Child, Adolescent, and Family Nursing: Joshua's Cystic Fibrosis Case

This case study focuses on Joshua, a 14-year-old diagnosed with cystic fibrosis at birth, who also developed type 1 diabetes. The assignment details Joshua's medical history, including a recent episode of gastroenteritis, and presents his symptoms: vomiting, diarrhea, fever, and deteriorating condition. The study emphasizes the importance of a comprehensive nursing care plan using the APIE framework (Assess, Plan, Implement, Evaluate). It covers a detailed assessment of Joshua's vital signs, including blood sugar levels, respiratory rate, and urinalysis results, highlighting the presence of ketoacidosis and related comorbidities. The treatment plan involves rehydration, oxygen therapy, and medical nutritional therapy tailored to Joshua's specific needs, including considerations for his diabetes and cystic fibrosis. The discussion also includes the pathophysiology of cystic fibrosis and diabetes, emphasizing the need for a multidisciplinary approach to care, including the involvement of physicians, nurses, dietitians, and psychologists. The case study underscores the importance of monitoring and evaluating the patient's psychological symptoms, as well as the implementation of appropriate interventions to improve his health outcomes. Finally, the case study highlights the importance of continuous assessment and planning of care strategies based on the physical and mental health needs of the patients.

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

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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

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condition includes nausea and vomiting, abdominal pain, excessive urination, fast pulse,
drowsiness and a fruity smell from the breath is usually observed. His respiratory rate was
recorded as 28 indicating the urgency of putting him on oxygen therapy.
The urinalysis report indicates acidic and concentrated urine with a lot of dissolved
chemicals. The urinalysis report also showsthe presence of high amount of protein in the urine
that can lead to proteinuria contributing to the development of kidney diseases. The presence of
glucose in his urine indicates a condition called glycosuria that again triggers persistent kidney
problems when diabetes mellitus is not properly controlled.
The vital signs and symptoms that Joshua was diagnosed with indicate a condition of
diabetes along with ketoacidosis and the related comorbidities.
Treatment plan for Joshua
APIE plan of care- Assess, Planning, Implement and Evaluate
Nursing care plan includes a systematic approach to deliver quality care. Prior to
administration of any medication and commencing treatment nurses must first assess the
patient’s condition by recording or collecting both subjective and objective data through
thorough analysis and monitoring of the patients vitals. Following assessments, nurses must plan
the appropriate care strategies according to the health needs of the patients. While planning the
nursing care, nurses must use a holistic approach to deliver care to ensure an effective and
quality care. Nurses must implement the care plan through specific nursing interventions based
on their knowledge, expertise and clinical judgment to improve the patient’s health outcome.

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After the above stages of the problem solving approach are implemented, nurses need to evaluate
the overall improvement of the patient’s health.
Assess- While assessing Joshua’s vitals, nurses needs to collect information based on qualitative
and quantitative data which includes height, weight, body temperature, blood pressure and
pulserate as quantitative information and social background, family status, patient’s likes and
dislikes, mental state, as qualitative information. All these data and information helps in planning
the treatment strategies for an improved health outcome. Nurses must also validate the data
recorded with the patient to ensure the accuracy and appropriateness, There is a need to involve a
multidisciplinary team to care for Joshua in all aspects. Each and every vital sign indicates the
urgency of putting him on different therapies and medications to manage his condition.
Therefore, every subjective and objective data should be accessed by the nurses to help them
guide the treatment process both within the medical diagnosis as well as from the psychological,
spiritual aspects related with the illness.
To monitor his blood sugar level, there are several screening tests that can be employed
including 50g glucose test, 75 g Oral Glucose Tolerance Test, fasting plasma glucose, 60
minutes glucose level, serial capillary blood glucose monitoring, HbA1c and a continuous
glucose monitoring (CGM).
Joshua was lethargic and has no energy. He has tired, weak, clammy and sleepy. This
indicates that Joshua needs extra care to make him feel good at this vital point of time. Although,
Joshua cannot be involved in any physical activity, but, there is a need to communicate with
Joshua in order to understand his current concerns. It is likely for Joshua to feel depressed and
lonely. Evidences have shown the prevalence of depression is higher in the diabetic patients.

