Term
| Sickled Hemoglobin results in: |
|
Definition
- Red blood cell trapping
- Impedance of blood flow (obstruction, hypoxia, infarction)
- Break down of damaged, rigid cells resulting in anemia
|
|
|
Term
| Signs/Symptoms of Sickle Cell Disease |
|
Definition
- Chronic pain
- Anemia
- Fatigue
- Pallor
- Abdominal pain
- Hematuria
|
|
|
Term
| The definitive test used to diagnose sickle cell syndromes is: |
|
Definition
| Hemoglobin electrophoresis |
|
|
Term
| Major Complications Associated with Sickle Cell Syndromes |
|
Definition
- Pain crisis
- Cerebrovascular accident
- Acute chest syndrome
- Dactylitis
- Priapism
- Splenic sequestration
- Aplastic crisis
- Infection
|
|
|
Term
| Cerebrovascular accident results in: |
|
Definition
- Hemiparesis
- Dysphasia
- Seizures
|
|
|
Term
| Acute chest syndrome: diagnosis |
|
Definition
- Presence of pulmonary infiltrate on chest x-ray
|
|
|
Term
| Acute chest syndrome: signs/symptoms |
|
Definition
- Fever
- Cough
- Tachypnea
- Chest pain
- Hypoxia
|
|
|
Term
| Splenic sequestration is characterized by: |
|
Definition
- Severe anemia
- Splenomegaly
- Hypovolemic shock
|
|
|
Term
| Splenic Sequestration: Signs/Symptoms |
|
Definition
- Irritability
- Unusual sleepiness
- Looks pale
- Weakness
- Fast heart beat
- Big spleen
- Pain on left side of abdomen
|
|
|
Term
| Presentation of Aplastic Crisis |
|
Definition
- Fatigue
- Severe anemia with few reticulocytes
- Dyspnea
|
|
|
Term
| Aplastic Crisis: Signs/Symptoms |
|
Definition
- Paleness
- Lethargy
- Headache
- Fever
- Low blood count
- Recent upper respiratory tract infection
- Fainting
|
|
|
Term
| Management of Pain: Pharm, NonPharm |
|
Definition
Nonpharm
- heating pad
- whirlpool
- relaxation techniques
Pharm
- Hydration
- Opiate analgesics
- NSAIDs for bone pain
- Bowel regimens for opioid-induced constipation (docusate, senna, miralax)
- Benadryl for opioid-induced itching
|
|
|
Term
|
Definition
MS Contin
- Peak analgesia: 3-4 hrs
- Duration of action: 8-12 hrs
- 24 hr IV morphine X 3 = 24 hr PO dose
Oxycontin
- Peak analgesia: 2-3 hrs
- Duration of action: 12 hrs
- 24 hrs IV morphine X 1.5 = 24 hr PO dose
|
|
|
Term
| Most likely pathogens to cause an infection in sickle cell syndrome |
|
Definition
- Streptococcus pneumoniae
- Haemophilus influenzae
- Salmonella
|
|
|
Term
| Antibiotic therapy: treatment |
|
Definition
Cephalosporins
Cefotaxime
Cefuroxime
Do not use Ceftriaxone -- high rate of hemolysis
Vancomycin
Clindamycin |
|
|
Term
| Antibiotic therapy: prophylaxis |
|
Definition
Penicillin
6mo - 5 yrs
No benefit after 5 yrs
Alt Therapies
Erythromycin
TMP/SMX |
|
|
Term
| Antibiotic therapy: Immunizations |
|
Definition
- Pneumococcal vaccines (pneumovax, prevnar)
- Influenza vaccine
|
|
|
Term
| Transfusion therapy is used in patients with: |
|
Definition
- Aplastic crisis
- Sequestration crisis
- Priapism
- Acute or suspected CVA
- Acute chest syndrome
- Severe symptomatic anemia
|
|
|
Term
| Chronic transfusion is used to: |
|
Definition
- prevent recurrent CVA
- treat recurrent episodes of acute chest syndrome
- treat chronic CHF
|
|
|
Term
| Pathophysiology of Sickle Cell Disease |
|
Definition
- Hemoglobin A consists of 2 alpha and 2 beta chains
- Substitution of valine for glutamic acid in the 6th position of the beta chain results in hemoglobin S or sickle hemoglobin
- Under certain conditions, such as low oxygen levels, the RBCs with the mutation will sickle
- These sickled cells can block small vessels producing microvascular occlusions which may cause necrosis of tissue
|
|
|
Term
| The leading cause of hospitalization in sickle cell disease patients is: |
|
Definition
|
|
Term
| The first manifestation of sickle cell disease is usually: |
|
Definition
|
|
Term
| Patients at highest risk for splenic sequestration are: |
|
Definition
|
|
Term
| Treatment of Cerebrovascular Accident |
|
Definition
- Acute management
- Chronic transfusion therapy
|
|
|
Term
| Treatment of Acute Chest Syndrome |
|
Definition
- Oxygen
- IVFs
- Narcotics
- Antibiotics
- Transfusion
|
|
|
Term
|
Definition
|
|
Term
|
Definition
|
|
Term
| Treatment of Splenic Sequestration |
|
Definition
|
|
Term
| Treatment of Aplastic Crisis |
|
Definition
- Supportive care
- May include transfusion
|
|
|
