Here is a list of Cystic Fibrosis Facts that you can use in raising awareness. I will continue to add facts as I discover more, if you have any suggestions feel free to comment below and I will add it! You can see the facts below in a sharable format on our Facebook page.
Sharable Fact Photo Albums: 2015 2016 2017
- You have a 1 in 4 change of having CF if both of your parents are carriers of a mutated gene (it doesn’t have to be the same gene)
Carriers only have 1 gene
Cystic Fibrosis needs 2 genes
There are more than 1,800 known gene mutations for CF. - In CF the mutated gene causes thick sticky Mucus
to build up in the lungs and other major organs. It
clogs the airways and traps bacteria in the lungs
leading to infection and long term damage. It also
stops the digestive enzymes in the pancreas from
breaking down food and absorbing vital nutrients. - The average life expectancy for a person with CF is 37 years old.
About 150 to 200 people with Cystic Fibrosis have received a lung transplant each year since 2007.
Organ transplant is one of the many ways CF is
battled. - CF is not just a terminal Lung Disease.
95% of men with CF and 20% of women with CF are infertile.
85% of people with CF are pancreatic insufficient and have to take enzymes to aid digestion. - All 50 States were required to add CF to the
Newborn Screening by 2010. Only 5 states were
doing so in 2005.
Approximately 1,000 new cases of CF are
diagnosed each year.
About 30,000 currently have CF. - No two CF cases are alike, even if they have the
same two mutations. This creates a wide variety of
doctor opinions and treatment variations.
Most with CF take daily pills, liquid medication,
neutralized medication and vest treatments. - Located on human chromosome 7, the CFTR gene is made up of 250,000 DNA nucleotides. CFTR stands for Cystic Fibrosis Transmembrane Conductance Regulator. The CFTR protein functions as a channel for the movement of chloride ions in and out of cells, which is important for the salt and water balance on epithelial surfaces, such as in the lungs or pancreas. In cystic fibrosis, mutations affect the composition of the mucus layer lining the epithelial surfaces in the lungs and pancreas. The disruption of ion transport affects the salt concentration in sweat.
- If you were to kiss the skin of a CF patient, you would find they taste salty. This is due to the high concentration of salt from the faulty protein channels on the cell surface.Doctors use a Sweat Chloride Test as a method to test for CF, commonly known as a Sweat Test. This test measures the amount of Salt that is in a persons sweat. People with CF have 2 – 3 times more Sodium and Chloride in their sweat than normal people.
- Daily Medications Common for CFers to take:
~ Enzymes for digesting (20+ pills a day)
~ Special Vitamin due to Malabsorption
~ Albuterol inhaled or nebulizer, opens airways
~ Hypertonic saline nebulized, Mucus Thinner
~ Pulmozyme nebulized, Mucus Thinner
~ Airway clearance via Vest or by Hand to move mucus (1+ hours daily)
~ Plus any allergy or antibiotics they might currently be on - In 1965, the term 65 Roses was coined by a 4
year old boy after listening to his Mother making awareness calls for donations to support research of CF. When he tried to say Cystic Fibrosis it
come out more like 65 Roses. This made way for
the rose to be a symbol for Cystic Fibrosis. - Aquagenic wrinkling of the palms often occurs in CF
patients and at times carriers. It is rapid pruning of
the hands and feet when exposed to water. (washing hands, holding a drink that is sweating)It may be caused by a salt imbalance in the skin cells, which results in increased water retention within
these cells and increased transepidermal water loss. - Clubbing of the Nails can occur in people with Cystic Fibrosis. Clubbing is a clinical finding characterized by bulbous fusiform enlargement of the distal portion of a digit.
- About the Cystic Fibrosis Foundation:
1955 – Foundation Created
1980 – Research Development Program
1989 – Program discovers CF Gene
1990s – National Network of Centers
created to streamline therapies and research.
11 Research Centers in the US Today
70 Local CFF Chapters in the US Today
120+ CF Care Centers in the US Today - Cystic fibrosis-related diabetes (CFRD) is a unique type of diabetes that is common in people with CF. With early diagnosis and proper treatment, CFRD can be managed successfully. CFRD shares some features with both type 1 and type 2 diabetes. The pancreas scarring from the mucus can make them insulin deficient. They can also be insulin resistant.
- CFTR gene mutation is in 1 in 25 of Caucasian decent (White European) people. It was discovered that this was so high due to the advantage carriers had over non carriers during the outbreaks of Cholera and Typhus. These diseases create an electrolyte imbalance and acute diarrhea which leads to dehydration. Carriers of CFTR only secreted half the fluid which was enough to flush the toxins.
- The malabsorption found in people with CF leads to a High Fat, High Calorie diet. Early into research Doctors would suggest High Fat, High Calorie foods that wouldn’t normally be eaten in excess by people due to cholesterol levels and other long term body complications. Now that CF patients are living well into adulthood the focus has turned to Healthy Whole Foods that are High Fat and High Calorie.A healthy diet seems to have a positive effect on people with CF but more research is being done on the Gut health connect.
- Nasal polyposis is a sinonasal manifestation of Cystic Fibrosis. Up to 67% of all CF patients will manifest polyps in their lifetime.The polyps are probably a consequence of chronic inflammation; however, their specific etiology is unclear. Treatment includes antibiotics, topical nasal steroids, decongestants, and mucolytics. Surgery is necessary when conservative measures fail.
- Cystic Fibrosis is often called an Invisible Illness. 96% of people with chronic medical conditions show no outward signs of their illness.
