Smith k pulmonary firbosis ppt
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IDIOPATHIC PULMONARY FIBROSIS
(IPF)Kimberly A Smith
Liberty University Advanced Pathophysiology NURS 506
Introduction A progressive, irreversible, devastating
interstitial lung disease
Etiology unknown (duBois, Weycker, Albera, Bradford, & Costabel ,2011)
Disease of the basal and peripheral lungs that progresses centrally and toward apices of the lungs over time (Leslie, 2012)
Lungs contain excessive amount of fibrous or connective tissue
Fibrotic process causes lungs to become stiff and difficult to ventilate (McCance & Heuther, 2010)
X-ray of fibrotic lung evidencing
excessive amount of fibrotic tissue
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Incidence and Prevalence of
IPF• Incidence• No differentiation found among ethnicities• Rising• Estimated to be between 4.6 and 16.3 per
100,000• Median survival post diagnosis is 2 to 4 years
• Prevalence• More predominant in men than women (1.7:1)• Frequency increases with age• Occurs in middle aged and elderly adults (median
age at diagnosis-66 years old, range 55-75) (King, Pardo, Selman, 2011)
Assessment
Probable Causes: Exposure to inhaled harmful substances (toxic fumes, organic/inorganic dusts, smoking) (McCance & Huether, 2010)
• Signs & Symptoms• Slow progressive breathlessness, especially with
exertion• Non-productive cough• Decreased oxygen saturation with exercise• Diffuse inspiratory crackles (Leslie, 2012)
• Clubbing of fingers (King et al., 2011)
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Assessment (continued)
Diagnostics Lab studies—reveal mild non-specific
elevation of antinuclear antibodies Pulmonary Function Test
Decreased lung capacity Decreased forced vital capacity Diffusing capacity for CO2
Arterial Blood Gas Decreased oxygen (pO2) levels Increased carbon dioxide (pCO2) levels (Leslie, 2012)
Assessment (continued)
Chest X-Ray Will demonstrate fibrotic patches Computed Tomography more definitive
High Resolution Computed Axial Tomography (HRCT) Patchy, coarse, subpleural reticulation Distortion of lung architecture Presence of pleural-based cysts (required
feature for a confident diagnosis) Subpleural “honeycombing” at bases (Leslie,
2012)
Assessment (continued)
Lung Biopsy Partially or completely scarred lobules devoid
of alveolar spaces Coarse peripheral lobar fibrosis Scar tissue demonstrates small cysts lined by
respiratory epithelium Fibroblast foci exist at the interface between
fibrosis and uninvolved lung tissue Microscopic “honeycombing” nearly always
present (Leslie, 2012)
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Pathophysiology of IPF
A heterogenous disease
The result of abnormal behavior of alveolar epithelial cells that: Provoke migration, proliferation, and activation of
mesenchymal cells Initiate formation of fibroblast and myofibroblast
foci
Activated fibroblasts secrete exaggerated amounts of extracellular matrix molecules
Subsequent destruction of lung architecture with alveolar collapse (King, Pardo, & Selman, 2011)
Pathophysiology (continued)
Gene expression of CCNA2 and {alpha}-Defensins up-regulated in patients with exacerbation of IPF, localized in the alveolar epithelium
{Alpha}-Defensin and ST2 protein levels in serum found to be elevated (Bhatti, Girdhar, Usman, & Abubakr, 2013)
Pathological Process of IPF & Activation of
Coagulation Cascade and Procoagulant
Signaling
Tissue factor-Factor VIIa-Factor X complex assembles on alveolar epithelium
Factor X activation stimulates fibroblasts within underlying fibrotic regions
Thrombin and activated Factor X induce differentiation of lung fibroblasts to myofibroblasts via the proteinase-activated receptor (King, Pardo, & Selman, 2011)
Proposed Pathological Sequence (Leslie, 2012)
1. Stretch injury to
epithelial-mesenchymal
transition
2. Formation of the Fibroblastic Reticulum-Type 2 cells proliferate over tear and
reconstitute the alveolar interface with air
3. Local alveolar collapse
4. Collagen deposition
5. Vascular growth
6. “Simplification” of lobules-devoid of alveoli, consist only of terminal
airways and dilate over time
7. Honeycomb lung
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Genetic/Genomic Implications for Care & Treatment
Genetics/Genomics Genetic transmission occurs in approximately 0.5-
3.7% of patients with IPF Effected families have autosomal dominant vertical
transmission patters of inheritance with reduced penetrance
In some familial cases, alterations in unfolded protein response occur with mutations in surfactant protein C– a hydrophobic protein expressed exclusively by AEC type II (King, Pardo, Selman, 2011)
Genetic/Genomic Implications for Care & Treatment (continued)
