Pharmacotherapy for Hematologic Disorders: Thalassemia – Advanced Pharmacology week 9 response 1
Type of document Essay
1 Page Double Spaced
Subject area Pharmacology
Academic Level Master
Style APA
References 3
Order description:
Please respond to Catherines post by Provide recommendations for alternative drug treatments and patient education strategies for treatment and management. Please use these readings and resources for at least 2 references or I dont get credit
Learning Resources
This page contains the Learning Resources for this week. Be sure to scroll down the page to see all of this week’s assigned Learning Resources. To access select media resources, please use the media player below.
Required Readings
Arcangelo, V. P., Peterson, A. M., Wilbur, V., & Reinhold, J. A. (Eds.). (2017). Pharmacotherapeutics for advanced practice: A practical approach (4th ed.). Ambler, PA: Lippincott Williams & Wilkins.
Review Chapter 50, “Pharmacotherapy for Venous Thromboembolism Prevention and Treatment, Stroke Prevention in Atrial Fibrillation, and Thromboembolism Prevention with Mechanical Heart Valves” (pp. 863-886)
This chapter covers drug therapy options for three disorders requiring anticoagulants: venous thromboembolism, atrial fibrillation, and ischemic stroke. It also explains the process of initiating and managing drug therapy for patients with these disorders.
Chapter 51, “Anemias” (pp. 891-906)
This chapter examines causes of various types of anemia and associated cell alterations. It also explores types of drugs used for treatment and patient factors to consider when initiating drug therapy.
Drugs.com. (2012). Retrieved from http://www.drugs.com/
This website presents a comprehensive review of prescription and over-the-counter drugs including information on common uses and potential side effects. It also provides updates relating to new drugs on the market, support from health professionals, and a drug-drug interactions checker.
Optional Resources
Refer to the Optional Resources listed in Week 1.
week 9 Discussion
COLLAPSE
Pharmacotherapy for Hematologic Disorders: Thalassemia
Thalassemia is an inherited hematologic disorder in which the body makes an abnormal form of hemoglobin and red blood cells. Hemoglobin is the protein particle in red blood cells that carries oxygen to the entire body. The disorder results in the extreme destruction of red blood cells, which leads to anemia (Huether and McCance 2012).The inherited syndrome is a disorder of α- or β-globin biosynthesis resulting in an inadequate supply of globins. The reduced amount of globin reduces production of hemoglobin tetramers, causes hypothermia and microcytosis (Huether and McCance 2012).
There are two broad classes of thalassemia blood disorders namely beta-thalassemia and alpha-thalassemia. There is a difference in the clinical signs and symptoms of these two class, beta-thalassemia manifest with microcytic hypochromic anemia, which is usually mild to moderate, splenomegaly, bronze coloring of the skin, and hyperplasia of bone marrow (Huether and McCance 2012). Alpha-thalassemia is minor and may be asymptomatic, symptoms when present are mild and include bone marrow hyperplasia, an increase in serum iron concentration, and moderate splenomegaly (Kasper D. et al. 2014). β-thalassemia diagnosis is identified during childhood; it presents with severe anemia, hepatosplenomegaly, microcytosis, and elevated levels of HbF, HbA2, or both. Red blood cells that are low in number, the size and shape are variable but usually smaller than average (Kasper D. et al. 2014). The symptoms of thalassemia can differ. Some of the most common signs include bone deformities, especially in the face, dark urine, delayed growth and development excessive tiredness and fatigue, and yellow or pale skin. Not every individual has visible symptoms of thalassemia. Signs of the disorder shows up later in childhood or adolescence (Holm G & Cherney K (2017).
Drug Treatment Therapy
Treatments for thalassemia is based on the type and severity of the disorder, those who present with mild or no symptoms may need little or no treatment (Kasper D. et al. 2014). However, the standard procedures include blood transfusions, iron chelation therapy, bone marrow transplant or stem cell transplant and folic acid supplements (Kasper D. et al. 2014). Blood transfusions can also lead to a buildup of iron in the blood because the hemoglobin in red blood cells is iron-rich protein. Iron overload otherwise known as hemochromatosis results when excess iron is deposited in the heart, liver, pancreas, and other organs. Hematochromatosis may result in organ failure and death, to prevent this, iron chelation therapy is an option to remove excess iron from the body. Deferasirox, Butyrate, and its derivatives are some of the most common and well-known medicines which are used for increasing the HbF percentage and iron chelation (Kasper D. et al. 2014).
There are several drugs responsible for inducing the γ globin gene expression among thalassemia patients like Hydroxyurea (inhibitor of Ribonucleotide Reductase), Sodium Butyrate (inhibitor of Histone Deacetylase), 5’-Azacytidine (DNA Methylating agents). These drugs help to improve α:β ratio in the erythroid progenitor cells and thus develop the major complication among transfusion-dependent thalassemia primary patients (Basu & Panja 2015).
