Abstract: This paper provides an overview of the inherited illness, sickle cell anemia, its diagnosis, treatment and containment of its health implications for patients young and old. It devotes special attention to the difficult subject of how to counsel children through the frightening aspects of the disease, such as chronic pain and promising new treatments for the disease overall. Despite the illness’ chronic nature, it is a containable illness for most patients, and enables them, with proper care, to have full lives, even though there is as yet no cure. Textbook definition of the disability.
According to the National Institute of Health, (2005) sickle cell anemia is an inherited blood disease. This means individuals are born with the illness. One cannot catch the disease from other people but parents can pass the illness on to their children through their genetic material. (“What is Sickle Cell Anemia,” NIH, 2003) Sickle cell anemia lasts throughout the duration of an individual’s lifetime and affects the red blood cells. Normal red blood cells are smooth and round like doughnuts. They move easily through blood vessels to carry oxygen to all parts of the body.
In sickle cell anemia, the red blood cells become hard. They are sticky, in other words, they do not proceed through the blood vessels freely, because they are shaped like sickles or crescents. When these hard and pointed red cells go through the small blood vessels, they tend to get stuck and block the flow of blood. This can cause pain, damage, and a low blood count of red cells, a condition known as anemia. (“What is Sickle Cell Anemia,” NIH, 2003) Key diagnostic procedure(s) for determining the disability The sickle cell genes tell the body to make the variant hemoglobin that results in sickle-shaped red blood cells.
Hemoglobin is the protein in red blood cells that carries oxygen to all parts of the body. Children who inherit sickle cell genes from both parents will have the variant hemoglobin and have sickle cell anemia. Children who inherit the sickle cell gene from only one parent will not have the disease. They will have sickle cell trait, generally have no symptoms but can act as carriers of the illness and pass the sickle cell gene on to their children. (“What Causes Sickle Cell Anemia,” NIH, 2003) Today, screening tests are done on newborn infants in most states for anemia.
(“Diagnosis,” NIH, 2003) This screening includes a simple blood test for sickle cell anemia on all newborn infants. This test uses blood from the same blood samples as other routine newborn screening tests. If the first test shows that the sickle hemoglobin is present, a second blood test is done to confirm the diagnosis. These tests also tell whether the child carries the sickle cell trait. It is also possible to identify sickle cell anemia before birth by getting a sample of amniotic fluid or tissue taken from the placenta. This test can be done as early as the first few months of pregnancy.
(“Diagnosis,” NIH, 2003) Contributing causes of the disability As sickle cell anemia is inherited there are no contributing causes as such, although sickle cell anemia has a higher instance in individuals of African heritage. . (“What is Sickle Cell Anemia,” NIH, 2003) Relation of the disability to other diagnosis Sometimes, anemia-like symptoms are similar between individuals with sickle cell anemia and other forms of anemia. But as the cause of the low iron is different in sickle cell patients, and is a blood absorption issue rather than a dietary issue, the two illnesses are not related.
(“What is Sickle Cell Anemia,” NIH, 2003) Treatment(s) for the condition such as physical or occupation therapy Sickle cell anemia is a serious disease and there is no cure. Bone marrow transplantation offers a cure, but very few patients have matched donors. Some patients also do not want bone marrow transplants because of the risks involved. However, o the past 30 years, doctors have learned a great deal about the disease. They know what causes it, what it does to your body, and how to treat many of the complications.
Today, with good preventative and maintenance health care, many people with the disease: are in reasonably good health much of the time live fairly normal lives from40 to 50 years and longer. (“What is Sickle Cell Anemia,” NIH, 2003) Assisting devices to manage the condition Blood transfusions are used to treat worsening anemia and sickle cell complications. A sudden fall in the patient’s blood count resulting from infection or spleen enlargement is a common reason for the need for a transfusion. Some, but not all, patients need regular transfusions to prevent life-threatening events such as stroke or pneumonia.
