VON WILLEBRAND DISEASE Type 3 is the most dangerous type of von Willebrand disease.

If you have this type,

your body won’t produce any VWF. As a result, your platelets won’t be able to clot.
Von Willebrand disease (VWD)- is a blood disorder in which the blood does not clot
This will put you at risk of severe bleeding that’s difficult to stop.
properly.
CAUSES:
*People with VWD are either missing or low in the clotting protein von Willebrand
factor (VWF) – or it doesn’t work as it’s supposed to. *Inherited from mother/father who has a history of VWD.

PATHO: *Can acquire later called Acquired VWD on due to underlying medical condition.

*Many different proteins are needed to make a person’s blood clot successfully. SIGNS AND SYMPTOMS:
VWF is a protein that helps platelets clump together, or clot.
The main symptoms of VWD are:
*Normally, when a person is injured and starts to bleed VWF binds to factor VIII (8),
another clotting protein, and platelets in blood vessel walls. This helps the platelets  Frequent (more than 5 a year) nosebleeds that last longer than 10 minutes
stick together, like glue, to form a clot at the site of injury and stop the bleeding.  Bleeding from cuts or injuries that lasts longer than 10 minutes
 Bruising easily, with bruises that are raised and larger than a quarter
PROBLEM:
 For women, girls and those who menstruate:
*When a person has VWD, because the VWF doesn’t work the way it should, the o Heavy periods, also called heavy menstrual bleeding, (having to
clot might take longer to form or form incorrectly and bleeding might take longer to change one pad or tampon every hour) or periods that last longer
stop. This can lead to heavy, hard-to-stop bleeding. than 7 days
o Heavy bleeding after childbirth or miscarriage
*When you have low levels of this protein or it doesn't work as it should, small
blood cells called platelets cannot stick together properly nor attach themselves Type 3 is the most severe form of the condition. If you have this type, you have no
normally to the blood vessel walls when an injury has occurred. This interferes with VWF in your body. This makes episodes of bleeding difficult to control. It also raises
the clotting process and can sometimes cause uncontrolled bleeding. your risk of internal bleeding, including bleeding in your joints and gastrointestinal
system.
TYPES:
DIAGNOSIS:
Type 1 is the most common type of von Willebrand disease. It causes lower-than-
normal levels of VWF to occur in your body. You still have small amounts of VWF in *Personal and family histories of bleeding.
your body to help clot blood.
*Check for unusual bruising or other signs of recent bleeding and order some blood
Type 2 von Willebrand disease, you have normal levels of VWF but it won’t work tests to measure how the blood clots
properly due to structural and functional defects.
  Prothrombin Time (PT) Test- measures the time it takes for blood *Monitor hemoglobin and hematocrit levels; assess for inhibitor antibody to factor
to clot. It measures primarily the clotting ability of factors I (1), II VIII; anticipate or instruct in the need for prophylactic treatment before high-risk
(2), V (5), VII (7), and X (10). situations, such as invasive diagnostic or surgical procedures, or dental work; and
 Activated Partial Thromboplastin Time (APTT) Test- This test provide replacement therapy of deficient clotting factors.
measures how long it takes for blood to clot. It measures the
*Utilize appropriate toys (soft, not pointed or small sharp objects); for infants, may
clotting ability of factors VIII (8), IX (9), XI (11), and XII (12).
need to use padded bed rail sides on crib; avoid rectal temperatures; provide
 Complete Blood Count (CBC)- This common test measures the
appropriate oral hygiene (use of a water irrigating device; use of a soft toothbrush
amount of hemoglobin (the red pigment inside red blood cells that
or softening the toothbrush with warm water before brushing; use of sponge-tipped
carries oxygen), the size and number of red blood cells, and the
toothbrush); and avoid contact sports such as football, soccer, ice hockey, karate.
numbers of different types of white blood cells and platelets found
in blood.

MEDICAL MANAGEMENT:

Desmopressin Acetate Injection- is injected into a vein to treat people with milder
forms of VWD (mainly Type 1).

Factor Replacement Therapy- are used to treat people with more severe forms of
VWD or people with milder forms of VWD who do not respond well to the nasal
spray.

Birth control pills can increase the levels of VWF and factor VIII in the blood and
reduce menstrual blood loss.