A genome wide scan of several families with familial IPF identified shared haplotype on chromosome 4g31 that harbored ELMOD2—a gene expressed in the lung ELMOD2 expressed slightly less in IPF lung when
compared to healthy lung ELMOD2 essential for cellular process
Mutations of telomerase also implicated in familial IPF (King, Pardo,& Selman, 2011)
50% of asymptomatic members have evidence of alveolar inflammation—a possible precursor to IPF (Doyle, Hunninghake, & Rosas, 2012)
Genetic/Genomic Implications for Care & Treatment (continued)
Some suggest that increased levels of matrix metalloproteinase-7 (MMP7) predict disease progression and mortality
Biomarker serum CC-chemokine ligand 18 as well as CXCL9 & CXCL10 have shown to be a predictive value in IPF
Others suggest further study of biomarkers neutrophilelastase, KL-6, and lactate dehydrogenase for disease determinant (Doyle, Hunninghake,& Rosas, 2012)
Care & Treatment of Patients with IPF
Pharmacological Corticosteroids (Methylprednisolone,
Prednisolone) Immuno-suppressants (Cyclosporin A,
Cyclophosphamide) Antifibrotic compounds (Pirfenidone—not yet
available in the United States for Rx) Efficacy unknown
Antioxidant Amino Acid/Mucolytic (Acetylcysteine) (Lee,
McLaughlin, & Collard, 2011)
Care & Treatment of Patients with IPF
Non-pharmacological Non-invasive ventilation (NIV)
High-flow oxygen for patients with resting hypoxia Continuous positive airway pressure (CPAP)
Mechanical Ventilation Once patient advances to mechanical ventilation,
probability of ventilator removal is poor, as is prognosis
<15% of patients requiring mechanical ventilation survive to hospital discharge (Lee, McLaughlin, & Collard, 2011)
Care & Treatment of Patients with IPF
Surgical – Lung Transplantation
Only therapy proven to increase long-term survival Problems:
Not all patients qualify for transplant Few hospitals have the capability for
transplantation Donor lungs not readily available (Bharri et al., 2012)
Patient Education
Disease Management Initial Teaching
Disease Pathophysiology Types of Diagnostic testing, indications Prognosis Disease- and symptom-centered management
Oxygen therapy Medications (indications, actions, possible
complications/side effects) (Lee, McLaughlin, & Collard, 2011)
Goal: Maintain maximal level of wellness and quality of life
Patient Education
Supplemental teaching Advanced Care Planning
Goal set within context of patient’s values and preferences
Initiated at a non-critical time (when death is imminent)
Palliative care/End-of-Life care Symptom control Relief of suffering (Lee, McLaughlin, & Collard, 2011)
Continual Reassessment
Patient Education
Cultural Teaching specific to language of patient Utilizing language-appropriate materials
and interpretive modalities AT&T language line Language Services Associates (LSA)
video communicator Providing care according to cultural
beliefs (Lever, 2011)
Patient Education
Spiritual Considerations Significant when dealing with advanced
planning and end-of-life care Encourage support of church family (if
affiliated with a church/religious organization) Provide pastoral care if requested Allow patient to express concerns and initiate
interdisciplinary modalities
NOTE: All education will utilize teach-back method to enhance/confirm understanding.
ReferencesBhatti, H., Girdhar, A., Usman, F., Cury, J. Bajwa, A. (2013). Approach to acute exacerbation of idiopathic pulmonary fibrosis. Annals of Thoracic Medicine, 8(2), 71-77. doi: 10.4103/1817-1737.109815
Doyle, T. Hunninghake, G., Rosas, I. (2012). Subclinical interstitial lung disease: Why you should care. American Journal of Respiratory and Critical Care Medicine, 185 (11), 1147-1153. doi: 10218100114
duBois, R., Weycker, D., Albera, C., Bradford, W., Costabel, U. (2011). Ascertainment of individual risk of mortality for patients with idiopathic pulmonary fibrosis. American Journal of Respiratory and Critical Care Medicine, 184(4), 459-466. doi: 884295098
King, T., Pardo, A., Selman, M. (2011). Idiopathic pulmonary fibrosis. The Lancet, 378(9807) , 1949-1961. doi: 910067528
Lee, J., McLaughlin, S., Collard, H. (2011). Comprehensive care of the patient with idiopathic pulmonary fibrosis. Current Opinion in Pulmonary Medicine, 17, 348-354. doi: 10.1097/MCP.ob013e328349721b
Leever, M. (2011). Cultural competence: Reflections on patient autonomy and patient good. Nursing Ethics, 18(4), 560-670. doi: 10.1177/0969733011405936
Leslie, K. (2012). Idiopathic pulmonary fibrosis may be a disease of recurrent, tractional injury to the periphery of the aging lung. Archives of Pathology & Laboratory Medicine, 136(6), 591-600. doi: 10.5858/arpa.2011-0511-OA
McCance, K., Huether, S. (2010). Pathophysiology: The biological basis for disease in adults and children (6th ed.). Maryland Hieghts, MO: Mosby.