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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 growth6. “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.