1
, ii. Positive results on two separate tests performed on separate blood
specimens are required for the diagnosis of HIV infection. Infants born to
HIV-infected women are also considered HIV infected if they meet the
Centers for Disease Control and Prevention surveillance case definition
for AIDS. Before testing, provide counseling to the parent or guardian,
including an explanation of HIV infection, the reason for the test, and
implications of positive test results, confidentiality issues, risk reduction
behaviors, and beneficial effects of early intervention.
e. Nursing Management
i. Prevention is a key component of HIV education. Educating adolescents
about HIV is essential in preventing HIV infection in this age-group.
Education should include information on the routes of transmission, the
hazards of IV and other recreational drug use, and the value of sexual
abstinence and safe sex practices
f. Therapeutic Management
i. The goals of therapy for HIV infection include slowing the growth of the
virus, preventing and treating opportunistic infections, and providing
nutritional support and symptomatic treatment. Antiretroviral drugs work
at various stages of the HIV life cycle to prevent reproduction of
functional new virus particles. Antiretroviral therapy regimens are
continually evolving. Although antiretroviral drugs are not a cure, they can
delay progression of the disease
2. Hemophilia
a. Etiology
i. The term hemophilia refers to a group of bleeding disorders resulting from
congenital deficiency, dysfunction, or absence of specific coagulation
proteins or factors. Although the symptomatology is similar regardless of
which clotting factor is deficient, the identification of specific factor
deficiencies has allowed definitive treatment with replacement agents.
ii. In about 80% of all cases of hemophilia, the inheritance pattern is
demonstrated as X-linked recessive. (See Chapter 3.) The two most
common forms of the disorder are factor VIII deficiency (hemophilia A, or
classic hemophilia) and factor IX deficiency (hemophilia B, or Christmas
disease), with prevalence of approximately 1 in 5000 and 1 in 20,000 to
30,000 live male births.
b. Pathophysiology
i. The basic defect of hemophilia A is a deficiency of factor VIII
(antihemophilic factor). Factor VIII is produced by the liver and is
necessary for the formation of thromboplastin in phase I of blood
coagulation. The less factor VIII that is found in the blood, the more
severe the disease.
, ii.
c. Treatments
i. The primary therapy for hemophilia is replacement of the missing clotting
factor. The products currently available are factor VIII concentrates, either
produced through genetic engineering (recombinant form) or derived from
pooled plasma, which are reconstituted with sterile water immediately
before use. A synthetic form of vasopressin, 1-deamino-8-D-arginine
vasopressin (DDAVP), is the treatment of choice in mild hemophilia and
von Willebrand disease (vWD) (types I and IIA only) if the child shows an
appropriate response. After DDAVP administration a threefold to fourfold
rise in factor VIII activity should occur. Because the goal is to raise the
factor VIII level at least 30%, patients with moderate factor VIII
deficiency do not benefit. In addition, various therapies are employed
when bleeding occurs or is anticipated.
ii. In addition, the nurse maintains a high level of suspicion when a child
with hemophilia shows signs such as headache; slurred speech; loss of
consciousness (from cerebral bleeding); and black, tarry stools (from
gastrointestinal bleeding).
3. Hemarthrosis
a. Subcutaneous and intramuscular hemorrhages are common. Hemarthrosis, which
refers to bleeding into the joint cavities, especially the knees, elbows, and ankles,
is the most frequent form of internal bleeding. Bony changes and crippling
deformities occur after repeated bleeding episodes over several years. Early signs
of hemarthrosis are a feeling of stiffness, tingling, or ache in the affected joint,
followed by a decrease in the ability to move the joint. Obvious signs and
symptoms are warmth, redness, swelling, and severe pain, with considerable loss
of movement. Spontaneous hematuria is not uncommon. Epistaxis may occur but
is not as frequent as other kinds of hemorrhage. Petechiae are uncommon in
persons with hemophilia because repair of small hemorrhages depends on platelet
function, not on blood-clotting mechanisms.
4. Von Wildebrand Disease
a. Von Willebrand disease (vWD) is a hereditary bleeding disorder characterized by
a deficiency of or defect in a protein called von Willebrand factor (vWF). The
vWF protein contributes to the adherence of platelets to damaged endothelium
and serves as a carrier protein for factor VIII. This results in prolonged bleeding
time because platelets fail to adhere to the walls of the ruptured vessel to form a
platelet plug. Even though bleeding time is insensitive to platelet function defects,
it is still used by some centers. The platelet function analyzer (PFA 100) is a
rapid, accurate detection of platelet dysfunction and vWD used by many centers,
but its results may be affected by such conditions (e.g., sepsis, pregnancy, and
, certain medications) and therefore requires the need for further platelet
aggregation testing. The disease can cause mild, moderate, or severe bleeding.
