Exam 3: Sickle Cell Case Study

Question 1

SCD Pathophysiology (5)

Answer 1

RBC are sickle shaped, and unable to adequately perfuse the body. They also have a life span of 10-20 days vs. the normal 120 days

IN SUM:

1. RBC are crescent or sickle shaped
2. Inadequate perfusion
3. Shorter life span (10-20 days)
4. RBC can go back and forth between sickle and bioconcave shape
5. Not all the cells are sickled

Question 2

Vaso-occlusive crisis (4)

Answer 2

A common painful complication of sickle cell anemia, where patients will experience:

1. Increased temp
2. Sick-feeling
3. Poor fluid intake
4. Pain

*It is the most common crisis of SCD

Question 3

Vaso-occlusive crisis triggers

Answer 3

Dehydration related to fever and stomach viral infection

Question 4

Other SCD Complications (13)

Answer 4

1. Hyperbilirubinemia
2. Gallstones
3. Acute chest syndrome
4. Pulmonary HTN
5. Ischemia (stroke)
6. Leg ulcers that don’t heal
7. Eye damage
8. Aplastic crisis
9. Hypovolemic shock
10. Sequestration crisis
11. Decrease in hbt/hct
12. Megaloblastic crisis
13. Sluggish movements in liver or spleen

EVERY ORGAN IS AFFECTED BY SCD*

Question 5

Aplastic crisis

Answer 5

When RBC aren’t producing fast enough (temporary cessation of RBC production) causing a precipitous drop in hemoglobin and an increase in reticulocytes

Reticulocytosis= an elevation in immature RBC, commonly seen with anemia

• *Can be a complication of Parvovirus (5th disease), which can attack bone marrow and cause the aplastic crisis
• MAY REQUIRE BLOOD TRANSFUSION WITH FIFTH DISEASE TO PX OR TX APLASTIC CRISIS

Question 6

Hyperhemolytic

Answer 6

Hyperhemolytic= higher than normal rate of hemolysis

Increased RBC destruction, causing anemia, jaundice, and increase in reticulocytes

*Increased reticulocytes b/c bone marrow is affected, and it puts out more RBC to compensate

Question 7

Sequestration crisis

Answer 7

Pooling of blood in liver and spleen (heptaospleenomegaly)

Acute splenic sequesteraton crisis:

1. enlarged, tender spleen
2. drop in Hbg
3. thrombocytopenia
4. reticulocytosis

*Palpate spleen regularly!!!

Question 8

Acute chest syndrome (4)

Answer 8

1. Chest pain
2. Fever
3. Pneumonia-like cough
4. Severe anemia

Question 9

Megaloblastic crisis

Answer 9

Excessive need for folic acid and vitamin B12

*Occurs with non-compliance of SCD meds

Question 10

Pain management of vaso-occlusive crisis

Answer 10

1. PCA with either morphine or Dilaudid AND IV Toradol (an NSAID)
   * *MUST CHECK CREATININE BEFORE ADMINISTERING TORADEL, DO NOT GIVE IF CREATININE IS ELEVATED
2. Can be transition to Methadone PO (long half life)
3. Warm soaks

Question 11

Side effects of meds for vaso-occlusive crisis (5)

Answer 11

Diluadid, Morphine, Toradol (transition to Methadone)

1. Constipation
2. Itching
3. Nausea
4. Sleepiness
5. Decreased urination

~Think dry

Question 12

Penicillin and SCD

Answer 12

Penicillin is prescribed for SCD patients due to functional aspleenia

*Infection is the leading cause of death in children under 5 with SCD

Question 13

Hydroxyurea

Answer 13

Reactivates FETAL Hbg, increasing the oxygenation of RBC

• Can be given at 4 years old
• Is also used for chemp
• Watch RBC levels

Question 14

SCD Immunizations

Answer 14

No immunizations are contraindicated with SCD, but you may wait to give immunizations if patient is experiencing a crisis

1. Additional HibMenCY is given @ 2,4,6, and 12-15 months
2. Extra pneumococcal vaccine (polysaccharide) is given at 2 years old to cover more strains of the infection
3. KEEP ON PENICILLIN UNTIL ALL IMMUNIZATIONS ARE COMPLETE

Question 15

Indications for blood transfusions with SCD (4)

Answer 15

1. Hgb below 6-7
2. CVA
3. Patient should be screened for alloantibodies to avoid reaction
4. Administer Deferoxamine (Desferal) while receiving a blood transfusion

Question 16

Deferoxamine (Desferal)

Answer 16

An iron-chelating agent; binds with excess iron
*Administered to SCD patients receiving a blood transfusion

Patient should be notified that urine will turn orange and it may cause auditory or visual changes

Question 17

SCD Screening

Answer 17

Testing can be done before the baby is born using amniocentesis and chorionic villi testing

AMNIOCENTESIS AND CHORIONIC VILLI TESTING

*Since it is autosomal recessive, there is a 25% chance of having a child with SCD each pregnancy if it runs in the family

Question 18

SCD Test Post-birth

Answer 18

Hgb electrophoresis

Question 19

Long term complications of SCD (9)

Answer 19

1. Renal
2. Retinopathy
3. CHF
4. Liver failure
5. Functional asplenia
6. Avascular necrosis of hips and shoulders
7. Chronic hemolytic anemia
8. Poor wound healing
9. CVA

Question 20

Is there a cure for SCD?

Answer 20

Yes, stem cell transplant

Question 21

Patient teaching on SCD (7)

Answer 21

1. HYDRATION IS IMPORTANT!!
2. Inform about situations that cause dehydration
3. Notify HCP if fever is above 101.5
4. Avoid extreme cold
5. Pain managment
6. Know signs of splenic sequestration crisis and respiratory disease
7. Importance of medication adherence and immunizations

Question 22

Scarring of organs from sickling

Answer 22

Pulmonary embolisms and CVA are caused by the damage of the vessels due to sickling

causes shorter life expectancy