CF/SCD/ED/PHTN/MS Flashcards
(51 cards)
How to diagnose CF?
Early symptoms?
Sweat gland chloride test >60 mmol/L
Failure to gain weight, salty tasting skin, steatorrhea, episodes of pneumonia
What type of heriditary disease is CF?
Autosomal recessuve
What are the two major organ systems affected in CF?
Pancreas
-endocrine issue leased to CF related diabetes
-Exocrine issues leads to malnutrition
Lungs
-Causes bronchitis and pneumonia to occur more frequently
How do you manage malnutrition in CF?
High calorie diet
Vitamins like ADEK
Pancreatic enzymes
What organisms are common in CF patients?
PSA, MSSA, MRSA, h.influenza
What are the options for chronic PSA infections in CF patients?
Inhaled tobramycin BID 28 days on 28 days off
Inhaled aztreona e TID 28d on 28d off
Dornase alfa
Brand
ROA
Indication
Storage
Pulmozyme
Inhaled
Mucolytic for CF
Refrigerated and protected from light
What are your pancreatic enzyme options for CF?
Where to store?
When to take?
What to avoid?
Creon, pancrease, pancrelipase, pertyze, viokace
At room temp
Before meals
Avoid antacids
Antiinflammatory options for CF?
Ibuprofen
Ivacaftor
Brand
MOA
Indication/age
Kalydeco
CFTR modulator therapy
CF >4 months old with G551D mutation
Tezacaftor - ivacaftor
Brand
MOA
Indication
Symdeko
CFTR modulator
Cf >6y.o with F508del mutation
Lumacaftor-ivacaftor
Brand
MOA
Indication
Orkambi
CFTR modulator therapy
> 2y.o with F508del mutation
Trikafta
Generic
MOA
Indication
Elexacaftor-tezacaftor-ivacaftor + ivacaftor
CFTR modulator therapy
> 6y.o with F508del mutation
What kind of hereditary disease is sickle cell disease?
Autosomal recessive
When do symptoms start for SCD and what tests are there?
4 months old
Newborn screening, Hgb electrophoresis, high performance liquid chromatography
What labs change when sicked RBC’s breakdown prematurely?
Anemia
High bilirubin
What are the five types of sickle cell crisis?
Vasoocclusive - hydration, transfusion, pain management
Aplastic crisis
Hemolytic crisis
Splenic sequestration crisis
Acute chest pain
What vaccines do you give for asplenia?
Pneumoncocca
Hib
meningococcal
influenza
When do you give PCN prophylaxis for SCD and what do you give?
First 5 years - PenVK 125mg PO BID for 1st 3 years then increase to 250mg PO BID
Hydroxyurea
Brand
Indication
MOA
ADE
Hydrea, droxia, siklos
SCD
reactivating fetal hemoglobin
BMS, GI, rash, hepatitis, pulmonary fibrosis , pregnancy category D
Voxelotor
Brand
Indication
MOA
Oxbryta
SCD
Hbs polymerization inhibitor to prevent sickling
Crizanlizumab
Brand
Indication
MOA
Adakveo
SCD
IV mAb to reduce frequency of vaso-occlusive episodes
How does inhaled hypertonic saline work for CF? ADE?
It’s a mucolytic that is recommended alongside dornase alfa to help draw out water from the cells so it can mix with the mucus and expel it
CoughingE
Exa-cel
Brand
Indication
MOA
Casgevy
SCD
Gene editing to increase fetal Hgb and reduce vaso-occlusive crisis