Hematologic Disorders:

Definition/Etiology:

• Anemia: Low red blood cells
• Sickle Cell Disease: defective hemoglobin molecules cause the RBCs to assume a “sickled shape”. Because of the shape these cells tend to clump together and cause an increased viscosity of the blood resulting in capillary obstruction.
• Hemophilia: Clotting disorder identified by a deficiency in factor VIII and factor IX.

Pathophysiology:

There are several pathos on several different disorders. I recommend you seek out the information on your own if you are interested. Some of the major ones you will see are congenital, like hemophilia and sickle cell disease, but something like anemia can be caused by a variety of factors including blood loss, or destruction of RBCs

Clinical Presentation:

Anemia:

• Fatigue
• DOE
• Dizziness
• Pallor
• Feeling cold

Sickle Cell Disease:

• Acute, severe pain – most common reason for ED visits
• Weakness due to anemia
• Pallor
• Jaundice from rapid breakdown of RBC
• Infections from splenic damage

Hemophilia:

• Brusing with minor injuries
• Bleeding into soft tissues, muscles, or joints with pain and swelling
• Paresthesia that progresses to nerve injury following a compressing hematoma
• Epistaxis
• Hematuria
• AMS from intracranial bleeding
• Shock from blood loss

Collaborative Management:

With anemia, our primary goal is to identify the cause and then treat it. We need a full panel of labs including a type and cross. We should anticipate fluids and possible blood transfusions. As there is a drop in HgB, which carries O2, you might want to think of giving them some.

In sickle cell crises, pain management is usually the first thing they need. It’s important to assess where their pain is. Is this simple joint pain, or is this acute chest syndrome. The complications from SCD are numerous and include chronic anemia, aplastic crisis, frequent infections, renal failure, pulmonary embolism, jaundice, blindness, and chronic skin ulcers. So yea…a pretty thorough assessment is necessary with these patients as well as a full panel of labs which would include a reticulocyte count. In treating, we want to rehydrate, treat infections, maintain a warm environment, and give blood if anemic.

With hemophilia, we want to delay the blood draw until IV factor replacement has been initiated. Then get a CBC, platelets, fibrinogen level and factor levels. We do not want to delay treatment as they can bleed out pretty easily. Remember to initiate factor VIII replacement even before diagnostics in known hemophiliacs. Once you perform a venipuncture, we want to apply pressure to the area for 3-5 minutes.

Evaluation | Patient Monitoring | Education:

• Anemia – well…the evaluation is simply if they are no longer anemic. So serial blood work is necessary, especially after treatment.
• In the sickle cell patient, continuous monitoring of their pain level will help to determine the extent of their crisis.
• With hemophilia, well…. Are they still bleeding? And while that may seem like a simple statement, thorough assessment is necessary. It’s not enough that we don’t see external bleeding, but we need to assess for signs of internal bleeding as well.

Linchpins: (Key Points)

• Blood work
• Hold on Hemo
• Treat underlying cause