CYSTIC FIBROSIS

- An autosomal recessive disease CF gene

- A genetic condition that affects a protein in the body. People who have cystic
fibrosis have a faulty protein that affects the body's cells, its tissues, and the
glands that make mucus and sweat. Normal mucus is slippery and protects the
airways, digestive tract, and other organs and tissues.
- is a genetic disorder inherited in an autosomal recessive manner that impairs
the normal clearance of mucus from the lungs, which facilitates the colonization
and infection of the lungs by bacteria, notably Staphylococcus aureus.
- Viscous secretion in lungs, intestines, reproductive tract, pancreas, increase salt
in sweat.
 SIGNS AND SYMPTOMS
• Pulmonary exacerbation involves a worsening of lung symptoms, such as more
coughing or wheezing, chest congestion, and a change in mucus color. You may also
have weight loss, a poor appetite, or fever.
• Coughing or spitting up blood may be a sign that an artery has broken and is
bleeding into the airway. Thick mucus.
• Sudden shortness of breath or chest pain may be a sign of a pneumothorax, or
collapsed lung.
• Exercise intolerance
• Repeated lung infections
• Inflamed nasal passages or a stuffy nose
• Recurrent sinusitis
 SIGNS AND SYMPTOMS
• Wheezing and Productive Cough with mucus.
• Clubbing of fingers and toes due to less oxygen getting to the
hands and feet
• Delayed puberty
• Fertility problems, especially for males
• Fever, which may include night sweats
• Gastrointestinal symptoms such as severe belly pain, chronic
(long-term) diarrhea, or constipation, foul smelly greasy stools, poor
weight gain and growth, intestinal blockage.
 SIGNS AND SYMPTOMS
• Infections of sinuses and lungs
• Jaundice , or yellowing of the skin and eyes, for an abnormally long
time after birth
• Low body mass index (BMI) or being underweight
• Muscle and joint pain
• Pancreatitis ( inflammation of the pancreas)
• Salty skin and extra-salty sweat
• Slow growth and shorter height
 DIAGNOSTIC TEST
• Checking of Family History should be done since it is a genetic disease.
• Genetic Testing (Newborn Screening) - a blood sample is checked for
higher than normal levels of a chemical called immunoreactive trypsinogen
(IRT), which is released by the pancreas. A newborn IRT level may be high
because of premature birth or a stressful delivery. But other test may be
needed to confirm a diagnosis of cystic fibrosis.
• Sweat test- for evaluation of your CF. If an infant has cystic fibrosis, doctors
may also conduct a sweat test once the infant is at least 2 weeks old. A
sweat-producing chemical is applied to a small area of skin. Then the sweat
is collected to test it and see if it's saltier than normal.
 THE GOALS OF TREATMENT:

• Preventing and controlling infections that occur in the

lungs
• Removing and loosening mucus from the lungs
• Treating and preventing intestinal blockage
• Providing adequate nutrition
 MEDICAL MANAGEMENT
• Antibiotics to treat and prevent lung infections
• Anti-inflammatory medications to lessen swelling in the
airways in your lungs
• Mucus-thinning drugs, such as hypertonic saline, to help you
cough up the mucus, which can improve lung function.
• Inhaled medications called bronchodilators that can help
keep your airways open by relaxing the muscles around your
bronchial tubes.
 MEDICAL MANAGEMENT
• Oral pancreatic enzymes to help your digestive tract
absorb nutrients
• Stool softeners to prevent constipation or bowel
obstruction
• Acid-reducing medications to help pancreatic enzymes
work better
• Specific drugs for diabetes or liver disease, when
appropriate
 RESPIRATORY SYSTEM COMPLICATIONS
• Damaged airways (bronchiectasis). Cystic fibrosis is one of the leading
causes of bronchiectasis, a chronic lung condition with abnormal widening
and scarring of the airways (bronchial tubes). This makes it harder to
move air in and out of the lungs and clear mucus from the bronchial tubes.
• Chronic infections. Thick mucus in the lungs and sinuses provides an
ideal breeding ground for bacteria and fungi. People with cystic fibrosis
may often have sinus infections, bronchitis or pneumonia. Infection with
bacteria that is resistant to antibiotics and difficult to treat is common.
