Case Study

on
Wilson’s Disease

Submitted by-Mr. Deepak

M.Sc Nursing 2nd year
Submitted to- Ms Tarika Sharma
Lecturer, ILBS CON
INTRODUCTION ABOUT SELF
My name is Deepak, M.Sc. Nursing 2nd year student of ILBS Nursing College. I was posted to
HDU of ILBS hospital from 09-07-2018 to 12-07-2018 as a part of my out posting clinical
experience.

INTRODUCTION ABOUT THE CLIENT

Mr. Anuj Thirthani is a resident of Swasthya Vihar, Delhi. He has suffered Wilson’s Disease
(Hepatolenticular Degeneration). He was previously admitted to ILBS with c/o breathlessness,
fatigue, Ascites and palpitations nearly about 2.5 years back.

REASON FOR SELECTING THIS TOPIC FOR CARE NOTE

I found this case interesting and out of my curiosity selected Wilson’s Disease as my topic for
Case Study, to know the case in detail that, what happened with the client earlier and with what
type of problems he came here and got admitted? So, all these questions raised in my mind and
as a part of my case study selection, I wanted to learn the comprehensive care which is required
for such patients so that, this learning could help me develop and refine my nursing care
providing abilities for future purpose as well.
 CLIENT PROFILE

A. PERSONAL CHARACTERISTICS

 Name of Patient : Mr. Anuj Thirthani

 Age : 29 Years
 Sex : Male
 Ward & Bed No. : HDU (19) ILBS
 Admission No. : 44394
 Address : Swasthya Vihar, New Delhi
 Date of Admission : 02/07/2018
 Religion : Hindu
 Educational Qualification : Graduated
 Marital status : Unmarried
 Occupation : Freelance Photographer
 Diagnosis : Wilson’s Disease (Hepatolenticular
Degeneration)

B. FAMILY CHARACTERISTICS
Family members

FAMILY TREE

Mr NK Thirthani Ms Krishna Thirthani

57 yrs 52 yrs

Mr Anuj Thirthani 29 yrs

S. Name Relation Age Sex Educational Occupati Health

No With Head Status on Status
1 Mr. NK Head 57yr M Graduated Business Hypertension
Thirthani man and Diabetes
Mellitus
2 Mrs. Wife 52yr F B.A. pass Housewif Diabetes
Krishna e Mellitus
3 Mrs. Daughter 35yr F Graduated Housewif Healthy and
 Avantika e active
4 Mr. Son in law 37yr M MBA Business Healthy and
Vivek man active
5 Ms. Granddaugh 7 yrs F - Student Healthy and
Raashi ter active
6 Mr. Son 32yr M B.Tech Servicema Healthy and
Abhishek n active
7 Mrs. Daughter in 30yr F B.A. pass House Healthy and
Mansi law wife active
8 Mr. Grand son 5 yr M - Student Healthy and
Manik active
9 Mr. Anuj Son 29 yr M Graduated Photograp Healthy and
her active
FAMILY COMPOSITION

1. Types of family form

a. They live in Joint family.

b. He lives with his parents, brother, sister-in-law and nephew.

2.Family characteristics

a. Role structure: He is the youngest son of the family.

b. Value systems : They follow societal rules and regulations
c. Communication pattern: He speaks Hindi and English while communicating with
family as well as with others.
d. Power structure: Along with his family, he makes family decisions.

CHIEF COMPLAINTS

At the time of admission:

• Dyspnoea since 2-3 hours
• Altered Sensorium
• Tremors
• Abnormal behaviour
• Vomiting
• Jaundice

PATIENT HISTORY

PRESENT HISTORY OF ILLNES

Patient was asymptomatic before 1998, then he developed jaundice and ascites and he diagnosed
with Wilson’s diseases and he recovered. In 2014 again the symptoms of jaundice, vomiting,
abdominal pain, neuropsychiatric symptoms occur and get admitted in ILBS and gradually
recover and on follow-up for the medical condition. Suddenly on 02 july 2018, he got dyspnea,
altered sensorium episodes, tremors, abnormal behaviour and patient is highly agitated then got
admitted in ILBS.
PAST MEDICAL HISTORY
There was no past medical history of any communicable such as TB. Patient is having history of
jaundice and ascites since 1998
PAST SURGICAL HISTORY
There was no significant in the past surgical history.
FAMILY MEDICAL HISTORY
Father had hypertension and diabetes mellitus and mother had diabetes mellitus.

PERSONAL AND SOCIAL HISTORY

• Type of House: Pucca House

• No. Of Rooms: 4 Rooms
• Ventilation: Adequate Ventilation with Windows
• Electricity: Available
• Water supply: Supply Water & Hand Pump
• Drainage system: Open Drainage System
• Sanitation: Toilet Available
• Socioeconomic status: Gross Income is 3,00,000/Month
• Nutritional status: Poorly Nourished
• Habits:
o Elimination pattern - Bowel and bladder habits are normal
o Sleeping pattern - Insomnia due to tremors
o Eating pattern - Less Intake due to tremors and agitated behaviour
o Addiction - Chronic smoker and alcoholic

PHYSICAL EXAMINATION

A.GENERAL APPEARANCE

 Body build - Mesomorphic

 Nourishment - Poorly Nourished
 Level of conscious - Conscious
 Hygiene - Maintained
 Activity - Restricted due to disease, client is weak.
  Posture and Gait - Normal

B. GENERAL PHYSICAL EXAMINATION

 Vital sign
 Temperature – 98.8F
 Pulse - 86 beats/min
 Respiration - 26 breaths/min
 Blood Pressure – 110/90 mmHg
 Height - 170 cm
 Weight - 60 Kg
 BMI - 25.4

C. HEAD TO TOE EXAMINATION

 Integument
o Skin
 Color: skin is pallor, but there is no jaundice, cyanosis or any other
problem
 Texture: skin is dry & wrinkles are present in the skin
 Temperature: temperature is normal
 Lesions: there is no any macules, papules, vesicles present

The skin is uniform in color, unblemished and no presence of any foul odor. He has a
good skin turgor and skin’s temperature is within normal limit.

o Hair: The hair of the client is thick, silky hair is evenly distributed and has a variable
amount of body hair. There are also no signs of infection and infestation observed.
o Nails: The client has a light brown nails and has the shape of convex curve. It is
smooth and is intact with the epidermis. When nails pressed between the fingers
(Blanch Test), the nails return to usual color in less than 3 seconds.

Head
  Head: The head of the client is rounded; normocephalic and symmetrical. Colour of
hair is black. Scalp is clean, no swelling, no dandruff and no pediculi present.
 Skull: There are no nodules or masses and depressions when palpated.
 Face: The face of the client appeared smooth and has uniform consistency and with
no presence of nodules or masses.

Eyes and Vision

 Eyebrows: Hair is evenly distributed. The client’s eyebrows are symmetrically

aligned and showed equal movement when asked to raise and lower eyebrows.
 Eyelashes: Eyelashes appeared to be equally distributed and curled slightly outward.
 Eyelids: There were no presence of discharges, no discoloration and lids close
symmetrically with involuntary blinks approximately 15-20 times per minute.
 Eyes
o The pupils of the eyes are black and equal in size. The iris is flat and round.
PERRLA (pupils equally round respond to lightaccommodation),
illuminated and non-illuminated pupils constricts. Pupils constrict when
looking at near object and dilate at far object. Pupils converge when object
is moved towards the nose.
o When assessing the peripheral visual field, the client can see objects in the
periphery when looking straight ahead.
o When testing for the Extraocular Muscle, both eyes of the client
coordinately moved in unison with parallel alignment.
o The client was able to read the newsprint held at a distance of 14 inches.

