BLOOD DISORDERS

IN CHILDREN
ANEMIA:
• Anemia is a common problem in children.
About 20% of children in the U.S. will be
diagnosed with anemia at some point. A child
who has anemia does not have enough red
blood cells or hemoglobin. Hemoglobin is a
type of protein that allows red blood cells to
carry oxygen to other cells in the body.
There are many types of anemia:
• IRON DEFICIENCY ANEMIA-
Too little iron in the blood. Iron is needed to form
hemoglobin. This is the most common cause of
anemia. Iron plays an important role in muscle
function, energy creation, and brain development.
Causes-

• Toddlers can run into problems if they drink

too much cow's milk (more than 24 ounces a
day) and eat fewer iron-rich foods, like red
meat and green leafy vegetables. Cow’s milk
is not a good source of iron. In fact, milk
makes it harder for the body to absorb iron
and can contribute to iron-deficiency anemia.
CONT..
• Teenage boys sometimes develop iron deficiency
during the rapid growth of puberty. But teen girls
have iron deficiency more often because their
bodies can't store as much iron and lose blood
during menstruation. Young athletes who
exercise often tend to lose more iron and may
also become iron deficient.
Symptoms-
• tiredness and weakness
• pale skin, especially around the hands, nails, and
eyelids
• rapid heartbeat or a heart murmur
• irritability
• low appetite
• dizziness or feeling lightheaded.
• In rare cases, a child with iron-deficiency anemia
may develop pica, a craving to eat nonfood items
such as paint chips, chalk, or dirt.
Diagnosis-
• -Blood test to check for low hemoglobin or iron
levels
• -stool test because iron-deficiency anemia can
sometimes be caused by the gradual loss of
small amounts of blood through the intestinal
tract.
Treatment-

• Daily iron supplements to get their iron levels back up.

Multivitamins with iron and changes to a child's diet can

help, but usually are not enough on their own.

• Iron should be taken on an empty stomach .Avoid giving

a child iron with milk or caffeinated drinks because both

prevent the iron from absorbing well. orange juice foods

that are high in vitamin C can make iron absorb better.

Cont..

• Kids with severe cases of iron-deficiency

anemia may require blood transfusion.
Prevention-
• younger than 1 year old should drink only breast milk
or an infant formula that contains iron. Breastfed
infants should eat iron-fortified solid foods starting at
about 6 months of age.
• Kids under 2 should have no more than 24 ounces of
whole milk a day. Iron-fortified products such as
cereal can be a great way to help young children to
get more iron.
CONT.
• Good sources of iron include: red meats, dark poultry,
salmon, tuna, egg yolks, green leafy vegetables, dried
peas and beans, molasses, dried fruits and raisins, and
enriched whole-grain bread. Serve these alongside foods
or drinks rich in vitamin C (tomatoes, broccoli, orange
juice, strawberries, etc.), which improves the body's
absorption of iron.
• Try cooking food in a cast iron pan, which can help
enrich the food with iron.
MEGALOBLASTIC ANEMIA-
• Red blood cells are too large. One type of
megaloblastic anemia is pernicious anemia. In
this type, there is a problem absorbing
vitamin B12, important to making red blood cells.
Megaloblastic anemia is an uncommon problem
in childhood that is most frequently associated
with vitamin deficiency or gastrointestinal
disease.
CONT..
Causes-
• The 2 most common causes of megaloblastic anemia are
vitamin B-12 (cobalamin) deficiency and folic acid
deficiency.
Treatment-
• Treatment of megaloblastic anemia depends on the
underlying cause. Supplemental folate or vitamin B-12
may be indicated
HEMOLYTIC ANEMIA-
• Red blood cells are destroyed. There are many
different causes, such as serious infections or
certain medications.
• Intrinsic. This is when the destruction of red
blood cells is due to a flow within the red blood
cells. This type is often inherited. It includes
sickle cell anemia, thalassemia, or erythrocytosis.
These conditions produce red blood cells that
don't live as long as normal red blood cells.
CONT.

Extrinsic. This is when the destruction of red blood

cells is from a cause outside of the cells. Some of the
causes of extrinsic hemolytic anemia, also called
autoimmune hemolytic anemia, are:
– Infections. From bacteria or viruses.

