NURSING CARE for the PATIENT with CF

What NURSES can do for PATIENTS!

Overall Assessments and Possible Findings:

System	Assessment	Clinical Manifestations
Respiratory	Respiratory rate, rhythm, and effort Use of accessory muscles Pulse oximetry Presence of cough Presence of sputum – color, consistency, and amount Breath sounds	Fatigue Chronic cough Thick, yellow-grey mucous Positive sputum culture (e.g., Pseudomonas aeruginosa, Haemophilus influenzae) Fever Shortness of breath, dyspnea, and wheezing Cyanosis Difficulty exhaling air, resulting in hyperinflation of the lungs Barrel-shaped chest Clubbing of the fingers and toes
Gastrointestinal	I&O Weight Stool Bowel sounds Palpation for tenderness	Large, loose, fatty, sticky, foul- smelling stools Impaired digestion Failure to gain weight Delayed growth patterns Distended abdomen Thin arms and legs Atrophy of buttocks and thighs
Integumentary	• Color • Nails • Peripheral pulses	Positive sweat chloride test Sweat, tears, and saliva abnormally salty Report of salty taste when kissing the child
Growth and Reproduction	Height and weight Development of secondary sex characteristics	Delayed puberty Viscous cervical mucous Decreased or absent sperm count

Taken from NCLEX review website: 

http://www.atitesting.com/ati_next_gen/FocusedReview/data/datacontext/RM%20NCC%20PN%207.1%20Chp%2019.pdf

Interventions again:

Nursing Care of Children

• ∆  Respiratory Interventions
  
  • Promptly treat respiratory infections with antibiotic therapy.
    
    
  • Provide pulmonary hygiene with chest physiotherapy (CPT)
    
    

    -breathing exercises to strengthen thoracic muscles

    
    

    - minimum of twice a day (in the morning and at bedtime

    
    
    
    
  • Have the child use the Flutter mucus clearance device to assist with mucus removal.
    
    
  • Administer bronchodilators through metered dose inhalers (MDIs) or hand-held nebulizer to promote expectoration of excretions.
    
    
  • Administer dornase alfa (Pulmozyme) through a nebulizer to decrease viscosity of mucus.
    
    VERY IMPORTANT:
    *Promote physical activity that the child enjoys to improve mental well-being, self-esteem, and mucus secretion
    
    
• ∆  Gastrointestinal Interventions
  
  
•            give pancreatic enzymes with meals and snacks
•            capsules can be:
• swallowed whole  
• opened to sprinkle the contents on a small amount of food.

MEAL TIME

• ◊  Encourage the child to select meals and snacks if appropriate.
  
• ◊  Facilitate high-caloric, high-protein intake through meals and snacks.
  
• ◊  Multiple vitamins and water-soluble forms of vitamins A, D, E, and K are often prescribed.
  
  
  Hospitalization

• Perform CPT 1 hr before meals or 2 hr after meals if possible.
  
• Use oxygen with caution to prevent oxygen narcosis.
  
• Promote adequate nutritional intake, and provide pancreatic enzymes at meals and with snacks.
  
• Encourage adequate fluid and salt intake.
  
• Provide meticulous skin care and oral hygiene.
  
• Provide encouragement and support to the child/family by using family-centered nursing care.
  
  Care in the Home
  

• Ensure parents/caregivers have information regarding access to medical equipment.
  
• Provide teaching about equipment prior to discharge.
  
• Instruct parents/caregivers in ways to provide CPT and breathing exercises.
  
  For example, a child can “stand on her head” by using a large, cushioned chair placed against a wall.
  
  
• Administer antibiotics through a venous access port.
  Parents/caregivers need instruction in administration techniques, side effects to observe for, and how to manage difficulties with the venous access port.
  
  
• Promote regular primary care provider visits.
  
• Ensure up-to-date immunizations with the addition of initial influenza vaccine at 6 months of age and then a yearly booster.
  
• Encourage regular physical activity.
  
• Encourage participation in a support group(s) and involvement in community resources.

Primary Reference:
Hockenberry, M., Wilson, D., Winkelstein, M. (2005). Wong’s essentials of pediatric nursing care. (7th ed.). St. Louis, MO: Mosby. 


AND HAVE FUN!

OTHER NURSING CARE RESOURCES:  ie: for student nurses

• http://www.istudentnurse.com/peds/cf/
• http://www.elsevier.com/__data/promis_misc/2005.pdf
• http://www.nursingtimes.net/the-role-of-the-cystic-fibrosis-nurse-specialist/206445.article
• http://depts.washington.edu/pedspulm/cfcenter.htm

NURSING INTERVENTIONS

For Nursing Students and Nurses caring for the patient with Cystic Fibrosis...