Ears and Hearing

 Ears: The Auricles are symmetrical and has the same color with his facial skin. The
auricles are aligned with the outer canthus of eye. When palpating for the texture, the
auricles are mobile, firm and not tender. The pinna recoils when folded. During the
assessment of Watch tick test, the client was able to hear ticking in both ears.

Nose and Sinus

  Nose: The nose appeared symmetric, straight and uniform in color. There was no
presence of discharge or flaring. When lightly palpated, there were no tenderness and
lesions
 Mouth:
o The lips of the client are uniformly pink; moist, symmetric and have a
smooth texture. The client was able to purse his lips when asked to whistle.
o Teeth and Gums: There are no discoloration of the enamels, no retraction
of gums, pinkish in color of gums
o The buccal mucosa of the client appeared as uniformly pink; moist, soft,
glistening and with elastic texture.
o The tongue of the client is centrally positioned. It is pink in color, moist
and slightly rough. There is a presence of thin whitish coating.
o The smooth palates are light pink and smooth while the hard palate has a
more irregular texture.
o The uvula of the client is positioned in the midline of the soft palate.
 Neck:
o The neck muscles are equal in size. The client showed coordinated, smooth
head movement with no discomfort.
o The lymph nodes of the client are not palpable.
o The trachea is placed in the midline of the neck.
o The thyroid gland is not visible on inspection and the glands ascend during
swallowing but are not visible.

Thorax, Lungs, and Abdomen

 Lungs / Chest: The chest wall is intact with no tenderness and masses. There’s a full
and symmetric expansion and the thumbs separate 2-3 cm during deep inspiration
when assessing for the respiratory excursion. The client manifested quiet, rhythmic
and effortless respirations.
 The spine is vertically aligned. The right and left shoulders and hips are of the same
height.
 Heart: There were no visible pulsations on the aortic and pulmonic areas. There is no
presence of heaves or lifts.
  Abdomen: The abdomen of the client has an unblemished skin and is uniform in
color. The abdomen has a symmetric contour. There were symmetric movements
caused associated with client’s respiration.
o The jugular veins are not visible.
o When nails pressed between the fingers (Blanch Test), the nails return to
usual color in less than 4 seconds.
o Hepatomegaly present

Extremities

 The extremities are symmetrical in size and length.

 Muscles: The muscles are not palpable with the absence of tremors. They are
normally firm and showed smooth, coordinated movements.
 Bones: There were no presence of bone deformities, tenderness and swelling.
 Joints: There were no swelling, tenderness and joints move smoothly of right hand
but tenderness and swelling the distal and proximal phalengeal joints of left hand
present. Heberden nodes and bouchard’s nodes are not present.

Spine

 Spina Bifida - Absent

 Curves - Normal

Genitalia and rectum

 Abnormal Discharges - Absent

 STDs - Absent
 Any enlargement - Absent
 Haemorrhoids - Absent
 Pelvic Masses - Absent

Neurological test

 Coordination test - Abnormal due to hepatic encephalopathy

 Reflexes - Dystonia present
 Test for Sensation - Normal
 GCS - Normal
Patient was in hepatic encephalopathy grade-2 as per the West Haven’s Criteria

Investigations

Blood Studies

S.No Investigations Normal 02-04-2018 03-04-2018 04-04-2018

Value

1. Haemoglobin For 11.3 11.9 10.9

men, 13.5 to
17.5 grams
per deciliter.
For
women, 12.0
to 15.5
grams per
deciliter.
2 PCV PCV 32.7 36.5 32.7
< 0.55 (Hb
Conc. < 180
 g/L)

3 TLC WBC (Male) 7.0 7.4 6.8

3.8 – 11.0
10^3 / mm3
WBC
(Female) 3.8
– 11.0 10^3 /
mm3
4 Neutrophils 40% to 60% - - 74.4

5 Lymphocytes 20% to 40% - - 9.3

6 Monocytes 2% to 8% - - 14.4

7 Eosinophils 1% to 4% - - 1.5

8 MCV 80-96 fL/red 84.7 94.7 -

cell

9 MCH 27-33 6.5 4.1 -

picograms
(pg)/cell 

10 MCHC 33-36 g/dL 8.2 1.1 -

11 Retculocytes 0.5% to 2.5 0.2 - -

%

12 Platlets 150,000 to 280000 300000 260000

450,000 

13 RBC RBC (Male) 3.4 3 3.2

4.2 – 5.6
10^6 / µL
RBC
(Female) 3.8
– 5.1 10^6 /
µL
14 PT 11 to 13.5 22.4 22.4 28.1
seconds

15 INR 0.8 to 1.1 2.19 2.14 2.73

16 BU 7 to 20 45.8 44 42
mg/dL

17 Sr. Creatinine 0.6 to 0.44 0.96 0.33

1.2mg/dL

18 Sodium 135 132.7 129 130

-145mEq/L 

19 Potassium 3.5-5.0 4.2 3.1 4.3

mEq/L

20 Chlorine 98-106 90.6 90 97

mmol/L

21 Calcium 8.5-10.2 9.1 9.0 8.4

mg/dL

22 Magnesium 1.7 to 2.02 1.58 2.02

2.2 mg/dL

23 S. Billirubin 0.2 to 1.2 31.7 29.8 29.6

Total mg/dL

Direct 0 to 0.4 14.9 17 15.9

mg/dL

Indirect 0 to 0.8 16.8 12.8 13.7

mg/dL

24 AST 10 to 40 950 866 337

units per liter

25 ALP 7 to 56 541 509 302

units per liter

26 SAP 30-40 µg/ml. 201 385 165

27 GGTP 3.3 - 57 63 51
 35.0IU/L

28 T. Protein 6-8.3g/dL 6.4 6.1 5.6

29 Albumin 3.5 to 5.5 2.8 2.8 2.8

g/dL

30 Globulin  6 to 8 g/dl 3.6 - -

31 S. Ammonia 15 to 45 - 84 -
µ/dL

32 Uric Acid 3.4-7.0 3.0 3.4 2.4

mg/dL

33 Bicarbonates 23 to 30 31 20 27
mEq/L

34 PCT <0.15 ng/mL - 100 -

ABG Analysis

S.NO Parameters Normal Values Patient’s Value

1. pH 7.350-7.450 7.417

2 pCO2 32-48 42.2 mm Hg

3 pO2 83-108 32.7 mm Hg

4 cNa+ 136-146 139 mmol/L

5 cK+ 3.4-4.6 4.8 mmol/L

6 cCa2+ 1.15-1.29 1.30 mmol/L

7 cCl- 98-106 99 mmol/L

8 cLac 0.5-1.6 1.4 mmol/L

 9 ctHb 12-16 10.7g/dL

10 sO2 95-99 62.5%

11 cHCO3-(P)c 22-28 26.7 mmol/L

Note- c indicated calculated values

Malaria- Negative

Dengue- NR

Input/ Output charting

Day-1 Input- 780mL Output-750mL

Day-2 Input- 800mL Output- 790mL

Day-3 Input-1280mL Output-750mL

Radiological Investigations: X-Ray chest and USG abdomen done on 05-04-17.