– Medicines. Antibiotics like penicillin or sulfonamides,

medicines for malaria, or acetaminophen.
CONT..
- Cancers. Leukemia, lymphoma, or other
tumors.
– Autoimmune disorders. Systemic lupus
erythematous (SLE or lupus), rheumatoid
arthritis, Wiskott-Aldrich syndrome, or
ulcerative colitis.
– Hypersplenism. A condition in which the
spleen destroys more red blood cells than
normal.
Symptoms –
• Pale skin
• Jaundice, or yellowing of the skin and eyes
• Dark-colored urine
• Fever
• Weakness
• Dizziness
• Confusion
• Intolerance to physical activity
• Enlargement of the spleen and liver
• Fast heart beat
• Heart murmur
Diagnostic evaluation-
• Hemoglobin and hematocrit. It measures the
amount of hemoglobin and red blood cells in the
blood.
• Complete blood count, or CBC. This
test checks the red and white blood cells, blood
clotting cells (platelets), and sometimes, young
red blood cells (reticulocytes). It includes
hemoglobin and hematocrit and more details
about the red blood cells.
Cont.
• Peripheral smear. A small sample of blood is
viewed under a microscope. Blood cells are
checked to see if they look normal or not.
CONT..
• Other blood tests. These tests will check lactate
dehydrogenase, bilirubin, haptoglobin, and
certain antibodies.
• Urine tests. For example, to check for
hemoglobin from red blood cells breaking down.
Cont..
Bone marrow aspiration, biopsy, or both. A
procedure in which a small amount of bone
marrow fluid (aspiration), solid bone marrow
tissue (called a core biopsy) often from the hip
bones, or both are checked for the number, size,
and maturity of blood cells. This test may also
look for abnormal cells.
Treatment-
• Blood transfusions
• Corticosteroid or steroid medicines
• Intravenous immune globulin (IVIg)
• Rituximab, a medicine to control the immune
system
• Antibiotics to treat the infection if that is the
underlying cause
• Other medicines, depending on the type of
hemolytic anemia
• In some children, who don't respond to first-line
therapy, these treatments may be needed:
CONT..
In some children, who don't respond to first-line
therapy, these treatments may be needed:
• Splenectomy. Surgical removal of the spleen.
• Immunosuppressive therapy. Use of other
drugs to control the immune system.
• Plasmapheresis. Removal of antibodies from
the blood that are destroying red blood cells.
• Stem cell transplant. Damaged bone marrow
cells are replaced with donor bone marrow.
SICKLE CELL ANEMIA-
• An inherited type of anemia with abnormally
shaped red blood cells. It is a type of hemolytic
anemia.
Causes-Your child is born with SCD. SCD is passed
from parents to a child. The child is given two
abnormal genes for hemoglobin, one from each
parent.
Sign and symptoms-
• Pain anywhere in the body
• Swollen hands and fingers
• Feeling very tired
• Dizziness or weakness
• Poor feeding in babies or young children
• Yellow skin or eyes
• Pale skin
• Shortness of breath
Diagnosis-A blood test can check the shape and
number of your child's RBCs.
Treatment-
• Medicines may be given to decrease pain or
decrease sickling of your child's RBCs. Your
child may also need medicine to prevent a
bacterial infection.
• A blood transfusion increases the number of
healthy RBCs in your child's blood. Your child
may need a blood transfusion more than once.
CONT.
• Surgery may be done to remove your child's
spleen or gallbladder. Surgery may be needed if
RBCs often get stuck in your child's spleen or he
or she has gallstones.
• A stem cell transplant , also called a bone
marrow transplant, helps your child's body make
new, healthy blood cells
APLASTIC ANEMIA-
• Failure of the bone marrow to make blood cells.
Aplastic anemia is a serious condition in which
the bone marrow doesn’t make enough new blood
cells. With fewer blood cells, a child with aplastic
anemia has:
• Less oxygen sent to organs, tissues and cells
(from too few red blood cells)
• Increased risk of infection (from too few white
blood cells)
• Increased risk of bleeding problems (from too
few platelets
Causes-
• Infection. These include hepatitis or liver
infection, and many different viral illnesses,
such as Epstein-Barr virus (EBV),
cytomegalovirus (CMV), parvovirus B19, or
human immunodeficiency virus (HIV).
• Cancer. Some cancers affect the bone marrow.
• Autoimmune disease. For example, lupus and
rheumatoid arthritis
.
Cont..
• Medicines.
• Toxins. For example, heavy metals, pesticides,
benzene
• Radiation therapy and chemotherapy. For the
treatment of cancer.
Symptoms-
• These are the most common symptoms of aplastic
anemia. Symptoms may include:
• From decreased red blood cells:
– Headache
– Dizziness
– Shortness of breath
• Lack of energy or tiring easily (fatigue)
– Pale skin
– Chest pain
– Irregular heart beat
– Enlarged heart
CONT..
• From too few white blood cells:
– Fevers
– Mouth sores
– Infections
• From too few platelets:
– Easy bruising
– Nosebleeds
– Bleeding gums
– Blood in the stool
– Heavy bleeding with menstrual periods
• Other symptoms:
– Nausea
– Skin rashes
Diagnosis-
• Because anemia is common in children, doctors
do routine screening for it. Plus, it often has no
symptoms. Most anemia in children is diagnosed
with these blood tests:
• Hemoglobin and hematocrit. This is often the
first screening test for anemia in children. It
measures the amount of hemoglobin and red
blood cells in the blood.
Cont.
Complete blood count, or CBC. A complete
blood count checks the red and white blood cells,
blood clotting cells (platelets), and sometimes,
young red blood cells (reticulocytes). It includes
hemoglobin and hematocrit and more details
about the red blood cells.
CONT.

