Sickle cell anemia is an inherited disorder on the beta chain of the hemoglobin resulting to abnormally shaped red blood cells. Hemoglobin is a form of protein that contains iron which is responsible for transporting oxygen that is carried in blood. The more hemoglobin molecules the RBC contain, a higher amount of oxygen will they be able to carry. Erythrocytes in sickle cell anemia contain abnormal hemoglobin that affects the beta-chain producing hemoglobin S or HbS. The abnormally shaped cells become sharp and spiky when the RBCs are discharging oxygen molecules and in cases where the oxygen content in blood is low such as performing vigorous exercise and being in high altitude areas. Sickle cell crisis refers to episodes of acute and severe sickling that blocks the circulation posing a threat of extensive organ damage. A condition called a sickle cell trait is identified by the presence of a single defective gene, instead of two.
Priaprism (males) – due to pooling of abnormally shaped erythrocytes in the blood vessels of the penis. Pain relief with Acetaminophen (Tylenol) or IV of morphine to reduce metabolic demand of oxygen thereby, terminating cell sickling. Hydroxyurea – antineoplastic agent that increases production of fetal hemoglobin in children. Obtain blood and urine culture, chest x-ray and CBC results if infection is the cause of sickling. The American College of Surgeons (ACOS) standards for cancer care include the availability of palliative care services that are offered from diagnosis through bereavement. What Is the Difference Between Under- or Untreated Depression and Truly “Giving Up” on Treatment? What Should I Tell My Patients About Fertility Testing and Its Indications for Fertility Counseling?


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RBC’s assume a crescent or C-shape that decreases the cell’s life span and ability to function thereby, producing various complications.
Severe pain is noted during these incidents caused by occluded vessels in the bone possibly resulting to bone necrosis. Oncology nurses care for patients and families across the continuum, including during their final journey. Muehlbauer, RN, MSN, AOCNS®, is a clinical nurse specialist for VA San Diego Healthcare System and academic educator for San Diego State University in California.
These small cells are circular and flattened with depressed centers on both sides resembling to that of a doughnut when viewed under a microscope. A red blood cell is just a vessel; the one that performs the oxygen transportation is the hemoglobin.
Valine (an amino acid) takes the place of the normally appearing glutamic acid in beta-chains.
The deformed erythrocytes also rupture easily and they tend to be trapped in the microcirculation, obstructing blood flow and oxygen transport that might lead to painful episodes of ischemic injury.
The crisis is triggered by hypoxemia, acidosis, or other conditions such as dehydration, infection, vigorous exercise, pregnancy or cold weather.
Meaning two sickle cell trait carrier parents can contribute a defective gene to a child that will carry the sickle cell disorder. Their size and shape provides a large surface area for carrying oxygen in relation to their volume. Replacement of glutamic acid with valine causes the polymerization of HbS components to cohere forming long and insoluble particles.


It is essential that nurses have the tools to compassionately assist with this transition and work with other disciplines to provide the best care.Kim Slusser, RN, MSN, CHPNAn Interdisciplinary ApproachACOS standards require an interdisciplinary team approach to palliative care to provide the most comprehensive care. Barbara Head, PhD, RN, CHPN, ACSW, FPCN, assistant professor in the University of Louisville School of Medicine’s Interdisciplinary Program for Palliative Care and Chronic Illness (IPPCCI) in Louisville, KY, and president-elect of the national Hospice and Palliative Nurses Association, explains the ideal make-up of the palliative care team.“The team must have the expertise to address patients’ physical, psychosocial, and spiritual needs of the patient,” she says. Heme molecules, which are only found in mature RBC’s, enables the erythrocytes to pick oxygen from the lungs and transport it throughout the body. The rounds include nursing, medical team representation, social work, physical and occupational therapy, nutrition, chaplain services, and recreation therapy. However, practicing nurses may have gotten very little in nursing school, and those graduating recently might want to know more.”She endorses the End-of-Life Nursing Education Consortium (ELNEC) for education in basic palliative and end-of-life care. Head explains that ELNEC offers a standardized eight-module curriculum as well as specialized curriculums teaching pediatric, critical care, veteran centric, and geriatric palliative care.Slusser concurs with using ELNEC. As a nursing administrator, it is great to be able to support the professional development of staff while also providing the rest of the staff with colleagues they can go to for knowledge, insight, and support.In addition to ELNEC, Slusser says that oncology nurses should look to partner within their organization or affiliates to provide nursing staff shadow experiences in hospice and palliative care.
They must be comfortable with death and their own beliefs about spirituality, yet be open to the beliefs and practices of others,” Head says.Slusser adds that nurses need to remember that it is the patient and family’s journey and to let them help lead the way. But, what we really need to do is go back to our basic nursing assessment of identifying what the patient and family goals are, what are their identified needs, and how best to support them through the process. Active listening is crucial; validating feelings, and providing comfort whether by medical management or psychosocial support is all part of the nurse’s role. Sometimes, just being a calm, supporting presence is all a patient or family needs.”Although caring for patients and their loved ones at the end of life can seem emotionally daunting, Slusser says she finds it incredibly rewarding.



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