Blood Studies

Studies Normal Patient Value

Plasma caeruloplasmin >200 mg/l 180 mg/l

Urine Cu <0.6 µmol/24 h 2.6 µmol/24 h

Hepatic copper < 250 µg/g dry wt 265 µg/g dry wt

Treatment

 Syp Gutclear 30mL PO TDS

 Syp Zinc Acetate 50mg PO TDS
 T Baclofen XL 20mg PO BD
  T. Pacitane 2mg PO BD
 Inj Optineuron 1 amp OD
 Inj Albumin 5 % 250mg IV OD @ 50mL/hr

Drug Study

S. Name of Action Indication Contraindication Side-effects Nursing

No Drug Responsibilities
1. Gutclear GI agent Prevention Low galactose GI: Flatulence, In children if
the initial dose
and and treatment diet; pregnancy borborygmi, causes
Generic Hyperosmot of portal- (category C). belching, diarrhea,
Name- dosage is
ic Laxative systemic Safe use in abdominal reduced
Lactulos encephalopat lactation or cramps, pain, immediately.
e Discontinue if
hy (PSE), children is not and distention
diarrhea
including established (initial persists.
stages of dose); diarrhea 
Promote fluid
hepatic (excessive intake (
precoma and dose); nausea, 1500–2000
mL/d) during
coma, and by vomiting, colon
drug therapy
prescription accumulation of for
for relief of hydrogen gas; constipation;
older adults
chronic hypernatremia. often self-
constipation. limit liquids.
Lactulose-
induced
osmotic
changes in the
bowel support
intestinal
water loss and
potential
 hypernatremia
. Discuss
strategy with
physician.

2. Zinc Mineral  To treat a Hypersensitivity GI upset, Avoid taking

Acetate supplement certain liver d nausea, allergy medications with
isease foods containing
(Wilson's high calcium and
disease) phosphorus
3. Baclofen ANS Agent, To provide Safety during CNS: Transient Note: CNS
depressant
Muscle symptomatic pregnancy drowsiness, vert effects will be
Relaxant relief of (category C), igo, dizziness, additive to
other CNS
painful lactation; weakness, depressants,
spasms in coagulopathy, fatigue, including
alcohol.
multiple bacteremia, headache,
sclerosis and intramuscular or confusion, Monitor blood
in the intrathecal insomnia; glucose for
loss of
management administration, ataxia, loss of glycemic
of detrusor subcutaneous seizure control control if
diabetic.
sphincter administration. in epileptic
dyssynergia patients; abrupt Do not drive
in spinal cord discontinuation or engage in
other
injury or of intrathecal potentially
disease. administration hazardous
activities until
may result in the response
high fever, to drug is
altered mental known.

status, Report
exaggerated adverse
reactions to
rebound physician.
spasticity, and Most can be
reduced by
muscle rigidity,
decreasing
that in rare cases dosage.
has advanced to Incidence of
CNS
rhabdomyolysis, symptoms
multiple organ- (drowsiness,
dizziness,
system failure,
ataxia) are
and reportedly
death. CV: Hyp high in
patients >40 y
otension. Specia of age.
l
Do not self-
Senses: Tinnitus
dose with
, nasal OTC drugs
congestion; without
physician's
blurred vision, approval.
mydriasis,
Do not stop
nystagmus, this drug
diplopia, unless
strabismus, directed to do
so by
miosis. GI:Naus physician.
ea, constipation, Drug
withdrawal
vomiting; mild needs to be
increases in accomplished
gradually over
AST, and
a period of 2
alkaline wk or more.
phosphatase, Abrupt
withdrawal
jaundice. Uroge following
nital: Urinary prolonged
administration
frequency.
may cause
anxiety,
agitated
behavior,
auditory and
visual
hallucinations,
 severe
tachycardia,
acute
exacerbation
of spasticity,
and seizures.

Do not breast
feed while
taking this
drug without
consulting
physician.

4. Pacitane ANS Agent, Symptomatic Narrow-angle GI: Dry mouth, Be aware that

incidence and
Anticholine treatment of glaucoma. nausea, constipa severity of
rgic agent, all forms of Safety during tion. Special adverse
effects are
antimuscari parkinsonism pregnancy Senses: Blurred usually dose
nic, (arteriosclero (category C), vision, mydriasi related and
may be
Antiparkins tic, lactation, or in s, photophobia,
minimized by
onism, idiopathic, children is not angle-closure dosage
Antispasmo postencephali established. glaucoma. Urog reduction.
Older adults
dic tic). Also to enital: Urinary appear more
prevent or hesitancy or sensitive to
usual adult
control drug- retention. CNS: 
doses.
induced Dizziness,
extrapyramid nervousness,ins Monitor vital
signs. Pulse is
al disorders. omnia, a particularly
drowsiness, sensitive
indicator of
confusion, response to
agitation, drug. Report
delirium, tachycardia,
palpitations,
psychotic paradoxical
manifestations, bradycardia,
or fall in BP.
euphoria. CV: T
 achycardia,
Assess for and
palpitations, report severe
hypotension, CNS
stimulation
orthostatic (see ADVERS
hypotension. Bo E EFFECTS)
that occurs
dy as a
with high
Whole: Hyperse doses, and in
nsitivity patients with
arteriosclerosi
reactions. s, or those
with history
of
hypersensitivi
ty to other
drugs.

In patients
with severe
rigidity,
tremors may
appear to be
accentuated
during therapy
as rigidity
diminishes.

Monitor daily
I&O if patient
develops
urinary
hesitancy or
retention.
Voiding
before taking
drug may
relieve
problem.
5. Optineur Vitamin b 12 Hypersensitivity Body as a Record
patient's
on deficiencies Whole: Feeling dietary history
Acne of warmth, carefully as an
essential part
Nerve pain weakness, of vitamin
Mental
disorders sweating, replacement
therapy.
Muscle restlessness, Collaborate
cramps tightness of with
physician,
Arthritic throat, dietitian,
Migraine angioneurotic patient, and
Minor skin responsible
edema, anaphyla
family
injuries xis. Respirator member in
High developing a
y: Cyanosis,
cholesterol diet teaching
pulmonary plan that can
Diarrhea be sustained
edema. CV:Car
Alzheimer's by patient.
diovascular
disease
collapse, slight Note: Body
Attention requirement
fall in BP
deficit of thiamine is
following rapid directly
hyperactivity
IV proportional
disorder to
administration.  carbohydrate
Arthritis
GI: GI intake and
Vitamin b3
hemorrhage, metabolic
deficiency rate;
Addisonian nausea. Skin: U requirement
rticaria, pruritus. increases
anemia
when diet
Small bowel consists
bacterial predominantly
of
overgrowth carbohydrates.
Fish Total absence
tapeworm of dietary
thiamine
infestation produce
Malignancy deficiency
of pancreas or state in about
3 wk.
bowel
Folic acid Food–drug
relationships:
deficiency
Learn about
Heart disease rich dietary
Clogged sources of
 arteries
thiamine (e.g.,
Cervical yeast, pork,
cancer beef, liver,
Carpal tunnel wheat and
other whole
syndrome grains,
Cardiovascula nutrient-added
breakfast
r diseases
cereals, fresh
Sunburns vegetables,
Mild burns especially
peas and dried
Skin disorders beans).

