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27 Cards in this Set

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What is the most common type of Sickle Cell Disease?
Carrier State
(AS)
What are the 2 most common reasons that kids were dying from Sickle cell disease?

What has been done to prevent this?
Splenic sequestration (failure)
Blood infections (commonly by strep pneumo)

Penicillin prophylaxis until age 6
Vaccines (especially pneumovax and hep B)
Mandatory screening of all newborns
Why do you not see sx in babies right away?
Predominance of Hb F
What is the number one cause of death in sickle cell patients?
Organ Failure (usually lung failure)
and
Acute Crisis
What is the average age of death for M and F with SS? With SC?
SS:
M: 42
F: 48

SC
M: 60
F: 68
Common Complications in Sickle Cell disease?
#1: PAIN
involves long bones due to avascular necrosis

Spleen infection/ failure
Lung Disease
Acute chest syndrome
Sickle cell chronic lung dz
Pulmonary HTN
Gallstones, Liver failure, gallbladder dz

Proteinuria, CKD
Blindness, CVA, Retinopathy
Priapism
Leg ulcers
9 Factors that Increase morbidity and mortality
Acute chest syndrome
Baseline WBC count > 15,000
Iron Overload
Freq. Pain Crisis
Freq. Hospitalizations
Low Fetal Hemoglobin
Pulmonary HTN
Renal Failure
Seizures
Do you need to get different labs for each different type of sickle cell disease?
No
What Labs do you need to do?
CBC- Anemia
CMP- Creatinine > 1 = abnormal
Albumin: Creatinine ratio
Ferritin- keep accurate transfusion record
Hep Panel
Urinalysis
What are 3 additional tests you should do?
Annual eye exam: checking for retinopathy
ECHO: checking liver
PFT and Arterial Blood Gas: (ankle foot orthosis)
Why is Hep a problem in these patients?
1 out of 4 million will get hep from blood transfusions

Hep can lead to cirrhosis or hepatocellular carcinoma
What do the following Albumin: Creatinine ratios tell you

30-300 mg creatinine
>300
> 1g creatinine

why do you want to detect this?
30-300 = Microalbuminuria
>300 = Proteinuria
> 1g = Nephrotic range of proteinuria

Early ID and tx of proteinuria can help prevent renal disease
Treat with ACEI or ARB
What is the relationship between frequent transfusions and ferritin?
Mult. transfusions Increase Iron overload

Too much iron can cause fibrosis and other severe problems
What is considered iron overload?
2 baseline ferritins > 1000
10 units of RBC/ year will give you ___ iron

> 50 units will give you __ iron
2g iron/ year

10g iron = iron overload
When do you treat iron overload?

When can you stop treatment?
When 2 ferritins > 1500

Can stop treatment when Ferritin decreases to approx 500
Is everyone with Sickle Cell Disease anemic?
No
What is the relationship between HbSS and Creatinine?
HbSS has High GFR
= More tubular secretion of creatinine
= Low serum creatinine
What does proteinuria indicate?
Glomerular/ Renal Damage
What are 4 preventative measures for pts with Sickle cell disease?
Pneumo vax
Hep B vax
Hydroxyurea
RBC transfusions
Does Pneumovax eliminate sepsis?
No it just reduces it
When is Hydroxyurea indicated?

is it ok to use in kids?
For SS and Beta Thalassemia
with:
3 hospitalizations/ yr
or
2 chest episodes/ yr

Not FDA approved for kids but still used for them
What does hydroxyurea increase and decrease?
Increases:
Hb level, % Fetal Hb (even in adults), MCV, Leukocyte count

Decreases:
Pain, hospitalizations, blood transfusions, acute chest syndrome
What is an ADR of hydroxyurea?
Can cause sterility and harm to a fetus: Must stop tx months before trying to get pregnant- in males and females
When do you use Chronic Transfusion Therapy?
Acute Chest syndrome
Anemia assoc with CKD
CHF
Chronic Hypoxia
Pulmonary HTN
Stroke
Severe Pain
What is the greatest barrier in pain management?
No good pain assessment tool
Is addiction in Sickle cell pts common or rare?
Rare