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Both of these conditions are driven by a common underlying biological and behavioural
mechanism including activation of hypothalamic-pituitary-adrenal axis, sleep disturbance,
inflammation, poor dietary habits and environmental risk factors. Therefore, there is a need to
monitor and evaluate the psychological symptoms and plan for care strategies in accordance with
that. It has also been found in a study that depression and anxiety are elevated in the patients
with Cystic Fibrosis associated diabetes and therefore a psychological assessment besides
physical assessments needs to be taken into account (Quittner et al., 2014).
Planning-Treatment should be planned according to the physical and mental health needs of the
patients. Nurses needs to continuously access and monitor patient’s vital signs and collect data
and plan for intervention strategies. A multidisciplinary team should be employed in the
treatment plan for Joshua in order to get an improved health outcome. The involvement of a
multidisciplinary team has found to be effective in managing a diabetes patient. The team should
comprise of physicians from multiple specialty, nurses, dietarians, podiatrists and clinical
psychologists to monitor and give care to the patient from every aspects. This specialist team
helps to provide care according to the complex needs of the patients. Involving a
multidisciplinary team identifies the needs and preferences of the patient and implements the
intervention strategies through a collaborative consultation with the multi-disciplinary clinicians
enabling a shared decision making (Wiley et al., 2015).
Implementation-
Rehydration:A person with diabetes has a condition called hyperglycemia characterized with
high sugar levels in the blood along with many complication including diabetic neuropathy
which is a nerve damage affecting digestive systems of the patients. Evidences have shown the

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prevalence of diarrhea in diabetic patients along with chances of developing neuropathy.
Diarrhea can also be a result of the side effect of diabetic medications. To manage the condition
of diarrhea of Joshua, nurses must always keep him hydrated by the administration of fluid and
foods with high amount of liquid. The treatment plan should be implemented based on the
severity of the condition. He should be given fluids and an oral rehydration solution (ORS) to
restore salts and other nutrients and substances that are lost from the body. He should be given a
high potassium diet without sugar can help manage his control his condition.
Oxygen therapy: The respiratory rate recorded was 28 and heart rate 128 beats/min indicating the
urgency to put him on the oxygen therapy. The normal respiratory of a healthy individual 12 to
20 breaths per minute. A respiration rate below 12 or above 25 breaths per minute while resting
is considered to be abnormal. Similarly, the heart rate between 60-100 is considered normal.
Joshua’s heart rate was recorded as 128 beats/minute. The primary treatment given to Joshua
should be putting him on oxygen therapy. It is recommended to consider oxygen asca drug
prescribed and administered for specific indications along with a documented range and also
regular monitoring of the patient and how he responds. Patients who have a respiratory rate
greater than 24 breaths per minutes, must receive immediate medical review along with the
review of level of consciousness (Cretikos et al.,2008).
Medical Nutritional Therapy: Nutritional treatment should be planned according to age, lifestyle,
medical and personal factors considering the diabetes severity and complications. Nurses should
also take into account the patient’s culture, beliefs, food availability and eating patterns. The
nutrition should planned that will maintain the blood glucose concentrations by coordinating
with insulin therapy and physical activity.

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Joshua requires a high amount of calorie, salt and a high fat diet. To manage his weight, Joshua
should be given appropriate calories for ensuring normal growth and development both
physically and psychologically.
Psychological therapy: Diabetes is such a metabolic condition that impacts physical, social and
mental or psychological well being of people. The psychological problems can lead to serious
negative complications and slows down the recovery process. Therefore, nurses need to address
the both the physical and psychological factors and develop implementation strategies (Kalra,
Jena &Yeravdekar, 2018).
The psychological care should be implemented by integrating with a collaborative and
patient centred care with the aim to promote and enhance the health outcome. Nurses should
frequently monitor his vitals incusing diabetes distress, anxiety, depression and cognitive
capacities (Young-Hyman et al., 2016). His level of consciousness should be measured wuth the
AVPU scale as an emergency medicine protocol.
Evaluation- After implementing the strategies, the overall health of Joshua should be monitored
and evaluated to observe his progress. If he is found to be suitable for discharge, then, an
appropriate discharge fact sheet must be provided with the necessary information regarding his
health. A strict diet and lifestyle routine should also be incorporated in the discharge fact sheet.
CFCC- Child family centred care
Family Centred Care is a means of providing medical care that acknowledges the
importance of parents for a child in hospital. Families must be involved in the rehabilitation

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scheme for their children. In the view to promote healthy family functions, the aim is to preserve
and strengthen families and the bond between the family and the child (Festini, 2014).
It is very difficult for a family to bear with the stress when a close member, specially
their child is admitted to the hospital. Therefore, medical professionals including nurses must
effectively communicate with the family regarding their child’s health and give necessary
information regarding the treatment and procedures for an enhanced recovery. The health
professions must support and maintain a relationship with the family in which both of the parties
will involve in sharing the concern and responsibility for the treatment of the child (Shields,
2015).
Considering Joshua’s health complications, his family must be given all the information
and the progress report of his health. Health professions including nurses should always provide
both the patient and their family with information about the treatment approaches or options
available for an enhanced recovery and respect their decisions. Miranda at this stage should be
informed about her child’s condition and must be given all information regarding Joshua’s health
and the treatment approaches available.
Conclusion
The treatment and care plan must include intervention strategies aiming at providing the
best available care based on Evidence Based Practice (EBP) and the use of knowledge and
appropriate skill set possessed by the nurses to get an improved heath outcome. To manage
Joshua’s condition, health assessments are needed to be conducted by the nurses and they should
also be able to understand and interpret the results indicated by the vital sign of the patient that