6. Albumin Plasma To restore Severe anemia; Body as a Monitor BP,

pulse and
volume plasma cardiac failure, Whole: Fever, respiration,
expanders, volume and patients with chills, flushing, and IV
albumin flow
Anticoagula maintain normal or increased rate. Adjust
nt cardiac increased salivation, flow rate as
needed to
output in intravascular headache, back
avoid too
hypovolemic volume. Safety pain. Skin: Urti rapid a rise in
shock; for during caria, BP.
prevention pregnancy rash. CV: Circul Lab tests:
and treatment (category C) or atory overload, Monitor
dosage of
of cerebral lactation is not pulmonary
albumin using
edema; as established. edema (with plasma
adjunct in rapid infusion); albumin
(normal): 3.5–
exchange hypotension, 5 g/dL; total
transfusion hypertension, serum protein
(normal): 6–
for dyspnea, 8.4 g/dL;
hyperbilirubi tachycardia. GI:  Hgb; Hct; and
nemia and Nausea, serum
electrolytes.
erythroblasto vomiting.
sis fetalis; to Observe
closely for
increase S&S of
plasma
circulatory
protein level overload and
in treatment pulmonary
edema. If
of S&S appear,
hypoproteine slow infusion
rate just
mia; and to
sufficiently to
promote keep vein
diuresis in open, and
report
refractory immediately
edema. Also to physician.
used for
Observe for
blood bleeding
dilution prior points that did
not bleed at
to or during lower BP with
cardiopulmon injuries or
surgery and as
ary bypass BP rises.
procedures.
Has been Monitor I&O
ratio and
used as pattern.
adjunct in Report
changes in
treatment of urinary
adult output.
Increase in
respiratory
colloidal
distress osmotic
syndrome pressure
usually causes
(ARDS). diuresis,
which may
persist 3–20 h.

Withhold
fluids
completely
during
succeeding 8
h, when
 albumin is
given to
patients with
cerebral
edema.

Anatomy and Physiology of Liver

Human liver development begins during the third week of gestation and does not
achieve mature architecture until about 15 years of age. It reaches its largest relative
size, 10% of fetal weight, around the ninth week. It is about 5% of body weight in the
healthy neonate. The liver is about 2% of body weight in the adult. It weighs around
1400g in an adult female and about 1800g in the male.

The liver is located in the right upper quadrant of the abdomen, just below the
diaphragm. It is almost completely behind the rib cage but the lower edge may be
palpated along the right costal margin during inspiration. A connective tissue layer
called Glisson's capsule covers the surface of the liver. The capsule extends to invest
all but the smallest the vessels within the liver. The falciform ligament attaches the
liver to the abdominal wall and diaphragm and divides the liver into a larger right lobe
and a smaller left lobe.

In 1957, the french surgeon Claude

Couinaud described 8 liver segments.
Since then, radiographic studies
describe an average of twenty
segments based on distribution of
blood supply. Each segment has its
own independent vascular and biliary
branches. Surgeons utilize these
independent segments when
performing liver resection for tumor or
transplantation. There are at least three reasons why segmental resection is superior to
simple wedge resection. First, segmental resection minimizes blood loss because
vascular density is reduced at the borders between segments. Second, it results in
improved tumor removal for those cancers which are disseminated via intrasegmental
branches of the portal vein. Third, segmental resection spares normal liver allowing
for repeat partial hepatectomy.

Each segment of the liver is further divided into

lobules. Lobules are usually represented as discrete
hexagonal aggregations of hepatocytes. The
hepatocytes assemble as plates which radiate from a
central vein. Lobules are served by arterial, venous
and biliary vessels at their periphery. This model is
useful for teaching purposes but more closely
resembles the adult pig lobule than the human.
Human lobules have little connective tissue
separating one lobule from another. The paucity of
connective tissue makes it more difficult to identify
the portal triads and the boundaries of individual
lobules. Central veins are easier to identify due to
their large lumen and because they lack connective
tissue that invests the portal triad vessels.
Lobules consist of hepatocytes and the spaces between them. Sinusoids are the spaces between
the plates of hepatocytes. Sinusoids receive blood from the portal triads. About 25% of total
cardiac output enters the sinusoids via terminal portal and arterial vessels. Seventy-five percent
of the blood flowing into the liver comes through the portal vein; the remaining 25% is
oxygenated blood that is carried by the hepatic artery. The blood mixes, passes through the
sinusoids, bathes the hepatocytes and drains into the central vein. About 1.5 liters of blood exit
the liver every minute. The liver is central to a multitude of physiologic functions, including:

 Clearance of damaged red blood cells & bacteria by phagocytosis

 Nutrient management
 Synthesis of plasma proteins such as albumin, globulin, protein C, insulin-like growth
factor, clotting factors, etc.
 Biotransformation of toxins, hormones, and drugs
 Vitamin and mineral storage
 Wilson Disease

Wilson disease is a genetic disease that prevents the body from removing extra copper. The body
needs a small amount of copper from food to stay healthy; however, too much copper is
poisonous. Normally, the liver filters extra copper and releases it into bile. Bile is a fluid made
by the liver that carries toxins and wastes out of the body through the gastrointestinal tract. In
Wilson disease, the liver does not filter copper correctly and copper builds up in the liver, brain,
eyes, and other organs. Over time, high copper levels can cause life-threatening organ damage.

Causes

Wilson disease is caused by an inherited autosomal recessive mutation, or change, in

the ATP7Bgene. In an autosomal recessive disease, the child has to inherit the gene mutation
from both parents to have an increased likelihood for the disease. The chance of a child
inheriting autosomal recessive mutations from both parents with a gene mutation is 25 percent,
or one in four. If only one parent carries the mutated gene, the child will not get the disease,
although the child may inherit one copy of the gene mutation. The child is called a “carrier” of
the disease and can pass the gene mutation to the next generation. Genetic testing is a procedure
that identifies changes in a patient’s genes and can show whether a parent or child is a carrier of
a mutated gene. Autosomal recessive diseases are typically not seen in every generation of an
affected family.

The following chart shows the chance of inheriting an autosomal recessive mutation from
parents who both carry the mutated gene.
 The chance of a child inheriting autosomal recessive mutations from both parents with a gene
mutation is 25 percent, or one in four.
Genetic Diseases

Each cell contains thousands of genes that provide the instructions for making proteins for
growth and repair of the body. If a gene has a mutation, the protein made by that gene may not
function properly. Not all gene mutations cause a disease.

People have two copies of most genes; they inherit one copy from each parent. A genetic disease
occurs when one or both parents pass a mutated gene to a child at conception. A genetic disease
can also occur through a spontaneous gene mutation, meaning neither parent carries a copy of the
mutated gene. Once a spontaneous gene mutation has occurred in a person, that person can pass
the gene mutation on to a child.

Risk Factors

Men and women develop Wilson disease at equal rates. About one in 30,000 people have Wilson
disease. Symptoms usually appear between ages 5 and 35; however, new cases have been
reported in people ages 3 to 72.

