Sample Answer for NURS 6521 Week 9: Hematologic System Included After Question
In the 1970s, the average lifespan for patients diagnosed with sickle cell disease was 14 years. Today, the average lifespan has increased to 50 years and beyond (TriHealth, 2012). The patient prognosis for many other hematologic disorders such as hemophilia and cancer continue to improve as well. This can be attributed to advancements in medical care—specifically drug therapy and treatment. When managing drug therapies for patients, it is essential to continuously examine current treatments and evaluate the impact of patient factors on drug effectiveness. To prepare for your role as an advanced practice nurse, you must become familiar with common drug treatments for various hematologic disorders seen in clinical settings.
To prepare:
- Select one of the following hematologic disorders: anemia, hemophilia, cancer, sickle cell anemia, thalassemia, thrombolytic disorders, or white blood cell disorders. Consider the types of drugs that would be prescribed to patients to treat symptoms associated with this disorder.
- Select one of the following factors: genetics, gender, ethnicity, age, or behavior. Reflect on how this factor might impact the effects of prescribed drugs, as well as any measures you might take to help reduce negative side effects.
With these thoughts in mind:
By Day 3
Post a description of the hematologic disorder you selected including types of drugs that would be prescribed to patients to treat associated symptoms. Then, explain how the factor you selected might impact the effects of prescribed drugs, as well as any measures you might take to help reduce negative side effects.
By Day 6
Read a selection of your colleagues’ responses and respond to at least two of your colleagues on two different days who selected a different hematologic disorder than you did. Provide recommendations for alternative drug treatments and patient education strategies for treatment and management.
Note: For this Discussion, you are required to complete your initial post before you will be able to view and respond to your colleagues’ postings. Begin by clicking on the “Post to Discussion Question” link and then select “Create Thread” to complete your initial post. Remember, once you click on Submit, you cannot delete or edit your own posts, and you cannot post anonymously. Please check your post carefully before clicking on Submit!
Submission and Grading Information
Grading Criteria
Once properly diagnosed, many hematologic disorders are very manageable with the appropriate treatment plan (Cancer Institute of Florida, n.d.). As an advanced practice nurse, it will be your responsibility to diagnose, treat, and/or recognize when to refer patients with these disorders. Many patients, however, struggle with diagnoses related to hematologic disorders that require extensive treatment and long-term care such as cancer or hemophilia prompting them to seek second opinions or alternative care. In your role as advanced practice nurse, you must be able to ease these concerns by answering patient questions about the disorders, related drug therapies, and effects of drug therapies based on individual patient factors.
This week you examine drugs prescribed to treat hematologic disorders, as well as the impact of patient factors on the effects of the drugs. You also explore ways to improve patient treatment plans including suggested drug therapies.
Learning Objectives
By the end of this week, students will:
- Analyze types of drugs prescribed to treat hematologic disorders
- Evaluate the impact of patient factors on the effects of prescribed drugs for disorders of the hematologic system
- Evaluate drug therapy plans to treat disorders of the hematologic system
- Analyze patient education strategies for treatment and management of hematologic disorders
- Understand and apply key terms, concepts, and principles related to prescribing drugs to treat hematologic disorders
Photo Credit: N/A – Laureate Image
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.
Optional Resources
Refer to the Optional Resources listed in Week 1.
A Sample Answer For the Assignment: NURS 6521 Week 9: Hematologic System
Title: NURS 6521 Week 9: Hematologic System
Definition of Sickle Cell Anemia
Sickle Cell Anemia is the most severe homozygous form of Sickle Cell Disease that can be lifelong condition (Huether & McCance, 2017). A genetic mutation, a replacement of the amino acid valine for amino acid glutamine acid, causes an abnormal hemoglobin S within the erythrocytes that reacts to dehydration and deoxygenation by solidifying and stretching the cells into sickle shapes that are elongated causing hemolytic anemia (Huether & McCance, 2017)
Treatment
The treatment goal of SCA is to prevent and reduce the complications of the condition and a potential cure (Arcangelo, Peterson, & Reinhold, 2017). Immunizations are significant in reduction of possible infections that can have a greater impact on the patient with sickle cell (Huether & McCance, 2017). Bone marrow is a potential cure of the disease (Mayo Clinic, 2019). Penicillin is also prescribed prophylactically to prevent any infection that can be detrimental to the patient (Mayo Clinic, 2019). Hydroxyurea is prescribed to decrease the number of attacks and pain management ranges from Tylenol to opioids such as hydromorphone.
Hydroxyurea is a prophylactic medication utilized to decrease the number of attacks or crisis the patient experiences. It increases the level of hemoglobin F levels, increases the water content of red blood cells, increases the deformity of the sickled cells, alters the adhesion of the red blood cells, and can reverse organ dysfunction (Arcangelo, Peterson, & Reinhold, 2017). Dosing is usually 15 mg/kg/day in adults rounding to the next five hundred, and 20 mg/kg/day for children (Arcangelo, Peterson, & Reinhold, 2017). A CBC and retic should be conducted every four weeks to monitor for side effects of the medication. Side effects can include neutropenia and thrombocytopenia (Arcangelo, Peterson, & Reinhold, 2017).
Hydromorphone is an opioid used to treat sever pain. Dosing usually ranges from 2.5-10mg by mouth every 3 to 6 hours (Connective Rx, 2018). It is available in pill form, intravenous form, rectal suppositories, and can be administered in an epidural (Connective Rx, 2018). Dosage adjustments should be made with patients who have hepatic and renal impairments (Connective Rx, 2018). Adverse reactions can include constipation, depression, and hyperreflexia (Connective Rx, 2018). Hydromorphone is contraindicated with alcohol (Connective Rx, 2018). It is 8-19% plasma protein bound, has a half-life of 2-3 hours, duration of 3-4 hours, and an onset of 30 minutes for the oral form (Connective Rx, 2018).
