Exam 3: Sickle Cell Case Study Flashcards
SCD Pathophysiology (5)
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:
- RBC are crescent or sickle shaped
- Inadequate perfusion
- Shorter life span (10-20 days)
- RBC can go back and forth between sickle and bioconcave shape
- Not all the cells are sickled
Vaso-occlusive crisis (4)
A common painful complication of sickle cell anemia, where patients will experience:
- Increased temp
- Sick-feeling
- Poor fluid intake
- Pain
*It is the most common crisis of SCD
Vaso-occlusive crisis triggers
Dehydration related to fever and stomach viral infection
Other SCD Complications (13)
- Hyperbilirubinemia
- Gallstones
- Acute chest syndrome
- Pulmonary HTN
- Ischemia (stroke)
- Leg ulcers that don’t heal
- Eye damage
- Aplastic crisis
- Hypovolemic shock
- Sequestration crisis
- Decrease in hbt/hct
- Megaloblastic crisis
- Sluggish movements in liver or spleen
EVERY ORGAN IS AFFECTED BY SCD*
Aplastic crisis
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
Hyperhemolytic
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
Sequestration crisis
Pooling of blood in liver and spleen (heptaospleenomegaly)
Acute splenic sequesteraton crisis:
- enlarged, tender spleen
- drop in Hbg
- thrombocytopenia
- reticulocytosis
*Palpate spleen regularly!!!
Acute chest syndrome (4)
- Chest pain
- Fever
- Pneumonia-like cough
- Severe anemia
Megaloblastic crisis
Excessive need for folic acid and vitamin B12
*Occurs with non-compliance of SCD meds
Pain management of vaso-occlusive crisis
- PCA with either morphine or Dilaudid AND IV Toradol (an NSAID)
* *MUST CHECK CREATININE BEFORE ADMINISTERING TORADEL, DO NOT GIVE IF CREATININE IS ELEVATED - Can be transition to Methadone PO (long half life)
- Warm soaks
Side effects of meds for vaso-occlusive crisis (5)
Diluadid, Morphine, Toradol (transition to Methadone)
- Constipation
- Itching
- Nausea
- Sleepiness
- Decreased urination
~Think dry
Penicillin and SCD
Penicillin is prescribed for SCD patients due to functional aspleenia
*Infection is the leading cause of death in children under 5 with SCD
Hydroxyurea
Reactivates FETAL Hbg, increasing the oxygenation of RBC
- Can be given at 4 years old
- Is also used for chemp
- Watch RBC levels
SCD Immunizations
No immunizations are contraindicated with SCD, but you may wait to give immunizations if patient is experiencing a crisis
- Additional HibMenCY is given @ 2,4,6, and 12-15 months
- Extra pneumococcal vaccine (polysaccharide) is given at 2 years old to cover more strains of the infection
- KEEP ON PENICILLIN UNTIL ALL IMMUNIZATIONS ARE COMPLETE
Indications for blood transfusions with SCD (4)
- Hgb below 6-7
- CVA
- Patient should be screened for alloantibodies to avoid reaction
- Administer Deferoxamine (Desferal) while receiving a blood transfusion
Deferoxamine (Desferal)
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
SCD Screening
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
SCD Test Post-birth
Hgb electrophoresis
Long term complications of SCD (9)
- Renal
- Retinopathy
- CHF
- Liver failure
- Functional asplenia
- Avascular necrosis of hips and shoulders
- Chronic hemolytic anemia
- Poor wound healing
- CVA
Is there a cure for SCD?
Yes, stem cell transplant
Patient teaching on SCD (7)
- HYDRATION IS IMPORTANT!!
- Inform about situations that cause dehydration
- Notify HCP if fever is above 101.5
- Avoid extreme cold
- Pain managment
- Know signs of splenic sequestration crisis and respiratory disease
- Importance of medication adherence and immunizations
Scarring of organs from sickling
Pulmonary embolisms and CVA are caused by the damage of the vessels due to sickling
causes shorter life expectancy
