RECAP:

• Blood Component • BT
• Disorder afffecting RBC – Plasma,RBC,WBC,Plt, WB
– Anemia – Complications (first 15mins)
Fe Deficiency Anemia • Leukemia
Sickle Cell Anemia
– ALL
• Blood D/O – AML
– Thalassemia Chemotherapy
– Hemophilia Radiation
– ITP Corticosteriods
Nursing Care of a Family When a Child
Has a Endocrine Disorder

Aileene D. Mendoza, RN, MAN

ENDOCRINE SYSTEM
 Pediatric Differences in the Endocrine System 

• The endocrine system is less developed at birth than any

other body system
• Hormonal control of many body functions is lacking until
12-18 months of age
• Infants might manifest imbalances in concentration of
fluids, electrolytes, amino acids, glucose, and trace
substances
 Understanding the Endocrine System in Children

Puberty brings many

changes :
• ↑GH released
• ↑ production of LH
and FSH in girls
• Development of
sexual characteristics
 Collecting data during an Endocrine Assessment

• Percentiles on weight and height

• Distinguishing facial features, abdominal fat
• Onset of puberty
• Routine NB screening
• Blood glucose levels
• Detection of chromosomal disorders
 Phenylketonuria (PKU) 

• Genetic metabolic disorder

characterized by absence of
enzyme phenylalanine
hydroxylase to breakdown
phenylalanine to the amino
acid Tyrosine.

• As a result, excessive
phenylalanine builds up in
the blood stream causing
permanent damage to brain
 Phenylketonuria (PKU) 

Clinical Manifestations:
• Musty or Mousey body and urine odor
• Irritability, hyperactivity
• Vomiting
• Hypertonia, hyperreflexivity
.... Complication:
• Seizure disorder
• Untreatable mental retardation
Goal of Therapy:

•  Keep serum phenylalanine level at 2-6mg/dl

 Newborn Screening 
• Should not be done until
feeding on breast milk or
formula
• Should be done ~48 hrs.
after birth
• If test shows elevated
levels of plasma
phenylalanine, repeat test
done and if that is elevated,
treatment started.
 Treatment and Nursing Care for PKU

• Special low-phenylalanine formula - Lofenalac, Minafen,

and Albumaid XP
• Diet low in phenylalanine – for life
• Avoid high-protein foods such as meats, fish, eggs,
cheese, milk, and legumes.
• Avoid aspartame (artificial non-saccharide sweetener 200 times sweeter than sucrose)

• Take a phenylalanine-free protein supplement to maintain

growth
• Family support
 Inborn Errors Of Metabolism
1.Galactosemia 

2. Maple syrup urine

3. Tay-Sachs disease
– is a fatal genetic disorder, most commonly occurring in
children, that results in progressive destruction of the nervous
system. Tay-Sachs is caused by the absence of a vital enzyme
called hexosaminidase-A (Hex-A).
 Galactosemia
– refers to a group of
inherited disorders that
impair the body's ability
to process and produce
energy from a sugar
called galactose
 Galactosemia

Signs and Symptoms

• Poor sucking
• Failure to gain weight / IUGR
• Vomiting and diarrhea
• Hypotonia
• Cataracts
• Infections
• Treatment
• Lactose-free formula and diet
 Maple syrup urine
– is an inherited disorder
in which the body is
unable to process
certain protein building
blocks (amino acids)
properly.
 Maple Syrup Urine Disease
• Disorder of amino acid metabolism
• Diagnosis made by UA

Signs and Symptoms:

• Poor appetite
• Lethargy
• Vomiting
• High-pitched cry
• Seizures
• Sweet odor of maple syrup in body fluids
Treatment/management:

• Removal of the amino acids and metabolites

• Diet low in proteins and amino acids
 Tay-Sachs disease

– is a fatal genetic disorder,

most commonly occurring
in children, that results in
progressive destruction of
the nervous system.

– Tay-Sachs is caused by
the absence of a vital
enzyme called
hexosaminidase-A (Hex-
A).
 Tay-Sachs Disease

• Deadly inherited disease

• No treatment
• Death by age 4
• Absence of hexosaminidase A,
a protein in nerve tissue which
breaks down gangliosides (fatty
substances that build up in nerve
cells of the brain)
 Tay-Sachs Disease 

Signs & Symptoms

• Deafness
• Blindness
• Decrease muscle tone
• Loss of motor skills
• Delayed mental & social skills
• Dementia
• Paralysis
• Seizures
 Nursing Measures for Metabolic Disorders
• Genetic counseling
• Dietary teaching
• Compliance Mixing special preparations Mainly
supportive
 Diabetes – Type 1
• Inability of the body to produce or excrete insulin
 When are Children most likely to be diagnosed
with Diabetes?

