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27 Cards in this Set
- Front
- Back
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What is the most common type of Sickle Cell Disease?
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Carrier State
(AS) |
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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 |
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Why do you not see sx in babies right away?
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Predominance of Hb F
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What is the number one cause of death in sickle cell patients?
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Organ Failure (usually lung failure)
and Acute Crisis |
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What is the average age of death for M and F with SS? With SC?
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SS:
M: 42 F: 48 SC M: 60 F: 68 |
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Common Complications in Sickle Cell disease?
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#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 |
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9 Factors that Increase morbidity and mortality
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Acute chest syndrome
Baseline WBC count > 15,000 Iron Overload Freq. Pain Crisis Freq. Hospitalizations Low Fetal Hemoglobin Pulmonary HTN Renal Failure Seizures |
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Do you need to get different labs for each different type of sickle cell disease?
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No
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What Labs do you need to do?
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CBC- Anemia
CMP- Creatinine > 1 = abnormal Albumin: Creatinine ratio Ferritin- keep accurate transfusion record Hep Panel Urinalysis |
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What are 3 additional tests you should do?
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Annual eye exam: checking for retinopathy
ECHO: checking liver PFT and Arterial Blood Gas: (ankle foot orthosis) |
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Why is Hep a problem in these patients?
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1 out of 4 million will get hep from blood transfusions
Hep can lead to cirrhosis or hepatocellular carcinoma |
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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 |
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What is the relationship between frequent transfusions and ferritin?
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Mult. transfusions Increase Iron overload
Too much iron can cause fibrosis and other severe problems |
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What is considered iron overload?
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2 baseline ferritins > 1000
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10 units of RBC/ year will give you ___ iron
> 50 units will give you __ iron |
2g iron/ year
10g iron = iron overload |
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When do you treat iron overload?
When can you stop treatment? |
When 2 ferritins > 1500
Can stop treatment when Ferritin decreases to approx 500 |
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Is everyone with Sickle Cell Disease anemic?
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No
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What is the relationship between HbSS and Creatinine?
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HbSS has High GFR
= More tubular secretion of creatinine = Low serum creatinine |
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What does proteinuria indicate?
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Glomerular/ Renal Damage
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What are 4 preventative measures for pts with Sickle cell disease?
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Pneumo vax
Hep B vax Hydroxyurea RBC transfusions |
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Does Pneumovax eliminate sepsis?
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No it just reduces it
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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 |
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What does hydroxyurea increase and decrease?
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Increases:
Hb level, % Fetal Hb (even in adults), MCV, Leukocyte count Decreases: Pain, hospitalizations, blood transfusions, acute chest syndrome |
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What is an ADR of hydroxyurea?
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Can cause sterility and harm to a fetus: Must stop tx months before trying to get pregnant- in males and females
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When do you use Chronic Transfusion Therapy?
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Acute Chest syndrome
Anemia assoc with CKD CHF Chronic Hypoxia Pulmonary HTN Stroke Severe Pain |
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What is the greatest barrier in pain management?
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No good pain assessment tool
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Is addiction in Sickle cell pts common or rare?
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Rare
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