• Growths in the nose (nasal polyps). Because the lining inside the nose
is inflamed and swollen, it can develop soft, fleshy growths (polyps).
 RESPIRATORY SYSTEM COMPLICATIONS
• Coughing up blood (hemoptysis). Bronchiectasis can occur
next to blood vessels in the lungs. The combination of airway
damage and infection can result in coughing up blood. Often this
is only a small amount of blood, but it can also be life-threatening.
• Pneumothorax. In this condition, air leaks into the space that
separates the lungs from the chest wall, and part or all of a lung
collapses. This is more common in adults with cystic fibrosis.
Pneumothorax can cause sudden chest pain and breathlessness.
People often feel a bubbling sensation in the chest.
 RESPIRATORY SYSTEM COMPLICATIONS
• Respiratory failure. Over time, cystic fibrosis can damage lung tissue so
badly that it no longer works. Lung function usually worsens gradually, and
it eventually can become life-threatening. Respiratory failure is the most
common cause of death.
• Acute exacerbations. People with cystic fibrosis may experience
worsening of their respiratory symptoms, such as coughing with more
mucus and shortness of breath. This is called an acute exacerbation and
requires treatment with antibiotics. Sometimes treatment can be provided
at home, but hospitalization may be needed. Decreased energy and
weight loss also are common during exacerbations.
 DIGESTIVE SYSTEM COMPLICATIONS
• Nutritional deficiencies. Thick mucus can block the tubes that carry digestive
enzymes from your pancreas to your intestines. Without these enzymes, your
body can't absorb protein, fats or fat-soluble vitamins, so you can't get enough
nutrients. This can result in delayed growth, weight loss or inflammation of the
pancreas.
• Diabetes. The pancreas produce insulin, which your body needs to use sugar.
Cystic fibrosis increases the risk of diabetes. About 20% of teenagers and 40%
of adults.
• Liver disease. The tube that carries bile from your liver and gallbladder to your
small intestine may become blocked and inflamed. This can lead to liver
problems, such as jaundice, fatty liver disease and cirrhosis — and sometimes
gallstones.
 DIGESTIVE SYSTEM COMPLICATIONS
• Intestinal obstruction. Intestinal blockage can happen to
people with cystic fibrosis at all ages. Intussusception, a
condition in which a segment of the intestine slides inside
an adjacent section of the intestine like a collapsible
telescope, also can occur.
• Distal intestinal Obstruction Syndrome (DIOS). Is
partial or complete obstruction where the small intestine
meets the large intestine.
 REPRODUCTIVE SYSTEM COMPLICATIONS
• Infertility in men. Almost men with CF are infertile because
the tube that connects the testes and prostate gland (vas
deferens) is either blocked with mucus or missing entirely.
Certain fertility treatments and surgical procedures sometimes
make it possible for men with CF to become a biological father.
• Reduced fertility in women. Women may be less fertile than
other women, its possible for them to conceive and to have
successful pregnancies. Still, pregnancy can worsen the s/sx
of CF.
 OTHER COMPLICATIONS
• Thinning of the bones (osteoporosis). People with cystic fibrosis are at
higher risk of developing a dangerous thinning of bones. They may also
experience joint pain, arthritis and muscle pain.
• Electrolyte imbalances and dehydration. Because people with cystic
fibrosis have saltier sweat, the balance of minerals in their blood may be
upset. This makes them prone to dehydration, especially with exercise or
in hot weather. Signs and symptoms include increased heart rate, fatigue,
weakness and low blood pressure.
• Mental health problems. Dealing with a chronic illness that has no cure
may cause fear, depression and anxiety
 SURGICAL AND OTHER PROCEDURES
• Nasal and sinus surgery- remove nasal polyps that obstruct breathing. Sinus
surgery may be done to treat recurrent or chronic sinusitis.
• Oxygen therapy. For blood oxygen level declines. Pure oxygen should be given
to prevent high blood pressure in the lungs (pulmonary hypertension).
• Noninvasive ventilation. Used while sleeping, noninvasive ventilation uses a
nose or mouth mask to provide positive pressure in the airway and lungs when
you breath in. this increase air exchange in the lungs and decrease the work
breathing. This may also help with airway clearance.
