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Case Study 1: Cystic Fibrosis with Cystic Fibrosis Related Diabetes

Table of Contents

Introduction

Case Study 1: Cystic Fibrosis with Cystic Fibrosis Related Diabetes

Identification of disease

Pathophysiology

Symptoms

Testing and Treatment

Potential Complications

Psychosocial aspects

Priorities of nursing management

Conclusion

References

Introduction

Cystic Fibrosis is an autosomal recessive genetic disease that affects the exocrine glands – primarily the respiratory and digestive systems. It is congenital, and is usually diagnosed soon after birth. The disease is more common in persons of European descent. About one fourth of Caucasians are carriers of the recessive gene that causes the condition, but fortunately, the number of patients that actually suffer from cystic fibrosis is quite low – about 6% of the world population. ("What is Cystic Fibrosis-related Diabetes?", 2020).

Cystic fibrosis related diabetes is a type 1 diabetes caused due to the scarring and other issues of cystic fibrosis. It causes an extreme deterioration of the life quality of the patient and is the most common cause of death for cystic fibrosis affected people. It occurs in about 20% of the adolescents and more than 40 percent adults. ("What is Cystic Fibrosis-related Diabetes?", 2020).

Children suffering from cystic fibrosis and related conditions have a decreased quality of life and development. There are also the factors of lack of socialisation and a social stigma of disease involved.

This report aims to study the case of Sam, a six year old with cystic fibrosis, and recently diagnosed with cystic fibrosis related diabetes.

Case Study 1: Cystic Fibrosis with Cystic Fibrosis Related Diabetes

Identification of Disease

The patient, 6 year old Adam, has a chronic condition of cystic fibrosis, leading to him continually getting respiratory infections. He has also recently been diagnosed with Cystic fibrosis-related diabetes (CFRD).

He is on recovery from a recent respiratory infection, after a two week period of hospitalisation, and is facing increased fatigue. The presenting condittions are the CFRD and fatigue, which is affecting his quality of life.

Pathophysiology

Adam’s cystic fibrosis is an autosomal recessive condition. It is caused by a mutated CFTR gene. The defective cystic fibrosis transmembrane conductance regulator protein leads to a glitch in the chloride channels of the cell membranes, particularly in exocrine tissue. These exocrine glands – such as mucus glands, lacrimal glands, sweat glands, salivary glands, bile glands in the liver, gastric glands and others in the body – produce an abnormally thick, viscous secretions. So, instead of being protective secretions that move easily and lubricate tissues, they accumulate and block different pathways in the body ("5 Cystic Fibrosis Nursing Care Plans - Nurseslabs", 2020).

The patients have a distinct predisposition to respiratory infections due to the fluid accumulation in the bronchi and clogged cilia, and are very likely to have long term respiratory complications, like emphysema. ("Cystic fibrosis – Knowledge for medical students and physicians", 2020). The severe mucociliary blockages and the difference of ion transport for the chloride and sodium together make the respiratory tract favourable for the growth of bacterial colonies, and cause repeated inflammation of the respiratory tract. The most common infections are caused due to these bacterial colonies, namely Pseudomonas, Haemophilus influenza, and Staphylococcus aureus.

The problems due to accumulation and inefficiency of the bile and pancreatic juices often cause several digestive problems, and is the chief reason for the development of Cystic Fibrosis-related Diabetes.

Symptoms

Cystic fibrosis is usually diagnosed soon after birth, but some infants and children may present less symptoms and remain undiagnosed for months and years. The patterns and severity vary from patient to patient.

The first observed symptom of CF is usually the inability to pass meconium ileus in neonates. In children, CF is often characterised by chronic, persistent coughing with phlegm, gastrointestinal absorption problems, poor growth and frequent loss of weight. The skin is likely to be salty due to sweat problems.

The respiratory tract is one of the most affected systems of the body in cases of CF. The repeated lung infections, bronchiectasis, haemoptysis, nasal polyps, continual wheezing and shortness of breath due to fluid accumulation lead to more and more severe health conditions.

CF also affects the reproductive system, with a marked decrease in the fertility, or even sterility. Males may have undescended testicles and a congenital absence of the vas deferens on both sides.

CFRD is often asymptomatic, especially in children. Also, some of the symptoms for diabetes – such as the fatigue, weight losses and a decline in respiratory function – are the same as the CF symptoms, so the CFRD is often undetected without a specific test for diabetes.

Type 1 diabetes mellitus is characterised by hyperglycemia, and a related increase in thirst and micturition.

Testing and Treatment

Cystic fibrosis and its related complication are mainly treated by treating and managing the symptoms. It is a genetic disease, and so far, there is no cure for it. Some aspects of nanotechnology and biotechnological medicine show promise, but they are still a long way to go.

With proper care, a patient of cystic fibrosis has a life expectancy of about forty years. Complications in the respiratory system, cystic fibrosis related diabetes and gastrointestinal problems are the greatest causes for the death of cystic fibrosis patients, statistically.

The sweat test (pilocarpine iontophoresis) is considered the best test for cystic fibrosis. It tests the level of chloride in sweat.

Cystic fibrosis related diabetes is rarely visible in the list of experienced symptoms, so regular screening for diabetes are recommended.

While most diabetes patients fear the blood pressure aspects of the disease more, patients with cystic fibrosis must pay more attention to their respiratory and nutrition. ("Preschool-Aged Care Clinical Care Guidelines", 2020)

Sam is must undergo annual screening to check his CFRD levels and conditions. Since he already has CFRD, his nutrition and development require close monitoring. His fatigue needs to be controlled by proper nutrition, and if possible, medication.

Since he already faces significant serious respiratory problems, he may often require treatment with glucocorticoids. In such a situation, he must be monitored closely and his blood sugar levels must be checked regularly for any fluctuations.

