DELA CRUZ JESTIAN RYLL B.

BSN-IV
CASE STUDY ON HEPATIC FAILURE: WILSON’S DISEASE
PART I. BASED ON THE VIDEO PRESENTED ANSWER THE FOLLOWING:
1.  the medical diagnosis of Sarah?
 Wilson’s disease is a rare inherited disorder that causes copper to accumulate in your
liver, brain and other vital organs.Copper plays a key role in the development of healthy
nerves, bones, collagen and the skin pigment melanin. Normally, copper is absorbed
from your food, and excess is excreted through a substance produced in your liver (bile).
But in people with Wilson’s disease, copper isn’t eliminated properly and instead
accumulates, possibly to a life-threatening level. When diagnosed early, Wilson’s
disease is treatable, and many people with the disorder live normal lives.
2. List down the possible risk factors
 A mutation in the ATP7B gene, which codes for copper transportation, causes Wilson’s
disease. You must inherit the gene from both parents in order to have Wilson’s disease.
This can mean that one of your parents has the condition or carries the gene.
3. Summarize the signs and symptoms that were mentioned in the video.
 The patient’s signs and symptoms were jaundice, back pain, anasarca, pruritus, and
weakness.
4. Prepare a Medical-Surgical treatment plan including the appropriate diagnostic procedures for
Sarah. Elaborate each treatment and emphasize your nursing responsibilities.
 Tests and procedures used to diagnose Wilson's disease include:
 Blood and urine tests. Blood tests can monitor liver function and check the level of a
protein that binds copper in the blood (ceruloplasmin) and the level of copper in the
blood. A blood test can also identify the genetic mutations that cause Wilson’s disease.
Knowing the mutations in your family allows doctors to screen siblings and begin
treatment before symptoms arise. The doctor also might want to measure the amount
of copper excreted in the urine during a 24-hour period.
 Nursing Responsibilities for Urine Test:
 Explain to the patient that this kind of urine collection involves first voiding
approximately one half of the urine into the toilet, urinal, or bedpan, then
collecting a portion of midstream urine in a sterile container, and allowing the
rest to be pass into the toilet. Discuss that this is done to detect the presence or
absence of infecting organisms and, therefore, must be free from contaminating
matter that may be present on the external genital areas.
 If it is necessary to measure the total amount of soluble substances excreted in
a 24-hour period, a strictly timed 24-hour specimen is required, because many
soluble substances exhibit diurnal variations. Collect the specimen in one or
more disposable, wide-mouthed, clean plastic container(s) with a plastic lid
large enough to hold about 3 L.  Amber-colored containers may be required for
light-sensitive analytes. Determine if the collection will require a preservative,
ensuring the collection container has the appropriate preservative, at the
correct concentration, along with a warning label indicating the preservative in
use. Label the collection container including the patient identification (name
and hospital number), test(s) required, and preservative used. The start date
and time plus the finish date and time should be recorded on the container and
requisition at the beginning and end of the collection period. The 24-hour
 collection should begin by having the patient empty his or her bladder or
catheter bag at a fixed time and discard the specimen.  Record this start date
and time on the collection container and on the laboratory requisition. If a
preservative is required, the patient must be advised to collect the urine in a
separate clean container and then carefully transfer the urine to the collection
container that will be transported to the laboratory.  Comment:  Assume that all
preservatives are hazardous (most are). Instruct the patient  to collect all voided
urine during the 24-hour collection period and add it to the collection container.
 The collection should end exactly 24 hours after it began, by having the patient
empty his or her bladder, or catheter bag, and adding this specimen to the
collection container. Record the ending date and time on the collection
container and on the laboratory requisition. Carefully seal the cap tightly so as
to avoid leakage.
 Nursing Responsibilities for Blood Test:
 Explain to the patient the procedure and obtain informed consent. Monitor
patients during testing. Patients are monitored by nurses during diagnostic
testing. This includes monitoring their current medical condition, especially in
those patients deemed unstable. 
