KAWASAKI

o Kawasaki disease is an acute systemic vasculitis of unknown cause (Hockenberry et.al, 2019). This means that
it is an illness that causes inflammation in the blood vessels throughout the body.
o Children who are younger than 5 years old are the ones who are often affected with this disease (Hockenberry
et al, 2019).
o This illness is self-limiting and resolves in 6 to 8 weeks. However, 20-25% of children would develop cardiac
sequelae if no treatment has been done (Hockenberry et.al, 2019).

II. Statistics

A. Incidence Rate

 -  Kawasaki Disease is seen in children of most racial and ethnic backgrounds

(Hockenberry et.al, 2019). However, children of Asian or Pacific Island descent, such as Japanese or
Korean, have higher rates of Kawasaki disease (Mayoclinic, 2020).

 -  The incidence rate is estimated to be at 112 cases per 100,000 children, 76% of which are younger
than 5 years old (Hockenberry et.al, 2019).

B. Occurrence in Males and Females

- Kawasaki Disease occurs 1.5 to 1.7 times more frequently in males compared to females.

III. Etiology
The etiology of Kawasaki Disease remains unconfirmed. Although KD is not spread by person-to-person
contact, several factors support an infectious cause or trigger (Hockenberry et al, 2019).

1. Autoimmune
The body is induced to attack its own cells. Antibodies, the immune system's attack proteins, are
supposed to attack germ invaders like bacteria and viruses, but can sometimes turn on the body itself.
Antibodies seem to do the majority of the damage in Kawasaki disease. High levels of autoantibodies or
antibodies against the body are present in Kawasaki patients (Msd Manual, 2019).

2. Virus
A virus may trigger the autoimmune process. Evidence for a virus includes the fact that the disease tends
to be self-limited, occurs in clusters in certain geographical locations, tends to affect young children,
produces a fever and a rash, and is characterized by periodic epidemics that tend to move geographically
in a wavelike distribution. This is just how viruses behave. But it is not yet known what virus may cause
Kawasaki disease (Msd Manual,2019).

3. Genetic
Even though a virus may trigger this disease, only certain children develop it. Such children may have a
genetic predisposition that makes them vulnerable when infected by the virus that causes it. Another
clue that genes might be contributing is that Kawasaki disease disproportionately affects children of
Asian descent. Children with Japanese heritage are particularly affected. Males are more affected than
females, and most cases occur in children younger than five years old (Msd Manual,2019).

4. Vaccine-associated
Vaccines have been implicated in Kawasaki disease. Reports from FDA and others reported an
 increased risk for Kawasaki disease from the RotaTeq vaccine licensed for the prevention of rotavirus in
children.(NCBI, 2015)

However, recent studies show no association with rotavirus vaccine to the disease (Front. Pharmacol.,
2019). The Bacille Calmette-Guérin vaccine, used in some parts of the world to prevent tuberculosis, has
been noted to help in the diagnosis of Kawasaki disease in cases when the vaccine scar became red and
irritated. It is not clear that there is any causal relationship (NCBI, 2019).

IV. Risk Factors

The following factors would increase a child’s risk of developing Kawasaki disease (Mayoclinic, 2020):

o Age
Children who are under the age of 5 years old are the ones who are at most risk of Kawasaki disease.
o Sex
Boys are slightly more likely than girls to develop the disease.
o Ethnicity
Children of Asian or Pacific Island descent, such as Japanese and
Korean are found to be more likely to develop Kawasaki disease.

V. Complications
The most serious complication of Kawasaki Disease is the development of coronary aneurysms and the
potential for myocardial infarction in children with aneurysm formation (Hockenberry et al, 2019).

Kawasaki disease is self-limiting. However, without the proper treatment, approximately 20-25% of
children would develop cardiac sequelae. The most common sequelae is the damage towards the
coronary arteries, which is the blood vessels that would supply the heart muscle (Hockenberry et.al,
2019). This would then involve the dilation of the coronary arteries and or coronary artery aneurysm
formation (Hockenberry et.al, 2019).
Infants younger than 1 year of age are at the greatest risk for heart involvement and children who are
older than 5 years of age are also at an increased risk of developing coronary sequelae since KD is often
not suspected in older children thus leading to a delay in diagnosis and treatment (Hockenberry et.al,
2019).