Diagnoses:

1. Ineffective airway clearance, related to accumulation of mucus.
2. Imbalanced nutrition, less than body requirements, related to malabsorption from ineffective production of pancreatic enzymes
3. Deficient knowledge, related to the diagnosis and condition of child.
4. Risk of infection, related to bacterial invasion of respiratory system.
5. Ineffective coping related to new diagnosis and management of disease.


More specific diagnosis with what you can do as a nurse to help!

Diagnosis:  
                    Ineffective airway clearance, related to accumulation of mucus.

PLAN/ OUTCOME

NURSING INTERVENTION

-absence of dyspnea and tachypnea (breathing improved to normal range based on age) -can effectively remove mucus from lungs through coughing -improved heart rate for age -maintain oxygen saturation above 93%

ASSESSMENTS: -lung sounds -breathing rate and rhythm -heart rate -oxygen saturation THERAPY/TREATMENT: -assist with chest physiotherapy ·      Chest percussion and drainage ·      Apply external percussion vest ·      Use of airway oscillating device -administer medications per orders ·      Bronchodilators ·      Nebulizers ·      Antibiotics ·      Anti-inflammatories -provide oxygen as needed based on saturations EDUCATION: -breathing exercises -medications MONITOR AND REASSESS: -effectiveness of treatment

References:

Price, D., & Gwin, J. (2008). Respiratory Disorders. In Nurse's Pocket Guide: Diagnoses, Prioritized Interventions, and Rationales (11th ed.). F A Davis Company.

http://www.elsevieradvantage.com/samplechapters/9781437717099/9781437717099.pdf

How do they stay healthy anyway?

PREVENTATIVE THERAPIES:

   -vaccinations: influenza and pneumococcal: reduce viral infections
   -unfortunately... set limitations for proximity to other CF patients reduces exchange of infection



TREATMENT RECOMMENDATIONS:

1. CHEST PHYSIOTHERAPY:

COUGH IT OUT!

there are different methods of shaking up the chest walls to break free that THICK, STICKY MUCUS from the chest walls

-Chest percussion and drainage: physical slapping on with hands on different parts of the chest

Details from Cystic Fibrosis Foundation: You can Percuss to!

-external percussion vest (does the slapping for you and makes you an incredible singer)

-airway oscillating devices

if > 6 years old...

Other therapies:

NEWEST and COOLEST fighting at the CORE of the problem! 

Ivafactor:  (a CFTR modulator) 150 mg every 12 hours by mouth
              -restores function of the mutant CF protein at the base!
              -improves chloride movement
              -reduces pulmonary exacerbations
              -improves cough clearance!  (helps COUGH IT OUT!)

  **only works on specific mutations so genotyping is needed


Nebulizers:  inhaled medications

      mucolytics: Mucus busters
      hypertonic saline: draws water into airways to thin mucus and COUGH IT OUT!
      bronchodilators: open up airways before chest physiotherapy


Antibiotic Therapies:  dependent on the type of bacterial infection, give after Chest Physiotherapy

                    *popular antibiotics for popular infections

AZITHROMYCIN: an anti-inflammatory and antibiotic 
                            -research shows chronic usage improved chronic cough and lung function!

for P. aeruginosa bacteria
      -Tobramycin or gentamycin (aminoglycides) 
      -"Cillins": penicillin, amoxicillin, Cephalosporin, Carbopenams (beta-lactams) 

Intravenous antibiotics for treating exacerbations of cystic fibrosis 

A TABLE OF ANTIBIOTICS

ANTI-INFLAMMATORY:

chronic high does ibuprofen: new research proven helpful if CF lung function > 60%

      
Other choices for more severe CF:

-supplemental oxygen
-BiPAP: to help with difficulty expanding chest walls (positive pressure ventilation)
-Lung Transplant: contraindicated for patients < 12 y.o.
                            -none for infection with Burkholderia gladioli


MULTISYSTEM TREATMENTS:

may also need to treat for CF-related:
    -nutritional deficiencies: provide enzymes, tube feeding
    -diabetes: insulin management
    -osteoporosis: exercise or calcium supplements


References:

Cystic Fibrosis Foundation - Home. (n.d.). Retrieved February 14, 2015, from http://www.cff.org

Simon, R. (2014, December 18). Cystic fibrosis: Overview of the treatment of lung disease. Retrieved February 14, 2015, from http://www.uptodate.com/contents/cystic-fibrosis-overview-of-the-treatment-of-lung-disease?source=search_result&search=cystic fibrosis treatment&selectedTitle=1~150

Seattle Children's Hospital Policies and Procedures: Cystic Fibrosis Pathway

What to look for.. Signs and Symptoms

Signs vs. Symptoms

What do these mean?

