Chapter 47: The Child with a Hematological Alteration n226

What is Beta-Thalassemia

- Abnormality in hemoglobin synthesis - Chronic production and destruction of RBCs

Describe care/management for hemophilia

- Avoid Aspirin & NSAIDS - NO HEAT on injury - use RICE - No injury prone activities - Aminocaproic acid or tranexamic acid (bleeding)

What is hemophilia?

- Bleeding disorder - Hemarthrosis bleeding into joint - Causes joint destruction

What are manifestations of Thalassemia

- Bossing - Bronze skin tone (jaundice) from ↑bilirubin

What is iron deficiency anemia?

- Decreased intake (r/t cow's milk intake) - Increased iron or blood loss - Periods of rapid growth - Peak 9-24 months, when transitioning to whole milk

How do you care for DIC?

- Plasma, RBC, platelets - What caused this? - Heparin to dissolved clots initially, controversial

Describe Thalassemia-Management

- RBC/Erythrocyte transfusions - Chelation therapy to help w/ iron overload - Splenectomy - Keep Hbg around 11 g/dl

Describe care for ITP (immune thrombocytopenic purpura)

- Steroids to block autoimmune destruction - IVIG to stimulate platelet production - splenectomy (increased infection risk)

Describe care von Willebrand disease?

- desmopressin acetate - Activity limitation - No NSAIDS!

What can cause a sickle cell crisis?

- extreme stress - vigorous exercise - high altitudes (low 02) - Cold exposure - Dehydration - Infections!!

Describe care for aplastic anemia

- stem cell transplant - immunosuppressants - Positive pressure outflow room placement

A nurse is caring for a child with von Willebrand disease. The nurse is aware that which of the following is a (are) clinical manifestation(s) of von Willebrand disease? Select all that apply. 1. Bleeding of the mucus membranes. 2. The child bruises easily. 3. Excessive menstruation. 4. The child has frequent epistaxis. 5. Elevated creatinine levels. 6. The child has a factor IX deficiency.

1. Bleeding of the mucus membranes. 2. The child bruises easily. 3. Excessive menstruation. 4. The child has frequent epistaxis.

Which test provides a definitive diagnosis of aplastic anemia? 1. Complete blood count with differential. 2. Bone marrow aspiration. 3.Serum immunoglobulin G (IgG) levels. 4. Basic metabolic panel.

2. Bone marrow aspiration.

The nurse is caring for a child with immune thrombocytopenic purpura (ITP) with a thrombocyte count of 5000/mm3. Which of the following should the nurse administer? 1. Platelets 2. Intravenous immunoglobulin 3. Packed red blood cells 4. White blood cells

2. Intravenous immunoglobulin

A nurse is doing discharge teaching with a parent who has a child with beta-thalassemia (Cooley anemia). The nurse informs the parent that the child is at risk for which of the following conditions? 1. Thyroid hypertrophy 2. Polycythemia vera 3. Thrombocytopenia 4. Chronic hypoxia and iron overload

4. Chronic hypoxia and iron overload

Avoid infection (biggest trigger) with sickle cell. What infection should be avoided the most?

Avoid 5ths disease (Slap cheek), encourage hydration!!!

Should you give aspirin, ibuprofen or acetaminophen to a child with sickle cell?

Avoid aspirin. Ibuprofen and acetaminophen are ok to give

What is von Willebrand disease?

Deficiency or defective von Willebrand's factor (vWF) w/ low levels of Factor VIII. This binds to platelets & attracts them to the site of injury. - Usual concurrent deficiency of clotting factor 8

What type of hemophilia can Desmopressin be used for

Desmopressin acetate nasal spray (vasoconstrictor) for TYPE A only

At what age can you have cows milk?

Do not consume if under 1 years old because it can break down protein. >1 yr, only have 24oz per day

How do you administer iron?

Given between meals & take with ORANGE JUICE. Drink w/ straw, avoid teeth. Stools may become tarry.

What is DIC (Disseminated Intravascular Coagulation)?

Initially, uncontrolled clotting, then they deplete and uncontrolled bleeding occurs. - Bruising, petechiae, oozing from puncture sites then quickly worsens - High D-Dimer

What is sickle cell disease?

It is an inherited blood disorder that causes the change in the primary structure of the hemoglobin, and it makes the cells look like sickles. Primarily in black individuals.

How long does a RBC live with sickle cell disease?

Live only 10-20 days instead of 3-4 months

What medication is commonly given when a child comes into the hosp with a sickle cell crisis?

Morphine

What is ITP (immune thrombocytopenic purpura)?

Platelets are coated w antigens so they are destroyed. Autoimmune - Thrombocytopenia (<150) - usually follows viral illness - Purpura (large masses of petechiae)

What is aplastic anemia?

destruction or inhibition of red bone marrow - Pancytopenia (↓ WBC, plts, Hgb) - Yellow, fatty bone marrow