Condition Info - BotMed Application

ConditionName

Sickle cell disease

ConditionDescription

Symptoms

Acute painful vaso-occlusive crisis caused by:
Hypoxia;
Dehydration;
Infection;
Trauma;
Cold temp;
Pain in chest, back, thighs;
May have fever;
Anemia;
Jaundice;
Cholelithiasis;
Murmurs;
Delay growth;

PhysicalFindings

ClinicalTests

Best initial test: peripheral smear (sickle cells);

(Howell-Jolly bodies – after splenectomy);
Most accurate test: electrophoresis (HbS);
↑LDH, ↑indirect bilirubin;
Renal manifestation is a only significant manifestation  - blood in UA;

RCC is high if pt doesn’t has folate or B12 deficiency, parvovirus;

If Hematocrit drops too fast in few days think about PB19 virus --> PCR test (most accurate) or ABs;

RiskFactors

Genetic (position 6 in beta globin chain: Valine replace glutamic acid.
Autosomal recessive;

African Americans;

IsRedFlag

IsNPLEX

Consequence

Bilirubin  gallstones;
Splenomegaly, jaundice;
Chronic hemolysis, leg ulcers;
Increased infections from auto splenectomy (encapsulated);
Osteomyelitis (Salmonella);
Retinopathy;
Dyspnea;
Swelling of hand and feet;
Stroke;
Sepsis;
Enlarged heart with systolic murmur;
Skin ulcers;
Avascular necrosis or femoral head;
Children – dactylitis (inflammation of fingers);
Death from infection;

ProgressionFromCondition

TreatmentOptions

Oxygen;
Hydration;
Prevent stress! It can cause vasoconstriction ;

PharmOptions

Analgesia;
If fever or high WBC: antibiotics for encapsulated;

Folic acid - always;
Pneumococcal vaccine and influenza;
Avoid crises;
After splenectomy they are immunocompromised!
Crises during severe hypoxia and acidosis;
Bone marrow transplantation cure. ONLY!

WebLink

https://www.merckmanuals.com/en-ca/professional/hematology-and-oncology/anemias-caused-by-hemolysis/sickle-cell-disease