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Smith k pulmonary firbosis ppt
1.
2. 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)
3. X-ray of fibrotic lung evidencing excessive amount of
fibrotic tissue
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4. 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)
5. 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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6. 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)
7. 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)
8. 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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9. 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)
10. 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)
11. 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)
12. 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
6. “Simplification” growth
of lobules-devoid of
alveoli, consist only of
terminal airways and
dilate over time
7. Honeycomb
lung
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13. 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)
14. 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)
15. 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)
16. 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)
17. 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)
18. 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)
19. Patient Education
Goal: Maintain maximal level of wellness and quality of life
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)
20. 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
21. 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)
22. 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.
23. References
Bhatti, 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
24. 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.