Cystic Fibrosis' Description, Symptoms & Treatment
Cystic Fibrosis
Description
Cystic Fibrosis (CF) is when mucus in the lungs is thicker and stickier than normal making it harder to breathe. CF occurs when there are two defective copies of the gene responsible for cystic fibrosis, one from each parent. [1] Cystic Fibrosis is a disease affecting the respiratory and digestive system. CF mostly affects the lungs. A defective gene causes the cells that produce mucus, sweat and digestive juices to be sticky and thick. This can plug up tubes, ducts, and passageways especially in the pancreas and lungs.
Symptoms
The symptoms of CF are not identical from person to person, but here are some of the most common symptoms: Respiratory symptoms:
- A continuous cough producing thick mucus
- Wheezing
- Breathlessness
- Exercise intolerance
- Repeated lung infections
- Inflamed nasal passage or stuffy nose
Digestive symptoms:
- Foul smelling, greasy stools
- Poor weight gain and growth
- Intestinal blockage (particularly in newborns)
- Severe constipation
- Images are numerically labeled to match their corresponding symptoms
Daily Life
CF does require daily care, but due to new treatment people can usually do normal daily activities. For example, most people do treatments in the morning, evening and night for twenty minutes each. Besides for those treatments they live normal lives. Treatment: The treatment of CF can affect people in many different ways. For the most part, it affects patients positively by helping them to breathe and to live a better life. Once a patient receives a lung transplant CF cannot occur in a new set of lungs symptoms still of CF can still occur. For example, after receiving a lung transplant some symptoms that may still occur are sinus infections, diabetes, pancreas problems, and osteoporosis.
Diagnosis
Newborn screening and diagnosis
A blood sample can be checked for higher than normal levels of immunoreactive trypsinogen (IRT) released by the pancreas. After, a sweat test can be done when the baby is at least 2 weeks of age. The most common way to diagnose CF is with a screening of newborns which is done on most babies prior to or just after birth. Many people know of the condition within the first month of life before symptoms develop. [2]Testing of older children and adults: If a physician suspects a patient to have CF tests can be recommended if they weren't done at birth. Genetic and sweat tests for cystic fibrosis will be done if you have any CF-related symptoms. A doctor applies a sweat-producing chemical to the skin. They will then collect a sweat sample and test it see if its saltier than usual.
Risks
Having tests done may cause unnecessary stress.One test being positive can make people think they have CF when further testing shows otherwise.
Benefits
The tests are cheap and easily done. Early genetic testing can prepare you for what is to come.Once you are diagnosed treatment options can be discussed to improve your way of life.
Treatment
Although CF requires daily care, new treatment makes it so that people can usually do normal daily activities. Improvements with screening and treatments have made it so people can live from the mid to late thirties and some even live into their fifties. There is not a cure for CF but treatments can ease symptoms and reduce the risk of complications.
Medication
Up to $200,000 per year - Can be covered in Canada) Antibiotics treating and preventing lung infections.Anti-inflammatory medications can lessen swelling in airways of lungs.Mucus-thinning drugs- Making it easier to cough up mucus.Bronchodilators can help keep your airways open by relaxing the muscles around your bronchial tubes.Medication is usually effective but is not very popular due to the price. Sometimes medication does not have any effect on patients with CF.
Chest Physical Therapy
(Can be done for free)This therapy loosens the mucus in the lungs making it easier to cough up. Most of the time it is done one to four times a day, the most common way this is done is by hitting cupped hands on the front and back of the chest. Breathing techniques also can be used to help loosen the mucus. Although this is cheap (Can be done at home) this treatment is often done incorrectly making it not effective.
Vest Therapy
($15,000 - $20,000 for vest) This therapy involves a machine which is made up of two pieces. First is an inflatable vest which is connected to an air-pulse generator by hoses. The generator pushes air through the hoses, causing the vest to inflate and deflate quickly, up 20 times per second. The inflating at deflating is similar to chest physical therapy. The vibrations separate mucus from the airway walls. Usually, the vest is used for five minutes and then the person will cough to clear the mucus. Typical sessions last about 20 to 30 minutes. Vest therapy is very effective, it can be expensive but it is a one time cost.
Surgical + Other Procedures
($2,000 - $100,000+ Usually covered in Canada)Endoscopy and lavage - Mucus can be sucked out from obstructed or blocked airways through an endoscope. Bowel surgery - If a blockage develops in your bowel, you may need surgery to remove it. Lung transplant - If you have a severe breathing problem or a life-threatening lung infection one or both lungs may need to be replaced. These treatment options usually come last when the disease gets uncontrollable. When surgeries go successfully they can be very effective but are still not a long-term fix.
Stem Cells
There was research done on stem cell transplantation, this involves collecting stem cells from the lungs of CF patients, correcting them with gene therapy, and finally reintroducing those cells back into the patient.The new stem cells pass on their healthy genes to their 'daughter cells' replenishing the airways with healthy cells and combating cystic fibrosis airway disease.When testing this on animals they first removed the existing surface cells, which created space needed to introduce the new cells. [5]
Alternative Medicine
You can eliminate foods that increase mucus production, including dairy (milk, cheese, sour cream, and ice cream), wheat (gluten), soy, corn, potatoes, cabbage, bananas, sugar, preservatives, food additives, and excessive salt and meats. Acupuncture can alleviate some of the symptoms of CF. Acupuncture may help to improve immune function, normalize digestion, as well as improving respiratory function. [3]*There is no real “effectiveness” for the treatments since none of them will ever cure CF. However, studies do show that western medicine allows CF patients to have a longer and better quality of life compared to patients who only use alternative medicine.
Prognosis
CF cannot be cured, so there is no cure rate. The average age CF patients live until is 51.8. A person with CF lives with this disease from the time they are born until they pass away due to the disease.
Prevention
If you or your partner know a close relative with CF it may benefit you to undergo genetic testing before having children. Genetic testing is done in a lab with a sample of blood, it can help determine the risk of having a child with cystic fibrosis. Since CF is genetic there is no true way to prevent it. The only thing is to be tested for the gene. If two people have the gene that makes children have CF they may decide not to have children do to the likelihood of CF occurring.
References:
- What is Cystic Fibrosis? (2018). Cystic Fibrosis Canada. Retrieved from https://www.cysticfibrosis.ca/about-cf/what-is-cystic-fibrosis
- Cystic fibrosis. (2016, October 13). Mayo Clinic. Retrieved from https://www.mayoclinic.org/diseases-conditions/cystic-fibrosis/symptoms-causes/syc-20353700
- Complementary and Alternative Medicine. (n.d.). St. Mikes Hospital. Retrieved from https://www.stlukes-stl.com/health-content/medicine/33/000045.htm
- Robinson, P., & Armstrong, D. S. (2017). How Well Do Children with Cystic Fibrosis Sleep? The Journal of Pediatrics, 182, 170-176. Retrieved September 20, 2018.
- Dr. Farrow. (2018). Stem Cell Research For Cystic Fibrosis Moves Forward. Biospectrum Asia. Retrieved September 21, 2018.
- Cystic Fibrosis Statistics. (n.d.). Cystic Fibrosis News Today. Retrieved September 28, 2018, from https://cysticfibrosisnewstoday.com/cystic-fibrosis-statistics/CF Facts. (n.d.).
- Cystic Fibrosis Canada. Retrieved September 25, 2018, from https://www.cysticfibrosis.ca/blog/introducing-cf-registry -facts/