LEUKEMIA

● Leukemia is a neoplastic proliferation of one particular cell type (granulocytes, monocytes,

lymphocytes [type of white blood cell involved in immune functions], or, infrequently,
erythrocytes or megakaryocytes).
● The leukemias are commonly classified according to the stem cell line involved, either
lymphoid (referring to stem cells that produce lymphocytes) or myeloid (referring to stem cells
that produce nonlymphoid blood cells).
● Leukemia is also classified as acute (abrupt onset) or chronic (evolves over months to years).
Its cause is unknown. There is some evidence that genetic influence and viral pathogenesis
may be involved.

II. Acute Lymphoblastic Leukemia (ALL)

● Acute lymphocytic leukemia (ALL) results from an uncontrolled proliferation of immature cells
(lymphoblasts) from the lymphoid stem cell.
 A. Development of ALL

● In healthy bone marrow, stem cells become mature, adult blood cells through the process called
“differentiation.” When an immature lymphoid blood cell in the bone marrow becomes damaged and
develops errors in its DNA, ALL develops.

● These genetic errors can give rise to a leukemic blast cell (lymphoblast) that is stuck in the earliest
stages of cell development. This immature blast cell cannot mature into a functioning blood cell.

● Genetic errors in the mutated cell tell the cell to keep growing and dividing when a healthy cell would
typically stop dividing and eventually die. Every cell that arises from the initial leukemia blast also has
the mutated DNA. As a result, the leukemia cells multiply uncontrollably. The leukemic blasts quickly
accumulate in the bone marrow, suppressing the development of normal, healthy blood cells. As a
result, there are too many leukemic blast cells that cannot function and too few mature, functioning
blood cells.

B. Risk factors

1. Previous exposure to chemotherapy and radiation therapy.

2. Genetic disorders including: Down syndrome, neurofibromatosis, Klinefelter syndrome, Fanconi anemia,
Schwachman-Diamond syndrome, Bloom syndrome and ataxia telangiectasia have been associated with an
increased risk of developing ALL.
3. Age. Children, adolescents or adults older than 70 years are at greater risk of developing ALL.
4. Gender. Men are more likely to develop ALL than women.
5. Race/ethnicity. In the United States, ALL is more common in Hispanics and whites.

C. Signs and Symptoms

● Patients with ALL do not have sufficient numbers of mature red blood cells, white blood cells and
platelets.

● Symptoms of a low red blood cell count (anemia) include

○ Fatigue
○ Shortness of breath during normal physical activities
○ Dizziness
○ Pale complexion

● Symptoms of a low white blood count (leukopenia) include:

○ Frequent infections
○ Fever

● Symptoms of a low platelet count (thrombocytopenia) include

○ Bruising easily
○ Prolonged bleeding from minor cuts
○ The appearance of pinhead-sized red spots on the skin, called “petechiae”
○ Frequent or severe nosebleeds
○ Bleeding gums
○ Blood in the urine.

● Other general symptoms of ALL include

○ Night sweats
○ Discomfort in bones or joints
○ Enlarged spleen, liver or lymph nodes
○ Pain or feeling of fullness below the ribs
○ Unexplained weight loss or loss of appetite.

D. Diagnostic Testing

1. Bone Marrow Aspiration and Biopsy

● These two procedures are generally done at the same time in a doctor's office or a hospital. For both
procedures, the patient is given medication to numb the area, or given a general anesthesia. The samples are
usually taken from the hip bone using specialized needles.
○ A bone marrow aspiration removes a liquid marrow sample
○ A bone marrow biopsy removes a small amount of bone filled with marrow

2. Complete Blood Count (CBC) with differential

● This test is used to measure the number of red blood cells, white blood cells and platelets in a sample
of blood. It measures the amount of hemoglobin in the red blood cells.
● The CBC should include a differential. The differential measures the numbers of the different types of
white blood cells in the sample.
● People with acute lymphoblastic leukemia (ALL) may have a high number of white blood cells and a
low number of red blood cells and platelets. This is because too many lymphoblasts are being made
in the bone marrow. These lymphoblasts crowd the bone marrow so that too few normal, healthy
blood cells are made.
● Even if CBC findings suggest leukemia, an ALL diagnosis is usually made only after a
hematopathologist has examined a sample of bone marrow cells.

