Hemophilia: Signs and Diagnosis (Pediatric Nursing)

00:01 So the signs and symptoms of hemophilia are related to the excessive bleeding and the easy bruising.

00:06 The extent depends on the severity of the hemophilia and patients can have internal and external bleeding.

00:12 Let's talk about external bleeding.

00:14 This can be inside the mouth, this can be from toothbrushing.

00:18 These patients sometimes present with ongoing epistaxis or a nose bleed.

00:23 Remember, a normal nosebleed last between 10 and 20 minutes and sometimes these patients will bleed for hours There is heavy bleeding from a minor cut.

00:32 Internal bleeding - this can be a bit sneaky.

00:34 The patients can have hematuria and this can be gross on inspection or can be microscopic.

00:40 The patient can have blood in their stool - that's hematochezia, and they may show up with bruising inside.

00:47 Now the joints - this can bleed into the knees and elbows and we'll cover this and just a minute.

00:51 Let's talk about bleeding in the brain.

00:53 This is a very serious complication of hemophilia, it can happen after a simple bump on the head or a more serious injury.

01:01 The signs and symptoms include a long-lasting painful headache, the patient may have some neck pain and stiffness, they may have repeated vomiting because remember that 5-HT3 vomiting center is found in the brain, excessive sleepiness or changes in behavior, sudden weakness or clumsiness of the arms or legs or maybe they'll have problems walking, and your patient may develop seizures.

01:24 Now let's talk about bleeding in the joints.

01:26 Bleeding in the knees, elbows and other joints is a common form of internal bleeding in people have hemophilia.

01:32 This bleeding can occur without obvious injury so it's spontaneous.

01:36 At first the bleeding causes a tightness in the joint with no real pain or any visible sign of bleeding and then the joints gonna become swollen, hot to the touch and painful to bend.

01:46 Swelling continues as the bleeding continues and eventually, the movement in the joint is temporarily lost.

01:52 They will lose their range of motion.

01:54 Pain can be severe at this point and joint bleeding that isn't treated can damage the joint.

02:01 How do we diagnose hemophilia? Well first, we'll obtain a medical history.

02:04 There are some screening tests that can be done in these include clotting factors.

02:09 And if there is a family history, the baby can be tested immediately after birth by testing their cord blood.

02:16 If there's no family history, it raises the clinical concern if there's prolonged bleeding after a heel stick which we do on newborns for newborns screen, or after a circumcision.

02:27 So labs that we use to evaluate, we do a CBC.

02:30 This includes a red blood cell count, a white blood cell count including the differential, a platelet count, The reticulocyte distribution width, a hematocrit level and the hemoglobin.

02:43 Now it's good to remember the CBC is usually normal in patients with hemophilia.

02:49 Let's take a look at the clotting studies.

02:51 Next we can check clotting tests, the first is a PTT - partial thromboplastin time, you might see APTT written as well and this is, how long does it take to clot? This is going to measure your factors VIII, IX, XI and XII.

03:06 Another is the PT or the prothrombin time and this is another time, how long it takes to clot.

03:11 This measures factors I, II, V,VII and X.

03:15 Finally we'll look at the fibrinogen and that's gonna be a measure of the Factor I.