• Feeding tube- to absorb nutrients from food very well. For feeding to deliver
extra nutrition by giving extra calories.
SURGICAL AND OTHER PROCEDURES
• Bowel Surgery. For the blockage in your bowel to remove it.
Intussusception, where a segment of intestine has telescoped
inside an adjacent section of intestine, also may require
surgical repair.
• Lung transplant. For life threatening lung complications or
increasing resistance to antibiotics for lung infections. Lung
transplantation is done because bacteria line the airways in
diseases that cause permanent widening of the large airways
(bronchiectasis).
 NURSING MANAGEMENT
• Chest physiotherapy - relieve mucus obstruction and help to reduce
infection and inflammation in the airways. These techniques loosen
the thick mucus in the lungs, making it easier to cough up.
• Fluid and dietary intake
• Reduce risk for infection.
• Pulmonary rehabilitation – for long term treatment. Physical
exercise that may improve your condition. Breathing techniques that
may help loosen mucus and improve breathing. Nutritional
counseling. Counseling and support, Education about your condition.
 NURSING DIAGNOSIS
• Imbalanced Nutrition: Less Than Body Requirements
Related to: Malabsorption, Poor digestion ,Nutrient deficiencies
As Evidence by: Delayed growth, Diarrhea or constipation , Foul-smelling
and greasy stools , Poor weight gain , Intestinal obstructions , Abdominal
distention or bloating
• Impaired Gas Exchange
Related to: Accumulation of tenacious secretions, Infectious process,
Inflammation of airways
As Evidence by: Abnormal ABGs, Tachypnea, Dyspnea, Hypoxia,
Restlessness, Tachycardia, Altered respiratory pattern
 NURSING DIAGNOSIS
• Impaired Spontaneous Ventilation
Related to:Excessive mucus production, Respiratory fatigue, Airway blockage,
Respiratory infections
• As Evidence by: Accessory muscle use, Shortness of breath, Apprehension,
Decreased cooperation, Restlessness
• Ineffective Airway Clearance
Related to: Increased mucus production , Infectious process , Inflammation of the
bronchioles
As Evidence by: Persistent cough , Productive cough, Wheezing ,Shortness of
breath, Nasal congestion , Sinusitis , Abnormal chest x-ray , Adventitious breath
sounds
 NURSING DIAGNOSIS
• Risk For Infection
Related to: Thick mucus causing pneumonia or bronchitis, Antibiotic resistance
causing MRSA, Weakened immune system , Poor environmental conditions
 BRONCHIECTASIS
• Chronic, irreversible dilation of bronchi and bronchioles.
• Predisposing Factors: Recurrent Respiratory Infection,
PTB
• Inflammation process with pulmonary infection damage
the bronchial wall result to thick sputum obstructing the
bronchi
 SIGNS AND SYMPTOMS
• Chronic cough and purulent sputum
• Shortness of breath
• wheezing
• Coughing up blood bloodstained phlegm
• chest pain
• joint pain
• clubbing of the fingertips – the tissue beneath the nail thickens and
the fingertips become rounded and bulbous
 BRONCHIECTASIS LEADS TO LUNG
INFECTION
Signs and symptoms of a serious lung infection include:
• a blueish tinge to the skin or lips (cyanosis)
• confusion
• a high temperature
• rapid breathing (more than 25 breaths a minute)
• severe chest pain that makes it too painful to cough and clear
your lungs
 WHY IT HAPPENS?
Bronchiectasis can develop if the tissue and muscles that surround the bronchi
are damaged or destroyed.
The 3 most common causes:
• having had a lung infection in the past, such as pneumonia or whooping cough,
that damages the bronchi
• underlying problems with the immune system (the body's defense against
infection) that make the bronchi more vulnerable to damage from an infection
• Aspergillosis– an allergy to a certain type of fungi that can cause the bronchi to
become inflamed if spores from the fungi are inhaled
But in many cases, no obvious cause for the condition can be found. This is
known as idiopathic bronchiectasis.
 WHY IT HAPPENS?
• Mucociliary Dysfunction – chloride is not excreted leads to
thickening of mucus.