Someone with Cystic Fibrosis won’t look “sick” on the outside. On the inside their body is fighting a losing battle. It takes them more energy to do the simplest tasks, from walking into a store to just breathing.When someone with CF is showing outer signs of being sick, it is much worse on the inside, and they may be in End Stages of the disease. - People with Cystic Fibrosis cannot be together in the same room or area. (Siblings and Family with CF excluded)The community has a 6 foot rule, due to the bacteria that sticks around in the mucus in the lungs of someone with CF.Germs can spread as far as 6 feet when released into the air through coughing or sneezing. It is important to stay home if you are sick, you never know who you are spreading to.
- Pseudomonas Aeruginosa (P. aeruginosa) is a common bacteria that is considered opportunistic. It only affects people with CF & compromised immune systems. It is extremely difficult to get rid of, and is nearly half of all with CF. (70% of adults with CF) It is found in damp places, drains, standing water, and contact with someone infected, or something they’ve touched such as doorknobs. Bacteria will begin to grow in standing water after about 24 hours!
- Burkholderia Cepacia Complex (B. cepacia) – Consists of about 20 different species of bacteria that are found in the natural environment, 6 of which are found most often in CFers that contract B. Cepacia. Most are resistant to many antibiotics so it is hard to rid after contracting, it can cause a rapid decline of lung function if left untreated. This bacteria can spread person to person or object. It can be found in soil and water and has been linked to rotting onions specifically.
- MRSA stands for methicillin-resistant Staphylococcus aureus is a bacteria that can cause an infection on the skin & in the lungs. It is resistant to most antibiotics. It is spread through infected person or object (doorknob) contact. To prevent contracting this it is important to wash hands with soap & water, keep wounds covered until healed, always cough and sneeze in a tissue, don’t share personal items, and wipe down shared equipment before using with alcohol wipes.
- Influenza or “the flu” is a highly contagious respiratory illness that is caused by a virus. This virus is well know for its severity in all people but for those that have CF it can be hospitalizing, & it can lead to a severe lung infections such as pneumonia.It is important for everyone to stay home if sick, including children. Where some might contract the Flu and be back to full health afterwords, someone with CF will not be able to be full health as quickly or easily if at all.
- Gastronomy or G Tube can help people with CF get the nutrition they need to thrive when faced with weight gain, breathing or infection issues that harbor calorie absorption and intake. There are 4 different tube options, depending on the persons need. In 2014 about 11% of CFers were using a G Tube to help supplement nutrition.Doctors look for these signs: Weight for height less than 85%, Weight loss for 2 consecutive months, no weight gain for 2 – 3 months in children under age 5, and no weight gain for 6 months for children 5 years +.
- CF Clinic – Visits can rage from Monthly to every three months depending on lung function. Clinic can last from 2-5 hours, and can include visits with a Pulmonologist, Respiratory Therapist, Dietitian, Child Life Specialist, Social Worker, & Team Registered Nurse. At each visit they may require blood draws, x-rays, throat swabs, lung function tests, and more depending on the case. This is on top of still visiting the childs Pediatrician so newborn CF babies could be a the doctor weekly just for checkups. Many with CF are hospitalized 1-4+ times a year for tune ups or illnesses.
- CF Game Changers:
Early 1990’s: Vest Developed
Assists Clearing Mucus
1993: Pulmozyme FDA approved
Proven to thin mucus
1997: TOBI FDA approved
Proven to improve CF health
2004: Hypertonic Saline
Proven to help clear mucus
2012: Kalydeco FDA approved, ages 6+
For G551D, G1244E, G1349D, G178R, G551S, S1251N, S1255P, S549N, or S549R gene types
2015: Orkambi FDA Approved for age 6+
For two copies of F508del (,most common) - Meconium ileus is a bowl obstruction that occurs when the meconium in your child’s intestine is even thicker and sticker than normal meconium, creating a blockage in a part of the small intestine called the ileum. MI can lead to bowel perforation, a twisting of the bowel, or inflammation and infection of the abdominal cavity. About 18% of CF patients are affected by this. Many discover their child has CF because they were born with MI, about 98% born with MI have CF.
- Second Hand Smoke is harmful to all but when you already have lung and body complications it can lead to long term damage.
Some affects of being exposed:
~ Slows lung growth, develop bronchitis and pneumonia more often, and wheezing and coughing are more common, all around get sick more often than children not exposed.
~ Ear infections are more common, as is fluid in the ears, and tubes being needed.
~ Slows weight and height growth. - CF Straw ChallengeHold your nose with a straw in your mouth. Breathe through the straw for 1 minute.
Not that hard right?
Keep going, your lungs will begin to strain, your head hurts, and your palms will sweat. Thankfully you can stop when you want.
This is breathing with CF 24/7.How long did you last? - Liver Disease & Cystic Fibrosis
Damage to the liver starts in the small bile ducks. The bile produced by the liver cells into these ducts become sticker than normal and blockage of the ducts follows (this very occasionally causes jaundice.) The surrounding liver tissue then becomes damaged and scarred, which may be referred to as biliary fibrosis. This is not unlike the underlying process occurring in the lungs. In time the fibrosis processes and eventually spreads throughout the liver. This causes the liver to become hard and the blood flow through it more difficult. Liver transplants are needed in most cases. - Ways YOU can help
- Learn about CF and spread awareness
- Join our Great Strides Team, don’t live in our area? No worries, start your own branch of our National Team! It’s easy and fun! Message us!
- Interact with our posts! The best way for us to reach our supporters is for people to interact with us! It spreads our reach bubble!
- Visit the CFF.ORG and find events near you!
- Whether it’s virtual or in person, your support, understanding ear, and reassuring friendship makes every day easier to get through.
