Alpha-1 Antitrypsin Deficiency

Posted on: 16th May 2023

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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