3. Blood Chemistry Profile

● Blood chemistry test findings indicate how well a person’s kidneys, liver and other organs are
working. These test results, although not used to diagnose leukemia, may show an abnormal amount
of a particular substance in the blood that may be a sign of disease or some other health problem. A
blood chemistry profile also provides helpful information about any potential organ damage caused
by leukemia cells or ALL treatments.

4. Coagulation Test
● This is a blood test that measures how well the blood is able to clot and determines whether there are
deficiencies in some proteins, such as fibrinogen.

5. Cell Assessment
● A hematopathologist will examine a sample of blood cells or bone marrow cells under the
microscope to determine the size, shape, and type of cells as well as to identify other features of the
cells. A significant finding is the appearance of the cells—whether the cells look more like normal,
mature blood cells or more like abnormal, immature blood cells (blast cells).
● The percentage of blast cells identified in the blood sample is very important. Typically, there are no
blast cells in the blood and no more than 5 percent of the cells in the bone marrow are blast cells.
Generally, a diagnosis of ALL requires a finding that shows that 20 percent or more of the cells in the
bone marrow are lymphoblasts.

6. Flow Cytometry
● This test is used to classify cells in a blood sample. The classification is based on the types of
antigens, or markers/proteins, on the surface of the cells. The pattern of the surface proteins is called
the “immunophenotype.” A sample of bone marrow is often used for this test, but a blood sample
may also be used. The sample of cells is treated with special man made antibodies that only stick to
the cells if the cells have a specific antigen on them. The cells are then passed through a laser beam.
The cells with antibodies attached to them will give off light. Leukemia cells are different from healthy
cells; they can have different antigens on their surface depending on whether the cells are myeloid or
lymphoid and their stage of development. Flow cytometry helps to confirm an ALL diagnosis. It is also
used to determine the type of lymphocytes in which ALL originated and to assess the maturity of the
cells. Flow cytometry is also used to check treatment results.

7. Genetic Test

a. Cytogenetic Analysis ( Karyotyping)

● In this test a hematopathologist uses a microscope to examine the chromosomes inside of cells.
Karyotyping is used to look for abnormal changes in the chromosomes of the leukemia cells of
patients with ALL.
● In many cases of ALL, the chromosomes of leukemia cells have abnormal changes that can be seen
under a microscope such as translocations and extra chromosomes

b. Fluorescence in situ hybridization (FISH)

● This is a cytogenetic laboratory technique that is used to identify and examine genes or
chromosomes in cells and tissues. In cases of ALL, doctors use FISH to detect certain abnormal
changes in the chromosomes and genes of leukemia cells.

c. Polymerase Chain Reaction (PCR)

● A PCR is a very sensitive laboratory technique that is used to detect and measure some genetic
 mutations and chromosomal changes that are too small to be seen with a microscope. Polymerase
chain reaction testing essentially increases or “amplifies” small amounts of specific pieces of either
RNA (ribonucleic acid) or DNA to make them easier to detect and measure. This test can find a single
leukemia cell among more than 500,000 to one million normal cells. Polymerase chain reaction
testing is one method used to determine the amount of minimal residual disease (MRD), the small
amount of cancer cells left in the body after treatment. This testing can be done on a bone marrow or
a blood sample.

