Child Adolescent and Family Nursing Assignment 2022

Running head: CHILD, ADOLESCENT AND FAMILY NURSING
CHILD, ADOLESCENT AND FAMILY NURSING
Name of the student
Name of the university
Author’s name

1CHILD, ADOLESCENT AND FAMILY NURSING
Introduction
Cystic fibrosis is a genetic condition characterised by the presence of a faulty protein
affecting the cells and tissues of the body and the glands that makes up mucus and sweat. People
affected by cystic fibrosis have thick and sticky mucus that can lead to blockages and damage
and even cause infection in the affected area. Inflammation can cause damage to organs like
pancreas and lungs. Since 1990s it has been a great challenge to manage the children with cystic
fibrosis (O’Riordan, Dattani, & Hindmarsh, 2010). However, it has come to a long way through
the advancements in both medical and nutritional care and increased the life expectancy among
children with cystic fibrosis. It has been that cystic fibrosis affects many parts and system of the
body. Complications arising from cystic fibrosis depends on the organ that is affected and the
severity of the disease(O’Riordan, Dattani, & Hindmarsh, 2010). People suffering from this
disease produces a thick and sticky mucous that causes problem in the lungs and in the digestive
system contributing to the development of serious complications(Nhlbi.nih.gov, 2019). When
mucus is build up in the lugs it promotes the growth of bacteria leading to serious lung infections
and diseases. People with cystic fibrosis also faces problem with nutrition because of the
impaired functionality of their pancreas(Nhlbi.nih.gov, 2019). Some of the possible
complications include allergic Broncho pulmonaryAspergillus, which is an allergic reaction
affecting lungs by the fungus Aspergillus. One of the common complication arising due to cystic
fibrosis is diabetes in children since this disease affects pancreas where insulin is
made(Nhlbi.nih.gov, 2019).
As it is depicted in the case study that the child, Joshua, was diagnosed with cystic
fibrosis during her birth develops type 1 diabetes. Cystic fibrosis can have lifelong implications

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like diabetes and other complicated disease having detrimental effect on people’s lives. It can
also lead to serious kidney problem and also cardiac diseases. It is one of the most prevalent
genetic autosomal and recessive diseases and reported to have a prevalence of 1 in 2500 live
births. Evidences have shown the prevalence of glucose intolerance in the cystic fibrosis patient
and have been reported to be rising(O’Riordan, Dattani, & Hindmarsh, 2010). The paper focuses
on the treatment plan for Joshua along with the nursing care strategies implemented for an
improved health outcome.
Discussion
Pathophysiology
In Cystic Fibrosis the abnormal chloride channel secretes thick viscous secretions that
causes obstructive damage to the exocrine pancreas with a gradual and progressive fibrosis and
fatty infiltration. An abnormal glucose homeostasis disrupts the islet architecture eventually
leading to loss of endocrine Beta, alpha and also polypeptide cells. Postmortem studies of people
suffering from cystic fibrosis have shown an increasing loss of pancreatic islets when compared
of the individuals with normal glucose intolerance. However, Beta cell dysfunction is not
observed in the patients suffering from Cystic fibrosis. Whereas, some studies have showed the
prevalence of Type 1 diabetes associated autoantibodies in the patients with cystic fibrosis.
However, some studies have suggested that both of these diseases, Cystic fibrosis as well
as diabetes type 1 are caused by a decreased insulin production and there is no difference in the
occurrence of auto-antibodies between the patients with or without Cystic fibrosis. Therefore, the
pathogenesis of cystic fibrosis is different from that of the diabetes in the cellular, organ as well

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as in the endocrine level and also in the system level of dysfunction. Cystic related diabetes is
linked with the decrease phase insulin release, where the second phase insulin remains stable and
intact (Kayani, Mohammed &Mohiaddin, 2018).
Some studies have suggested that Cystic Fibrosis related diabetes is caused by the
destruction of pancreatic islets due to fatty infiltration and fibrosis. An abnormal chloride
channel function in Cystic Fibrosis results in viscous and thick secretions that act as a barrier in
the pancreatic ducts and leads to interstitial edema, death of beta cells and ischemic damage of
the exocrine pancreas. The loss of beta cells contributes to insulin deficiency making the primary
cause of Cystic fibrosis related diabetes (Vagula, Mastro& Wessel, 2013).
Joshua has a medical history of gastroenteritis which is genetically carried throughout the
generations. The night before he was admitted to the hospital, his signs were deteriorating
progressively with time. Joshua was found vomiting in the morning with fever and diarrhea. The
nurse, Grace, commenced the treatment plan of hourly blood sugar levels for managing his
condition and immediately gave sub cutaneous insulin titrated and sips of oral replacement
therapy for six hours. Despite treatment plan, his condition seemed to be deteriorating and he
was immediately shifted to the hospital with symptoms like weak, excessive thirst, sleepy, tired,
sweaty and cold to touch. The vitals recorded were blood sugar level, 29 mmol/L and blood
ketons 2.2 mmol/L indicating a high glucose level in the blood along with a high rate of blood
ketone. The acceptable and normal range of blood ketone is less than 0.6 mmol/L, when the
ketone in the blood ranges between 1.5- 3.0 mmol/L, it indicates a high risk of developing a
condition called ketoacidosis. Ketoacidosis is developed as a result of high blood glucose level
usually more than 14.0 mmol/L. The common symptoms contributing to diagnosis of this