Thalassemia: Signs and Symptoms (Pediatric Nursing)

00:01 These are the signs and symptoms of thalassemia.

00:03 In beta-thalassemia minor, the patient remember is usually asymptomatic but they may have a very mild persistent anemia.

00:11 In contrast, patients with beta thalassemia major usually have pallor, fatigue, growth delay because the anemia can slow the growth, Recurrent infections - predisposing factors for infections in patients with thalassemia include their severe anemia, iron overload, splenectomy and a range of other immune abnormalities.

00:33 Splenomegaly - this means enlarged spleen.

00:36 Remember the spleen helps you fight infection and filter unwanted materials such as old, damaged blood cells.

00:42 Thalassemia is often accompanied by this increased destruction of a large number of red blood cells.

00:47 This causes your spleen to enlarge and work harder than it should.

00:51 Splenomegaly can make anemia worse and it can reduce the life of these transfused red blood cells.

00:56 Once the spleen gets too large, it's surgically removed.

01:00 Bone deformities.

01:01 Thalassemia can make the bone marrow expand as it's trying to compensate and churn out more cells.

01:07 This can actually cause the bones to widen.

01:10 Deformities of the face and skull can develop and the expansion of the bones can also make the bones thin and brittle, increasing the risk for fracture.

01:19 Heart problems.

01:20 Severe thalassemia is associated with congestive heart failure and abnormal heart rhythms known as arrythmias.

01:27 These are the signs and symptoms based on insufficient transfusion.

01:30 The first is facial deformity, the patient may develop hypertrophy of the upper maxillary bones.

01:36 In the blood we will see the patient has anemia.

01:39 The patient's also predisposed to this pathologic fractures and premature closure of the lower femoral epiphysis and this is due to the bone marrow expansion.

01:48 At the same time, when the patient does get a blood transfusion, there are also complications that develop due to the iron overload if there is insufficient chelation which is iron removal.

01:58 This iron is going to wreak havoc on many of the body system and organs including the pituitary system, the thyroid gland, the heart and of course this is the most important organ that can be affected, the pancreas, the genitals and this can cause difficulty in function and development, the parathyroid gland causing a hypoparathyroidism that's going to affect the calcium level, the liver can become enlarged that's hepatomegaly, skin pigmentation - the patient can get a slate-grey discoloration, and bone and joint pain related to the hypoparathyroidism.

02:33 Moderate to severe thalassemias are often diagnosed before the age of 2 because of the severity of symptoms.

02:40 Most patients with thalassemia trait or thalassemia minor are found incidentally when they have a routine CBC and it's gonna show a microcytic hypochromic anemia.