• Peripheral smear. A small sample of blood is examined under

a microscope to see if they look normal.

• To get a blood sample, a healthcare provider will insert a

needle into a vein, usually in the child's arm or hand. A
tourniquet may be wrapped around the child's arm to help the
healthcare provider find a vein. Blood is drawn up into a
syringe or a test tube. In some cases, blood can be taken using
a needle prick.
Cont..
Blood tests may cause a little discomfort while the
needle is inserted. It may cause some bruising or
swelling. After the blood is removed, the
healthcare provider will remove the tourniquet,
put pressure on the area, and put on a bandage.
Cont.
Depending on the results of the blood tests, your child
may also have a:
• Bone marrow aspiration, biopsy, or both. This
involves taking a small amount of bone marrow fluid
(aspiration) or solid bone marrow tissue (called a core
biopsy). The fluid or tissue is examined for the
number, size, and maturity of blood cells and/or
abnormal cells.
COMPLICATIONS OF ANEMIA-
• The complications of anemia depend on what is
causing it. Some types have few complications,
but others have frequent and serious
complications. Some anemias may cause:
• Problems with growth and development
• Joint pain and swelling
• Bone marrow failure
• Leukemias or other cancers
Prevention of anemia-
• Some types of anemia are inherited and can’t be
prevented. Iron deficiency anemia, a common
form of anemia, may be prevented by making sure
your child gets enough iron in his/her diet. This is
done by the following:
• When your baby is breast fed he or she will get
enough iron from the breast milk.
• Cow’s milk does not provide enough iron. It
should not be given to babies until after 1 year of
age, when he or she is eating enough other food.
Cont..
• If your child is on formula, use formula with
added iron.
• Choose foods that are good sources of iron
including: iron-enriched grains and cereals, egg
yolks, red meats, potatoes, tomatoes, and
raisins.
Key points about anemia in children
• Anemia is a low red blood cell count or a low
hemoglobin level.
• There are many different causes of anemia.
• Children are usually screened for anemia. Often
there are no symptoms.
• The treatment of anemia depends on what is
causing it.
 COOLEY'S ANEMIA (THALASSEMIA)
• Thalassemia is a blood disorder passed down
through families (inherited) in which the body
makes an abnormal form of hemoglobin.
Hemoglobin is the protein in red blood cells that
carries oxygen. The disorder results in large
numbers of red blood cells being destroyed, which
leads to anemia.
Causes:
• The blood disorder is caused by a defect in the
gene that controls the production of hemoglobin.
The defective gene causes an inability to
produce normal hemoglobin. Children inherit
this gene from one or both parents. If a child
inherits the faulty gene from both parents, the
child will have thalassemia major.
Cont..

If the faulty gene is only passed on by one parent, the

child has thalassemia minor. The child then becomes a
carrier of the defective gene.

