Thalassemia: Management and Complications (Pediatric Nursing)

00:01 So how is thalassemia managed? Well standard treatments for patients with thalassemia major are blood transfusions and then iron chelation.

00:09 The patient needs the components of the blood that they don't need this extra iron that comes with the transfusion.

00:15 Patients with moderate to severe thalassemia are going to get a transfusion to restore their healthy red blood cells and hemoglobin approximately every 4 months And patients with beta thalassemia major, they might require blood transfusions every 2 to 4 weeks So every treatment has a risk and there is a risk of iron overload in patients who received these blood transfusions.

00:37 These patients have to undergo iron chelation to remove the extra iron and from the body since the body cannot rid the extra iron on its own.

00:45 Iron overload is very dangerous to your organs including the liver, the heart, the kidneys and endocrine system.

00:52 So patients who receive frequent transfusions also require this chelation and this can be given in an oral pill.

00:59 Patients with thalassemia may also require nutritional supplementation.

01:03 Nutritional supplements including folic acid and B12 may be necessary as these are the nutrients and key components to make a healthy red blood cells.

01:11 W hile the aforementioned treatments are helpful, they're not a cure.

01:15 Bone marrow and stem cell transplant from a compatible related donor is the only treatment that can cure thalassemia.

01:21 It is the most effective treatment.

01:23 Compatible means that the donor has the same types of proteins called human leukocyte antigens or HLA on the surface of their cells as the person who's going to be receiving the transplant.

01:35 Bone marrow transplant from a compatible sibling offers the best chance for a cure.

01:39 Most patients with thalassemia however lack of suitable sibling donor.

01:44 Within one month, the transplanted bone marrow stem cells are gonna start to make new healthy blood cells.

01:50 Given the high risk of complications associated with bone marrow transplant, it's not routinely recommended for those with mild or moderate thalassemia because it's a very high risk procedure.

02:01 Thalassemia is a complex condition and it can cause complications in multiple organ systems due to the effects of the chronic anemia on the body or the consequence of the iron overload from trying to fix the anemia.

02:12 This is going to occur across your patient's life span.

02:15 This is a chronic blood disorder but the complications vary based on the patients age.

02:20 Here in pediatrics, we see clinical concerns include growth failure, poor development and problems with skeletal deformities.

02:27 This can also impact your patient's school.

02:30 Throughout the lifespan, the patient's going to be anemic and have a risk for iron overload from their transfusions.

02:36 In adolescents, the patient may start to develop heart, liver and endocrine disease and again this is a consequence of receiving those frequent blood transfusions.

02:44 There can be complications in pregnancy and delays with sexual development.

02:48 The anemia can also affect your patient in regard to sports and hobbies and in their sexual life and relationships.

02:54 And finally in adulthood, this can contribute to the development of malignancies and survival stress.

03:01 Patients with thalassemia have ineffective erythropoesis which means they're not able to make red blood cells fast enough to counteract the destructive phase, this causes a chronic anemia.

03:13 Patients are also at an increased risk for infectious blood-borne diseases from these repeated blood transfusions.

03:19 These patients have an increased risk of cancer.

03:23 When studied, the overall incidence of hematologic malignancies and abdominal cancers was much higher in patients with thalassemia when compared to patients without thalassemia.

03:32 This is thought to be due to the iron overload and the risk for hepatitis which can increase the risk for liver cancer.

03:40 The bone marrow in patients with thalassemia is under constant stress.

03:44 This oxidative stress is going to increase the risk of these blood cancers.

03:49 Another common complication is iron overload and this is the end-organ damage.

03:54 This happens in the heart, the liver and the endocrine glands.

03:58 So we monitor for this iron overload in these patients because we expect it to happen and you're gonna be checking a liver iron concentration and also monitoring the serum ferritin level.

04:08 You can prevent this by helping your patient optimiz their chelation to match their iron intake and you also want to ensure adherence to their chelation therapy, You can treat this by optimizing their chelation to the baseline iron levels and this is gonna be different and patient-specific and you also want to ensure their adherence.

04:28 Patients with thalassemia are at an increased risk of acquiring viral hepatitis due to these repeated blood transfusions.

04:35 You can monitor this by checking bloodwork for HCV and HBV every one year in these transfused patients.

04:42 You can prevent by vaccinating your patients for Hepatitis A and Hepatitis B.

04:47 And you can treat the patient by consulting with a hepatologist to administer antivirals as recommended.

04:54 Hepatic disease.

04:55 Remember, these patients are getting frequent blood transfusions and the liver is under constant assault by this extra iron every time the patient's transfused.

05:03 Over time, this can wreak havoc on the liver resulting in hepatic disease.

05:08 You can monitor this by checking your patient's liver function tests or LFTs and you'll also perform liver ultrasounding to check the size and density of the liver.

05:17 You can prevent this by optimizing the chelation of their iron overload and optimizing antivirals to treat for viral hepatitis.

05:26 There are also lifestyle modifications for your patient including eliminating or limiting alcohol intake and liver toxic medications.

05:35 You can treat this by again optimizing the iron chelation getting rid of that iron in the body and consulting with the specialist, a hepatologist.