A person’s risk of being a carrier or having Wilson disease increases when his or her family has
a known history of Wilson disease. Some people may not know about a family history of the
condition because the mutation is often passed to a child by a parent who is a carrier. A person’s
chances of having Wilson disease increase if a health care provider has diagnosed one or both
parents with the condition.
Pathophysiology

Copper is needed by the body for a number of functions, predominantly as a cofactor for a

number of enzymes such as ceruloplasmin, cytochrome c oxidase, dopamine β-
hydroxylase, superoxide dismutase and tyrosinase.
Copper enters the body through the digestive tract. A transporter protein on the cells of the small
bowel, copper membrane transporter 1 , carries copper inside the cells, where some is bound
to metallothionein and part is carried by ATOX1 to an organelle known as the trans-Golgi
network. Here, in response to rising concentrations of copper, an enzyme called ATP7A releases
copper into the portal vein to the liver. Liver cells also carry the CMT1 protein, and
metallothionein and ATOX1 bind it inside the cell, but here it is ATP7B that links copper
to ceruloplasmin and releases it into the bloodstream, as well as removing excess copper by
secreting it into bile. Both functions of ATP7B are impaired in Wilson's disease. Copper
accumulates in the liver tissue; ceruloplasmin is still secreted, but in a form that lacks copper
(termed apoceruloplasmin) and is rapidly degraded in the bloodstream.

Signs and Symptoms

The signs and symptoms of Wilson disease vary, depending on what organs of the body are
affected. Wilson disease is present at birth; however, the signs and symptoms of the disease do
not appear until the copper builds up in the liver, the brain, or other organs.

When people have signs and symptoms, they usually affect the liver, the central nervous system,
or both. The central nervous system includes the brain, the spinal cord, and nerves throughout the
body. Sometimes a person does not have symptoms and a health care provider discovers the
disease during a routine physical exam or blood test, or during an illness. Children can have
Wilson disease for several years before any signs and symptoms occur. People with Wilson
disease may have

 liver-related signs and symptoms

 central nervous system-related signs and symptoms
 mental health-related signs and symptoms
 other signs and symptoms
Liver-related Signs and Symptoms

People with Wilson disease may develop signs and symptoms of chronic, or long lasting, liver
disease:

 weakness
 fatigue, or feeling tired
 loss of appetite
 nausea
 vomiting
 weight loss
  pain and bloating from fluid accumulating in the abdomen
 edema—swelling, usually in the legs, feet, or ankles and less often in the hands or
face
 itching
 spiderlike blood vessels, called spider angiomas, near the surface of the skin
 muscle cramps
 jaundice, a condition that causes the skin and whites of the eyes to turn yellow

Some people with Wilson disease may not develop signs or symptoms of liver disease until they
develop acute liver failure—a condition that develops suddenly.

Central Nervous System-related Signs and Symptoms

Central nervous system-related symptoms usually appear in people after the liver has retained a
lot of copper; however, signs and symptoms of liver disease may not be present. Central nervous
system-related symptoms occur most often in adults and sometimes occur in children.Signs and
symptoms include

 tremors or uncontrolled movements

 muscle stiffness
 problems with speech, swallowing, or physical coordination

Mental Health-related Signs and Symptoms

Some people will have mental health-related signs and symptoms when copper builds up in the
central nervous system. Signs and symptoms may include

 personality changes
 depression
 feeling anxious, or nervous, about most things
 psychosis—when a person loses contact with reality
Other Signs and Symptoms

Other signs and symptoms of Wilson disease may include

 anemia, a condition in which red blood cells are fewer or smaller than normal, which
prevents the body’s cells from getting enough oxygen
 arthritis, a condition in which a person has pain and swelling in one or more joints
 high levels of amino acids, protein, uric acid, and carbohydrates in urine
 low platelet or white blood cell count
 osteoporosis, a condition in which the bones become less dense and more likely to
fracture
Complications

People who have Wilson disease that is not treated or diagnosed early can have serious
complications, such as

 cirrhosis—scarring of the liver

 kidney damage—as liver function decreases, the kidneys may be damaged
 persistent nervous system problems when nervous system symptoms do not resolve
 liver cancer—hepatocellular carcinoma is a type of liver cancer that can occur in
people with cirrhosis
 liver failure—a condition in which the liver stops working properly
 death, if left untreated
Diagnosis

To diagnose Wilson disease, including the following:

 medical and family history

 physical exam
 blood tests
 urine tests
 liver biopsy
 imaging tests
Medical and Family History

A health care provider may take a medical and family history to help diagnose Wilson disease.

Physical Exam

A physical exam may help diagnose Wilson disease. During a physical exam, a health care
provider usually

 examines a patient’s body

 uses a stethoscope to listen to sounds related to the abdomen

A health care provider will use a special light called a slit lamp to look for Kayser-Fleischer
rings in the eyes.

Blood Tests

A nurse or technician will draw blood samples at a health care provider’s office or a commercial
facility and send the samples to a lab for analysis. A health care provider may
 perform liver enzyme or function tests—blood tests that may indicate liver
abnormalities.
 check copper levels in the blood. Since the copper is deposited into the organs and is
not circulating in the blood, most people with Wilson disease have a lower-than-
normal level of copper in the blood. In cases of acute liver failure caused by Wilson
disease, the level of blood copper is often higher than normal.
 check the level of ceruloplasmin—a protein that carries copper in the bloodstream.
Most people with Wilson disease have a lower-than-normal ceruloplasmin level.
 conduct genetic testing. A health care provider may recommend genetic testing in
cases of a known family history of Wilson disease.
Urine Tests

24-hour urine collection. A patient will collect urine at home in a special container provided by
a health care provider’s office or a commercial facility. A health care provider sends the sample
to a lab for analysis. A 24-hour urine collection will show increased copper in the urine in most
patients who have symptoms due to Wilson disease.

Liver Biopsy

A liver biopsy is a procedure that involves taking a small piece of liver tissue for examination
with a microscope for signs of damage or disease. The health care provider may ask the patient
to stop taking certain medications temporarily before the liver biopsy. He or she may also ask the
patient to fast—eat or drink nothing—for 8 hours before the procedure.

Parameter Normal Wilson's

Plasma
>200 mg/l <200 mg/l
caeruloplasmin
Urine Cu <0.6 µmol/24 h >1.6 µmol/24 h
Hepatic copper < 250 µg/g dry wt > 250 µg/g dry wt
Kayser-Fleischer Present in neurological cases,
Absent
rings but may be absent in younger children
Cerebral imaging
Normal May be abnormal
(MRI)
 

Imaging Tests

A health care provider may order imaging tests to evaluate brain abnormalities in patients who
have nervous system symptoms often seen with Wilson disease, or in patients diagnosed with
Wilson disease. Health care providers do not use brain imaging tests to diagnose Wilson disease,
though certain findings may suggest the patient has the disease.
Magnetic resonance imaging (MRI). An MRI is a test that takes pictures of the body’s internal
organs and soft tissues without using x-rays. A specially trained technician performs the
procedure in an outpatient center or a hospital, and a radiologist—a doctor who specializes in
medical imaging—interprets the images. The patient does not need anesthesia, though people
with a fear of confined spaces may receive light sedation, taken by mouth. An MRI may include
the injection of a special dye, called contrast medium. With most MRI machines, the patient will
lie on a table that slides into a tunnel-shaped device that may be open ended or closed at one end.
Some machines allow the patient to lie in a more open space. The technician will take a sequence
of images from different angles to create a detailed picture of the brain. During sequencing, the
patient will hear loud mechanical knocking and humming noises. MRI can show if other diseases
or conditions are causing the patient’s neurological symptoms.