The Impact of Age on the Treatment of Sickle Cell Anemia
Sickle cell is a genetic disease that manifests early in the patient’s life. Dosage adjustments exist for younger patients versus older patients with hydroxyurea (Arcangelo, Peterson, & Reinhold, 2017). Hydromorphone is not recommended for children, infants, and neonates (Connective Rx, 2018). Caution is advised when utilizing it in the elderly population (Connective Rx, 2018).
Measures to Reduce the Negative Side Effects
In order to reduce the side effects of hydroxyurea, close laboratory monitoring can allow the practitioner to identify the presence of the side effects early. The prompt identification of the side effects of hydroxyurea and prompt intervention with dose adjustments would decrease the impact of the negative side effects. Side effects of hydromorphone can be reduced by close monitoring of the patient’s bowel status and encouraging increasing fiber and fluids in the patient’s diet.
Conclusion
Sickle cell anemia is a genetic disease the causes the shape of the red blood cell to be sickled. Treatment options include prevention of infections and attacks, pan management, and cure with bone marrow transplant. Dosage adjustments are required with age for hydroxyurea and hydromorphone is not indicated in children, infants, and neonates. Measures to reduce negative side effects include monitoring and timely intervention.
References
Arcangelo, V. P., Peterson, A. M., & Reinhold, J. A. (2017). Pharmacotherapeutics for Advanced Practice: A Practical Approach. Ambler, PA: Lippincott Williams & Wilkins.
Connective Rx. (2018). Hydromorphone. Retrieved from PDR: https://www.pdr.net/drug-summary/Dilaudid-Injection-and-HP-Injection-hydromorphone-hydrochloride-490.901
Huether, S. E., & McCance, K. L. (2017). Understanding Pathophysiology. S. Louis, Missouri: Elsivier.
Mayo Clinic. (2019). Sickle Cell Anemia. Retrieved from Mayo Clinic: https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/diagnosis-treatment/drc-20355882
A Sample Answer 2 For the Assignment: NURS 6521 Week 9: Hematologic System
Title: NURS 6521 Week 9: Hematologic System
Thalassemia is a condition resulting from the destruction of red blood cells. The condition is cause by the suppression of the rate of synthesis of globin chains due to mutant genes (Copestead and Banasik, 2014). The disruption of the synthesis causes normal chains to accumulate and be deposited within the cytoplasm causing damage to cell membranes. This damage leads to cell destruction. The condition is congenital and affects at least 1,000 people in the United States (CDC, 2018). Diagnosis of the condition can be attained through a thorough patient and family history as well as diagnostic testing. This testing can be completed with a blood draw of a CBC. Patients with the condition will have a mean corpuscular volume less than 75 with a normal ferritin count (Muncie and Campbell, 2009).
Patients with this condition will present with fatigue, weakness, dyspnea, decreased stamina, and tachycardia (Vichinsky, 2013). Patients with severe to moderate thalassemia are usually diagnosed in early childhood. The condition is most often seen in those of Asian, Mediterranean and African descent (NIH, 2018). If the condition is left untreated in moderate to severe cases the results can include deformities of the skull bones, mongoloid facies, bowing and rarefaction of long bones, extension of bone marrow into the intraabdominal and paraspinal tumors, hepatomegaly, splenomegaly, cardiac failure, and icterus. Other possible effects of the condition are hypogonadism and diabetes mellitus (Copestead and Banasik, 2014).
Treatment of the condition varies based on intensity. Patient with mild thalassemia will require not intervention. Those with the hemoglobin H form will need to take folate supplements. These should avoid drugs with iron or oxidative drugs. Those with severe thalassemia will need to have a folate supplement as well as regular transfusions. Deferoxamine mesylate may be needed for iron chelation therapy when tissue become overloaded with iron deposits (Arcangelo et al, 2018).
Deferoxamine mesylate functions by binding to free iron within a stable complex which inhibits its ability to be part of chemical reactions. This is then excreted by the kidneys and in fecal matter from bile (Pubchem, 2018). Common adverse reactions are nausea, vomiting, diarrhea, abdominal discomfort, tachycardia, hypotension, pruritis, injection site irritation and ocular and auditory disturbances. Patients receiving this medication need to have annual hearing and visual examinations.
Arcangelo, V., Peterson, A., Wilbur, V., and Reinhold, J. (2017) Pharmacotherapeutics for advanced practice: A practical approach (4th ed.). Ambler, PA: Lipincott Williams and Wilkins
CDC (2018). Living with thalassemia. Centers for Disease Control and Prevention. Retrieved from: https://www.cdc.gov/features/international-thalassemia/index.html
Copestead, L. and Banasik, J. (2013). Pathophysiology (5th ed.). St. Louis, MO: Elsevier Inc.
Muncie, H. and Campbell, J. (2009). Alpha and beta thalassemia. American Family Physician. 80(4): 339-344
NIH (2018). Thalassemias. National Heart, Lung, and Blood Institute. Retrieved from: https://www.nhlbi.nih.gov/health-topics/thalassemias
Pubchem (2018). Deferoxamine mesylate. National Center for Biotechnology Information. Retrieved from: https://pubchem.ncbi.nlm.nih.gov/compound/Deferoxamine_mesylate
Vichinsky, E. (2013). Clinical manifestation of a-thalassemia. Cold Spring Harbor Perspective in Medicine. 3(5): a011742
A Sample Answer 3 For the Assignment: NURS 6521 Week 9: Hematologic System
Title: NURS 6521 Week 9: Hematologic System
Sickle Cell Anemia
To explain Sickle Cell anemia, one must know about Sickle Cell Disease (SCD). SCD is an inherited disorder that affects the red blood cells. Patient’s inherited the sickle cell gene from both parents; thus, causing SCD (Moerdler & Manwani, 2018). SCD is when the red blood cells are not flexible and form a “sickle” shape that doesn’t allow them to flow in the vessels. The sickle cells adhere to the vessel walls causing blood flow to slow or stop. When this happens the patient experiences severe pain that can occur without warning.
