Download Effects on the Respiratory System: Pulmonary Fibrosis

Effects on the Respiratory System:
Pulmonary Fibrosis
Effects on the Respiratory System:
Pulmonary Fibrosis and Non-cardiogenic Pulmonary Edema
Author: Ayda G. Nambayan, DSN, RN, St. Jude Children’s Research Hospital
Content Reviewed by: Cindy Burleson, RN, MSN, MS, CPON, St. Jude Children’s Research
Hospital
Cure4Kids Release Date: 6 June 2006
Pulmonary damage associated with treatments includes pulmonary fibrosis, hypersensitivity lung
disease and non-cardiogenic pulmonary edema (NCPE) also referred to acute respiratory
distress syndrome [ARDS]). Although these complications have similar presenting symptoms,
they differ in terms of time-relation to cancer treatment and long-term outcomes and are
associated with differing radiographic findings and types of pulmonary damage. Often,
pulmonary complications present as a nonspecific cough along with progressive dyspnea and
low-grade fever.
The most common mechanisms by which specific drugs can -induce pulmonary complications
include the following.
1.
direct damage to the alveoli
2.
immunologic respiratory response
3.
metabolic damage by the chemotherapy metabolite such as acrolein
4.
antitumor actions of the drug
Pulmonary Fibrosis
The most common chemotherapy-associated lung injury is drug-induced pneumonitis, associated
with most pulmonotoxic antineoplastic agents such as bleomycin and nitrosureas. The major
concern regarding pneumonitis is the potential of progression to irreversible pulmonary fibrosis.
Pulmonary fibrosis (A – 1) is the formation of fibrous scar tissue in the lungs as a consequence
of inflammation or injury or both. Subsequently, fibrosis progresses to sclerosis of pulmonary
vessels and bronchi, leading to bronchiectasis. Patients at risk of pulmonary fibrosis are those
who have received high doses of radiation therapy to the lungs and those who have received
chemotherapy consisting of drugs such as busulfan, methotrexate, melphalan,
cyclophosphamide, mitomycin, carmustine and bleomycin.
Assessment
In children and adolescents, the most common symptoms are restlessness, anxiety and a
progressive cough. Signs and symptoms of pulmonary fibrosis are the same as those of
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Effects on the Respiratory System:
Pulmonary Fibrosis
respiratory distress: tachypnea, dyspnea, cough, compromised oxygenation levels, increased
respiratory effort and chest pain.
Pulmonary symptoms usually occur 7 to 10 days after administration; symptoms may also have a
delayed presentation of up to 10 years. Delayed presentation is usually due to irreversible lung
tissue damage.
Children and adolescents with respiratory fibrosis should be monitored for signs and symptoms
of congestive heart disease and respiratory distress. Physical assessment of patients with such
conditions may detect rapid weight changes, peripheral edema, skin mottling, cyanosis, use of
respiratory muscles to breath and easy fatigability. In severe cases, muscle wasting may be
evident.
Physical assessments may also reveal the use of accessory muscles of breathing, shallow
respirations, decreased breath sounds, possible asymmetry of chest expansion and the presence
of adventitious breath sounds.
The risk of developing pulmonary damage is increased by cumulative doses of chemotherapy
(especially of bleomycin); the presence of comorbidities such as respiratory syncytial virus
(RSV) infections, asthma, and other factors such as age and premature birth.. Therefore, the
nurse’s assessment should identify any of these factors, if present.
Laboratory diagnostic procedures such as pulmonary function tests and radiologic examinations
may also be done. Pulse oximetry may show decreased oxygenation levels.
Planning
A plan of care should result in the following.
 Maintaining adequate oxygenation and respiratory function
 An understanding by the patient and family of pulmonary fibrosis and the
chronic-care requirements
 An understanding by the patient and family of the need for long-term follow-up
 Compliance with the recommended therap y
Implementation
The goal of care should be maintenance of adequate oxygenation levels and prevention and early
recognition of cardiopulmonary complications such as congestive heart failure. The patient with
pulmonary fibrosis is often given oxygen therapy and corticosteroids. In addition, the nurse can
implement supportive care designed to help alleviate the work of breathing such as raising the
head of the bed, providing small frequent meals and foods that are easy to chew and digest.
Supportive medical care such as administration of diuretics and vasopressors is designed to
relieve pulmonary congestion; artificial ventilation may be used, if necessary.
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Effects on the Respiratory System:
Pulmonary Fibrosis
The nursing care plan should also include monitoring fluid intake and output, measuring daily
weight and teaching the patient and family alternative activities that will not compromise the
cardiopulmonary status of the patient. Play and other activities may be modified to better meet
the patient’s tolerance level.
Evaluation
The desired outcomes for the patient with pulmonary fibrosis include adaptation and successful,
continued management of an added chronic condition.
Patient and Family Education
The patient and family education should be focused on the following.