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will help to estimate the severity of the illness and hence guide the treatment process. The nurses
must possess the appropriate skill set and core competencies when taking care of the patients,
especially in the Emergency Department. Considering the severity of Joshua’s condition, health
professionals including nurses must prioritize the treatment plan according to the health needs of
the patient. While dealing with patients in the Emergency Department, nurses must use problem
solving approach to explore the patient’s condition and plan intervention strategies. They must
critically think and analyse the situation and access, plan, implement and evaluate the care
strategies for an effective delivery of an enhanced nursing care to promote health of the patient.
Besides giving medical treatments, nurses must also plan the treatment strategies through a Child
Family Centred Care approach; “family centred care is a way of caring for children and their
families within health services which ensures that care is planned around the whole family, not
just the individual child/person and in which all the family members are recognized as care
recipients” (Shields, Pratt & Hunter, 2006).

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References:
Abdelghaffar, S. (2015). Nutritional Management of Type 1 Diabetes. Major Topics in Type 1
Diabetes, 121. doi: 10.5772/61150
Cretikos, M. A., Bellomo, R., Hillman, K., Chen, J., Finfer, S., &Flabouris, A. (2008).
Respiratory rate: the neglected vital sign. Medical Journal of Australia, 188(11), 657-
659. doi:10.5694/j.1326-5377.2008.tb01825.x
Festini, F. (2014,) Family-centered care. In Italian journal of pediatrics 40 (1), p. A33. BioMed
Central. doi: 10.1186/1824-7288-40-S1-A33
Kalra, S., Jena, B. N., &Yeravdekar, R. (2018). Emotional and psychological needs of people
with diabetes. Indian journal of endocrinology and metabolism, 22(5), 696. doi:
10.4103/ijem.IJEM_579_17
Kayani, K., Mohammed, R., &Mohiaddin, H. (2018). Cystic fibrosis-related diabetes. Frontiers
in endocrinology, 9, 20. doi: 10.3389/fendo.2018.00020
Nhlbi.nih.gov. (2019). Cystic Fibrosis | National Heart, Lung, and Blood Institute (NHLBI).
Retrieved 30 August 2019, from https://www.nhlbi.nih.gov/health-topics/cystic-fibrosis
O’Riordan, S. M., Dattani, M. T., & Hindmarsh, P. C. (2010). Cystic fibrosis-related diabetes in
childhood. Hormone research in paediatrics, 73(1), 15-24. doi: 10.1159/000271912
Quittner, A. L., Goldbeck, L., Abbott, J., Duff, A., Lambrecht, P., Solé, A., ...& Blackwell, L.
(2014). Prevalence of depression and anxiety in patients with cystic fibrosis and parent

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caregivers: results of The International Depression Epidemiological Study across nine
countries. Thorax, 69(12), 1090-1097. doi: 10.1136/thoraxjnl-2014-205983
Shields, L. (2015). What is “family-centered care”?.European Journal For Person Centered
Healthcare, 3(2), 139-144. Retrieved from
https://researchoutput.csu.edu.au/ws/portalfiles/portal/11519449/81682_1000005125post
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Shields, L., Pratt, J. & Hunter, J. (2006). Familycentred care: a review of qualitative studies.
Journal of Clinical Nursing 15 (10)1317-1323. doi: 10.1111/j.1365-2702.2006.01433.x
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Young-Hyman, D., De Groot, M., Hill-Briggs, F., Gonzalez, J. S., Hood, K., &Peyrot, M.
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Appendix:
Top to toe assessment:
Presenting complaint: Cystic fibrosis along with diabetes type 1, a condition of ketoacidosis
and diarrhoea.
History: Type 1 diabetes.
Allergies/ Current medication: NA
Developmental history: He is raised in a family with a good financial status and a caring nature.
Family History:His sibling has asthma.
Social History:Joshua is very active and participates in the local church group, scouts, and
music and drama productions and engaged well with his peers and the community. Joshua
dreams to be an actor in Hollywood.
Subjective data collected:
Nature of the child:He is a very active child and wants to pursue his career as a Hollywood
actor.
Colour of the child: NA
Obvious appearance: Distressed and sick.
Interaction with people and Interaction with environment: No such information is given.
Dysmorphic features: No such features are observed in Joshua.
Position: NA
General appearance: No such information is given.
Obvious birthmarks, bruises or rashes: No such information is given.
Physical assessments:

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Basic physical recordings:
Blood sugar- 29mol/l
Blood ketones- 2.2 mol/l
Respiratory assessments:
Heart rate- 20
Respiratory rate- 28
Level of hydration:10% dehydration
Urinalysis:
pH- 5
Specific gravity- 1.035
Glucose- 2mmol/l
Protein- >30
Neurological status: NA
Head circumference: NA
Skin assessment: NA
Weight: Not provided in the case scenario.
Height: Not provided in the case scenario.
BMI: NA
Centile charts: NA

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