Living with Cystic Fibrosis

Many of our readers had no idea that CF (Cystic Fibrosis) can be treated through PICC lines and ports so we wanted to share more on Cystic Fibrosis below. Feel free to continue to send us other topics that you’re interested in learning about!

What is Cystic Fibrosis?

Cystic fibrosis is a life-threatening, genetic disease that causes persistent lung infections and progressively limits the ability to breathe. It is a genetic disorder that affects mostly the lungs but also the pancreas, liver, kidneys and intestines. It is a disease of the secretory glands, which include glands that make mucus and sweat. In the lungs, the mucus clogs the airways and traps bacteria leading to infections, extensive lung damage and eventually, respiratory failure. In the pancreas, the mucus prevents the release of digestive enzymes that allow the body to break down food and absorb vital nutrients.

Cystic fibrosis is a recessive disorder, which means that both parents must pass on the defective gene for their children to get the disease. If a child receives only one copy of the faulty gene from one parent, it will make him or her a carrier but he or she will not have the disease. Carriers do not have the disease because it requires both copies of the faulty genes, however, they can pass it on to their children. If both parents are carriers there is a 1 in 4 chance that both will pass on the non-functioning gene.

The main signs of cystic fibrosis are the accumulation of thick and sticky mucus, salty-tasting skin, poor growth and poor weight gain. Many times frequent chest infections, coughing or shortness of breath may be indications of cystic fibrosis. Symptoms of cystic fibrosis may start occurring in infancy and childhood. A more comprehensive list of symptoms include:

• Very salty-tasting skin
• Persistent coughing, at times with phlegm
• Frequent lung infections including pneumonia or bronchitis
• Wheezing or shortness of breath
• Poor growth or weight gain in spite of a good appetite
• Frequent greasy, bulky stools or difficulty with bowel movements
• Male infertility

 You can learn more from the Cystic Fibrosis Foundation.

Respiratory System Signs and Symptoms

People who have cystic fibrosis have thick, sticky mucus that builds up in their airways. This buildup of mucus makes it easier for bacteria to grow and cause infections. Infections can block the airways and cause frequent coughing that brings up thick spit or mucus that is sometimes bloody.

People who have cystic fibrosis tend to have lung infections caused by unusual germs that don't respond to standard antibiotics. For example, lung infections caused by bacteria called mucoid pseudomonas are much more common in people who have cystic fibrosis than in those who do not. An infection caused by these bacteria may be a sign of cystic fibrosis. Those infected tend to have frequent infections of the sinuses, hollow air spaces around the eyes, nose, and forehead. These infections can cause long-term lung damage.

As cystic fibrosis gets worse, you may have more serious problems such as bronchiectasis or even need nasal surgery. Bronchiectasis is when there is extensive damage to the lung’s airways that cause them to widen and become flabby and scarred. It injures the walls so much that the airways are unable to clear mucus, which can lead to severe infections and lack of oxygen flow to other parts of your body. Lung function often starts to decline in early childhood in people who have cystic fibrosis. Over time, damage to the lungs can cause severe breathing problems. Respiratory failure is the most common cause of mortality in people who have cystic fibrosis.

Digestive System Signs and Symptoms

In cystic fibrosis, mucus can block tubes or ducts in your pancreas. These blockages prevent enzymes from reaching your intestines. As a result, your intestines cannot fully absorb fats and proteins. This can cause ongoing diarrhea or bulky, smelly, greasy stools. Intestinal blockages also may occur, especially in newborns. Too much gas or severe constipation in the intestines may cause stomach pain and discomfort.

A hallmark of cystic fibrosis in children is poor weight gain and growth. These children are unable to get enough nutrients from their food because of the lack of enzymes to help absorb fats and proteins. As cystic fibrosis gets worse, other problems may occur, such as:

• Pancreatitis: this is a condition in which the pancreas becomes inflamed, which causes pain.
• Rectal prolapse: Frequent coughing or problems passing stools may cause rectal tissue from inside you to move out of your rectum.
• Liver disease due to inflamed or blocked bile ducts.
• Diabetes
• Gallstones

Diagnosis of CF

Newborn Test

Since cystic fibrosis can begin showing symptoms in infants, testing approaches vary on age. All states screen newborns for cystic fibrosis using a genetic test or a blood test. The genetic test shows whether a newborn has faulty CFTR genes. The blood test shows whether a newborn's pancreas is working properly.

Sweat Test

If a genetic test or blood test suggests cystic fibrosis, a doctor will confirm the diagnosis using a sweat test. This test is the most useful test for diagnosing cystic fibrosis. A sweat test measures the amount of salt in sweat. For this test, the doctor triggers sweating on a small patch of skin on an arm or leg. He or she then rubs the skin with a sweat-producing chemical and then uses an electrode to provide a mild electrical current. This may cause a tingling or warm feeling. Sweat is collected on a pad or paper and then analyzed. The sweat test usually is done twice. High salt levels confirm a diagnosis of cystic fibrosis.