Hemoglobin is made of 2 proteins:

• Alpha globin

• Beta globin

• Thalassemia occurs when there is a defect in a gene

that helps control production of 1 of these proteins.
Types-
There are 2 main types of thalassemia:
• Alpha thalassemia occurs when a gene or genes related to the
alpha globin protein are missing or changed (mutated). To
make alpha globin, the child need to have four genes, two from
each parent.
• Beta thalassemia occurs when similar gene defects affect
production of the beta globin protein.
• Alpha thalassemias occur most often in people from Southeast
Asia, the Middle East, China, and in those of African descent.
Cont..
• Beta thalassemias occur most often in people of
Mediterranean origin. To a lesser extent, Chinese,
other Asians, and African Americans can be
affected.
• Both alpha and beta thalassemia include the
following 2 forms:
• Thalassemia major

• Thalassemia minor
Cont..

Child must inherit the gene defect from both

parents to develop thalassemia major. Thalassemia
minor occurs if you receive the faulty gene from
only 1 parent. People with this form of the
disorder are carriers of the disease. Most of the
time, they do not have symptoms. Beta
thalassemia major is also called Cooley anemia.
Risk factors for thalassemia include:
• Asian, Chinese, Mediterranean, or African American
ethnicity
• Family history of the disorder

Sign and symptoms-

• The most severe form of alpha thalassemia major
causes stillbirth or hydrops fetalis( all four alpha genes
are missing or mutated) (death of the unborn baby
during birth or the late stages of pregnancy).
Cont..
• Children born with beta thalassemia major (Cooley
anemia) are normal at birth, but develop severe
anemia during the first year of life.
Other symptoms can include:
• Bone deformities in the face
• Fatigue
• Growth failure
• Shortness of breath
• Yellow skin (jaundice)
• People with the minor form of alpha and beta
thalassemia have small red blood cells but no
symptoms.
Exams and Tests-
• Your health care provider will do a physical exam
to look for an enlarged spleen.
• A blood sample will be sent to a laboratory to be
tested.
• Red blood cells will appear small and abnormally
shaped when looked at under a microscope.
• A complete blood count (CBC) reveals anemia.
• A test called hemoglobin electrophoresis shows
the presence of an abnormal form of hemoglobin.
• A test called mutational analysis can help detect
alpha thalassemia.
Treatment-
• Treatment for thalassemia major often involves
regular blood transfusions and foliate
supplements. If you receive blood transfusions,
you should not take iron supplements. Doing so
can cause a high amount of iron to build up in the
body, which can be harmful.
Cont..