Sickle Cell anemia is when patient’s have sickle cell disease and their red blood cells only last 10 to 20 days instead of 90 to 120 days (Moerdler & Manwani, 2018). Their bodies have trouble making new red blood cells to replace the lost ones, this causes the patient to have anemia due to a low red blood cell count.
Treatment
Patient’s with Sickle Cell anemia should follow a healthy lifestyle to avoid situations that may put them in a crisis as there is no cure. When patients need treatment to help with symptoms, they will be prescribed antibiotics, pain relivers, Hydroxyurea, childhood immunizations, and blood transfusions. In rare cases, for those with sever SCD, patients may get a bone marrow or stem cell transplant; for this to work a close match is required, usually a sibling (Moerdler & Manwani, 2018).
Antibiotics are given to patients to help them avoid life-threatening infections such as pneumonia (NIH, 2018). Children start taking penicillin when they are two months old and continue until at least age five, this age is at high risk for contracting an infection and this helps prevent those illnesses, such as pneumonia (NIH, 2018). Adults may need to continue this therapy if they have had pneumonia in the past or have had a splenectomy.
Some patients will require a blood transfusion to replace the red blood cells. This treatment comes with the risk of excess iron buildup. If there is excess iron built up patients will need to have treatments to reduce the iron level (Moerdler & Manwani, 2018).
Hydroxyurea is prescribed to patients with SCD to help reduce the frequency of crisis episodes and can help reduce the likelihood of a blood transfusion or hospital stay (NIH, 2018). Hydroxyurea is an antineoplastic agent that inhibits DNA synthesis (Drug Bank, 2019). It is believed to work by increasing the production of fetal hemoglobin in the red blood cells (Drugs, 2017). Fetal hemoglobin is a type of hemoglobin found in newborns that helps prevent the formation of sickle cells (Moerdler & Manwani, 2018). It is metabolized in the liver with a half life of 3-4 hours and is eliminated through the kidneys.
Factor-Age
Sickle Cell Anemia is most common in African American’s; 1 in 365 African American babies is born with sickle cell disease (NIH, 2018). Treatment can now begin earlier in life as all states now have SCD as a part of their newborn screening (NIH, 2018).
Hydroxyurea has not been studied in children and should not be given to patients under the age of 18 (Drugs, 2017). Thus, making it very important to keep children up to date on immunizations and to take antibiotics to help prevent infections. Children also need to maintain a healthy lifestyle; staying hydrated, adequate rest, balanced meals, and proper hand washing. Those over the age of 65 may need a smaller dose as the elderly may be more sensitive to the drug (Drugs, 2017).
References
Arcangelo, V. P., Peterson, A. M., Wilbur, V., & Reinhold, J. A. (2017). Pharmacotherapeutics for advanced practice: A practical approach (4th ed.). Ambler, PA: Lippincott Williams & Wilkins.
Drug Bank. (2019, January 1). Retrieved from https://www.drugbank.ca/drugs/DB00451
Drugs. (2017, January 20). Retrieved from Drugs: https://www.drugs.com/
Moerdler, S., & Manwani, D. (2018, November). New Insights into the pathophysiology and development of novel therapies for sickle cell disease. Hematology, 2018(1), 493-506.
NIH. (2018, July 30). Medline Plus. Retrieved from Sickle Cell Disease: https://medlineplus.gov/sicklecelldisease.html
A Sample Answer 4 For the Assignment: NURS 6521 Week 9: Hematologic System
Title: NURS 6521 Week 9: Hematologic System
Introduction
Hematological disorders are the diseases associated with the components of blood such as red blood cells, white blood cells, and platelets. For example, conditions associated with red blood cells include anemia, thalassemia, iron-deficiency anemia, hemolytic anemia, and sickle-cell anemia. Those associated with WBC include leukemia and lymphoma while those associated with platelets include thrombocytosis purpura.
Anemia
Sickle cell anemia (Sickle Cell Disease, SCD) is an autosomal recessive single gene defect in the beta chain of hemoglobin in which hemoglobin leads to chronic hemolytic anemia with numerous clinical consequences (Arcangelo, Peterson, Wilbur & Reinhold, 2017). Sickle cell anemia is linked with varying degrees of anemia, red cell hemolysis, and blockage of small blood capillaries triggering painful crises, damage to major organs, and increased susceptibility to severe infections (Epocrates, 2019). In this discussion, I will emphasis on anemia as the most prevalent hematological disorder that is derived from the red blood cells.
Anemia is a disease in which the concentration of hemoglobin or erythrocytes in the blood is below average (Arcangelo, Peterson, Wilbur & Reinhold, 2017. It is the most frequent disorder of the blood and statistics from the National Heart, Lung and Blood Institute indicates that the disease affects more than three million people in the United States. Anemia occurs when a person does not have adequate red blood cells or when the RBC do not work proficiently. RBC carries hemoglobin throughout the blood. People that are suffering from anemia do not have enough oxygen in their bodies, and they experience various symptoms such as dizziness, weakness, shortage of breath, irregular heartbeat, headache, yellow skin, cold hands and feet, and chest pain (Maakaron, 2018).