Other Tests

If you or your child has cystic fibrosis, your doctor may recommend other tests, such as:

• Genetic tests to find out what type of CFTR defect is causing your cystic fibrosis
• A chest x-ray: This test creates pictures of the structures in your chest, such as your heart, lungs, and blood vessels. A chest x-ray can show whether your lungs are inflamed or scarred or whether they trap air.
• A sinus x-ray: This test may show signs of sinusitis, a complication of cystic fibrosis.
• Lung function tests: These tests measure how much air you can breathe in and out, how fast you can breathe air out and how well your lungs deliver oxygen to your blood.
• A sputum culture: For this test, your doctor will take a sample of your spit to see whether bacteria are growing in it. If you have bacteria called mucoid Pseudomonas,you may have more advanced cystic fibrosis that needs aggressive treatment.

Prenatal Screening

If you're pregnant, prenatal genetic tests can show whether your fetus has CF. These tests include amniocentesis and chorionic villus sampling (CVS). In amniocentesis, your doctor inserts a hollow needle through your abdominal wall into your uterus. He or she removes a small amount of fluid from the sac around the baby. The fluid is tested to see whether both of the baby's CFTR genes are normal. In CVS, your doctor threads a thin tube through the vagina and cervix to the placenta. The doctor removes a tissue sample from the placenta using gentle suction. The sample is tested to see whether the baby has cystic fibrosis.

Cystic Fibrosis Carrier Testing

If you have a family history of fibrosis or a partner who has it and you are planning a pregnancy, you may want to find out whether you're a fibrosis carrier. A genetics counselor can test a blood or saliva sample to find out whether you have a faulty CF gene. This type of testing can detect the faulty genes in 9 out of 10 cases.

Living with Cystic Fibrosis

Cystic fibrosis currently has no cure, but there are several treatments that patients can utilize to help manage this disorder. Recent developments in treatment methods have enabled individuals to lead a fuller life without much disruption from the disorder. Pulmonary rehabilitation is a form of cystic fibrosis management that continues throughout the individual’s life, maximizing organ function and quality of life. Treatment is meant to delay the decline in organ failure. Currently, these are the different treatments for cystic fibrosis:

1. Antibiotics
   Many patients diagnosed with cystic fibrosis are on one or more different medications, healthy or not, to suppress future infections that may occur and to keep the lungs clear. It is absolutely needed whenever pneumonia is suspected and there is a noticeable decline in lung functions through sputum (spit) analysis and assessments. Prolonged therapies for patients with a more serious case of cystic fibrosis are often hospitalized and antibiotics are transported through a PICC line. Inhaled antibiotics are used to help fight infections and oral antibiotics are used to prevent or control ongoing infections. Make sure to consult with your healthcare provider about any medications being taken since many medications also come with side effects.
2. Mechanical Techniques
   Usually, this treatment is utilized in hospital settings. A respiratory therapist will perform a chest physiotherapy (CPT), which is basically percussing or lightly tapping a patient’s chest with their hands several times a day to loosen secretions. Other devices that can mimic this therapy is:

• ThAIRapy Vest: This is a device and system for clearing excess mucus from lung airways (bronchi and bronchioles)
• Intrapulmonary Percussive Ventilator (IPV): This is a pneumatic positive pressure device used to move mucus
• Biphasic Cuirass Ventilation: This is a non-invasive method of negative pressure ventilation which requires the patient to wear an upper body shell or cuirass
• If lung function worsens, patients may need to sleep with breathing supports, like special masks to push air into the lungs to prevent low blood oxygen levels with a machine called bi-level positive airway pressure ventilators (BiPAP).
• Transplant Individuals who choose to have a transplant are considered when lung function seriously declines and these individuals must have both lungs replaced because the remaining lung might contain bacteria that could infect the new transplanted lung.
• Nutrition and Lifestyle Keeping good nutrition and maintaining a healthy lifestyle can help manage cystic fibrosis. Eat whole foods while meeting the daily nutritional values and keeping an active lifestyle helps fight off infections and keep the body healthy. Because gaining weight is a difficult thing to do for a person with cystic fibrosis, it is important to keep nutrition and exercise a constant. 

Your cystic fibrosis doctor may prescribe many medications to help keep your lungs clear, prevent or fight infections and help correct the underlying cause of the disease. Medications that people need to fight infections for a long time may require additional devices such as PICC lines and ports.

At Care+Wear, we believe that everyone should feel like a person first and a patient second. As a result, we have created a PICC line cover and port access shirt that you can check out here. You can also read about other patient stories here!