• People who receive a lot of blood transfusions

need a treatment called chelation therapy. This
is done to remove excess iron from the body.
• A bone marrow transplant
Outlook (Prognosis)-
• Severe thalassemia can cause early death
(between ages 20 and 30) due to heart failure.
Getting regular blood transfusions and therapy
to remove iron from the body helps improve the
outcome.
Cont..
• Less severe forms of thalassemia often do not
shorten lifespan.
• You may want to seek genetic counseling if you
have a family history of the condition and are
thinking of having children
Possible Complications-
• Untreated, thalassemia major leads to heart
failure and liver problems. It also makes a
person more likely to develop infections.
• A child with this blood disorder may have
gallstones. Other children may have poor growth
the major complications are related to iron
overload.
Prevention-
• People with any form of thalassemia should get
genetic counseling. Prenatal tests are also
available.
• Blood transfusions can help control some
symptoms, but carry a risk of side effects from
too much iron
• A low fat ,plant based diet.
HEMOPHILIA
• Hemophilia is a rare disorder in which your
blood doesn't clot normally because it lacks
sufficient blood-clotting proteins (clotting
factors). If you have hemophilia, you may bleed
for a longer time after an injury than you would
if your blood clotted normally.
Cont..
• Small cuts usually aren't much of a problem.
The greater health concern is deep bleeding
inside your body, especially in your knees,
ankles and elbows. That internal bleeding can
damage your organs and tissues, and may be
life-threatening.
cont,.
• Hemophilia is an inherited (genetic) disorder.
There's no cure yet. But with proper treatment
and self-care, most people with hemophilia
can maintain an active, productive lifestyle.
Symptoms-
• Signs and symptoms of hemophilia vary,
depending on your level of clotting factors. If
your clotting-factor level is mildly reduced,
you may bleed only after surgery or trauma. If
your deficiency is severe, you may experience
spontaneous bleeding.
Signs and symptoms of spontaneous bleeding
include:
• Unexplained and excessive bleeding from cuts
or injuries, or after surgery or dental work
• Many large or deep bruises
• Unusual bleeding after vaccinations
• Pain, swelling or tightness in your joints
• Blood in your urine or stool
• Nosebleeds without a known cause
• In infants, unexplained irritability
Emergency signs and symptoms of hemophilia
include:
• Sudden pain, swelling and warmth in large joints,
such as knees, elbows, hips and shoulders, and in
your arm and leg muscles
• Bleeding from an injury, especially if you have a
severe form of hemophilia
• Painful, prolonged headache
• Repeated vomiting
• Extreme fatigue
• Neck pain
• Double vision
Causes-
• When you bleed, your body normally pools blood
cells together to form a clot to stop the bleeding.
The clotting process is encouraged by certain
blood particles (platelets and plasma proteins).
Hemophilia occurs when you have a deficiency in
one of these clotting factors.
• Hemophilia is inherited. However, about 30
percent of people with hemophilia have no family
history of the disorder. In these people hemophilia
is caused by a genetic change (spontaneous
mutation).
Types-
There are several types of hemophilia. They are
classified according to which clotting factor is
deficient:
• Hemophilia A, the most common type, is
caused by insufficient clotting factor VIII.
• Hemophilia B, the second most common type,
is caused by insufficient clotting factor IX.
• Hemophilia C, in which signs and symptoms
are often mild, is caused by insufficient clotting
factor XI.
Hemophilia inheritance/Pathophysiology
• Everyone has two sex chromosomes, one from
each parent. A female inherits an X
chromosome from her mother and an X
chromosome from her father. A male inherits an
X chromosome from his mother and a Y
chromosome from his father.
Cont..
Hemophilia inheritance depends on your type of
hemophilia:
• Hemophilia A or B. The gene that causes them is
located on the X chromosome, so it can't be
passed from father to son. Hemophilia A or B
almost always occurs in boys and is passed from
mother to son through one of the mother's genes.
Most women with the defective gene are simply
carriers and experience no signs or symptoms of
hemophilia.
Cont..
Women can experience bleeding symptoms if
their factor VIII or IX is moderately decreased.
Hemophilia C. This disorder can be passed on to
children by either parent. Hemophilia C can occur
in girls and boys.
Diagnosis
• In children and adults, a blood test can show a
clotting-factor deficiency. Hemophilia is
diagnosed at an average age of 9 months and
almost always by age 2. Sometimes, mild
hemophilia isn't diagnosed until a person
undergoes surgery and experiences excessive
bleeding.
Treatment-
• While there's no cure for hemophilia, most
people with the disease can lead fairly normal
lives.
Treatment for bleeding episodes
Therapies to stop bleeding depend on the type of
hemophilia:
• Mild hemophilia A. Slow injection of the
hormone desmopressin (DDAVP) into a vein
can stimulate a release of more clotting factor to
stop bleeding. Occasionally, DDAVP is given as
a nasal medication
cont..
• Moderate to severe hemophilia A or hemophilia
B. Bleeding may stop only after an infusion of
recombinant clotting factor or clotting factor
derived from donated human blood. Repeated
infusions may be needed if internal bleeding is
severe.
• Hemophilia C. Clotting factor XI, the factor
missing in this type of hemophilia, is available only
in Europe. In the United States, plasma infusions
are needed to stop bleeding episodes.
Ongoing treatment-
• Regular infusions of DDAVP or clotting
factor. The infusions can help prevent bleeding.
This approach may reduce time spent in the
hospital and limit side effects such as damage to
joints. Your doctor can show you how to
perform the infusions.
Cont..
• Clot-preserving medications
(antifibrinolytics). These medications help
prevent clots from breaking down.
• Fibrin sealants. These medications can be
applied directly to wound sites to promote
clotting and healing. Fibrin sealants are
especially useful in dental therapy.
Cont.
• Physical therapy. It can ease signs and symptoms if
internal bleeding has damaged your joints. If internal
bleeding has caused severe damage, you may need
surgery.
• First aid for minor cuts. Using pressure and a
bandage will generally take care of the bleeding. For
small areas of bleeding beneath the skin, use an ice
pack. Ice pops can be used to slow down minor
Cont..
Vaccinations. Although blood products are
screened, it's still possible for people who rely on
them to contract diseases. If you have hemophilia,
consider receiving immunization against hepatitis
A and B.
Complications-
• Deep internal bleeding. Bleeding that occurs in deep
muscle can cause your limbs to swell. The swelling
may press on nerves and lead to numbness or pain.
• Damage to joints. Internal bleeding may also put
pressure on your joints, causing severe pain. Left
untreated, frequent internal bleeding may cause
arthritis or destruction of the joint.
Cont.
• Infection. People with hemophilia are likelier to have blood
transfusions, increasing their risk of receiving contaminated
blood products.
• Adverse reaction to clotting factor treatment. In some
people with hemophilia, the immune system has a negative
reaction to the clotting factors used to treat bleeding. When
this happens, the immune system develops proteins (known
as inhibitors) that inactivate the clotting factors, making
treatment less effective.
Nursing considerations-