Activity levels
-
play that does not require a great expenditure of energy
diversional activities
activities patterned to meet the patient’s ability to tolerate respiratory
exertion
activities that are directed to energy conservation
Prevention of complications such as respiratory infections and burns from O2 combustion
- avoidance of crowds and people with infections
- hand washing and general cleanliness
- oxygen safety (prevention of oxygen combustion)
Monitoring of the patient’s condition and early recognition of complications
- signs and symptoms of increased respiratory effort
- signs and symptoms of congestive heart failure – rapid weight gain,
distal edema, increased work of breathing, decreased urinary output
- respiratory compromise (increased need for oxygen) – cyanosis,
headache, irritability, changes in mentation
- identification of the conditions in which a health care provider should
be contacted
Ways to manage or decrease dyspnea
- teaching the patient exercises that would assist with dyspnea such as
pursed-lip breathing, blowing out--candle exercises
- the importance of not smoking and/or being exposed to secondhand
smoke
- use of medications such as low-dose opioids
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Effects on the Respiratory System:
Pulmonary Fibrosis
Non-cardiogenic pulmonary edema (NCPE)
Non-cardiogenic pulmonary edema (NCPE) is a rare and less recognizable pulmonary toxicity
associated with cancer treatment. NCPE is characterized by the simultaneous presence of severe
hypoxemia, the collection of fluids in the lungs (bilateral alveolar infiltrates on chest x-rays) with
no evidence of left atrial hypertension (congestive heart failure).
NCPE is diagnosed by exclusion of other possible causes such as infections and other metabolic
and cancer-related causes. A supportive rationale for a diagnosis of NCPE is sometimes provided
by the proximity between the time at which NCPE appears and the time that the drugs known to
cause NCPE were administered. Cancer therapeutic agents often associated with NCPE (A - 2)
include the following.
cytarabine
gemcitabine
interleukin-2 (IL-2), which causes increased capillary permeability
all-trans retinoic acid (ATRA), which is used to treat acute promyelocytic
leukemia
Early signs of NCPE may include coughing and restlessness during sleep. Later, the patient may
experience trouble breathing when awake and at night. Coughing usually produces white- or
pink-tinged frothy sputum. Noisy breathing (wheezing and bubbly sounds), bluish nailbeds and
lips, sweating, and a fast heartbeat are other signs. The patient may also feel very anxious.
NCPE is reversible and can be successfully managed with high-dose corticosteroids, diuretics
and oxygen supplementation. When lung toxicity is suspected, immediate discontinuation of
chemotherapy is recommended.
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Effects on the Respiratory System:
Pulmonary Fibrosis
APPENDIX
A – 1 Radiation Fibrosis
Loyola University Medical Education Network - LUMEN
http://www.meddean.luc.edu/lumen/meded/medicine/pulmonar/cxr/atlas/radpneumonitis.htm
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Effects on the Respiratory System:
Pulmonary Fibrosis
A – 2 Cancer Therapeutic Agents Associated with the Development of Non-cardiogenic
Pulmonary Edema
Incidence Comments/Characteristics
Therapy or Medication
Cytarabine - moderate to high doses (1-1.5
2
g/m given by continuous infusion, or >3
g/m2 as 2-h IV infusion per 12 h) over 3 to
4 days
High
Potentially fatal toxicity observed in patients with
leukemia. It develops 1 to 2 weeks after chemotherapy
(usually at the initial course). Pathophysiology:
increased permeability of alveolar capillaries
Recombinant IL-2 (high-doseIV) given to
patients with renal cancer or melanoma
3% - 20%
Bone marrow transplantation
Moderate
All-trans-retinoic acid (ATRA)
12%
Usually described in the context of generalized
vascular leak syndrome. Severe but reversible on
discontinuation of IL-2. Pathophysiology: a damaging
effect on vascular endothelial cells by cytokines
Bone marrow transplant recipients may develop NCPE
in the context of systemic capillary leak syndrome 1 to
2 months after high-dose chemotherapy. A pivotal
contribution by circulating leukocytes has been
suggested.
NCPE occurs in the context of ATRA syndrome,
which may develop in patients with acute
promyelocytic leukemia who are undergoing remission
induction treatment consisting of ATRA. The
syndrome is characterized by fever, respiratory
distress, peripheral edema and pleural or pericardial
effusions. Treatment with corticosteroids, if started
early, is effective.
Arsenic trioxide (As203 )
15%
Same as ATRA
Gemcitabine
0.1%
Gemcitabine plus docetaxel
rare
Mitomycin plus vinblastine
2%
Intrathecal methotrexate
Occasional
NCPE usually occurs after several courses, but it may
also occur after a single course. It is life-threatening
but also reversible upon treatment discontinuation and
the start of intensive supportive therapy and IV
corticosteroid therapy.
In patients with solid tumors. Similar clinical features
and outcome as those seen with gemcitabine alone.
NCPE tends to occur shortly after administration of
this combination.
Three cases of rapidly developing respiratory distress
that followed the administration of methotrexate into
the cerebrospinal fluid have been reported.
G – CSF
(Granulocyte
Colony
Stimulating
Factor
Occasional
Evangelos Briasoulis, Nicholas Pavlidis Noncardiogenic Pulmonary Edema: An Unusual and Serious Complication of
Anticancer Therapy. The Oncologist, Vol. 6, No. 2, 153-161, April 2001
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Effects on the Respiratory System:
Pulmonary Fibrosis
Acknowledgments
Author: Ayda G. Nambayan, DSN, RN, St. Jude Children’s Research Hospital
Content Reviewed by: Cindy Burleson, RN, MSN, MS, CPON, St. Jude Children’s Research
Hospital
Edited by: Julia Cay Jones, PhD, ELS, Freelance Biomedical Editor, Memphis, TN
Cure4Kids Release Date: 6 June 2006
Cure4Kids.org
International Outreach Program
St. Jude Children's Research Hospital
332 N. Lauderdale St.
Memphis, TN 38105-2794
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Last printed 6/5/2008 10:23 AM
Last Updated: 3 July 2006; AS
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