8. Spinal Fluid Test – Lumbar Puncture

● Acute lymphoblastic leukemia can spread to the cerebrospinal fluid (CSF), the fluid that flows around
the brain and spinal cord. In order to determine whether or not leukemia cells have spread to this
area, a sample of the CSF is tested. A lumbar puncture (also called a “spinal tap”) is a procedure that
is used to collect the CSF from the spinal column. After the area over the spine in the lower part of the
back has been numbed with a local anesthetic, a thin needle is inserted between two bones
(vertebrae) and into the CSF. A sample of the fluid is withdrawn and examined under a microscope to
look for leukemia cells that may have spread to the brain and spinal cord.

9. Imaging Tests

a. CT scan
● A CT scan may be used to look for enlarged lymph nodes, liver or spleen caused by an accumulation
of leukemia cells in the chest, abdomen and pelvis.

b. MRI Scan
● An MRI scan of the head and/or spinal cord should be done if a patient has symptoms (such as
headache or seizures) that suggest that ALL cells may have spread to the brain and spinal cord.

c. Ultrasonography
● Testicular involvement can occur so an ultrasound examination of the scrotum (the external sac that
contains the testicles) may be needed to see if a mass is present.

d. Echocardiogram
● Some treatments for ALL can damage the heart so the doctor may want to evaluate a patient’s heart
and cardiac function in order to plan the best treatment.

E. Acute Lymphoblastic Leukemia Subtypes

1. B-Cell Lymphoblastic Leukemia/Lymphoma

● B-cell acute lymphoblastic leukemia is a cancer that affects your "B lymphocytes" -- white blood cells
that grow in the soft center of your bones, called marrow.
● B lymphocytes are supposed to grow into cells that help you fight infections. But in this disease, they
turn into "leukemia" cells that live longer than normal cells and reproduce quickly. They build up in
your bone marrow and move into your bloodstream. From there they can spread to other organs in
your body.

2. T-Cell Lymphoblastic /Lymphoma

● A type of non-Hodgkin lymphoma in which too many T-cell lymphoblasts (immature white blood
cells) are found in the lymph nodes and spleen.
● They leave the bone marrow and go to lymph nodes or lymphatic organs or extralymphatic sites, and
do what all other cancers do: occupy space, hog resources, and make it very difficult for that part of
the body to do the job it is required to do. The patient's immune system can be compromised,
allowing for infections to run riot in the body or they can form bulky masses and get in the way of
organ function.
● B cells produce the antibodies that target diseased cells, T cells directly destroy bacteria or cells
infected with viruses.

F. Treatment

1. Chemotherapy
● Chemotherapy, which uses drugs to kill cancer cells, is typically used as an induction therapy for children and
adults with acute lymphocytic leukemia. Chemotherapy drugs can also be used in the consolidation and
maintenance phases.

2. Stem Cell Transplantation

● A bone marrow transplant is a medical treatment that replaces your bone marrow with healthy cells. The
replacement cells can either come from your own body or from a donor.

3. Ph-positive ALL therapy

● Tyrosine kinase inhibitors (TKIs) are used to treat Ph+ ALL by blocking (inhibiting) the BCR-ABL protein from
sending signals that cause leukemia cells to form. TKIs are a type of targeted therapy. Targeted therapy uses
drugs or other substances that target and attack specific cancer cells but are less likely to harm normal cells.
● Tyrosine kinase inhibitors alone are generally not used to treat ALL. Instead, they are added to a combination
chemotherapy regimen. These drugs are taken daily as pills.

4. Clinical trials

● Clinical trials are experiments to test new cancer treatments and new ways of using existing treatments. While
clinical trials give you or your child a chance to try the latest cancer treatment, treatment benefits and risks
may be uncertain. Discuss the benefits and risks of clinical trials with your doctor.
 G. Childhood Acute Lymphoblastic Leukemia
● Childhood acute lymphoblastic leukemia (ALL) is a type of cancer in which the bone marrow makes too many
immature lymphocytes (a type of white blood cell).
● Signs of childhood ALL include fever and bruising
● Childhood acute lymphoblastic leukemia (also called ALL or acute lymphocytic leukemia) is a cancer of the
blood and bone marrow. This type of cancer usually gets worse quickly if it is not treated.