Patient factors, effects of age and Gender on Anemia
Patient factor: Hereditary factor has more effects on sickle cell anemia. Around 8% of African American people carry the sickle cell gene (Epocrates, 2019). However, based on the research study, each year, 4000 to 5000 pregnancies are at risk for sickle cell disease in the United States (Epocrates, 2019). In the United States, many people are at risk of anemia due to their poor diet, chronic diseases, intestinal disorders, infections, and other conditions. Research indicates that women who are menstruating or pregnant, as well as people suffering from chronic medical conditions, are at high risk of contradicting the disease (Arcangelo, Peterson, Wilbur & Reinhold, 2017. Concerning the age factor, old people also have a high probability of becoming anemic. For instance, people of above 50 years of age usually get infected with chronic conditions that might result in anemia(Maakaron, 2018). These conditions include cancer, thyroid disease, ulcerative colitis , kidney disease, liver disease, and rheumatoid arthritis.
Pharmacotherapy for Anemia
Healthcare providers such as Advanced Nurse Practitioner routine use numerous therapeutic methods in the treatment management of anemia, which contain the use of blood and blood products, immunotherapies, nutritional therapies, and adjunctive therapies. In acute anemia, the role of therapies is mainly restoring the hemodynamics of the vascular systems as well as replacing the lost red blood cells (Arcangelo, Peterson, Wilbur & Reinhold, 2017). Most of the time and Advance Nurse practitioner might use mineral and vitamin supplements combined with blood transfusions, vasopressors, glucocorticosteroids, and histamine antagonists to achieving a better outcome. The selection of appropriate therapy is determined by the documentation of the etiology of anemia. Also, all microcytic anemia do not originate from iron deficiency, but some are iron-overloading disorders.
The administration of oxygen, provision of pain-relieving drugs together with intravenous fluids that reduce pain and prevent complications are some of the sickle-cell anemia treatment regimen. Pregnant women and old adults, the healthcare providers regularly recommend blood transfusions, antibiotics, and folic acid supplements. Bone marrow transplant, also identified as stem cell transplant is an effective treatment, that offers the only potential possible cure for sickle cell anemia patients (Mayo Clinic, 2019). A treatment regimen of Nitric oxide ,and folic acid supplements are vital for people with sickle cell anemia because they have low levels of them in their blood cells. Also, a cancer drug known as hydroxyurea can be used in the treatment of sickle-cell anemia (Maakaron, 2018). Children with sickle cell anemia can take penicillin antibiotic for infection prevention.
Drug Therapy
Before initiating drug therapy, it is imperative to examine a peripheral blood smear for sickling and checking a reticulocyte count can provide supportive data to diagnose SCD (Arcangelo, Peterson, Wilbur & Reinhold, 2017). A reduced hemoglobin and RBCs count are the laboratory results, and chorionic biopsy is the test during early gestation (Arcangelo, Peterson, Wilbur & Reinhold, 2017). A therapeutic transfusion is reserved for a person with acute stroke, acute chest syndrome, acute multiorgan failure, and acute symptomatic anemia (Arcangelo, Peterson, Wilbur & Reinhold, 2017). he management treatment of SCD primarily emphasis on the main prevention of the complication, children with SCD should be immunized against S. pneumonia, and H. influenzae and hepatitis B and meningococcal vaccine (Arcangelo, Peterson, Wilbur & Reinhold, 2017). Hydroxyurea is used in some patient as a prophylaxis treatment to reduce the number of crisis (Arcangelo, Peterson, Wilbur & Reinhold, 2017).
Another alternative treatment is the management of painful episodes consists of exclusion of causes such as infection, hydration by oral or intravenous fluid resuscitation, and aggressive pain relief, including analgesics and opiates (Arcangelo, Peterson, Wilbur & Reinhold, 2017). Vaso-occlusive crisis is treated with analgesia, antihistamine, supportive care and correction of the cause, hydration, antibiotics and blood transfusion (Epocrates, 2019). Acute chest syndrome is treated with oxygen and incentive spirometry, analgesia, antihistamine, broad-spectrum antibiotics, blood transfusion and hydration (Epocrates, 2019). For chronic disease, continuing treatment with supportive care, prevention of complications, hydroxyurea, repeated blood transfusions and bone marrow transplantation is required (Epocrates, 2019).
Measures to reduce the adverse effects
While numerous types of anemia cannot be prevented, consumption of a healthy diet is vital in avoiding both iron-deficiency and vitamin-deficiency anemia. The affected people necessitate including food in their diet that includes high levels of iron such as beef, dried fruits, and nuts. Folic acid foods include citric juices, legumes, and dark green leafy vegetables. Although there is no way to prevent sickle cell disease, genetic counseling can help to counsel parents who are heterozygous and are therefore at risk of producing homozygous offspring (Epocrates, 2019). Prevent dehydration by drinking lots of water, because dehydration can increase the risk of a sickle cell crisis. Individual with Sickle Cell Anemia should avoid extreme temperature. Since, exposure to extreme heat or cold can increase the risk of a sickle cell crisis.
Conclusion
Hematological disorders such as anemia require specific drug therapies and interventions that will control the prevalence. Women and older adults are risky to anemic infections hence the need for proper prevention strategies.
References
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.
Epocrates. (2019). Sickle cell anemia. Retrieved from https://online.epocrates.com/diseases/Sickle-cell-anemia/Epidemiology
Maakaron, J. E. (2018, January 20). Anemia Medication. Retrieved January 20, 2019, from Medscape: https://emedicine.medscape.com/article/198475-medication
Mayo Clinic. (2019). Sickle cell anemia. Retrieved from https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/treatment/
A Sample Answer 4 For the Assignment: NURS 6521 Week 9: Hematologic System
Title: NURS 6521 Week 9: Hematologic System
Sickle Cell Anemia
Alterations of erythrocyte functions resulting in too few or an insufficient volume of erythrocytes or a reduction in the quality or quantity of hemoglobin in the circulating blood is a condition called anemia. Anemias occur from a plethora of reasons and, therefore, are classified by their cause or by the changes that affect the shape, size, or substance of the erythrocyte (Huether & McCance, 2017). The specific anemia this discussion focuses on is sickle cell anemia. Sickle cell anemia is an inherited, autosomal recessive disorder. It is characterized by the production of abnormal hemoglobin S (Hb S) within the erythrocyte. Hb S reacts to deoxygenation and dehydration by bending the erythrocyte into a stretched-out sickle shape, thus, producing hemolytic anemia (Huether & McCance, 2017). The shape of the sickle cell makes it prone to becoming lodged within the blood vessel, causing a reduced blood supply to organs as well as damaging the endothelium of both arterial and venous circulation systems. Under these circumstances, a sickle cell crisis may ensue. Furthermore, those who suffer from sickle cell anemia are also predisposed to gallstones, renal failure and Streptococcus pneumoniae (S. pneumoniae) and Haemophilus influenzae (H. influenzae) infections (Arcangelo, Peterson, Wilbur & Reinhold, 2017).