NURSING MANAGEMENT:

*Relieve pain. Immobilize joints and apply elastic bandages to the affected joint if
indicated; elevate affected and apply a cold compress to active bleeding sites, but
must be used cautiously in young children to prevent skin breakdown.

*Provide gentle, passive ROM exercise when the child’s condition is stable; educate
on preventive measures, such as the application of protective gear and the
administration of factor products; and refer for physical therapy, occupational
therapy, and orthopedic consultations, as required.
THALASSEMIA TYPES:

THALASSEMIA - is an inherited blood disorder that causes your body to have less There are two main types of thalassemia:
hemoglobin than normal.  Alpha thalassemia occurs when a gene or genes related to the alpha globin
protein are missing or changed (mutated).
PATHO:
 Beta thalassemia occurs when similar gene defects affect production of the
*Red blood cells carry oxygen to all the cells of the body. beta globin protein
*Oxygen is a sort of food that cells use to function.
SIGNS AND SYMPTOMS:
*Hemoglobin is the substance in red blood cells that carries oxygen throughout your
body. Thalassemia signs and symptoms can include:
*Hemoglobin is made up of two different components (subunits): beta globin and
alpha globin.  Fatigue
 Weakness
*The HBB gene provides instructions for making beta globin, while the HBA1 and
 Pale or yellowish skin
HBA2 genes provide instructions for making alpha globin.
 Facial bone deformities (the bone marrow expands, deforming the
CAUSES: bone around it, especially the bones of the skull and face. The bone
*Thalassemia is caused by mutations in the DNA of cells that make hemoglobin — can become brittle, increasing the risk of fracture)
the substance in red blood cells that carries oxygen throughout your body.  Slow growth
*In thalassemia, the production of either the alpha or beta chains are reduced,  Abdominal swelling (The spleen recycles red blood cells. In
resulting in either alpha-thalassemia or beta-thalassemia. thalassemia, the red blood cells may have an abnormal shape, making
it harder for the spleen to recycle them. The cells accumulate in the
PROBLEM:
spleen, making it grow.)
*Thalassemia occurs when there is a defect in a gene that helps control production  Dark urine
of one of these proteins. (Depends on your diagnosis either it is thalassemia A or
thalassemia B).
MEDICAL MANAGEMENT:

 blood transfusions
 bone marrow transplant
 SICKLE CELL ANEMIA
 medications and supplements
 possible surgery to remove the spleen or gallbladder SICKLE CELL ANEMIA- is a genetic disease of the red blood cells (RBCs) which there
aren't enough healthy red blood cells to carry oxygen throughout your body.

NURSING MANAGEMENT: PATHO:

*Assist the client in planning and prioritizing activities of daily living (ADL); assist the *Normally, RBCs are shaped like discs, which gives them the flexibility to travel
client in developing a schedule for daily activity and rest; and stress the importance through even the smallest blood vessels.
of frequent rest periods.
PROBLEM:
*Explain the importance of the diagnostic procedures (such as complete blood
*The abnormal hemoglobin causes red blood cells to become rigid, sticky and
count), bone marrow aspiration and a possible referral to a hematologist; and
misshapen.
explain the hematological vocabulary and the functions of blood elements, such as
white blood cells, red blood cells, and platelets. * These rigid, sticky cells can get stuck in small blood vessels, which can slow or
block blood flow and oxygen to parts of the body.
*Assess for local or systemic signs of infection, such as fever, chills, swelling, pain,
and body malaise; instruct the client to avoid contact with people with existing * Both mother and father must pass the defective form of the gene for a child to be
infections. affected.

*Assess for any frank bleeding from the nose, gums, vagina, or urinary or SIGNS AND SYMPTOMS:
gastrointestinal tract and monitor platelet count.
* Anemia. Sickle cells break apart easily and die, leaving you with too few red blood
cells. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells
(anemia).

* Swelling of hands and feet. The swelling is caused by sickle-shaped red blood cells
blocking blood flow to the hands and feet.

* Frequent infections. Sickle cells can damage your spleen, leaving you more
vulnerable to infections.

* Episodes of pain. Periodic episodes of pain, called pain crises, are a major
symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells
block blood flow through tiny blood vessels to your chest, abdomen and joints.