H. Risk Factors for Childhood Acute Lymphoblastic Leukemia

● Possible risk factors for ALL include the following:
● Being exposed to x-rays before birth.
● Being exposed to radiation.
● Past treatment with chemotherapy.
● Having certain genetic conditions, such as: Down syndrome and Bloom syndrome.

I. Medical Management

● Vincristine. Vincristine is an anti-cancer (antineoplastic or cytotoxic) chemotherapy drug and is

classified as a plant alkaloid.
● Prednisone. This drug works by altering the body’s normal immune system responses.
● Cytarabine. Cytarabine belongs to the category of chemotherapy called antimetabolites, wherein
When the cells incorporate these substances into the cellular metabolism, they are unable to divide
and they attack cells at very specific phases in the cycle.
● L-asparaginase. Asparaginase breaks down asparagine in the body, so since the cancer cells cannot
make more asparagine, they die.
● Daunorubicin. Daunorubicin is classified as an antitumor antibiotic which is made from natural
products produced by species of the soil fungus Streptomyces, and these drugs act during multiple
phases of the cell cycle and are considered cell-cycle specific.
● Antibiotic, Antifungals, and Antivirals. These control infection, a common complication of acute
leukemias

J. Nursing Management
 The interventions included in the care plan of the patient are the following:
● The nurse should explain the disease course, treatment, and adverse effects
● Promote good nutrition. The nurse should explain that chemotherapy causes weight loss and
anorexia, so the patient must be encouraged to eat and drink high-calorie and high-protein foods and
beverages.
● Infection. The nurse should teach the patient and his family how to recognize symptoms of infection.
● Infection control. Control infection by placing the patient in a private room and instituting neutropenic
precautions.
● Skincare. Provide thorough skin care by keeping the patient’s skin and perianal area clean, applying
mild lotions and creams to keep skin from cracking and drying, and thoroughly cleaning skin before
all invasive skin procedures.
● Psychological support. Provide psychological support by establishing a trusting relationship to
promote communication.
● Manage stress. Minimize stress by providing a calm, quiet atmosphere that is conducive to rest and
relaxation