• Decrease in Ciliary Function – increase build up of mucus
because mucus will not be expelled
• Airway Obstruction – Tumor, COPD, Foreign body
blockage.
 COMPLICATIONS
Potential complications include:
• Atelectasis. Collapse of the alveoli is a common complication.
• Pneumonia. Infection is recurrent in patients with
bronchiectasis.
• Empyema. Overproduction of sputum causes the bronchi to be
filled with pus.
 ASSESSMENT AND DIAGNOSTIC TEST

• History of productive cough. A definite sign is a prolonged

history of productive cough, with sputum negative for
tubercle bacilli.
• CT scan. The diagnosis is established by a CT scan, which
reveals bronchial dilation.
 NURSING ASSESSMENT
Nursing assessment of a patient with bronchiectasis
include:
• Evaluation of current smoking status.
• Evaluation of current exposure to occupational
toxins or pollutants and in indoor/outdoor pollution.
• Assess the patient’s current level of functioning.
 SURGICAL MANAGEMENT
Surgical intervention may be indicates for patients who continue to
expectorate large amounts of sputum and have repeated bouts
of pneumonia.
• Segmental resection. The diseased segment of a lobe is removed.
• Lobectomy. The diseased lobe is removed.
• Pneumonectomy. The entire diseased lung segment is removed,
but this rarely happens.
 MEDICAL MANAGEMENT
• Antibiotics
• Bronchodilators relax the muscles around your airways. This helps open your
airways and makes breathing easier. Most bronchodilators are inhaled
medicines. You will use an inhaler or a nebulizer to breathe in a fine mist of
medicine. Inhaled bronchodilators work quickly, because the medicine goes
straight to your lungs. Your provider may recommend that you use a
bronchodilator right before you do chest physical therapy.
• Inhaled corticosteroids treat inflammation in the airways but should be used
only if asthma or COPD is present and your provider prescribes them.
• Mucus thinners make it easier to clear the mucus from your airways. These
treatments are taken by inhaling them.
 NURSING INTERVENTION
• Smoking cessation. Patient teaching targets smoking and
other factors that increase the production of mucus and hamper
its removal.
• Postural drainage. Perform postural drainage with percussion
and vibration in the morning and at night as prescribed.
• Activities. Encourage alternating activity with rest periods.
• Chest physiotherapy. Chest physiotherapy,
including percussion and postural drainage, is important in the
management of secretions.
 NURSING MANAGEMENT
• Encourage patient to stop smoking.
• Postural drainage –promotes and clears secretion.
• Encourage oral fluid intake - Drinking plenty of fluid,
especially water, helps prevent airway mucus from
becoming thick and sticky. Good hydration helps keep
airway mucus moist and slippery and easier to cough up.
 NURSING DIAGNOSIS
• Impaired gas exchange related to ventilation-perfusion
imbalance.
• Ineffective airway clearance related to increased mucus
production.
• Ineffective breathing pattern related to mucus and airway
irritants.
• Activity intolerance related to hypoxemia and ineffective
breathing patterns.
 HOME INSTRUCTION
• Smoking cessation. Smoking cessation is important, because smoking
impairs bronchial drainage by paralyzing ciliary actions, increasing bronchial
secretions, and causing inflammation of the mucous membranes
• Postural drainage. Patient and families are taught to perform postural
drainage.
• Exposure to infections. The nurse educates the patient and the family to
avoid exposure to people with upper respiratory or other infections.
• Signs of infection. The patient is taught about the early signs of respiratory
infection and the progression of the disorder, so that appropriate treatment can
be implemented promptly.
• Nutrition. The patient’s nutritional statuss is assessed and strategies are
implemented to ensure adequate diet at home.
 NURSING CARE PLAN AND GOAL
•Improvement in gas exchange.
•Achievement of airway clearance.
•Improvement in breathing pattern.
•Improvement in activity intolerance.
 DOCUMENTATION GUIDELINES
• Respiratory rate, character of breath sounds, and presence of
cyanosis.
• Frequency, amount, and appearance of secretions.
• Character of cough.
• Relevant history of the problem.
• Respiratory pattern.
• Use of respiratory aids.
 DOCUMENTATION GUIDELINES
• Level of activity.
• Vital signs before, during, and after the activity.
• Plan of care.