 Preparing a patient for diagnostic testing is a nurse’s responsibility. Drawing
blood or administering medication prior to testing is one way nurses help
prepare patients. Another way they help is by collecting specimens, such as
sputum or urine samples, and sending them to the lab. Patients may need to
have an area sterilized or shaved for a surgical procedure or the application of
electrodes. Nurses assist with mentally preparing patients for testing by
answering questions, explaining the procedure or test and possible outcomes.
 Patients are monitored by nurses during diagnostic testing. This includes
monitoring their current medical condition, especially in those patients deemed
unstable. They must check a patient’s vital signs (blood pressure, pulse,
breathing rate), assess physical condition and keep an eye on any monitors that
the patient needs to remain hooked up to during the tests, such as a heart
monitor or ventilator. Nurses may also be required to connect or disconnect any
monitors or devices that can interfere with the testing.
  It may be the nurse’s responsibility to check for the results of the tests as well.
They may be in charge of entering the results into the patient’s medical record.
Nurses must also notify the patient’s physician when abnormal or critical results
that require an immediate response, such as abnormal blood work with critical
potassium levels, are found.
 Eye exam. Using a microscope with a high-intensity light source (slit lamp), an
ophthalmologist checks your eyes for Kayser-Fleischer rings, which is caused by excess
copper in the eyes. Wilson's disease also is associated with a type of cataract, called a
sunflower cataract, that can be seen on an eye exam.
 Nursing Responsibilities
 Explain the procedure to the patient and secure informed consent. Educate the
patient of the Kayser-Fleischer rings that they may see in their eye. Explain that
it is formed by the deposition of copper in the Descemet membrane in the
limbus of the cornea. The color may range from greenish gold to brown. They
are well-developed rings may be readily visible to the naked eye or with an
ophthalmoscope set at +40. When not visible to the unaided eye, the rings may
 be identified using slit-lamp examination or gonioscopy. Kayser–Fleischer rings
are seen in most of the patients with neurologic involvement from Wilson
disease. However, it may not be seen in approximately 5% of these patients.
They are present in only 50% of the patients with isolated hepatic involvement
and in pre-symptomatic patients.
 Removing a sample of liver tissue for testing (biopsy). Your doctor inserts a thin needle
through your skin, into your liver and draws a small sample of tissue. A laboratory tests
the tissue for excess copper.
 Nursing Responsibilities
 Explain the procedure to the patient and secure informed consent. Before to
the procedure, the patient may be asked to stop taking medications and
supplements that can increase the risk of bleeding, including: Aspirin, ibuprofen
(Advil, Motrin IB, others) and certain other pain relievers. Blood-thinning
medications (anticoagulants), such as warfarin. Certain dietary supplements that
may increase the risk of uncontrolled bleeding
 Instruct the patient to remain NPO 8 hours prior to procedure. Educate the
patient that a the type of liver biopsy will vary depend if the patient have a
history of or likelihood of bleeding problems or blood-clotting disorders, might
have a tumor involving blood vessels in the liver. Have an abnormal amount of
fluid in your abdomen (ascites). The patient is very obese, and have a liver
infection. Also educate the patient that just before the biopsy, the patient will
have an IV line placed, possibly be given a sedative to help you relax during the
procedure, he/she will be advised to empty the bladder. After the biopsy, the
patient can expect to be taken to a recovery room, where a nurse will monitor
your blood pressure, pulse and breathing. The patient may rest quietly for two
to four hours, or longer if you had a transjugular procedure, he/she may feel
some soreness where the needle was inserted, which may last as long as a
week. Advice the patient to avoid lifting more than 10 to 15 pounds for one
week and that the patient may be able to get back to their usual activities
gradually over a period of a .

PART II.
Create your own video discussing/explaining the following to Sarah:
a. Nursing Responsibilities pre-post diagnostic procedures.
b. Your nursing responsibilities prior to OR.
c. Explain your plan of care for her.
d. Explain your nursing responsibilities prior to giving her medications.
e. Discuss to her your discharge planning.