Most cases of vWD are mild and require intervention only for dental and surgical
procedures.
b. The most characteristic clinical feature of vWD is an increased tendency toward
bleeding from mucous membranes. The most common symptom is frequent
nosebleeds, followed by gingival bleeding, easy bruising, and excessive menstrual
bleeding (menorrhagia) in females. Unlike hemophilia, vWD affects both males
and females because its inheritance shows an autosomal dominant pattern.
However, the treatment and final outcome is similar in both disorders. Treatment
of bleeding is with DDAVP and/or a specially concentrated clotting factor known
as Humate-P.
c. Nursing Care Management
i. The nursing goals are similar to those for hemophilia, with special
considerations related to epistaxis. Nosebleeds are often a frightening
experience for the child and parents. A calm, reassuring manner can
alleviate anxiety and promote the child's cooperation.
5. Epistaxis
a. Have child sit up and lean forward (not lie down).
b. Apply continuous pressure to nose with thumb and forefinger for at least 10
minutes.
c. Insert cotton or wadded tissue into each nostril and apply ice or cold cloth to
bridge of nose if bleeding persists.
d. Keep child calm and quiet.
6. Menorrhagia
a. For menorrhagia, factor replacement therapy or the administration of DDAVP
may be beneficial on the first day of the menstrual cycle to lessen the flow.
Teaching the adolescent methods to prevent embarrassing accidents during
menstruation, such as using double sanitary pads, helps her adjust to the
inconvenience. Interestingly, these females frequently do not experience
excessive bleeding during childbirth. This is thought to be due to the increased
levels of factor VIII during pregnancy.
7. Sickle Cell Disease
a. Sickle cell anemia (SCA) is one of a group of diseases collectively termed
hemoglobinopathies, in which normal adult hemoglobin (hemoglobin A [HgbA])
is partly or completely replaced by abnormal sickle hemoglobin (HgbS)
b. The gene that determines the production of HgbS is situated on an autosome.
When both parents have sickle cell trait, there is a 25% chance with each
pregnancy of producing an offspring with SCA.
c. The clinical manifestations of SCA are primarily the result of (1) obstruction
caused by the sickled RBCs, (2) vascular inflammation, and (3) increased RBC
destruction. The abnormal adhesion, entanglement, and enmeshing of rigid sickle-
shaped cells accompanied by the inflammatory process intermittently blocks the
microcirculation, causing vasoocclusion (Fig. 30-3). The resultant absence of
blood flow to adjacent tissues causes local hypoxia, which leads to tissue
ischemia and infarction (cellular death)
, ii. Positive results on two separate tests performed on separate blood
specimens are required for the diagnosis of HIV infection. Infants born to
HIV-infected women are also considered HIV infected if they meet the
Centers for Disease Control and Prevention surveillance case definition
for AIDS. Before testing, provide counseling to the parent or guardian,
including an explanation of HIV infection, the reason for the test, and
implications of positive test results, confidentiality issues, risk reduction
behaviors, and beneficial effects of early intervention.
e. Nursing Management
i. Prevention is a key component of HIV education. Educating adolescents
about HIV is essential in preventing HIV infection in this age-group.
Education should include information on the routes of transmission, the
hazards of IV and other recreational drug use, and the value of sexual
abstinence and safe sex practices
f. Therapeutic Management
i. The goals of therapy for HIV infection include slowing the growth of the
virus, preventing and treating opportunistic infections, and providing
nutritional support and symptomatic treatment. Antiretroviral drugs work
at various stages of the HIV life cycle to prevent reproduction of
functional new virus particles. Antiretroviral therapy regimens are
continually evolving. Although antiretroviral drugs are not a cure, they can
delay progression of the disease
2. Hemophilia
a. Etiology
i. The term hemophilia refers to a group of bleeding disorders resulting from
congenital deficiency, dysfunction, or absence of specific coagulation
proteins or factors. Although the symptomatology is similar regardless of
which clotting factor is deficient, the identification of specific factor
deficiencies has allowed definitive treatment with replacement agents.
ii. In about 80% of all cases of hemophilia, the inheritance pattern is
demonstrated as X-linked recessive. (See Chapter 3.) The two most
common forms of the disorder are factor VIII deficiency (hemophilia A, or
classic hemophilia) and factor IX deficiency (hemophilia B, or Christmas
disease), with prevalence of approximately 1 in 5000 and 1 in 20,000 to
30,000 live male births.
b. Pathophysiology
i. The basic defect of hemophilia A is a deficiency of factor VIII
(antihemophilic factor). Factor VIII is produced by the liver and is
necessary for the formation of thromboplastin in phase I of blood
coagulation. The less factor VIII that is found in the blood, the more
severe the disease.