Deferoxamine is isolated from Streptomyces pilosus (Lexicomp 2017). It binds loosely to iron in iron-carrying proteins hemosiderin and ferritin; it does not compete for biologically chelated iron, that is present in microsomal and mitochondrial cytochromes and hemoproteins (Lexicomp 2017). The IM or IV route is preferred because it is poorly absorbed when given orally and may increase iron absorption through this route (Lexicomp 2017). The adult dose is IM: 90 mg/kg/dose every 8 hours (maximum: 6,000 mg/24 hours). When given IM, this route is less preferred compared to the IV route 15 mg/kg /hour (maximum: 6,000 mg/24 hours) when given through IV route. In children over three years old and Adolescents, the IV dose is 20 to 40 mg per kg per day over eight to 12 hours for five to seven days; dose should not exceed 40 mg per kg in a day until growth has stopped (Lexicomp 2017). The medication is eliminated through the kidney and the liver, often turning the urine to an orange-red color. IV route should be given slowly to avoid hypotension. Side effects include flushing, abdominal discomfort, and rash. Other adverse effects include acute respiratory distress syndrome when it is administered continuously for over 24 hours (Kasper D. et al. 2014).
Factor Relationship
In 2016 the Mayo Clinic findings revealed significant factors of thalassemia which have evidence of ethnicity and genetic traits. The prevalence of thalassemia relates to the appearance of a mutant hemoglobin gene. A persons’ risk increases with a family history of this hematologic disorder. For this reason, thalassemia tends to transmit from parents to children. Second, diagnosis of thalassemia, most often, presents with a particular ancestry. Thalassemia is prevalent among people of a specific descent; these include Italian, Greek, Asia, and African (Huether and McCance 2012). The confirmation of this disorder is accessible through an evaluation of the blood test. The blood test usually reveals red blood cells that are low in number, smaller than usual, pale, and variable in size and shape. Notably, a clear uneven distribution of red blood cells is apparent.
Drug therapy: Reducing Negative Side Effects
Practitioners must attain a baseline study of Liver test and serum creatinine and serum creatinine clearance before and during therapy to decrease the chance of renal and liver failure. Serum creatinine and serum creatinine clearance should be accessed before and during therapy to reduce the chance of renal failure. It is even more crucial in elderly patients who have a hematologic advancement malignancy or a low platelet count (Trevor AJ et al. 2015). A dose reduction, interruption or discontinuation are recommended for the prevention of hepatic failure. Providers should make dosage adjustment using the creatine clearance of the patients as follows (Lexicomp 2017):
CrCl >50 mL/minute: No adjustment necessary
CrCl 10 to 50 mL/minute: Reduce the dose of deferoxamine to between 25% to 50% of normal dose
CrCl<10 mL/minute, hemodialysis, peritoneal dialysis: It is not advisable to use deferoxamine in these patients.
Liver function test is essential every two weeks during the first month of treatment, and, at least, every month after that. It is of medical importance that the practitioner stops Deferasirox treatment with suspicion of gastrointestinal ulcers or gastrointestinal bleeding.
The clinician should start ascorbic acid only after one month of regular deferoxamine treatment if both medications are necessary (Lexicomp 2017). The dose of ascorbic acid should not exceed 200 mg/day for adults, 100 mg/day for children over ten years of age, or 50 mg/day in children less than10 years of age. Providers should not give deferoxamine in combination with ascorbic acid in patients with preexisting cardiac disease (Lexicomp 2017).
References
Basu A, & Panja A (2015). Pharmacogenomics of the drugs used for the treatment of
Thalassemia. J Cytol Histol 6:360. doi:10.4172/2157-7099.1000360
Holm G & Cherney K (2017). Thalassemia: Causes, Symptoms, And Diagnosis – Healthline.
Retrieved from https://www.healthline.com/health/thalassemia
Huether, S. E., McCance, K. L. (2012). Understanding pathophysiology (Laureate custom ed.). St. Louis, MO: Mosby.
Kasper D, Fauci A, Hauser S, Longo D, Jameson J, Loscalzo J (2014). Harrison’s Principles of Internal Medicine, 19e New York, NY: McGraw-Hill. Lexicomp (2017). Deferoxamine. Retrieved from https://fco.factsandcomparisons.com/lco/action/doc/retrieve/docid/patch_f/6699
Mayo Clinic. (2016). Thalassemia: risk factors. Retrieved from http://www.mayoclinic.org/diseases-conditions/thalassemia/basics/risk-factors/con-20030316
Trevor AJ, Katzung BG, Kruidering-Hall M. (2015). Pharmacology: Examination & Board Review, 11e New York, NY: McGraw-Hill