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Thalassemia, care checklist, complications, nursing management

Comphrehensive care checklist

Check List 

  • Cardiac Evaluation – The following tests are standard protocol and should be performed at least once a year

o   Cardiac Echo

o   24 hour Holter monitor

o   EKG

o   Cardiac Stress Test ( for patients 18years and older)

o   Iron related Heart problems can be determined by (MRI) testing. It determines the degree of iron overload in the heart

  • Liver Function Tests

o   Annual Hepatitis C Surface antibodies if hep C Ab positive, draw hep C

o   SGOT and SGPT (every three months)

o   Hepatitis B

o   If you have elevated ALT, it is recommended repeat the test every month. If persistently elevated then should consider a liver biopsy.

  • Ophthalmology Evaluation:

o   Annual Evaluation for cataracts, night blindness, decreased visual field (recommended for patients on Desferal and those who are diabetic)

  • Audiological Evaluation:

o   Annual Audiological Evaluation (every 6 months if on Desferal) for hearing changes and Tinitus (ringing in the ears).

  • Others

o   Annual HIV

Focus On A Cure For Thalassemia

Stem Cell Transplantation

Stem Cells, present in the bone marrow, give rise to red blood cells that produce Hemoglobin. In Thalassemia, it’s the defect in the stem cells that leads to deformed red blood cells that produce the abnormal hemoglobin and transfusion – dependent anemia. Stem Cell transplantation provides a thalassemia patient with healthy stem cells that will produce normal blood cells.

Stem Cells can be transplanted into a patient’s body through different methods, such as Bone Marrow Transplantation (BMT)

In BMT, chemotherapy is used to kill the patient’s stem cells in the bone marrow. They are then replaced with healthy stem cells from a compatible donor. Bone marrow transplants are generally most successful when the donor and host are genetically compatible and when the patient is younger and less transfused.

Cord Blood Transplantation

Instead of harvesting stem cells from a donors bone marrow or blood, stem cells are taken from the “cord blood” found in the umbilical cord/placenta of a newborn sibling. Unlike regular bone marrow transplantation, the stem cells taken from the cord blood do not have to be a perfect genetic match. There appears to be a lower instance of rejection in cord blood transplantation.

Gene Therapy

The objective of GENE therapy is to insert a normal beta – globin gene into the patients stem cells, thus allowing increased production of beta globin and healthy red blood cells

Hydroxyurea

Hemoglobin is composed of two protein chain-alpha globin chain and beta globin chains. Patients with beta-thalassemia do not make beta globin, they often require frequent red blood cell transfusions.

Some drugs, including hydroxyurea, can stimulate production of a third type of protein chain called gamma chains. In the womb, the foetus makes this type of protein instead of beta globin. It is not until after birth, when the foetus no longer produces gamma globin that the beta globin deficiency becomes apparent.

Hydroxyurea has been shown to induce production of fetal hemoglobin. Fetal hemoglobin has a pair of gamma-globin molecules in place of the typical beta-globins of adult hemoglobin. Higher-than-normal levels of fetal hemoglobin can ameliorate some of the symptoms of thalassemia. Effects in patients with -thalassemia major are controversial. We now report a marked elevation of total Hb levels with HU that permitted regular transfusions to be stopped in some children with transfusion-dependent -thalassemia.

Complications

  • Iron overload: People with thalassemia can get an overload of iron in their bodies, either from the disease itself or from frequent blood transfusions. Too much iron can result in damage to the heart, liver and endocrine system, which includes glands that produce hormones that regulate processes throughout the body. The damage is characterized by excessive iron deposition. Without adequate iron chelation therapy, almost all patients with beta-thalassemia will accumulate potentially fatal iron levels.[21]
  • Infection: People with thalassemia have an increased risk of infection. This is especially true if the spleen has been removed.
  • Bone deformities: Thalassemia can make the bone marrow expand, which causes bones to widen. This can result in abnormal bone structure, especially in the face and skull. Bone marrow expansion also makes bones thin and brittle, increasing the risk of broken bones.
  • Enlarged Spleen: the spleen aids in fighting infection and filters unwanted material, such as old or damaged blood cells. Thalassemia is often accompanied by the destruction of a large number of red blood cells, and the task of removing these cells causes the spleen to enlarge. Splenomegaly can make anemia worse, and it can reduce the life of transfused red blood cells. Severe enlargement of the spleen may necessitate its removal.
  • Slowed growth rates: Anemia can cause a child’s growth to slow. Puberty also may be delayed in children with thalassemia.
  • Heart problems: Such as congestive heart failure and abnormal heart rhythms (arrhythmias), may be associated with severe thalassemia.

Nursing diagnosis

 

  • Activity intolerance R/T impaired oxygen transport
  • High risk for infection R/T decreased resistance secondary to hypoxia
  • Altered body image R/T skeletal changes.
  • High risk for altered health maintenance R/T lack of knowledge.
  • Altered nutrition: less than body requirement related to inadequate nutritional intake and anorexia.
  • Ineffective management of therapeutic regimen related to lack of knowledge about appropriate nutrition and medication.

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