Stay alert for bleeding-

• Monitor the patient for signs and symptoms of
bleeding. Pain is among the first symptoms of a joint
or soft-tissue bleed; the patient may complain of pain
in a localized area but lack outward signs of bleeding.
• Eventually, a bleed causes redness, warmth, and
swelling. As ordered, administer replacement factor
promptly and use rest, ice, compression, and elevation
Precautions for patients undergoing invasive
procedures-
• Surgical patients with hemophilia require close
monitoring, factor administration before the
procedure, and consideration of deep vein
thrombosis prophylaxis.. Many patients require
multiple infusions after major surgery
• Other invasive procedures that require pretreatment
with medication include colonoscopy, biopsy,
steroid injections, arthrocentesis, and dental
procedures. Consult a hematologist if a patient with
a bleeding disorder requires one of these procedures
Home treatment-
• Most patients with bleeding disorders know a
great deal about their disorder. At an early age,
they’re taught to recognize symptoms.
• Also, many patients infuse their own
replacement factor at home; many have
implanted ports for easier administration.
Children as young as age 6 are taught how to use
a butterfly needle to infuse factor.
POLYCYTHEMIA-
• Polycythemia is a rare and serious blood disease that
causes the bone marrow to produce too many red
blood cells (adjusted for race, age, sex and altitude) to
circulate in the blood stream. This causes the blood to
become thicker, makes it more difficult for blood to
flow throughout the body, and can lead to blood clots.

Polycythemia can be divided into:

• Primary or intrinsic polycythemia
• Secondary or extrinsic polycythemia
Cont..
Primary polycythemia has three forms:
• Newborn polycythemia
• Primary familial/congenital polycythemia
(PFCP)
• Polycythemia vera
Secondary polycythemia may be congenital
(e.g.hemoglobin variants) or acquired.
Causes of polycythemia-
• Primary polycythemia is caused by an acquired or
inherited gene mutation (change). Secondary
polycythemia is due to outside factors like lack of
oxygen from lung cardiac, kidney, or liver
disease, high altitude or abnormal hemoglobins
(in the newborn baby). These all stimulate
erythropoiesis.
Symptoms of polycythemia-
• Children with polycythemia frequently have no
symptoms. When present, common symptoms
include;
• Red/purple color
• feeding problems
• headaches
• trouble breathing
• dizziness
• itchiness, and
• weakness/tingling of hands and feet.
Diagnosis-
• Hemoglobin and hematocrit. It measures the
amount of hemoglobin and red blood cells in the
blood.
• Complete blood count, or CBC. A complete
blood count checks the red blood cells, white
blood cells, blood clotting cells (platelets), and
sometimes, young red blood cells
(reticulocytes). It includes hemoglobin and
hematocrit and more details about the red blood
cells.
Cont..
• Peripheral smear. A small sample of blood is
examined under a microscope. Blood cells are
checked to see if they look normal or not.
• Erythropoietin (EPO) level. This test
measures the amount of EPO in the blood. EPO
is a hormone involved in red blood cell
production.
Treatment-
• Venipuncture or removing some blood, thus
reducing the number of red blood cells (similar
to a person donating blood)
• Low-dose of aspirin to prevent blood clots.
• Medications to decrease the production of red
blood cells in the bone marrow.
Complications-
• Complications depend on how severe the
polycythemia is. They include:
• Blood clots in the veins of the liver

• Deep vein thromboses or blood clots in the veins

• Heavy bleeding

• Stroke

• Leukemia
THROMBOCYTOPENIA-
• Thrombocytopenia is defined as a platelet count
of <150,000/microL. It is clinically suspected
when there is a history of easy bruising or
bleeding, or it may present as an incidental
finding during routine evaluation or during
investigations performed for other reasons.
Causes-
most commonly infections (especially viral
infections) and destruction of platelets by the
immune system (called
immune thrombocytopenia or ITP).
Symptoms and diagnosis-

• Excessive or unusual bruising

• Petechiae (small red/purple spots on the skin)

• Blood blisters in the mouth

• Overt signs of bleeding

• A physician will take a complete medical history

and perform a physical examination.

