Alpha-1 Antitrypsin Deficiency
Question
I have attached the instructions below. Just an FYI, I am not a nurse. I am a registered respiratory therapist that is in school with RRT’s. So minor terms and “common sense” things for our field do not need to be explained or defined. Please include an abstract! It does not have to be on a separate page. Thank you!!!
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Solution
Abstract
Alpha-1 antitrypsin (AAT)
is a protein produced mainly through your liver, circulating throughout your
body. This protein helps to protect your lungs and vital body organs from
irritants and infections. Alpha-1 is an uncommon genetic (inherited) condition
in which people's blood AAT levels are abnormally low. This condition raises
your chances of getting lung and liver disorders like emphysema (affected air
sacs in the lungs) and cirrhosis (liver scarring). The lungs are not secured
because AAT levels are low, and the protein build-up harms the liver. Adults
can develop lung difficulties, while adults and children can develop liver
problems. Some of these conditions are potentially fatal. This paper outlines
the signs and symptoms, causes, epidemiologic data, diagnosis and treatment
options. Also, it describes a respiratory care plan for alpha-1 antitrypsin
deficiency.
Alpha-1 Antitrypsin Deficiency
Signs
and Symptoms
Shortness of breath is
persistent, especially when exercising. COPD is a category of lung diseases that
can cause symptoms such as Allergies that last the entire year, extreme
exhaustion, colds in the chest, breathing problems, unprecedented weight loss,
and wheezing. Liver disease affects roughly 10% of individuals with Alpha-1 and
10% of newborns with Alpha-1 (Strnad et al., 2020). Alpha-1-related liver
disorders can cause the following signs and symptoms: easy bruising, yellowing
of the skin and eyes, fluid-related welling in the legs and abdomen, and
vomiting of blood.
Mutations cause Alpha-1
antitrypsin deficiency. It is a genetic condition. Therefore, it is passed on
to children by parents. Alpha-1 is a rare species. In the United States,
Alpha-1 affects around one out of every 3,500 persons (Curjuric et al., 2018).
However, the illness is underdiagnosed and under-recognized. People with this
condition are often diagnosed as having asthma. Some diagnostic procedures
include blood tests, genetic tests, and imaging tests such as X-rays and CT
scans. Treatment options for alpha-1 antitrypsin deficiency include
augmentation therapy, lung transplant procedure and medications such as
steroids and bronchodilators that open airways, making it easier to breathe.
Other options include oxygen therapy and pulmonary rehabilitation, which
includes exercises that relieve breathing problems and improve normal
functioning.
Respiratory Care Plan
|
Assessment Data |
Diagnosis |
Outcomes |
Interventions |
Rationale |
Evaluation |
|
Wheezing Excessive cough with phlegm Dyspnoea
|
Ineffective breathing pattern related to
bronchial tube spasms as evidenced by wheezing, excessive cough with phlegm
and dyspnoea. |
The patient will demonstrate and
maintain an optimal breathing pattern within three days. |
Administer medications such as
short-acting beta-2-adrenergic agonists such as albuterol and terbutaline.
Administer inhaled corticosteroids such
as beclomethasone and budesonide. Educate the patient on therapeutic
techniques such as maintaining the head of the bed elevated. |
These medications act as
bronchodilators; hence they aid in the relaxation of the muscles that line
the airway carrying air to the lungs (Ackley et al., 2019). Corticosteroids act by reducing
inflammation of the airways carrying air to the lungs and reducing mucus
production by bronchial tubes.
To promote the maximum expansion of the
lungs hence assisting in breathing. |
After three days of interventions, the
patient demonstrated an optimal breathing pattern. |
|
Lethargy Decreased exercise capacity
|
Activity intolerance related to fatigue,
as evidenced by lethargy and decreased exercise capacity (Ackley et al.,
2019). |
The patient will engage in daily
activities without exhaustion within 48 hours. |
Reinforce limitations to activity and
exercise as they trigger fatigue.
|
This provides a therapeutic, preventive
measure in offsetting fatigue and prevents changes in the patient's
respiratory status as energy reserves are conserved. |
After 48 hours, the patient engages in
everyday activities without fatigue and exhaustion. |
|
Restlessness Apprehensiveness Tachycardia |
Anxiety related to the disease process
and a change in health status as evidenced by restlessness, apprehensiveness,
and tachycardia. |
The patient will demonstrate an
effective coping mechanism in 24 hours. The patient will verbalize a decrease in
anxiety level experience after 24 hours. |
Provide suitable comfort measures such
as a calm environment. Explain procedures to the patient in a
simple way. Encourage deep, slow breathing and
assure the client and significant others. |
A calm environment reduces the
consumption of oxygen. Helping the client understand the
treatment and procedures decreases anxiety levels (Ackley et al., 2019). It ensures prompt intervention, and the
presence of their significant others provides a sense of security to the
client. |
After 24 hours, the patient demonstrated
an effective coping mechanism. After 24 hours, the patient verbalizes a
decreased anxiety. |
References
Ackley, B. J., Ladwig, G. B., Makic, M. B. F.,
Martinez-Kratz, M., & Zanotti, M. (2019). Nursing diagnosis
handbook E-book: An evidence-based guide to planning care. Elsevier Health
Sciences.
Curjuric, I., Imboden, M., Bettschart, R.,
Caviezel, S., Dratva, J., Pons, M., ... & Probst-Hensch, N. M. (2018).
Alpha-1 antitrypsin deficiency: From the lung to the heart?. Atherosclerosis, 270,
166-172.
Strnad, P., McElvaney, N. G., & Lomas, D.
A. (2020). Alpha1-antitrypsin deficiency. New England Journal of
Medicine, 382(15), 1443-1455.
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