, ii.
c. Treatments
i. The primary therapy for hemophilia is replacement of the missing clotting
factor. The products currently available are factor VIII concentrates, either
produced through genetic engineering (recombinant form) or derived from
pooled plasma, which are reconstituted with sterile water immediately
before use. A synthetic form of vasopressin, 1-deamino-8-D-arginine
vasopressin (DDAVP), is the treatment of choice in mild hemophilia and
von Willebrand disease (vWD) (types I and IIA only) if the child shows an
appropriate response. After DDAVP administration a threefold to fourfold
rise in factor VIII activity should occur. Because the goal is to raise the
factor VIII level at least 30%, patients with moderate factor VIII
deficiency do not benefit. In addition, various therapies are employed
when bleeding occurs or is anticipated.
ii. In addition, the nurse maintains a high level of suspicion when a child
with hemophilia shows signs such as headache; slurred speech; loss of
consciousness (from cerebral bleeding); and black, tarry stools (from
gastrointestinal bleeding).
3. Hemarthrosis
a. Subcutaneous and intramuscular hemorrhages are common. Hemarthrosis, which
refers to bleeding into the joint cavities, especially the knees, elbows, and ankles,
is the most frequent form of internal bleeding. Bony changes and crippling
deformities occur after repeated bleeding episodes over several years. Early signs
of hemarthrosis are a feeling of stiffness, tingling, or ache in the affected joint,
followed by a decrease in the ability to move the joint. Obvious signs and
symptoms are warmth, redness, swelling, and severe pain, with considerable loss
of movement. Spontaneous hematuria is not uncommon. Epistaxis may occur but
is not as frequent as other kinds of hemorrhage. Petechiae are uncommon in
persons with hemophilia because repair of small hemorrhages depends on platelet
function, not on blood-clotting mechanisms.
4. Von Wildebrand Disease
a. Von Willebrand disease (vWD) is a hereditary bleeding disorder characterized by
a deficiency of or defect in a protein called von Willebrand factor (vWF). The
vWF protein contributes to the adherence of platelets to damaged endothelium
and serves as a carrier protein for factor VIII. This results in prolonged bleeding
time because platelets fail to adhere to the walls of the ruptured vessel to form a
platelet plug. Even though bleeding time is insensitive to platelet function defects,
it is still used by some centers. The platelet function analyzer (PFA 100) is a
rapid, accurate detection of platelet dysfunction and vWD used by many centers,
but its results may be affected by such conditions (e.g., sepsis, pregnancy, and
, certain medications) and therefore requires the need for further platelet
aggregation testing. The disease can cause mild, moderate, or severe bleeding.
Most cases of vWD are mild and require intervention only for dental and surgical
procedures.
b. The most characteristic clinical feature of vWD is an increased tendency toward
bleeding from mucous membranes. The most common symptom is frequent
nosebleeds, followed by gingival bleeding, easy bruising, and excessive menstrual
bleeding (menorrhagia) in females. Unlike hemophilia, vWD affects both males
and females because its inheritance shows an autosomal dominant pattern.
However, the treatment and final outcome is similar in both disorders. Treatment
of bleeding is with DDAVP and/or a specially concentrated clotting factor known
as Humate-P.
c. Nursing Care Management
i. The nursing goals are similar to those for hemophilia, with special
considerations related to epistaxis. Nosebleeds are often a frightening
experience for the child and parents. A calm, reassuring manner can
alleviate anxiety and promote the child's cooperation.
5. Epistaxis
a. Have child sit up and lean forward (not lie down).
b. Apply continuous pressure to nose with thumb and forefinger for at least 10
minutes.
c. Insert cotton or wadded tissue into each nostril and apply ice or cold cloth to
bridge of nose if bleeding persists.
d. Keep child calm and quiet.
6. Menorrhagia
a. For menorrhagia, factor replacement therapy or the administration of DDAVP
may be beneficial on the first day of the menstrual cycle to lessen the flow.
Teaching the adolescent methods to prevent embarrassing accidents during
menstruation, such as using double sanitary pads, helps her adjust to the
inconvenience. Interestingly, these females frequently do not experience
excessive bleeding during childbirth. This is thought to be due to the increased
levels of factor VIII during pregnancy.
7. Sickle Cell Disease
a. Sickle cell anemia (SCA) is one of a group of diseases collectively termed
hemoglobinopathies, in which normal adult hemoglobin (hemoglobin A [HgbA])
is partly or completely replaced by abnormal sickle hemoglobin (HgbS)
b. The gene that determines the production of HgbS is situated on an autosome.
When both parents have sickle cell trait, there is a 25% chance with each
pregnancy of producing an offspring with SCA.
c. The clinical manifestations of SCA are primarily the result of (1) obstruction
caused by the sickled RBCs, (2) vascular inflammation, and (3) increased RBC
destruction. The abnormal adhesion, entanglement, and enmeshing of rigid sickle-
shaped cells accompanied by the inflammatory process intermittently blocks the
microcirculation, causing vasoocclusion (Fig. 30-3). The resultant absence of
blood flow to adjacent tissues causes local hypoxia, which leads to tissue
ischemia and infarction (cellular death)