Signs: what your doctor will find via examination or lab test results
Symtoms: what the patient feels


Presentation of CF in infancy:

*20% of CF infants or children have meconium ileum: baby's first poop blocks bowel

*45% of CF infants or children have

• respiratory symptoms: cough, wheezing difficulty breathing         
• respiratory signs: hyperinflation of lung fields,  obstructive airway disease determined from pulmonary function tests (PFTs)

*28% of CF infects or children have Failure to Thrive diagnosis: failure to meet growth milestones             despite adequate nutrition

Presentation of CF in adults (only 7% of patients with CF are diagnosed > 18)

• GI symptoms: failure to properly absorb nutrients due to abnormal enzyme production from pancreas
• diabetes mellitus: pancreatic insufficiency of insulin production (needed for glucose tolerance)
• infertility: defects in sperm transport or reduced fertility in women due to malnutrition or abnormal production of cervical mucus

Presentation if Pictures and Graphs:

BODY PARTS AFFECTED BY CF:

Add caption

SYSTEMS THROUGH THE AGES:

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OVERVIEW FROM A NURSING PERSPECTIVE: what to look out for as a healthcare professional

Diagnosis: How do you know if you have CF?

AGE OF DIAGNOSIS:  75% of CF patients diagnosed by AGE 2!


NEW NEWBORN SCREENINGS!

in 2001 less than 10% of cases were diagnosed in infancy..

in 2011, 60% of cases were diagnosed in infancy.

WHAT THEY FIND.. DIAGNOSIS MUST HAVE....

●Clinical SYMPTOMS consistent with CF in ONE organ (lungs, pancreas, liver, intestines, skin)

                     

AND

●Evidence of ABNORMAL CFTR:

•Increased sweat chloride on skin ≥60 mmol/L (2 times!)

•2- disease-causing mutations in CFTR from each parent's genes

•Abnormal nasal potential difference

HOW THEY FIND IT....

First...Blood Tests:

A provider will take a small amount of blood generally by poking the heel

What they are looking for...

• CF is associated with increased levels of IRT (immunoreactive trypsinogen) 
• IRT is a protein that is produced by the pancreas

If levels are high there are two options depending on the state you live in:

•  they will repeat the blood test in 2 weeks to rule out other causes of high IRT  
• they will complete a DNA test of the blood looking for mutations in CFTR

Results from DNA test:

• positive if they find 2 mutations in CFTR (one from mom, one from dad)
• negative with only 1 or 0 mutations in CFTR

Next.. SWEAT tests:

A provider will perform a test to make a small part of the baby's skin sweat for collection.  

• Applies an odorless, non-harmful chemical that causes sweat production (called Pilocarpine) over small area on arm or leg 
• covers this area with plastic wrap to promote moisture
• removes the covering and collects the sweat with gauze or filter paper
• sample sent to lab for analysis

Results from sweat tests:

For newborns and infants younger than six months of age:

●

≤29 mmol/L: Normal (CF very unlikely)

●

30 to 59 mmol/L: Intermediate (Possible CF)

●

≥60 mmol/L: Abnormal (Diagnosis of CF)

For infants ≥6 months, children, and adults:

●

≤39 mmol/L: Normal (CF very unlikely)

●

40 to 59 mmol/L: Intermediate (Possible CF)

●

≥60 mmol/L: Abnormal (Diagnosis of CF)

ONE MINUTE CLIP OF SWEAT TEST !

Generally this information can be useful in diagnosis but sometimes a 3rd test may be performed..

Third.. Nasal Potential Difference Test (NFD)

• a non-harmful, non-painful electrode is placed in the nose and a voltage is obtained before and after different chemicals are applied to determine the difference in chloride movement
• determining the movement of chloride is a way to check on the CFTR gene since it codes for this protein channel transporter of chloride

SYMPTOMS of CF in NEWBORNS: (will require testing)

• 20% of CF patients had Meconium Ileus at birth
• however...80-90% of newborns with meconium ileum have CF
• meconium: infant poop, blocks the digestive system
• 45% of CF patients had respiratory symptoms at birth
• 28% of CF patients were diagnosed as Failure to Thrive                                                                           (not meeting growth and development milestones despite proper nutrition)

LATE ONSET OF CF: adults typically present with gastrointestinal symptoms, diabetes mellitus, and infertility


References:
Katkin, J. (2014, June 25). Cystic fibrosis: Clinical manifestations and diagnosis. Retrieved January 30, 2015, from http://www.uptodate.com/contents/cystic-fibrosis-clinical-manifestations-and-diagnosis?source=search_result&search=cystic fibrosis diagnosis&selectedTitle=1~150

Pathophysiology a.k.a WHAT EXACTLY IS GOING ON HERE?!

Here is a very nicely put overview of the progression of the disease before we get into specifics..

https://www.youtube.com/watch?v=tWWpPAXFEFs


Another summary of what she is talking about.. Pathophysiology.