Pharmacological Therapies
Sickle cell disease (SCD) management focuses on the prevention and treatment of its complication. Prevention includes the immunization against influenza, hepatitis B, H. influenzae, meningitis, pneumococcal disease, and S. pneumoniae (Arcangelo et al., 2017). The only drug currently approved by the US Food and Drug Administration for the treatment of SCD is hydroxyurea. Opioid or nonsteroidal analgesics may be used for pain management, tricyclic antidepressants improve mood and also regulate pain signals, and folic acid supplementation helps replenish the depleted folate stores necessary for erythropoiesis. Furthermore, long-term hydroxyurea is also the currently accepted treatment for frequent and severe pain (Maakaron, 2017).
Hydroxyurea is an antineoplastic which mechanism of action is to inhibit DNA synthesis without interfering with RNA or protein synthesis. It is also an antimetabolite specific for S phase of a cell cycle (Skidmore-Roth, 2018). Hydroxyurea also increases total and fetal hemoglobin in children with SCD which delays gelation and sickling of red blood cells (RBCs) (Maakaron, 2017). Hydroxyurea is indicated for melanoma, recurrent or metastatic ovarian cancer, sickle cell anemia, and squamous cell carcinoma of the head and neck. It is administered by mouth as a capsule, readily absorbed, degraded in the liver, and excreted in urine (Skidmore-Roth, 2017). Side effects include alopecia, hyperpigmentation of the skin, dry skin, nail pigmentation, and leg ulcers (Arcangelo et al., 201). Serious adverse effects are hepatotoxicity, pancreatitis, seizures, leukopenia, thrombocytopenia, megaloblastic erythropoiesis, pulmonary fibrosis, and secondary cancers (Skidmore-Roth, 2018).
Opioid analgesics are used to control an acute sickle cell crisis. An example of an opioid is oxycodone with acetaminophen. Oxycodone with acetaminophen is used for moderate to severe pain. It inhibits ascending pain pathways in the central nervous system (CNS) and therefore alters pain perception and increases pain threshold. It is contraindicated in those with a history of opiate addiction, asthma, and ileus. Oxycodone with acetaminophen is available as a tablet or oral solution. Onset of the drug is 15-30 minutes with a duration of 2-6 hours. Metabolism occurs in the liver and excretion via the urinary system. It is 45% protein bound. Several common side effects may occur such as dizziness drowsiness, headache, sedation, confusion, euphoria, nausea, vomiting, constipation, anorexia, cramps, and a rash. Adverse reactions include bradycardia and respiratory depression (Skidmore-Roth, 2018).
A nonsteroidal anti-inflammatory drug (NSAID) such as ibuprofen may also add to the effects of opioids during a painful crisis. Ibuprofen has analgesic and antipyretic properties and is therefore used to reduce fever or treat pain or inflammation caused by a variety of conditions. Its mechanism of action is not entirely understood but may be related to prostaglandin synthetase inhibition (Drugs.com 2018). Administered orally as a capsule, tablet or liquid; ibuprofen has an onset of 30 minutes, with a peak of 1-2 hours. It is metabolized by the liver and excreted in urine. Adverse effects may occur such as headaches, nausea, vomiting, anorexia with more serious adverse effects of hepatitis, gastrointestinal (GI) bleeding or perforation, nephrotoxicity, myocardial infarction, and stroke (Skidmore-Roth, 2018).
An example of a tricyclic antidepressant used in some sickle cell anemia patients is amitriptyline. Amitriptyline inhibits presynaptic reuptake of serotonin and norepinephrine and blocks adrenergic, cholinergic, histaminergic, and sodium channels (Maakaron, 2017). Indications include major depression with unlabeled uses for neuropathic pain, fibromyalgia, diabetic neuropathy, and prevention of cluster migraines. It is taken by mouth as a tablet where it is then metabolized by the liver to nortriptyline and subsequently excreted in urine and feces. Side effects include dizziness, drowsiness, insomnia, orthostatic hypotension, dry mouth, constipation, urinary retention, and blurred vision (Skidmore-Roth, 2018).
Folic acid is a nutritional supplement that is administered orally as a tablet. Folic acid is essential for the formation of coenzymes in the metabolic systems (purine and pyrimidine synthesis required for maintenance in erythropoiesis), and it also stimulates platelet production in folate deficiency anemias. Absorption occurs in the proximal small intestine; metabolism occurs in the liver, and excretion via the kidneys. Side effects are minimal and rare (Folic acid Rx OTC, n.d.).
Effects of Patient Factors
Patient behaviors such as drinking alcohol, excessive sun exposure, or overuse of non-scheduled drugs such as opioid analgesics or NSAIDs will impact prescribed drugs for SCD. For instance, ibuprofen total daily dose should not exceed 3200 mg. And if GI complaints occur, administer ibuprofen tablets with meals or milk. In addition, the risk of bleeding occurs when taken with anticoagulants, prolonged use, smoking, drinking alcohol (Drugs.com, 2018). Furthermore, ibuprofen use should be avoided or minimized when taking hydroxyurea due to increased risk of thrombocytopenia. Similarly, amitriptyline should be taken with food or milk to avoid GI symptoms. Patients taking amitriptyline should also wear sunscreen or large hats when outdoors as photosensitivity may occur. And lastly, when taking amitriptyline or oxycodone with acetaminophen, alcohol is to be avoided to prevent further CNS depression and impaired psychomotor function (Skidmore-Roth, 2018).