Peak incidence is:

• 5-7 years of age
• Puberty It can occur at any age.
 Emerging Trends of Diabetes 

• Incidence of Type 1 diabetes increasing, the etiology is

unknown. This trend is most apparent in very young
children
• Obesity is causing increased incidence of Type 2 diabetes
in children and teens
• As children with chronic illness survive longer(i.e. cystic
fibrosis)with more extreme measures and (i.e. transplants),
diabetes becomes another side effect of their illness
Etiology :
• Autoimmune process
• Inflammatory process in the insulin secreting islet cells of
the pancreas
• Destruction of the islet cells
• Failure to produce or excrete insulin
 Pathophysiology
• Failure to produce insulin leads to elevated blood glucose
HYPERGLYCEMIA
 Clinical Manifestations Three P’s
• Polyuria – the need to
frequently urinate,
particularly at night.
• Polydipsia – increased
thirst & need for fluids.
• Polyphagia – an increased
appetite.
 How would you tell polyuria in a toddler? 

Answer: Enuresis (involuntary urination, especially by

children at night) in a toilet-trained child
Other manifestations of hyperglycemia :
• Fatigue – unexplained
• Weight Loss (gradual, over several weeks)
• Blurred vision
• Headache
• Hunger
Diagnosis Symptoms of diabetes plus Plasma Glucose
Levels of:

• Fasting plasma glucose ≥ 126 mg/dl or

• Two-hour plasma glucose ≥200 mg/dl or
• Random serum glucose concentration ≥200 mg/dl
**Ketonuria is a frequent finding**
 Goals of Diabetic Management
Therapeutic Management Managed and
educated by a multidisciplinary team of
experts in pediatric diabetes

These Goals are Met by: 

• Insulin Administration
• Regulations of nutrition and exercise
• Stress Management
• Blood glucose and urine ketone monitoring
 Insulin Therapy

Goal of Insulin Therapy:

• to replace the insulin the child

• Lower blood glucose levels
• Stabilize glucose levels
• Eliminate ketones
Goals of Insulin Therapy Maintain serum glucose levels from:
• Toddlers and preschoolers
100 – 180 before meals
110-200 at bedtime
• School-age
90- 180 before meals
100 – 180 at bedtime
• Adolescents
90 – 130 before meals
90 – 150 at bedtime
Types of Insulin 

• Rapid (Lispro/Humalog)
• Short acting (regular)
• Intermediate acting (NPH,
Lente)
• Long acting
(Lantus/Ultralente)
Factors which may affect insulin dosage in children 
• Stress
• Infection
• Illness
• Growth spurts (such as puberty)
• Meal coverage for finicky toddlers
• Adolescents concerned about weight gain not wanting to
eat AM snack
 About Insulin

• Store insulin in a cool, dry place; do not freeze or expose

to heat or agitation

• Check the expiration date on the vial before using

• Once opened, date the vial and discard as recommended

• When mixing two different types of insulin, inject the

appropriate amount of air into both vials, then withdraw the
short-acting (clear) first before coudy (long-acting).
 Treatment and Nursing Care
Signs of DKA:
• 3 P's
• Abdominal pain / “Stomachache”
• Nausea and vomiting
• Acetone (fruity)breath odor
• Dehydration
• Increasing lethargy
• Kussmaul respirations
• Coma
Treatment for DKA 
• IV Fluids (boluses)
• IV insulin - Wean off IV insulin when clinically stable
• Electrolyte replacement
• Oral feedings introduced when alert
• Prevention of future episodes
 Nursing Management at the time of diagnosis (admitted)

Nursing assessments directed toward:

• Vital Signs
• LOC
• Hydration
• Hourly monitoring of BS
• Dietary and caloric intake
• Ability of family to manage
 Focus of Child and Parent Education 
• Signs and symptoms of hypoglycemia and hyperglycemia
and related treatment
• Blood-glucose monitoring / urine ketone monitoring
• Administration of insulin
• “Sick day” guidelines
• Nutrition
 Sick Day Guidelines

• Monitor blood glucose levels more often

• Test urine ketones when blood glucose is high
• Do NOT skip doses of insulin
• Usual doses of insulin may be increased
• Encourage large fluid intake
 Hypoglycemia
• Blood Sugar < 70mg/dl 

Symptoms:
• Trembling
• Sweating, clammy skin
• Tachycardia
• Pallor
• Personality change/
irritable
• Slurred speech
 Hypoglycemia

Treatment:

• 15g carbohydrate
• Glucagon sub-q
• IV glucose
• OJ, sweet beverage, raisins, cheese and crackers, candy
 Hyperglycemia
• Blood Sugar > 160mg/dl 

Symptoms:
• Polyuria
• Polydipsia
• Fatigue
• Weight Loss
• Blurred vision
• Emotional lability
• Headache
Treatment:

• Insulin
• Increase oral fluids
Home Teaching 

• Incorporate into the family lifestyle

• “Honeymoon phase”
• Community resources
experience shortly after being diagnosed. During this time, a
• Recognizing
person withthe cognitive
diabetes seemslevels at time
to get better andofmay
teaching
only need
minimal amounts of insulin.
 Nutrition for the Child withType I Diabetes Mellitus

• Meals and snacks are balanced with insulin action

• Both the timing of the meal or snack and the amount of
food are important in avoiding hyperglycemia or
hypoglycemia
• Adherence to a daily schedule that maintains a consistent
food intake combined with consistent insulin injections aids
in achieving metabolic control
 Exercise for the Child withType I Diabetes Mellitis

• Exercise is encouraged

• Avoid exercising during insulin peak

• Add an extra 15 to 30g carbohydrate snack for each 45-60

minutes of exercise