I. HEMOPHILIA
A. History of Hemophilia
- Hemophilia has been called a “Royal Disease”. This is because the hemophilia gene was passed from Queen
Victoria, to the ruling families or Russia, Spain, and Germany.
- Among her children, one son had hemophilia whereas two daughters were carriers. One of which married into
the Spanish family and she ten passed the gene to the heir of the Spanish throne.
B. Definition of Hemophilia
- CDC defines Hemophilia as an inherited bleeding disorder in which there is a deficiency or lack of factor VIII
(hemophilia A) or factor IX (hemophilia B). This is a disorder in which the blood does not clot properly and this
may lead to spontaneous bleeding following injury or surgery.
C. Etiology
- According to the CDC, Hemophilia is caused by mutations or changes in the genes. These mutations and/or
changes, affect the proteins needed to form a blood clot. These genes being affected are found in the X
chromosomes, which we get from our mothers.
- The chances of males having hemophilia is if he inherits an affected X chromosome from his mother. Females
with an affected X chromosome is a “carrier”of hemophilia, and can also have the symptoms of hemophilia as
well.
D. Types of Hemophilia
i. Hemophilia A
- Also known as the “Classic Hemophilia”, which is caused by the lack or decrease of clotting factor VIII
(FVIII), a clotting protein.
- People with hemophilia A often bleed longer than other people. Bleeds can occur internally, into joints and
muscles, or externally, from minor cuts, dental procedures or trauma.
- Normal plasma levels of FVIII ranges from 50%-150%. Levels below 50% determine a person’s symptoms.
- Mild Hemophilia A- 6%-49% - generally experience bleeding only after serious injuries, or trauma.
- Moderate Hemophilia A- 1%-5%- Tend to have bleeding episodes after injuries, regardless of severity
- Severe Hemophilia A- <1%- Experience bleeding following injury and frequent spontaneous bleeding episodes,
often in their joints and muscles. Bleeds without obvious causes are called spontaneous bleeding episodes.
ii. Hemophilia B
- Also known as “Christmas Disease”, is caused by the lack or decrease of clotting factor IX (FIX), a clotting
protein
- People with hemophilia B often bleed longer than other people. Bleeds can occur internally, into joints and
muscles, or externally, from minor cuts, dental procedures or trauma.
 - Normal plasma levels of FIX ranges from 50%-150%. Levels below 50% determine a person’s symptoms.
- Mild Hemophilia B- 6%-49% - generally experience bleeding only after serious injuries, or truma.
- Moderate Hemophilia B- 1%-5%- Tend to have bleeding episodes after injuries, regardless of severity
- Severe Hemophilia B- <1%- Experience bleeding following injury and frequent spontaneous bleeding episodes,
often in their joints and muscles. Bleeds without obvious causes are called spontaneous bleeding episodes.
iii. Von Willebrand disease (VWD)
- is a genetic disorder caused by missing or defective von Willebrand factor (VWF), a clotting protein. VWF
binds factor VIII, a key clotting protein, and platelets in blood vessel walls, which help form a platelet plug
during the clotting process
- Frequent nosebleeds, easy bruising, and excessive bleeding during invasive procedures, such as tooth
extractions and surgery, are common symptoms of the disease.
- There are three main types of VWD based on defects in the VWF. The fourth type, is not hereditary:
- Type 1 (60%-80% of patients)- Levels of VWF in blood range from 20%-50% of normal. Usually mild
symptoms.
- Type 2 (15%-30% of patients)- Type 2 is broken down into four subtypes: type 2A, type 2B, type 2M and type
2N, depending on the presence and behavior of multimers, molecular chains of VWF. Symptoms are mild to
moderate.
- Type 3 (5%-10% of patients)- Have a qualitative deficiency of VWF. Symptoms are typically severe, and
include spontaneous bleeding episodes, often in the joints and muscles.
- Acquired VWD- Results after the diagnosis of an autoimmune disease, such as lupus or cancer. It may also
occur after taking certain medications.

II. Risk Factors

A. Genetics/ Family History of Hemophilia
- Hemophilia A and B are X-linked recessive disorder
- Severity level is consistent between family members ~30 % of cases of hemophilia are new mutations
- Female with one gene altered chromosome has 50% chance of passing the gene to her children. Typically they
are called “carrier”. She may not have the disease herself due to sufficiency in clotting factors from the normal x
chromosome but have often increased risk for bleeding.
B. Gender
- Hemophilia is typically expressed in males and carried by females
- Most males are affected by Hemophilia
- On the other hand, von Willebrand disease (vWD) affects both males and females
C. Age
- Recognized in early childhood specifically toddler age group
- Based on Centers for Disease Control data, the median age at diagnosis is:
❏ Mild hemophilia: 36 months
❏ Moderate hemophilia: 8 months
❏ Severe hemophilia: 1 month