References
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.
Drugs.com. (2018). Ibuprofen. Retrieved from https://www.drugs.com/pro/ibuprofen.html
(Folic acid Rx OTC). (n.d.). Folic acid (Rx, OTC). Retrieved January 23, 2019, from Medscape.
Huether, S. E. & McCance, K. L. (2017). Understanding Pathophysiology (6th ed.). St. Louis, MO: Mosby.
Maakaron, J. E. (2018). Sickle cell anemia. Retrieved January 23, 2019, from Medscape.
Skidmore-Roth, L. (2018). Mosby’s 2018 nursing drug reference (31st ed.). St. Louis, MO: Elsevier.
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A Sample Answer 5 For the Assignment: NURS 6521 Week 9: Hematologic System
Title: NURS 6521 Week 9: Hematologic System
Sickle cell anemia is an inherited disease where blood cells are sickle or crescent shape and are very sticky and ridged unlike regular blood cells which are round and flexible (Mayo Clinic, 2018).
The current treatment for sickle cell includes hydroxyurea which works by increasing the water content of red blood cells which increases the deformability of sickled cells and alters the adhesion of red blood cells to endothelium (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). Side effects include upset stomach, nausea, vomiting, diarrhea, constipation, skin changes (color changes or peeling), flu like symptoms, hair loss, rash, headache, dizziness, drowsiness, and weight gain. Serious side effects include anemia, myelosuppression, and leukemia (Rxlist, 2017).
In July 2017 the FDA approved a new therapy for Sickle cell anemia called L-glutamine oral powder “in phase 3 of the trial patients taking this drug had 25% fewer hospital visits for sickle cell crisis, were hospitalized 33% less often, were discharged an average of 4.5 days sooner, and were 65% less likely to experience acute chest syndrome compared with the placebo group” (Karon, 2017). The way L-glutamine oral powder works is not totally understood but what is believed is it “may improve the NAD redox potential in sickle RBCs through increasing the availability of reduced glutathione” (Rxlist, 2017). The side effects if this medication are constipation, nausea, headache, abdominal pain, cough, pain in extremities, back pain, chest pain, indigestion, burning sensation, and hot flashes (Rxlist, 2017). Other drugs used to help control sickle cell attacks are short and long acting opioids and NSAIDs.
Sickle cell anemia is a genetic disease that not only effects adults but also children. About 10% of people from African decent have sickle cell anemia. Treatment for children include pain medications, a blood transfusion, drinking plenty of water, folic acid to help prevent anemia and sometimes a bone marrow transplant (Stanford Children’s Health, n.d.). Life expectancy has increase for individuals with Sickle cell anemia over the last 30 years.
References
Arcangelo, V. P., Peterson, A. M., Wilbur, V., & Reinhold, J. A. (2017). Pharmacotherapeutics for advanced practice: a practical approach. Philadelphia, PA: Lippincott Williams & Wilkins.
Karon, A. (2017, December 15). A new drug and more on the way for sickle cell disease. Retrieved from https://acphospitalist.org/archives/2017/12/new-drug-and-more-for-sickle-cell-disease.htm
Mayo Clinic. (2018, March 8). Sickle cell anemia – Symptoms and causes. Retrieved from https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876
Rxlist. (2017, July 14). Common Side Effects of Hydrea (Hydroxyurea) Drug Center – RxList. Retrieved from https://www.rxlist.com/hydrea-side-effects-drug-center.htm
Rxlist. (2017, July 19). Common Side Effects of Endari (L-glutamine Oral Powder) Drug Center – RxList. Retrieved from https://www.rxlist.com/endari-side-effects-drug-center.htm
Stanford Children’s Health. (n.d.). Sickle cell disease in children. Retrieved from https://www.stanfordchildrens.org/en/topic/default?id=sickle-cell-disease-in-children-90-P02327
A Sample Answer 6 For the Assignment: NURS 6521 Week 9: Hematologic System
Title: NURS 6521 Week 9: Hematologic System
Adult hemoglobin is primarily comprised of hemoglobin A, which is made up of equal quantities of alpha and beta globin chains (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). Thalassemia is an inherited blood disorder that is caused by the body not making enough hemoglobin, resulting in anemia. Thalassemia is existent when there is a decreased production of either the alpha or beta globin or structurally abnormal globin chains (Arcangelo, Peterson, Wilbur, & Reinhold, 2017).
Individuals with milder forms of this disease will not require treatment. Hemoglobin H, should take a folate supplement and avoid iron and oxidative medications (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). Synthetic folic acid has a bioavailability between 85-100% (Folic Acid, 2019). Folic acid is distributed to all body tissues including CNS and is primarily stored in the liver where it is also metabolized (Folic Acid, 2019). Folic acid is mainly excreted through the urine (Folic Acid, 2019). Other categories of thalassemia include alpha-thalassemia, beta-thalassemia minor, beta-thalassemia intermedia, and beta thalassemia major. Individuals with severe thalassemia are managed through regular blood transfusions and receive folate supplementation (Arcangelo, Peterson, Wilbur, & Reinhold, 2017).
Deferoxamine
Deferoxamine mesylate may be used in patients with thalassemia to remove excess iron from the bloodstream. Deferoxamine chelates iron by forming a stable complex that stops it from entering into further chemical reactions (deferoxamine, n.d.). It is metabolized mainly by plasma enzymes and is excreted primarily through the urine (deferoxamine, n.d.). Adverse effects with a frequency that is not defined include injection site reactions, nausea, vomiting, diarrhea, hypotension with too rapid of IV infusion, blood dyscrasia (deferoxamine, n.d).