• Teaching plan.
• Client’s responses to treatment, teaching, and actions
performed.
• Attainment or progress towards desired outcomes.
• Modifications to plan of care
ACUTE RESPIRATORY DISTRESS SYNDROME (ARDS)
• is a life-threatening lung condition. It is a form of breathing failure that can occur
in very ill or severely injured people.
• It is not a specific disease.
• It starts with swelling of tissue in the lungs and build up of fluid in the tiny air
sacs that transfer oxygen to the bloodstream. This leads to low blood oxygen
levels.
• ARDS is similar infant respiratory distress syndrome, but the causes and
treatments are different. ARDS can develop in anyone over the age of one year
old.
• fluid builds up inside the tiny air sacs of the lungs, and surfactant breaks down.
 CAUSES
Direct injury to the lungs:
• Chest trauma, such as a heavy blow
• Breathing vomit
• Breathing smoke, chemicals, or salt water
• Burns
 CAUSES
Indirect injury to the lungs:
• Severe infection
• Massive blood transfusion
• Pneumonia
• Severe inflammation of the pancreas (pancreatitis)
• Overdoses of alcohol or certain drugs (eg. aspirin, cocaine, opioids,
phenothiazines, and tricyclic antidepressants)
• Lung and bone marrow transplantation–within few days of a lung
transplant, the recipient is prone to development of ARDS.
 RISK FACTORS
• ARDS usually develops in people who are already in the hospital
and are being treated for an injury.
• However, only a small number of people who have these injuries
actually develop ARDS.
• While none can predict who will get ARDS, cigarette smokers,
those with chronic lung disease, or those who are over age 65
are more at risk of developing ARDS.
• Lifestyle: Heavy alcohol use, Overdose of illegal drugs, Smoking
 HOW TO LOWER THE RISK OF ARDS?
You can help lower your risk of ARDS with the following steps:
• Get routine vaccines to prevent the flu, COVID-19, and other
infections.
• Avoid tobacco smoke.
• Limit the amount of alcohol you drink. (The recommended
amount is no more than 2 drinks per day for men and 1 drink
per day for women.)
• Limit exposure to pollution as much as possible.
 SYMPTOMS

• Shortness of breath
• Fast, labored breathing
• Bluish skin or fingernail color
• Rapid pulse
 DIAGNOSIS
A person suffering from severe infection or injury develops breathing problems
• Blood tests show a low level of oxygen in the blood A low blood oxygen level might be
a sign of ARDS. To confirm the cause of your symptoms, your doctor may also check
your blood for signs of infection or a heart problem, or to see how well other organs
are working.
• Other conditions that could cause breathing problems have been ruled out
• Blood pressure check
• Lung imaging tests, such as a chest X-ray or computerized tomography (CT) scan,
create detailed images of your lungs. This shows fluid in the air sacs of both lungs.
• A sputum culture can help find the cause of an infection. The culture is used to study
the phlegm you have coughed up from your lungs.
• Urine tests detect bacterial infections or rule out kidney problems.
 DIAGNOSIS
• Analysis of coughed-up matter
• Occasionally, an echocardiogram (heart ultrasound), to rule out congestive
heart failure.
• Pulmonary artery catheterization aid in diagnostic work-up
• Bronchoscopy to analyze airways. A laboratory examination may indicate
presence of certain viruses, cancer cells etc. Bronchoscopy can diagnose a
lung problem when there is no clear cause of your ARDS. As part of this test,
your doctor may rinse an area of your lung to get cells and examine them under
a microscope or with other tests.
• Open lung biopsy is reserved for cases when diagnosis is difficult to establish.
 COMPLICATIONS
• Atelectasis: This condition happens when small air pockets in
the lung collapse.
• Complications of treatment in a hospital: Such complications
include blood clots that can form from lying down for long
periods, weakness in muscles used for breathing or moving
around, infections, stress ulcers, and depression or other mood
disorders. Problems with thinking, memory, and judgment can
also result from the long-term use of sedative medicines.
 COMPLICATIONS
• Failure of multiple organs: The body’s organs may not work as well
or may stop working altogether if they do not get enough oxygen. This
lack of oxygen may cause several organs to stop working at the same
time, setting up a potentially life-threatening situation.