III. Signs and Symptoms

a. Bleeding into the joints. This can cause swelling and pain or tightness in the joints; it often affects the
knees, elbows, and ankles
b. Bleeding into the skin (which is bruising) or muscle and soft tissue causing a build-up of blood in the
area (called a hematoma)
c. Bleeding of the mouth and gums, and bleeding that is hard to stop after losing a tooth
d. Bleeding after circumcision (surgery performed on male babies to remove the hood of skin, called the
foreskin, covering the head of the penis)
e. Bleeding after having shots, such as vaccinations
f. Bleeding in the head of an infant after a difficult delivery
g. Blood in the urine or stool
h. Frequent episodes of epistaxis that are hard to stop.
 IV. Clinical Manifestations
a. Hemorrhage
- Uncontrolled hemorrhage due to after dental extractions, irritation of the gingiva with the use of hard bristle
toothbrush
- Tends to occur secondary to trauma
- Most dangerous site of hemorrhage is the head (intracranial or extracranial), it may cause nausea, vomiting,
headache, drowsiness, confusion, visual changes, and loss of consciousness
b. Bleeding from minor trauma or cuts
- Slow, persistent, prolonged bleeding from trauma
- Delayed bleeding may happen for several days or days
- For vWF bleeding tends to be mucosal
c. Bleeding in joints
- Called “hemarthrosis”
- May lead to severe joint injury and deformity to cause crippling
- Common affected joints site:
❏ Knees
❏ Elbows
❏ Shoulders
❏ Hips
❏ Ankles, wrists
d. Chronic pain or Ankylosis
- Occurs when there is recurrent joint hemorrhages
- Ankylosis refers to stiffness of the joint
e. Spontaneous hematuria and GI bleeding
- Hematuria is the presence of blood in the urine and along may cause potential renal failure from genitourinary
trauma and splenic rupture results in falls or abdominal trauma
f. Ecchymoses and subcutaneous hematomas
- Ecchymoses means the discoloration of skin due to bleeding underneath such as bruising
- Severe factor VIII deficiency, the hematomas may occur without known trauma and extends all directions
- Hematomas occurring in the muscles, peripheral nerves can be compressed that will result to decreased
sensation, weakness and atrophy
g. Neurologic signs: pain, anesthesia, and paralysis
- May develop from nerve compression caused by hematoma formation
V. Assessment and Diagnostics
a. CBC
- Measures the amount of hemoglobin, the size, and number of RBC, WBC, and platelets found in our blood.
People with hemophilia would have an RBC count that is low.
b. Activated Partial Thromboplastin Time (APTT) Test
- This test measures how long it takes for the blood to clot by measuring the different clotting factors. The results
of this test will show a longer clotting time among people with Hemophilia A or B
c. Prothrombin Time (PT) Test
- It is also another test that measures how long it takes for the blood to clot.
d. Bleeding time (BT)
- Usually normal in patients with hemophilia, although occasionally a patient with hemophilia may have a
prolonged BT. Bleeding times are not recommended to evaluate a patient for hemophilia or other bleeding
disorders because of their unreliability.
e. Fibrinogen Test
- This is another test used to assess a patient’s ability to form a blood clot. This is either ordered along with other
blood clotting tests for when a patient has an abnormal PT and APTT test.
f. Clotting factor measurement (factor assays)
- Shows the type of hemophilia and the severity of it.
i. Normal- 50% to 1000% levels of Factor VIII or IX
 ii. Mild - Greater than 5% but less than 50% levels of Factor VIII or IX
iii. Moderate- 1% to 5% levels of Factor VIII or IX
iv. Severe- Less than 1% levels of Factor VIII or IX
VI. Nursing Diagnoses
a. Risk for bleeding r/t decreased concentration of clotting factors in the blood
b. Risk for deficient fluid volume related to hemorrhage
c. Pain related to bleeding into the joints and muscles
d. Ineffective family -coping related to life threatening disease

VII. Nursing Interventions

a. Assessment and history taking

- A comprehensive and detailed assessment and history taking helps the health care team in planning for the
proper care needed for the patient
b. Recognizing and control of bleeding
- The goal is the prevention and the decrease for the risk of injury of the patients. Appropriate exercises to
strengthen the joints and muscles may be done according to the age and level of activity tolerance.
- RICE- rest, ice, compression and elevation can be done for parents as supportive measures
- This is also important so that the patients and their significant others may be taught beforehand and they will be
equipped to initiate immediate care needed.
c. Assist family and in coping
- This helps alleviate the anxiety felt by the family, especially the mother. Giving them an opportunity to discuss
their feelings may be of help in alleviating their anxiety by tenfold.
- Teach them to recognize which problems can be resolved at home and which require hospitalization:
a. Immediate medical attention for severe pain or swelling of a muscle or joint that restricts movement or inhibits
sleep and for a head injury, swelling in the neck or mouth, abdominal pain, hematuria, melena, and skin wounds
in need of suturing.
d. Encourage patients to be self-sufficient
- This is to encourage the patient to be involved in the plan of care
e. Health teaching on home-based care, prevention of bleeding and activity restrictions
- This is to avoid the risk of injury that may endanger the patient even more
- Teach the patient to perform daily oral hygiene without causing trauma such as soft bristled toothbrush.
- Advise the patient to participate only in noncontact sports (e.g., golf, swimming)
- Wearing of gloves when doing household chores to prevent cuts or abrasions from knives, hammers, and other
tools.