First...

CF begins with a gene mutation

1. A GENE MUTATION: a mistake in the DNA leading to a change in some protein
• the gene: CFTR gene: cystic fibrosis transmembrane conductance regulator protein
• What does CFTR do?
• this protein acts as a tunnel or channel which transports Na+ and Cl- across cell membranes
• proper transport of Na+ and Cl- is necessary for movement of water into and out of cells
• this channel is located in several areas of the body affecting the lungs, pancreas, liver, intestines and skin
• there are 1900 different ways this gene can change that causes CF
2. THE PROBLEM
• without proper movement of sodium, chloride and water SECRETIONS or MUCUS becomes VERY THICK and STICKY 
• this mucus is stuff that sometimes comes out of your nose when you cough or sneeze
• because it is very thick, it is difficult to push out of your lungs
• SEE LUNGS with CF in action!

Further complications:

1. Bacteria LOVE thick, sticky mucus and get trapped in CF lungs causing infection
2. LUNGS: Airways can be blocked by sticky mucus making it difficult to breathe!



Image from WebMD

3. Other passageways can be BLOCKED!

• PANCREAS: 
• provides enzymes to breakdown food and insulin to help regulate sugar in the blood
• BLOCKED pancreas: results in diabetes and problems digesting food
• LIVER: provides bile to breakdown food
• BLOCKED liver: problems in digesting food
• INTESTINES: help absorb nutrients from food
• BLOCKED intestines: do not get enough nutrients, intestines can become distended or obstructed (looks like a big belly!)

4. Sweating!

• when we sweat, water and Na and Cl are brought to the surface of their skin
• this Na and Cl remains on the skin and are not reabsorbed in a typical CF patient
• skin can taste SALTY!
• its important to reduce the amount of sweat produced and keep salt in the body by drinking fluids with electrolytes, like Pedialyte or Gatorade!

Due to the progression of the disease, people with CF:

• must EAT LOTS OF FOOD but gain very little weight
• must get treatments to break up the mucus in their lungs since they cannot cough it up themselves
• often get DIABETES and manage by monitoring blood sugars and taking insulin

References:

Katkin, J. (2014, September 2). Cystic fibrosis: Genetics and pathogenesis. Retrieved January 30, 2015, from http://www.uptodate.com/contents/cystic-fibrosis-genetics-and-pathogenesis?source=search_result&search=pathophysiology Cystic Fibrosis&selectedTitle=1~150#H14

CFTR gene. (2015, January 27). Retrieved January 30, 2015, from http://ghr.nlm.nih.gov/gene/CFTR

Epidemiology a.k.a. Who, What, Where and Why of Cystic Fibrosis!

What we learned last time:

• CF research and treatments have led to an increase in survival rates of those diagnosed early on..  = LONGER LIVES!
• Currently there are about 30,000 people living with CF in the US
• CF is genetic and affects families of every race.. but it can affect some more than others
• caucasians/northern europeans: 1 in 2,500 
• african americans: 1 in 15,100
• hispanic/latino: 1 in 13,500
• asian american/native hawaiian/pacific islanders: 1 in 31,000

**Statistics courtesy of the American Lung Association.

What we will learn today:

Although we know CF is genetic and passed on from parent to child, the severity of each patient's illness differs.  We know there are other NON-GENETIC FACTORS that can INCREASE the SEVERITY of the disease.. 

Some studies showed that CF patient's with low SOCIOECONOMIC STATUS, or income low enough to qualify for the government health insurance, MEDICAID, had significantly worsened lung function, more frequent outpatient visits and almost double the amount of hospitalizations for worsened lung functioning. Although this is a relationship seen from National Registries, it does not discuss why lower SES would influence lung function in CF patients.. so they looked further.

With a lower SES, CF patients may have experienced some of the things listed below which could increase the risk of worsening symptoms.

• Exposure to tobacco smoke
• Increased exposure to other infectious agents, viruses, bacteria affecting airways
• STRESS... when kids are sick, parents are stressed, families suffer, kids get sicker
• PoorER nutrition, increased variety of healthy foods can improve immune system!
• Lack of health care system resources, how far away is the clinic?  Is the primary caregiver educated enough to follow a rigorous medication program?  How similar is their access to clinical trials or new drugs that may improve lung function?

Diagram courtesy of Schlechter via PubMed

These questions are just a few that epidemiologists look at to understand the CF population and differences in individual signs and symptoms.

References:

 Schechter MS. Non-genetic influences on cystic fibrosis lung disease: the role of sociodemographic characteristics, environmental exposures, and healthcare interventions. Semin Respir Crit Care Med. 2003;24:639–652. 

n.d.). Retrieved January 26, 2015, from http://www.lung.org/assets/documents/publications/solddc-chapters/cf.pdf