Computerized tomography (CT) scan. A CT scan uses a combination of x-rays and computer
technology to create images. For a CT scan, a health care provider may give the patient a
solution to drink and an injection of contrast medium. CT scans require the patient to lie on a
table that slides into a tunnel-shaped device where a technician takes the x-rays. An x-ray
technician performs the procedure in an outpatient center or a hospital. A radiologist interprets
the images. The patient does not need anesthesia. A CT scan can show if other diseases or
conditions are causing the patient’s neurological symptoms.

Treatment

Wilson disease with a lifelong effort to reduce and control the amount of copper in the body.
Treatment may include

medications
 changes in eating, diet, and nutrition
 a liver transplant
Medications

The medications have different actions that health care providers use during different phases of
the treatment.

Chelating agents. Chelating agents are medications that remove extra copper from the body by
releasing it from organs into the bloodstream. Once the copper is in the bloodstream, the kidneys
then filter the copper and pass it into the urine. A health care provider usually recommends
chelating agents at the beginning of treatment. A potential side effect of chelating agents is that
nervous system symptoms may become worse during treatment. The two medications available
for this type of treatment include

 trientine (Syprine)—the risk for side effects and worsening nervous system symptoms
appears to be lower with trientine than d-penicillamine. Researchers are still studying
the side effects; however, some health care providers prefer to prescribe trientine as
the first treatment of choice because it appears to be safer.
  d-penicillamine—people taking d-penicillamine may have other reactions or side
effects, such as
o fever
o a rash
o kidney problems
o bone marrow problems

A health care provider will prescribe a lower dose of a chelating agent to women who are
pregnant to reduce the risk of birth defects. A health care provider should consider future
screening on any newborn whose parent has Wilson disease.

Changes in Eating, Diet, and Nutrition

People with Wilson disease should reduce their dietary copper intake by avoiding foods that are
high in copper, such as

 shellfish
 liver
 mushrooms
 nuts
 chocolate

People should not eat these foods during the initial treatment and talk with the health care
provider to discuss if they are safe to eat in moderation during maintenance treatment.

People with Wilson disease whose tap water runs through copper pipes or comes from a well

Liver Transplant

A liver transplant may be necessary in people when

 cirrhosis leads to liver failure

 acute liver failure happens suddenly
 treatment is not effective

A liver transplant is an operation to remove a diseased or an injured liver and replace it with a
healthy one from another person, called a donor. A successful transplant is a life-saving
treatment for people with liver failure.

Most liver transplants are successful. About 85 percent of transplanted livers are functioning
after 1 year.Liver transplant surgery provides a cure for Wilson disease in most cases.
Prevention

A person cannot prevent Wilson disease; however, people with a family history of Wilson
disease, especially those with an affected sibling or parent, should talk with a health care
provider about testing. A health care provider may be able to diagnose Wilson disease before
symptoms appear. Early diagnosis and treatment of Wilson disease can reduce or even prevent
organ damage.

People with a family history of the disease may also benefit from genetic testing that can identify
one or more gene mutations. A health care provider may refer a person with a family history of
Wilson disease to a geneticist—a doctor who specializes in genetic diseases.

Comparison between patient and book picture

Book Picture Patient Picture

Etiology Etiology

 Genetics  Genetic

Signs and Symptoms Signs and Symptoms

Liver related Symptoms Liver related Symptoms

 weakness  weakness
 fatigue, or feeling tired  fatigue, or feeling tired
 loss of appetite  loss of appetite
 nausea  nausea
 vomiting  vomiting
 weight loss  weight loss
 pain and bloating from fluid  pain and bloating from fluid
accumulating in the abdomen accumulating in the abdomen
 edema—swelling, usually in the  itching
legs, feet, or ankles and less often in  muscle cramps
the hands or face
 jaundice
 itching
CNS related Symptoms
 spiderlike blood vessels, called  tremors or uncontrolled movements
spider angiomas, near the surface of
 muscle stiffness
the skin
 problems with speech, swallowing,
 muscle cramps
or physical coordination
 jaundice, a condition that causes the
 Dystonia
 skin and whites of the eyes to turn  Dysarthia
yellow Mental health related symptoms
CNS related Symptoms  personality changes
 tremors or uncontrolled movements  feeling anxious
 muscle stiffness Other Symptoms
 problems with speech, swallowing,  low platelet or white blood cell
or physical coordination count
Mental health related symptoms
 Golden-brown eye discoloration
 personality changes
(Kayser-Fleischer rings)
 depression
 feeling anxious, or nervous, about
most things
 psychosis—when a person loses
contact with reality
Other Symptoms
 Anemia
 Arthritis
 high levels of amino acids, protein,
uric acid, and carbohydrates in urine
 low platelet or white blood cell
count
 osteoporosis
 Golden-brown eye discoloration
(Kayser-Fleischer rings)
Diagnosis Diagnosis

 History and Physical examination  History and Physical examination

 24 hrs urine  24 hrs urine
 Copper accumulation in plasma  Copper accumulation in plasma
 Liver biopsy  Hb, DLC, Na+, Albumin and
 MRI/ CT Globulin reduced
 Copper isotope studies  PCV, PT/INR, BU, Billirubin, AST,
 Blood Studies ALP, SAP, GGTP increased

Management Management

 Chelating agent  Syp Gutclear 30mL PO TDS

 Zinc
 Nutritional therapy  Syp Zinc Acetate 50mg PO TDS
 Liver transplantation  T Baclofen XL 20mg PO BD
 T. Pacitane 2mg PO BD
  Inj Optineuron 1 amp OD
 Inj Albumin 5 % 250mg IV OD @
50mL/hr

THEORY APPLICATION:
On the assessment of the patient, I came to know that the Physiological, Psychological,
Spiritual, Moral and sociological needs of the patient are not met. The patient is not able to
breathe adequately, fear and anxiety regarding disability is present and patient is not able to
feed himself adequately. So, I applied “Virginia Henderson Need Theory”

VIRGINIA HENDERSON’S NEED THEORY

“Nursing theories mirror different realities, throughout their development; they reflected the
interests of nurses of that time.”

INTRODUCTION
Throughout the development of nursing as a field of specialization, there were numerous
theories formulated by nurse scientists to explain the nature of nursing as a distinct science or
profession. Virginia Henderson was a nurse theorist who has contributed significantly to
nursing practice and nursing field. He has been called “The First Lady of Nursing &The First
Truly International Nurse”because of his writing research presentation.

ASSUMPTION OF THE THEORY

 Nurse’s care for patient until patient can care for themselves once again.
 Patients desire to return to health, but this assumption is not explicitly stated.
 Nurses are willing to serve and that “nurses will devote themselves to the patient day and
night.
 Henderson also believes that the “mind and body are inseparable and are interrelated.”

HENDERSON’S THEORY AND THE 4 MAJOR CONCEPT (METAPARADIGMS)

INDIVIDUAL

 Henderson states that individuals have basic needs that are component of health and require
assistance to achieve health and independence or a peaceful death. According to his, an
individual achieves wholeness by maintaining physiological and emotional balance.
 He defined the patient as someone who needs nursing care, but did not limit nursing to illness
care. His theory presented the patient as a sum of parts with biopsychosocial needs and the
mind and body are inseparable and interrelated.
 ENVIRONMENT

 Although the Need Theory did not explicitly define the environment, Henderson stated that
maintaining a supportive environment conducive for health is one of the elements of his 14
activities for client assistance.
 Henderson’s theory supports the tasks of the private and the public health sector or agencies
in keeping the people healthy. He believes that society wants and expects the nurse’s service
of acting for individuals who are unable to function independently.

