystic fibrosis is on chromosome 7. To have cystic fibrosis, a person must inherit two copies of the defective CF gene—one copy from each parent. If both parents are carriers of the CF gene (i.e., they each have one copy of the defective gene), their child will have a 25% chance of inheriting both defective copies and having cystic fibrosis, a 50% chance of inheriting one defective copy and being a carrier, and a 25% chance of not having CF or carrying the gene.
Who gets cystic fibrosis?
Cystic Fibrosis is the UK's most common, life-threatening, inherited disease. It affects over 7,500 people in the UK. In the UK, 2.3 million people carry the faulty CF gene - 1 in 25 of the population. If both parents are carriers of the faulty gene, there is a 1 in 4 chance with every pregnancy that their child will have Cystic Fibrosis. The disease is most common in Caucasians, but it can affect all races.
The severity of cystic fibrosis symptoms is different from person to person. The most common symptoms are:
♠ Very salty-tasting skin
♠ A large appetite, but poor weight gain
♠ Greasy, bulky bowel movements
♠ Constant coughing, wheezing or shortness of breath
♠ Many lung infections, like pneumonia and bronchitis
♠ Small, fleshy growths in the nose called nasal polyps
Sometimes people are told that they have asthma or chronic bronchitis when they really have cystic fibrosis. New research shows that the severity of CF symptoms is partly based on the types of CF gene mutations (defects). Scientists have found more than 1,500 different mutations of the CF gene.
How is CF diagnosed?
Most people are diagnosed with CF at birth or before the age of 3. A doctor who sees the symptoms will order either a sweat test or a genetic test to confirm the diagnosis.
A sweat test is the most common test used to diagnose cystic fibrosis. A small electrode is placed on the skin (usually on the arm) to stimulate the sweat glands. Sweat is then collected and the amount of chloride, a component of salt in the sweat, is measured. A high level of chloride—a score of more than 60 mmol/L (a measure of concentration)—means that the person has cystic fibrosis. Scores between 40 mmol/L and 60 mmol/L are considered to be on the borderline and need to be looked at on a case-by-case basis. Scores of less than 40 mmol/L are considered negative for CF. The best place to receive a reliable sweat test is at a Cystic Fibrosis Foundation-accredited care center.
In a genetic test, a blood sample or cells from the inside of the cheek is taken and sent to a laboratory to see if any of the various mutations of the CF gene are found. A genetic test is often used if the results from a sweat test are unclear.
How does CF affect the lungs?
Scientists have many different ideas about what goes wrong in the lungs of a person with cystic fibrosis, but it all begins with defective CF genes. Normally, the healthy CF gene makes a protein—known as CFTR (Cystic Fibrosis conductance Transmembrane Regulator)—that is found in the cells that line various organs, like the lungs and the pancreas. This protein controls the movement of electrically charged particles, like chloride and sodium (components of salt) in and out of these cells. When the protein is defective, as in cystic fibrosis, the salt balance in the body is disturbed. Because there is too little salt and water on the outside of the cells, the thin layer of mucus that helps keep the lungs free of germs becomes very thick and difficult to move. And because it is so hard to cough out, this mucus will clog the airways and lead to infections that damage lungs.
Is cystic fibrosis fatal?
Currently, there is no cure for cystic fibrosis. However, specialized medical care, aggressive drug treatments, and therapies, along with proper CF nutrition, can lengthen and improve the quality of life for those with CF.
The best way for people with cystic fibrosis to fight their disease is to work with their medical caregivers at a CF Foundation-accredited care center. The care center partners with people who have CF to help keep them in the best health possible.
What is a typical day for someone with CF?
Because the severity of CF differs widely from person to person, and CF lung infections flare up from time to time, there may not be a “typical” day. However, each day most people with CF:
♠ Take pancreatic enzyme supplement capsules with every meal and most snacks (even babies who are breastfeeding may need to take enzymes).
♠ Take multi-vitamins.
♠ Do some form of airway clearance at least once and sometimes up to four or more times a day.
♠ Take aerosolized medicines. These are liquid medications that are made into a mist or aerosol and then inhaled through a nebulizer.
What is the life expectancy for people who have CF?