When he is stable, and relatively healthy, he should have routine checkups. The diagnostic tests should span two separate days, so that laboratory errors can be ruled out.

The FPG and A1C tests can be used to confirm and test his blood sugar levels. An OGTT must also be performed. (Edward DeSimone, 2020)

The baseline statistics for a cystic fibrosis patient are as follows:

  • 2 hour OGTT plasma glucose less than or close to 200 mg/dl (11.1 mmol/l)
  • FPG less than or close to 126 mg/dl (7.0 mmol/l)
  • A1C less than or close to 6.5%
  • Casual glucose level around 200 mg/dl (11.1 mmol/l)

The patient must also continue the necessary physiotherapy and medications to regain strength and build up his immunity. Although unpleasant for him, the procedure is extremely beneficial for him.

Potential Complications

1. Diabetes mellitus: This greatly complicates the nutritional aspects of the CF patient. Also, diabetes increases the chances of a respiratory infections, which are greatly complex for all CF patients.

2. Respiratory Infections: They require great care and monitoring in all CF patients. They may get complicated very soon, therefore carers must be especially vigilant.

Psychosocial Aspects

Cystic fibrosis is a genetic disorder that severity affects all the organ of the body and greatly impacts the quality of life. The current median rate of survival is 37.4 years, and the largest number of them die of respiratory and lung infections.

Cystic fibrosis is chronic and steady in its progress to more and more systems of the body. From the initial fatigue and shortness of breath, cystic fibrosis patients move to having severely scarred and infected organs, and later, organ and system failures.

As such, this is not a disease that allows much in the way of a good quality of life, especially not for a six- year old child who already faces such severe and frequent respiratory infections requiring hospital care and has already developed diabetes (Ernst, et al. 2010).

Continued physiotherapy, nebulization and frequent checkups to remove accumulated fluids can help Sam feel better and healthier. His diet must be carefully controlled, considering both his digestive problems, gastrointestinal malabsorption and diabetes problems ("Cystic Fibrosis", 2020). Sam is a pre-school student. He is physically able to interact and play with his friends, and have a social life, even if limited. His parents and careers must continue to encourage him to keep that.

His parents and caregivers also need to keep a close eye on him for other psychological problems, like depression, which are very likely to happen in a child in his situation. His present unwillingness to interact with his friends may also stem from the aforementioned issue.

Priorities of Nursing Management

1. Adequate clinical knowledge:

A lack of adequate knowledge on the part of carers, especially in the case of congenital diseases like cystic fibrosis, where the patient themselves is often a baby or a child, is important. Nurses need to explain the problem and care instructions very clearly and patiently to the carers. It must be remembered that most of them are in a state of emotional tension, so they may need patience and understanding on the part of the professional.

2. Oxygenation:

An imbalance in the oxygenation or deoxygenation of blood, where one is more or less than required. It may be a symptom of a physical block in the airways, inflammation in the alveoli and respiratory tract, or infection. In this situation, the nurse must monitor the oxygenation and deoxygenating rates of the blood, look and assess the patient for other symptoms of gas exchange problems, and continue the examination for other complications. A full checkup must be done if this is evidenced for the first time, then the patient must be closely monitored.

3. Blocked airways:

The symptoms for blocked or improperly clean airways are the same as the symptoms for gas exchange problems. They may even be interlinked. The nursing procedure for if the nurse suspects a blocked airway is the same as the above.

Conclusion

Sam’s case of fatigue and unwillingness to go to prepare likely interconnected, and characteristic of any patient who is recovering from infections. There is, however, a chance of him being depressed due to the conditions pf his life. Sam’s physio, and nutrition must be taken care of properly. His medications must be properly administered routinely, and he must be encouraged to interact more with his peers. His diabetes requires daily monitoring and insulin administration. His parents must learn it well, and research more on the topic. The more knowledgeable they are, the better care they will be able to take of their child.

References

5 Cystic Fibrosis Nursing Care Plans - Nurseslabs. Nurseslabs. (2020). Retrieved 6 May 2020, from https://nurseslabs.com/cystic-fibrosis-nursing-care-plans/.

Brown, Sheena D., White, Rachel Tobin, Phil, Keep them breathing, Journal of the American Academy of PAs: May 2017 - Volume 30 - Issue 5 - p 23-27 doi: 10.1097/01.JAA.0000515540.36581.92

Cystic fibrosis – Knowledge for medical students and physicians. Amboss.com. (2020). Retrieved 6 May 2020, from https://www.amboss.com/us/knowledge/Cystic_fibrosis.

Cystic Fibrosis. Physiopedia. (2020). Retrieved 6 May 2020, from https://www.physio-pedia.com/Cystic_Fibrosis.

Edward DeSimone, N. (2020). Cystic Fibrosis: Update on Treatment Guidelines and New Recommendations. Uspharmacist.com. Retrieved 6 May 2020, from https://www.uspharmacist.com/article/cystic-fibrosis-update-on-treatment-guidelines-and-new-recommendations.

Ernst, M., Johnson, M., & Stark, L. (2010). Developmental and Psychosocial Issues in Cystic Fibrosis. Child And Adolescent Psychiatric Clinics Of North America, 19(2), 263-283. doi: 10.1016/j.chc.2010.01.004

Preschool-Aged Care Clinical Care Guidelines. Cff.org. (2020). Retrieved 6 May 2020, from https://www.cff.org/Care/Clinical-Care-Guidelines/Age-Specific-Clinical-Care-Guidelines/Preschool-Aged-Care-Clinical-Care-Guidelines/.

What is Cystic Fibrosis-related Diabetes? Beyond Type 1. (2020). Retrieved 6 May 2020, from https://beyondtype1.org/what-is-cystic-fibrosis-related-diabetes/.

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