f. Health teaching on agents to avoid

- This is to avoid accidents that may initiate or worsen the symptoms of the disease
- Avoid pain medications that could aggravate bleeding
g. Promote good dental hygiene
- Patients who have bleeding disorders need to be taught the proper dental hygiene based on their situation so as
to avoid accidents like the bleeding of gums while brushing their teeth
VIII. Medical Management
a. Replacement Therapy
- May be given on demand, before surgery, lumbar puncture, and dental care as prophylactic measure
- Concentrates are given when active bleeding occurs

i. Factors VIII concentrate

- Intravenous infusion
a. IV push
 b. Continuous infusion
c. Half-life: 8-12 hours
d. Each unit infused raises serum factor VIII level by 2 %
- Dose varies depending on type of bleeding
a. Ranges from 20-50+ units/kg. body weight

ii. Factors IX concentrates

- Intravenous infusion
a. IV push
b. Continuous infusion
c. Half-life: 12-24 hours
d. Each unit infused raises serum factor IX level by 1%
- Dose varies depending on type of bleeding: Ranges from 20-100+ units/kg. body weight

b. Antifibrinolytic therapy
- inhibits fibrinolysis by inhibiting plasminogen activation in the fibrin clot, enhancing clot stability. These agents
are used to stabilize clots in areas such as the oral cavity, and in patients with difficult episodes of epistaxis and
menorrhagia

i. Tranexamic acid
- about 10 times more potent in vitro than aminocaproic acid. It binds more strongly than aminocaproic acid
- Route: Oral
- Pediatric population dosage is in the region of 20 mg/kg/day.
- Side effects: nausea, diarrhea, stomach pain or discomfort, vomiting, chills and fever.

ii. epsilon-aminocaproic acid (amicar)

- Oral administration of ε-aminocaproic acid (Amicar) prevents clot destruction. Its use is limited to mouth or
trauma surgery
- Anti-fibrinolytic
- Used for mucocutaneous bleeding
- Route: IV/ Oral
- Dosing: 50-100 mg/kg. q 6 hrs
- Side effects: dizziness, headache, muscle pain or weakness, stomach pain
- Pediatric population: Give 200 mg/kg as a loading dose orally followed by 100 mg/kg every 6 hours.
(Maximum 3 grams per dose). Decrease to 50 mg/kg if side effects occur. In patients with mild/moderate
hemophilia or VWD with RCo >20, decrease the loading dose to 100 mg/kg (Maximum 3 grams per dose).

c. Desmopressin acetate (DDAVP)

- increases plasma factor VIII activity and is the treatment of choice in mild hemophilia and certain types of vWD
if the child shows an appropriate response. Not effective in severe hemophilia A, severe vWD, or any form of
hemophilia B.
- a synthetic analog of vasopressin, may be used to stimulate an increase in factor VIII and vWF. This drug
acts on platelets and endothelial cells to cause the release of vWF, which binds with factor VIII, thus increasing
their concentration.
- Route: IV, subcutaneously, or by intranasal spray.
- It decreases and corrects bleeding time of DDAVP: administered IV, seen within 30 minutes and can last for
more than 12 hours. Short lived, the patient must be closely monitored and repeated doses may be necessary
- It is an appropriate therapy for minor bleeding episodes and dental procedures.
- Contraindicated in patients with unstable coronary artery disease because can induce platelet aggregation
- Side effects: headache, facial flushing, tachycardia, hyponatremia
d. Plasmapheresis
- Plasmapheresis is the removal of plasma containing components causing or thought to cause disease. It can also
be used to obtain plasma from healthy donors to be administered. When removed, it is replaced by fluids such
as saline, fresh-frozen plasma, or albumin where plasma exchange may occur.