HEALTH

 Although not explicitly defined in Henderson’s theory, health was taken to mean balance in
all realms of human life. It is equated with the independence or ability to perform activities
without any aid in the 14 components or basic human needs.
 Nurses, on the other hand, are key persons in promoting health, prevention of illness and
being able to cure. According to Henderson, a good health is a challenge because it is
affected by numerous factors such as age, cultural background, emotional balance, and
others.

NURSING

 Henderson wrote his definition of nursing before the development of theoretical

nursing. The nurse’s goal is to make the patient complete, whole, or independent. In turn,
the nurse collaborates with the physician’s thistherapeutic plan.
 Nurses temporarily assist an individual who lacks the necessary strength, will, and
knowledge to satisfy one or more of the 14 basic needs. He states: “The nurse is
temporarily the consciousness of the unconscious, the love life for the suicidal, the leg of
the amputee, the eyes of the newly blind, a means of locomotion for the infant,
knowledge and confidence of the young mother, the mouthpiece for those too weak or
withdrawn to speak”

THE 14 COMPONENTS OF HENDERSON THEORY

 PHYSIOLOGICAL

 Breath Normally
 Eat and Drink Adequately
 Eliminate Body waste
 Moves & maintain desirable position
 Sleep & Rest
 Select suitable clothes-dress and undress
 Maintain body temperature within normal range by adjusting clothes and modifying
environment
 Keep the body clean and well groomed and protect the integument
 Avoid dangers in the environment and avoid injuring others

 PSYCHOLOGICAL ASPECTS OF COMMUNICATING & LEARNING

 Communicate with others in expressing emotions, needs, fears, or opinions.

  Learn, discover, or satisfy the curiosity that leads to normal development and health and
use the available health facilities.

 SPIRITUAL & MORAL

 Worship according to one’s faith

 SOCIOLOGICALLY ORIENTED TO OCCUPATION AND RECREATION

 Work in such a way that there is sense of accomplishment

 Play or participate in various forms of recreation

APPLICATION OF THEORY

 Name of Patient : Mr. Anuj Thirthani

 Age : 29 Years
 Sex : Male
 Ward & Bed No. : HDU (19) ILBS
 Admission No. : 44394
 Address : Swasthya Vihar, New Delhi
 Date of Admission : 02/07/2018
 Religion : Hindu
 Educational Qualification : Graduated
 Marital status : Unmarried
 Occupation : Freelance Photographer
 Diagnosis : Wilson’s Disease (Hepatolenticular
Degeneration)
 Theory applied: Virginia Henderson Need Theory

CONCEPT THEORY APPLICATION TO PATIENT

 Breathing pattern
 Eat and Drink
 Eliminate body waste
 Move and maintain
desirable posture
 Sleep and Rest
CONDITION
 Patient is not able to breath independently, he is very
anxious and in hepatic encephalopathy grade-2
 Patient is not able to eat due to anxiety, dystonia
 Patient has Constipation
 Activity level is decreased as patient is not able to move
independently due to muscular weakness, dystonia and
dysarthia
 Patient is advised for complete bed rest as hyperactivity

 Maintain body
temperature within
normal range by
adjusting clothing and
modifying environment
 Avoid dangers in the
environment and avoid
injuring others
furthers causes respiratory distress
 Patient is wearing cotton clothes to adjust his body
temperature according to environment
 As patient is conscious, disoriented. He is in HE, health
care team has to be alert to prevent patient from any
danger or fall and side-rails are also kept up 24 hours

 PSYCHOLOGICAL ASPECTS

 Communicate with others in  Patient is not able to communicate with health

expressing emotions, needs, care workers, he is very irritable, agitated and
fears and opinions not cooperate with health care team workers.

Nursing Diagnosis:-
1. Hyperthermia related to inflammatory damage due to progressive extrahepatic biliary
duct.
2. Ineffective breathing pattern related to an increase in abdominal distension.
3. Imbalanced Nutrition: Less Than Body Requirements related to anorexia and impaired
absorption of fat.
4. Impaired bowel elimination (diarrhea) related to intestinal malabsorption.
characterized by liquid stool, increased frequency of bowel movements (more than 3
times daily), increased bowel sounds.
5. Impaired skin integrity related to accumulation of bile salts and pruritis
characterized by pruritis.
6. Deficient fluid volume related to nausea and vomiting.
7. Altered skin colour related to accumulation of the bile secondary to fibrosis of the biliary
tract
8. Activity intolerance related to liver dysfunction
9. Disturbed thought processes related to HE
10. Low self-esteem related to disease condition
11. Risk for infection related to prolonged hospitalization
12. Anxiety and knowledge deficit related to disease condition as evidenced by verbalisation
13. Abnormal behavioural related to disease condition
14. Risk for adherence to treatment regimen
Assessment Nursing Diagnosis Goals Interventions
Increased  Hyperthermia relat To maintain body Assess the
temperature- ed to inflammatory temperature of the condition of patient.
101F damage due to patient  Monitor the vital
progressive sign of patient.
extrahepatic biliary Specially
duct and surgical Temperature.
procedure  Assess the
underlying condition
and body
temperature.
Give tepid
sponging to patient.
 Keep patient in
cool environment.
 Give the
antipyretic to patient.
 Assess neurologic
response, notify level of
consciousness and
orientation, reaction to
stimuli and presence of
posturing seizure
Secretions Ineffective To improve Elevate the head
present breathing pattern breathing pattern of the bed 30degrees.
related to an Position the patient
increase in
in a lateral or semi-
abdominal
distension. prone position.
Suctioning should
also be done.
Chest
Implementation Evaluation
Condition of the Exhibits normal
patient assessed. body temperature-37
Vital signs degrees.
monitored.
Temp- 38 degrees.
PR- 122/min
RR- 42/min
Tepid sponging
given to the patient.
Patient kept in a
cool and calm
environment.
Head of the bed Shows normal
elevated to 30 degrees, breathing pattern
Suctioning done every
8th hourly and when
needed.
Chest physiotherapy
and postural drainage
 physiotherapy  given.
&postural drainage Chest auscultated for
may be initiated. lung sounds and bilateral
air entry.
Auscultate the chest
every 8hours. ET secretion sent to
 Send secretion’s lab for culture.
culture and sensitivity for
lab test
Not able to take Imbalanced To maintain Assess the Condition of the Normal nutrition
orally Nutrition: Less normal nutrition of condition of patient. patient assessed. level maintained.
Than Body the patient  Insert Ryles tube. Ryles tube
Requirements relate  Check the bowel inserted.
d to anorexia and sound of patient. Bowel sounds
impaired absorption  Give extra sugar checked-normal.
of fat. with feed.
 Give ryles tube Ryles tube feed 50ml
feed.. every 2 hrly given.
 Aspirate before Aspirate checked
giving each feed. before each feed.
 Note the colour
and amount of
aspirant.

Inability to take Deficient fluid To maintain fluid Hydration status Skin turgor assessed.  maintains
fluids by mouth volume related to balance and should be assessed. IVF ½ DNS adequate fluid
nausea and managing Administering the administered. balance
vomiting. nutritional needs
required IV fluid. For Has no
Intake output chart
patient with intra- maintained. c l i n i c a l signs
cranial conditions, IV or symptoms
solutions must be of dehydration
administered slowly. Ryles tube feeding given.
 Monitor Intake and
output of patient Foleys catheterization
carefully. done.
Administer Ryles’s
tube feeding .
Foley’s
Catherterization done.