Deferasirox
Deferasirox is another medication that can be used to remove iron from the bloodstream. It is a oral active chelator that is selective for iron and reaches a peak in 1.5 to 4 hours (deferasirox, 2018). It is approximately 99% protein bound almost exclusively to serum albumin with a volume of distribution of 14.37 L (deferasirox2018). It is metabolized through glucuronidation and it is excreted in the feces (deferasirox, 2018). Adverse reactions include acute kidney injury, hepatic toxicity, GI hemorrhage, bone marrow suppression, and skin rash (deferasirox, 2018).
New treatments being designed
Luspatercept is a medication that is designed to initiate the production of healthy blood cells by regulating transforming growth factor beta proteins involved in late-stage red cell differentiation and maturation (Campbell, 2018). LentiGlobin inserts a functional human beta-globin gene into a patient’s own hematopoietic stem cells, following this re-engineering they are infused back into the patient (Campbell, 2018). Both of these therapies are currently in human research phases with good results.
Thalassemia, Genetics, and Treatment
Thalassemias are a group of recessively inherited conditions. Genetic testing can be done to determine the presence of these genes. Genetics and genetic mutations impact the disease and its severity. Depending on the severity of the particular type of thalassemia will determine the patient’s course of treatment.
Iron deficiency is the most common cause of microcytosis, increasing the risk that a provider may mistakenly prescribe an iron supplement. It is important that providers rule out thalassemia traits and perform lab tests to evaluate iron levels, as excessive iron can deposit in the body causing organ damage in the long-term (The Demographics of Thalassemia, n.d.). Presently the only cure that exists for this disease is stem cell transplant.
References
Arcangelo, V. P., Peterson, A. M., Wilbur, V., & Reinhold, J. A. (2017). Pharmacotherapeutics for Advanced Practice: A practical approach (4 ed.). Ambler, PA: Lippincott Williams & Wilkins.
Campbell, T. (2018). 2 Beta Thalassemia Drugs Are Fast Approaching the Finish Line. Retrieved from https://www.fool.com/investing/2018/04/22/2-beta-thalassemia-drugs-are-fast-approaching-the.aspx
deferasirox. (2018). Retrieved from https://www.drugs.com/pro/exjade.html
deferoxamine. (n.d.). Retrieved from https://reference.medscape.com/drug/desferal-deferoxamine-343722#10
Folic Acid. (2019). Retrieved from https://www.drugs.com/monograph/folic-acid.html
The Demographics of Thalassemia. (n.d.). Retrieved from http://thalassemia.com/demographics.aspx#gsc.tab=0
A Sample Answer 7 For the Assignment: NURS 6521 Week 9: Hematologic System
Title: NURS 6521 Week 9: Hematologic System
Week 9 Discussion: Pharmacotherapy for Hematologic Disorders: Thalassemia
Thalassemia’s, which are considered hypoproliferative anemias, are hemoglobin synthesis hereditary disorders (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). Thalassemia is present when there is either a decrease in production of alpha or beta-globins, or the globin chain is structurally abnormal (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). When there is an alteration of the alpha-globin chains a mutation of one, or four genes, can occur (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). A mutation of all four genes is incompatible with life and is also known as hydrops fetalis (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). An alteration of one of the four genes is considered a silent carrier (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). When two of the four genes are altered mild anemia and microcytosis results (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). When three or the four genes are altered extra beta-chains form tetramers and severe anemia and microcytosis develop (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). Pallor and splenomegaly will be noted upon physical examination (Arcangelo, Peterson, Wilbur, & Reinhold, 2017).
Beta-thalassemia is classified by the severity of the anemia (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). The beta-globin chain is controlled by two genes; when one gene is altered beta-thalassemia minor results and when both are dysfunctional, thalassemia intermedia is present (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). Thalassemia major, also known as Cooley anemia, results when both genes are altered (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). Red blood cell transfusions are required for life and growth failure, bony deformities, hepatosplenomegaly, jaundice, leg ulcers and cholelithiasis are noted (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). Frequent transfusions in these individuals leads to iron overload as witnessed by hemochromatosis, heart failure, cirrhosis, and endocrinopathies (Arcangelo, Peterson, Wilbur, & Reinhold, 2017).
Mild thalassemia, with either the alpha-thalassemia trait or beta-thalassemia minor, does not require treatment (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). Hemoglobin H requires folate supplements while iron and oxidative drugs should be avoided (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). Severe thalassemia requires folate suuplemantion and regular transfusions (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). Red blood cell transfusions are required every two to three weeks (Vigue, 2017). The goal of routine transfusions is to keep the hemoglobin above 9 grams/ deciliter (g/dl) (Kannan, & Singh, 2017). Iron chelation therapy with deferoxamine mesylate can be used if transfusions are not tolerated (Arcangelo, Peterson, Wilbur, & Reinhold, 2017).
Folate supplements (Folic Acid)
Folic acid is metabolized by the liver and converted to dihydrofolic acid and tetrahydrofolic acid, is highly bound to plasma proteins, and eliminated in the urine (Drugbank, 2019b). Most elimination is seen within six hours and is complete within 24 hours (Drugbank, 2019b). Folic acid can also be excreted in the milk of nursing mothers (Drugbank, 2019b).