There is no way to accurately predict how long people with cystic fibrosis will live, as many different factors may affect a person’s health. Severity of disease and time of diagnosis are two such factors. Many people have a mild case of CF, while others can have moderate or severe cases. In addition, some adults with cystic fibrosis have only recently begun to use new treatments, while an infant diagnosed at birth will have the advantages of starting specialized treatments that were not available even a decade ago.
Improved treatment and management of Cystic Fibrosis now results in a much longer life expectancy. Since the 1960's, average life expectancy has risen from 5 up to just over 30 years.
Transplants.
The success rate of Lung transplantation for CF patients is encouraging: 70% survive one or two years after transplantation and the longest surviving patients had their transplant operations over 15 years ago. Like any other major surgery it carries considerable risks and is appropriate only for a patient who is severely ill and has tried all other forms of conventional treatment. To these patients, lung transplantation offers a better quality of life.
There is a shortage of donor organs available for those awaiting transplants. If more people carry donor cards, then more lives that can be saved in this way.
CF in school.
It is important to remember that CF affects each child in different ways, with varying degrees of severity. Each child's health can change considerably from month to month or even from day to day.
The most noticeable feature of CF is a persistent cough. Although it is not infectious it may be embarrassing in front of other children, especially as a severe coughing attack occasionally leads to coughing up mucus or vomiting.
Parents or other carers may have to come into school to help their child with daily physiotherapy. Nebuliser treatment may also be necessary.
The number of physiotherapy sessions that take place each day varies according to the child's current state of health. The length of each session will vary from 15 minutes to an hour.
As CF causes malfunctioning of the pancreas, special supplements need to be taken with all meals and snacks. These are available in capsule form and need to be taken in large quantities.
Children with CF are as academically able as their peers, so teachers should expect similar standards. But hospitalisation or chest infections can result in prolonged absence from school, so extra help may be needed to catch up with the rest of the class. Examining boards make certain allowances for pupils with Cystic Fibrosis.
Some older children take advantage of portable intravenous antibiotic equipment, which enables them to attend lessons more regularly.
Physical exercise is beneficial for children with CF so they should participate in Games and PE lessons. However, illness can result in loss of energy, which should be taken into consideration.
Children with CF may be teased or picked on at school because of their persistent cough, and the fact that they may be underweight and small for their age. Taking tablets and capsules with their meals may also be embarrassing for them.Physiotherapy is very time consuming, possibly to the detriment of the child's social life. However, children with CF usually find supportive friends who help with care and physiotherapy.
During teenage years there is the possibility that physiotherapy and diet could be neglected. Delayed sexual maturity may cause anxiety or insecurity.
Teenagers may require sympathetic treatment and counselling to deal with some of these issues.
Cystic Fibrosis requires a degree of special involvement from teachers. This could include consultations with parents or even practical help.
GSCEs.
Although children with CF are as academically as able as their peers, some may have special educational needs. CF may also prevent or hinder the child from using certain educational facilities.
The joint council for the GCSE (standing agreement number four) advises that 'Examining groups are required to take all reasonable steps to enable candidates with permanent, long-term, or temporary handicaps or indispositions to demonstrate their attainments'. Such steps can mean that under suitable safeguards, a grade can be awarded if a candidate is absent from an exam for legitimate reasons. Additional time may be permitted (normally up to 25%) for all types of examination.
If necessary, a candidate can receive treatment during a supervised break. Arrangements can also be made (subject to examining board approval) for candidates to take examinations outside their own centre (ie at home or in hospital).
Disabled candidates may be given additional time to complete course work, or assigned a reduced amount of course work. Again, this is subject to the approval of the examining board.
In order for a pupil to be considered for concessions at GCSE level, they need to have documented their special needs over a number of years. A child with CF should be included on the school's register of special needs as soon as possible.
It is advisable to keep a record of any absences from school, so it can be presented at the request of the examining board.
Some other facts;
♠ The defective gene that causes Cystic Fibrosis was found in 1989.
♠ Cystic Fibrosis Trust is the only national charity dedicated to helping those affected by Cystic Fibrosis.
♠ The Cystic Fibrosis Trust needs to raise £8 million every year to fund its vital work.
♠ Each week 5 babies are born with Cystic Fibrosis in the UK.
♠ Each week three young lives are lost to Cystic Fibrosis.