• Lab test.
Treatment-
• Steroids, which help prevent bleeding by
decreasing the rate of platelet destruction.
Steroids, if effective, will result in an increase in
platelet counts seen within two to three weeks.
Side effects may include irritability, stomach
irritation, weight gain, hypertension and acne.
• Intravenous gamma globulin (IVGG), a
protein that contains many antibodies and also
slows the destruction of platelets. IVGG works
more quickly than steroids (within 24 to 48
hours).
Other treatments for ITP may include:
• Rh immune globulin — This medication
temporarily stops the spleen from destroying
platelets. Your child must be Rh positive and
have a spleen for this medication to be effective.
• Medication changes — If it is a medication is
the suspected cause of your child's ITP,
discontinuation or changing the medication may
be necessary.
• Infection treatment — If infection is the cause
for ITP, then treatment of the infection may
result in higher platelet count.
Cont..
• Splenectomy — In some cases , the child's spleen
may need to be removed since this is the site of
platelet destruction. This is considered more often in
older children with chronic ITP to decrease the rate of
platelet destruction.
• Hormone therapy — Teenage girls may need to take
hormones to stop their menstrual cycle when their
platelets are low if excessive bleeding occurs.
Follow-up care-
• If your child has ITP, you'll need to learn how to
best prevent injuries and bleeding. Consider the
following:
• For the young child, make the environment as safe
as possible. Padding a crib, wearing helmets, and
providing protective clothing are necessary when
platelet counts are low.
• Contact sports, riding bicycles and rough play
may need to be restricted.
• Avoid medications which contain aspirin, as they
may interfere with the body's ability to control
bleeding.
Nursing Interventions for Thrombocytopenia Disease:
• Various nursing intervention for thrombocytopenia disease are
mentioned in the following:
• Assess the Patient for any sign symptoms of unusual bleeding, such
as- Epistaxis, Hemoptysis, Gingival bleeding, Purpura.
• Monitor platelet and coagulation test results and report for any
abnormal values.
• Avoid any intramuscular or subcutaneous injection.
• Use small gauge needle when performing venous or arterial
punctures.
• Apply gentle prolonged pressure to venous and arterial or bone
marrow aspiration site.
• Avoid any anti-coagulant drugs without any doctor’s approval.
• Avoid any per rectal medication (Suppository) enemas, vaginal
douches.
• Avoid cutting nails, shaving with sharp blade.
Cont..
• Provide semi solid or liquid diet.
• Encourage the Patient to take adequate fluid to avoid
constipation.
• Use a soft bristle tooth brush and avoid dental floss.
• Do not strain to move bowels.
• Check BP only when necessary.
• Keep high fowlers position if epistaxis present and apply
pressure and ice pack to nasal area.
• Instruct client to avoid nose blowing and keep hydrate nasal
mucosa with nasal drop.
• Keep bed lower position with side rails up to prevent patient fall.
• Transfuse blood cells (Platelets, fresh frozen plasma or Packed
Red Blood Cell) as ordered.
• Check any transfusion reaction and report it.
Cont.
• Monitor intake output carefully to evaluate renal function.
• Administer medication like steroids, IVIG, Rituximab as ordered.
• Monitoring for side effects of the prescribed medications and their
interactions with other drugs.
• Encourage patient to give up alcoholism because these can worse
bleeding problem.
• Instruct patient to Avoid contact sports and other activities that
might cause injury.
• Instruct patient to avoid contact with toxic chemicals. Chemicals
such as pesticides, arsenic, and benzene can slow the production of
platelets.
• Avoid medicines that you know have decreased your platelet count
in the past.
• Instruct patient to avoid some medications that increase bleeding
tendency such as aspirin, ibuprofen.
Cont..
• Arrange and vaccinated patient against some
disease such as mumps, measles, rubella and
chickenpox.
• Provide good oral care (using oral swabs, a
normal saline rinse, or other mouthwash as
appropriate) helps to keep mucous membranes
moist and minimize bleeding.
• Instruct the patient to do not walk in bare feet.
• Instruct the patient to avoid wear tight clothing.
DISSEMINATED INTRAVASCULAR
COAGULATION-
• DIC, is a condition in which blood clots form
throughout the body's small blood vessels. These
blood clots can reduce or block blood flow
through the blood vessels, which can damage the
body's organs. DIC can cause life-threatening
bleeding.
Causes-
• Some diseases and conditions can disrupt the
body's normal blood clotting process and lead to
disseminated intravascular coagulation (DIC).
These diseases and conditions include:
• Sepsis (an infection in the bloodstream)
• Surgery and trauma
• Cancer
• Serious complications of pregnancy and
childbirth
Sign and symptoms-
Signs and Symptoms of Excessive Blood Clotting
• In DIC, blood clots form throughout the body's
small blood vessels. These blood clots can reduce or
block blood flow through the blood vessels. This can
cause the following signs and symptoms:
• Chest pain and shortness of breath
• Pain, redness, warmth, and swelling in the lower leg
• Headaches, speech changes, paralysis (an inability
to move), dizziness, and trouble speaking and
understanding if blood clots form in the blood
vessels in your brain
Signs and Symptoms of Bleeding:
• In DIC, the increased clotting activity uses up the platelets
and clotting factors in the blood. As a result, serious
bleeding can occur. DIC can cause internal and external
bleeding.
• Internal Bleeding