Deferoxamine (DFO)
DFO is referred to as a chelating agent and it works by binding to the free iron or aluminum in the bloodstream and enhances the elimination through the urine (Drugbank, 2019a). The chance of liver damage and other signs of iron overload, as mentioned above, are then reduced. DFO is rapidly absorbed following intramuscular or subcutaneous administration and should not be given orally (Drugbank, 2019a). DFO is primarily metabolized in the plasma and minimally by the liver; eliminated primarily by metabolism, feces, and bile; and has a half-life of one hour (Drugbank, 2019a). Deferoxamine is administered subcutaneously by a continuous infusion of 20- 50 milligrams/kilogram/day (mg/kg) for 10 to 24 hours per day; a dose of up to 60 mg/kg/day can be used also (Arcangelo, Peterson, Wilbur, & Reinhold, 2017, Saliba, Harb, & Taher, 2015). Dosing is also required five to seven days a week which leads to a much higher noncompliance rate and complications (Saliba, Harb, & Taher, 2015). Local injection site irritation, pruritus, hypotension, tachycardia, abdominal discomfort, diarrhea, nausea, and vomiting can occur (Arcangelo, Peterson, Wilbur, & Reinhold, 2017). Baseline and annual hearing and vision exams should also be performed due to the risk of ocular and auditory disturbances (Arcangelo, Peterson, Wilbur, & Reinhold, 2017).
Factor: Age
In children with severe thalassemia that requires regular transfusions, the use of DFO is crucial for proper development. Suboptimal iron chelation and hypoxia in these individuals leads to growth retardation and short stature (Kannan, & Singh, 2017). The
largest percentage of growth retardation was found in children whose hemoglobin was less than 8 g/dl (Kannan, & Singh, 2017). Body weight and growth needs to be followed closely and assesse every three months in children receiving routine therapy (Saliba, Harb, & Taher, 2015). Kannan, & Singh (2017), recommend oral chelation therapy and lots of parental education to increase compliance, since compliance is the main reason treatment is not effective. Two oral chelating agents are currently available: deferiprone (DFP), and deferasirox (DFX) and are described below (Saliba, Harb, & Taher, 2015).
DFP
DFP, which is currently only approved for adult dosing in the United States, is administered orally and is given in three divided daily doses of 75-100mg and is excreted mainly in the urine (Saliba, Harb, & Taher, 2015). Side effects include gastrointestinal symptoms (GI), neutropenia, agranulocytosis, arthralgia and increased liver enzymes (Saliba, Harb, & Taher, 2015). Weekly complete blood counts (CBC’s) are needed weekly to monitor for side effects (Saliba, Harb, & Taher, 2015). DFP can also be combined with DFO in individuals who have reached the maximum dose of DFP and has been proven to work very well (Saliba, Harb, & Taher, 2015). Altering the use of DFP and DFO is also effective for some individuals with transfusion- dependent thalassemia (Saliba, Harb, & Taher, 2015).
DFX
DFX is administered 20-40 mg once daily, is excreted in the feces and bile and has a half-life of 16-18 hours (Saliba, Harb, & Taher, 2015). Side effects include GI disturbances, GI bleeding, elevated serum creatinine, rash, elevated liver enzyme, liver failure and renal insufficiently; this drug is also expensive so use is limited for more patients (Saliba, Harb, & Taher, 2015). This drug should be taken on an empty stomach after being dissolved in water, apple juice, or orange juice to ensure adequate bioavailability (Saliba, Harb, & Taher, 2015). Combining DFO and DFX has been tolerated but has shown limited efficacy (Saliba, Harb, & Taher, 2015).
Combining or alternating DFX and DFP is also possible, and safe, but studies demonstrating the efficacy are limited (Saliba, Harb, & Taher, 2015).
US guidelines recommend maintaining the existing iron chelation therapy as long as the liver iron concentration is between 3 milligram/dry weight (mg/dw) and 7 mg/dw and serum ferritin is between 1,000 ng/ml and 2,500 ng/ml (Saliba, Harb, & Taher, 2015). DFX can also be given at a maximum tolerated dose, or DFO, over the course of 12 hours, if there is not cardiac dysfunction (Saliba, Harb, & Taher, 2015). DFO should be given 24 hours daily in the case of cardiac dysfucntion (Saliba, Harb, & Taher, 2015). An oral iron chelator SP-420 has been shown to be effective in the case of iron overload and is currently in the trial phases (Saliba, Harb, & Taher, 2015, Taher, et.al, 2017). Saliba, Harb, & Taher (2015), also recommend the use of JAK2 inhibitors as a means to reduce the need for blood transfusions (Saliba, Harb, & Taher, 2015).
References
Arcangelo, V. P., Peterson, A. M., Wilbur, V., & Reinhold, J. A. (2017). Pharmacotherapeutics
for advanced practice: A practical approach (4th ed.). Ambler, PA: Lippincott Williams & Wilkins.
Drugbank. (2019a). Deferoxamine. Retrieved from
https://www.drugbank.ca/drugs/DB00746
Drugbank. (2019b). Folic Acid. Retrieved from https://www.drugbank.ca/drugs/DB00158
Kannan, S., & Singh, A. (2017). Compliance score as a monitoring tool to promote treatment
adherence in children with thalassemia major for improved physical growth. Asian Journal of
Transfusion Science, 11(2), 108–114. https://doi-org.ezp.waldenulibrary.org/10.4103/ajts.AJTSpass:
Saliba, A. N., Harb, A. R., & Taher, A. T. (2015). Iron chelation therapy in transfusion-dependent
thalassemia patients: current strategies and future directions. Journal of blood medicine, 6, 197-209. doi:10.2147/JBM.S72463
Taher, A. L.,…Viprakasit, V. (2017, September). Safety and pharmacokinetics of the oral iron
chelator SP‐420 in β‐thalassemia. American Journal of Hematology, 92(12), 1356-1361.
https://doi-org.ezp.waldenulibrary.org/10.1002/ajh.24914
Vigue, C. L. . P. D. (2017). Thalassemia. Magill’s Medical Guide (Online Edition). Retrieved
from https://ezp.waldenulibrary.org/login?url=https://search.ebscohost.com/login.aspx? direct=true&db=ers&AN=86196391&site=eds-live&scope=site
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