Increased Impaired skin To maintain skin Regular turn by Patient positioned Exhibits improved
Bilirubin levels integrity related to integrity side every 2nd hourly. skin integrity.
accumulation Patient side by
of bile salts and Dragging and pulling
side Every 2nd hourly.
pruritis avoided.
characterized by After turning, the
pruritis. patient should be Air mattress u sed.
repositioned
carefully. S. Bilirubin checked
Dragging or daily to assess the
pulling the patient bilirubin levels.
should be avoided.
Maintain correct
body position and
passive exercise.
. Use of trochanter
rolls.
Fluidized or low-
air-loss beds may be
use.
Avoid soiling of bed
sheets.
 Avoid dehydration of
skin or dryness of skin.
Use coconut oil or
moisturizer to prevent dryness
of skin.
Surgical Risk for infection To reduce risk of  Assess the Condition of the patient Risk of infection
procedure and infection condition of patient. assessed. reduced.
related to
prolong  Monitor vital sign Vital signs checked.
hospitalization prolonged of patient.
 Use sterile Aseptic techniques used
hospitalization
technique . before touching and
 Hand-washing doing every procedure.
before and after Hand washing promoted.
procedure Vaccination of the
 Monitor white patient checked which
blood count (TLC) of was incomplete
patient. according to age because
 Assess the of disease condition.
vaccination status of Antibiotics given i.e.
patient. Inj Meropenem, Inj
 Monitor the Collistin and
Possible sign and Inj.Levoflox.
symptom of
infection.
 Administer
antibiotic to prevent
infection.

Family Anxiety and To reduce anxiety Assess the knowledge  knowledge level of Exhibits decrease
members looks and increase level of family members. family members level of anxiety and
knowledge deficit
anxious and knowledge level Provide knowledge assessed. increase knowledge
worried related to disease of family members regarding the disease  knowledge regarding
condition, etiology, the disease condition,
condition as management and the etiology, management
prognosis. and the prognosis
evidenced by
Provide psychological Provided.
verbalization support to the family  Psychological support
members. to the family members.
Emphasize on the Emphasize on the follow
follow ups after ups after discharge
discharge. provided.
 EVALUATION OF DAILY PROGRESS

DATE AND CONDITION OF PATIENT

DAY
 09/7/18  Patient was conscious but disoriented.
 1st Day  He was highly irritable, agitated and anxious.
 He constantly wants his mother to be with himself.
 He had dystonia, dysarthria and tremors in right hand.
 He had fever
 He was in HE grade 2

 10/07/18  He was conscious and oriented.

 2nd Day  He was afebrile
 He was cooperative as compared to previous day
 His investigations were better as compared to previous day
but still needs more improvement.

 11/07/18  He was conscious and oriented.

 3rd Day  He was afebrile
 He was cooperative as compared to previous days
 Improvements were distinguished
 Behaviour improved.
 Effects of HE also reduced.
 Dystonia and Dysarthria still present.
 Patient is hydrated and saturation maintained on room
atmosphere.
 Vital sign monitored.
 Nebulization given

Health Education
(Given to family members of the patients)
1. Life style modification :
 Educate to Patient’s relative about life style modification.
 Educate about food and water sanitation.
 Educate to about disease condition, etiology, prevention, surgical procedure
done and prognosis.
 Emphasis on hand hygiene.
 Educate to maintain balance between activity and rest to prevent himself from
fatigue or inability to perform ADL.
  Educate about importance of balanced and healthy diet.

2. Medication :
 Educate to adhere therapeutic regimen with special emphasis on the methods of
administration, rationale and side effects of the prescribed immunosuppressive
agents.
 Provide written as well as verbal instructions about how and when to take the
medicines.

3. Dietary modification :
 Provide a special diet plan for formula fed infants which includes vitamins-
especially fat-soluble vitamins-and medium-chain triglyceride (MCT) oil. MCT
oil adds calories to foods and is easier to digest without bile than other fats.
 Advise to monitor his weight and blood pressure on regular basis.
 Advise to reduce salt and sodium intake to maintain normal blood pressure.
 Advised to use a diet that is low in saturated fat, total fat and dietary
cholesterol.
 Foods containing copper to be eliminated.
 Not eating shellfish
 Not eating liver
 Limiting or not eating mushrooms
 Limiting or not eating nuts
 Limiting or  not eating dried fruits
 Limiting or not eating chocolate
 Not taking multivitamins that have copper

4. Personal hygiene :
 Educate about the importance of maintaining personal hygiene.
 Encourage to perform self-care activities from unaffected side.
 Encourage to perform oral hygiene on regular basis.
 Encourage for daily bath and change of clothes.
 Educate family members to take care of environmental hygiene also.

5. Psychological support :
 Encourage them to share feeling and concern with family and friends.
 Encourage regarding positive self-image.
 Encourage family members for family support and care.

6. Follow- up :
 Teach family members about medication, its use and importance and tell
patient to take medication daily.
  Teach about various side-effects of drugs and advise his to inform physician
when these side-effects arise.
 Advise for routine blood pressure and weight measurement and also to
maintain a dairy for same and to bring this dairy to doctor on appointment.
 Advise for routine check-up and follow-up.

Summary

I Mr. Deepak, MSc. Nursing 2nd Year was posted in HDU, from 09/04/2018-12/07/2018.
There I took this patient (Anuj Thirthani) for my Case Presentation was suffering from
Wilson’s Diseaseadmitted on 02ndApril 18 as follow up case of Wilson’s Diseases, as
dystonia, dysarthria, tremors, abnormal behaviour, agitation, vomiting and loss of appetite
develops and got admitted to ILBS.
I provided him care for 5days, and made this case presentation. In this case study I
discussed about the patient’s baseline data, history of present and past illness, dietary
history, family history, patient physical and systemic examination, diagnosis and
management of disease.
The comparison is made between the patients finding and book findings including causes,
risk factors, sign and symptoms, diagnostic evaluation and management strategies.
I have applied Virginia Henderson Theory of need of the patient. It focuses on basic
need of the patientbased on which nursing management was made.

Conclusion
I am very thankful to Ms Sarita, Lecturer ILBS CON who gave me this opportunity to
learn about this interesting case. I gained very good experience to provide care to these
patients.
Bibliography -

 Smeltzer,S.C,Bare,B.G,Hinkle,J.L,Cheever,K.H, (2010),Brunner and Suddarth’s

Textbook of Medical Surgical Nursing (12th ed.),LippincottWilliams and Wilkins,
1165-1167.
 Lewis (2004). Medical and Surgical nursing 10th ed., Lewis Blicher Heitkemper
Harding 1267-1272
 https://www.mayoclinic.org/diseases-conditions/wilsons-disease/symptoms-
causes/syc-20353251
 https://emedicine.medscape.com/article/183456-overview
 https://en.wikipedia.org/wiki/Wilson%27s_disease
 https://www.niddk.nih.gov/health-information/liver-disease/wilson-disease
 https://ghr.nlm.nih.gov/condition/wilson-disease
 https://patient.info/health/abnormal-liver-function-tests-leaflet/wilsons-disease