• Internal bleeding can occur in your body's organs, such as

the kidneys, intestines, and brain. This bleeding can be life
threatening. Signs and symptoms of internal bleeding
include:
Cont..
• Blood in your urine from bleeding in your
kidneys or bladder.
• Blood in your stools from bleeding in your
intestines or stomach. Blood in your stools can
appear red or as a dark, tarry color. (Taking iron
supplements also can cause dark, tarry stools.)
• Headaches, double vision, seizures, and other
symptoms from bleeding in your brain.
Cont.
• External bleeding may cause purpura .Purpura are
purple, brown, and red bruises. This bruising may
happen easily and often. Petechiae are small red
or purple dots on your skin.
• Other signs of external bleeding include:
• Prolonged bleeding, even from minor cuts.
• Bleeding or oozing from your gums or nose,
especially nosebleeds or bleeding from brushing
your teeth.
• Heavy or extended menstrual bleeding in women.
Diagnostic test- Complete Blood Count and
Blood Smear
Tests for Clotting Factors and Clotting Time
• The following tests examine the proteins active
in the blood clotting process and how long it
takes them to form a blood clot.
• PT and PTT tests. These tests measure how long
it takes blood clots to form.
• Serum fibrinogen. Fibrinogen is a protein that
helps the blood clot. This test measures how
much fibrinogen is in your blood.
Treatment-
• Acute Disseminated Intravascular
Coagulation- Treatment may include
blood transfusions, medicines, and oxygen therapy.
• Chronic Disseminated Intravascular Coagulation-
People who have chronic DIC are more likely to have
blood clotting problems than bleeding. If you have
chronic DIC, your doctor may treat you with
medicines called anticoagulants, or blood thinners.
• Blood thinners help prevent blood clots from
forming. They also keep existing blood clots from
getting larger.
Bibliopgraphy-
• Cappellini MD. The thalassemias. In: Goldman L, Schafer AI,
eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier
Saunders; 2016:chap 162.

• DeBaun MR, Frei-Jones MJ, Vichinsky EP. Hemoglobinopatiies. In:

Kliegman RM, Stanton BF, St Geme JW, Schor NF, eds. Nelson Textbook of
Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 462.
•
• Giardina PJ, Rivella S. Thalassemia syndromes. In: Hoffman R, Benz EJ Jr,
Silberstein LE, Heslop HE, Weitz JI, Anastasi J, eds. Hematology: Basic
Principles and Practice. 6th ed. Philadelphia, PA: Elsevier Saunders;
2013:chap 38.

• https://www.nhlbi.nih.gov/health-topics/disseminated-intravascular-
coagulation.