• Pulmonary hypertension: This condition is an increase in blood
pressure in the major artery leading from the heart to the lungs. This
can happen when the blood vessel narrows because of inflammation
or mechanical ventilation. ARDS may also cause tiny clots to form in
blood vessels in the lungs.
 NURSING DIAGNOSES

• Ineffective airway clearance

• Ineffective breathing pattern
• Impaired gas exchange
• Anxiety
 MEDICAL MANAGEMENT
• Lung Function Test: These tests will help your doctor check how well your lungs are
working. Your doctor may ask you questions about your symptoms, measure your
blood oxygen level, or do a spirometry test.
• Muscle strength and physical capacity tests: Weak muscles are a common result of
staying in bed for long-term treatment, especially if the doctor gave you a sedative.
One common test of endurance is the 6-minute walk test, which measures how far you
can walk in that time. Such tests will help your doctor know how you are recovering
and whether you need additional care, such as pulmonary rehabilitation or physical
therapy.
• Mental health screening: Your doctor may also ask questions to assess the state of
your emotional health.
 MEDICAL MANAGEMENT
• Acid-reducing medicines prevent stress ulcers, which can
cause bleeding in the intestines.
• Antibiotics treat or prevent infections. If you are on a ventilator,
your healthcare team may do tests, such as lung fluid lab tests or
CT scans, to look for signs of new infection.
• Blood thinners stop blood clots from forming or growing larger.
Heparin is a common blood thinner for adults.
 MEDICAL MANAGEMENT
• Muscle relaxants help prevent coughing or gagging while on a
ventilator or reduce the amount of oxygen your body needs.
• Sedatives help relieve anxiety, make it easier to breathe on a
ventilator, or lower your body’s oxygen needs. Sometimes your doctor
may pair a sedative with another medicine to make delivering the
oxygen easier. Complications vary depending on the sedative used,
the dose, and how long it is used. They can include depression, post-
traumatic stress disorder (PTSD), problems with thinking or memory,
or a delay in removing the ventilator.
 MEDICAL MANAGEMENT
• A feeding tube can ensure you get enough of the right nutrients while you
are on a ventilator.
• Blood transfusions treat low hemoglobin levels. Hemoglobin carries
oxygen in the blood, so a transfusion can improve the delivery of oxygen
to the body’s organs.
• Extracorporeal membrane oxygenation (ECMO) or a similar
device helps when ventilation alone cannot deliver enough oxygen or
while a patient waits for a lung transplant. ECMO works like an artificial
lung, removing carbon dioxide and pumping oxygen-rich blood back into
the body.
EXTRACORPOREAL MEMBRANE OXYGENATION
(ECMO)
 MEDICAL MANAGEMENT
• Fluid management through an intravenous (IV) line helps
restore fluid levels if needed. Low fluid levels in your blood
vessels can prevent oxygen from getting to your organs. If you
have too much fluid in the lungs, your doctor may give you
medicines that help your body get rid of the extra fluid.
• Lying facedown helps get more oxygen to your lungs.
• Physical therapy maintains muscle strength and prevent sores
from forming. Movement may help shorten the time you are on a
ventilator and improve recovery after you leave the hospital.
 TREATMENT
Treating the underlying cause or injury
• Providing support until the lungs heal:
• Mechanical ventilation (a breathing machine) through a tube placed in
the mouth or nose, or through an opening created in the neck
• Monitoring blood chemistry and fluid levels
• Often, ARDS patients are sedated to tolerate these treatments.
• Noninvasive ventilation, such as use of bilevel positive airway pressure
(BiPAP) or continuous positive airway pressure (CPAP) machines, which are
electronic breathing devices that help keep your airways open by blowing air
through a face mask.
 HOME INSTRUCTIONS
Your healthcare team can support your recovery or suggest additional resources,
including recommending that you:
• Keep regular appointments so that your doctor can monitor your recovery.
• Take all medicines regularly as prescribed. Do not change the amount of
your medicine or oxygen or skip a dose of medicine unless your doctor tells you
to.
• Work with therapists or other healthcare providers to gain strength and
speed your recovery.
• Between visits, call your doctor if you have any new symptoms or your
symptoms worsen.