Most complications of sickle cell anemia are treated as soon as they happen. (“Treatment,” NIH, 2003) Acute chest syndrome is a frequent cause of death in children and adults with sickle cell anemia. Treatment usually requires hospitalization and may include the assisting use of oxygen therapy, transfusions, antibiotics, and pain medication. Hand-foot syndrome is an early complication seen in sickle cell anemia that starts at less than 1 year of age and treatment usually includes the use of pain medication and fluids.
Leg ulcers may be treated with assisting cleansing solutions and zinc oxide. Skin grafts may be needed if the condition continues. Ulcers on the ankle are painful, and patients may be given strong pain medication. Bed rest and keeping the leg raised to reduce swelling are useful, though not always possible. (“Treatment,” NIH, 2003) Medications Penicillin is generally given to all children with sickle cell anemia until they are five to prevent complications such as hand-foot syndrome.
Although there is no cure, other than bone marrow transplantation, for sickle cell anemia, adult patients make take hydroxyurea for prevention and not to treat acute pain. It reduces the frequency of painful crises and of acute chest syndrome in sickle cell anemia patients, and patients taking the drug may also need fewer blood transfusions. There is concern, however, that this cause the lowering of the patient’s white blood count if the dose is too high, and the drug is not approved for use in children. (“Treatment,” NIH, 2003)
Psychosocial Issues The generalized weakness that accompany the illness can make it difficult for sickle cell patients to enjoy a full range of activities, although it should be stressed that most patients can and do lead normal lives when the illness is treated. (“What is Sickle Cell Anemia,” NIH, 2003) Vocational Issues Similarly, patients must be mindful of pacing their energy levels in their vocations and be especially mindful of proper diet, long-term maintenance care of their health, and not overextending themselves physically.
Access to health care and health insurance is critical for these patients throughout their lives. (“What is Sickle Cell Anemia,” NIH, 2003) Part II Vocational implications From the nurses’ point of view knowledge of the specific developmental characteristics of school-age children is important for nurses and other pediatric surgical team members when dealing with sickle cell child patients. Children ages 7 to 11 as in the concrete operations stage of cognitive development are often particularly interested in acquiring more detailed knowledge and gaining a sense of increased self-control.
Thus, the illness should be explained to them as best as possible. All efforts should be made to ensure that the child participaties in as manyschool-related activities, as possible, is able to attaining skills in competition and cooperation as much as possible through reasoable physical activity and develops an overall sense of competence. Nurses must work in dialogue with the child’s family, or even with a mature patient, to ensure that these needs are satisfied. (Hendricks-Ferguson & Nelson, 2003) Counseling implications
The nurse should be aware that school-age children may experience anxiety before an unfamiliar or familiar medical procedure because of their increased cognitive ability and may have many questions related to medical procedures that require health care providers to give age-appropriate explanations using correct scientific and medical terminology before all procedures. Explanation of these procedures can be enhanced by the use of simple anatomy and physiology diagrams and concrete terms explaining why and how equipment functions.
“According to developmental theory, school-age children are focused on gaining a sense of control and competence during various activities; therefore, health care providers should strive to keep children informed of what to expect before all medical procedures. Allowing time before procedures for patient questions and additional explanations is recommended. Health care providers also should educate school-age children about self-control strategies, such as helpful postoperative strategies,” including deep breathing exercises, relaxation techniques, and counting to “minimize feelings of anxiety.
” (Hendricks-Ferguson & Nelson, 2003) This is helpful as well to use with adults, again, even in the face of familiar procedures such as transfusions. Quality of life issues The Multicenter Study of Hydroxyurea in Sickle Cell Anemia (MSH) showed that hydroxyurea reduced the incidence of painful episodes and acute chest syndrome by nearly one half over 2. 5 years. Mortality rates were reduced by 40 percent during three-month intervals when patients were taking hydroxyurea (2. 6 versus 1. 5 deaths per three months).
(Wellbery, 2003) For adults, exploring the use of medications such as hydroxyeurea can be helpful in containing the reoccurance of painful episodes, however the risks must be made clear, and also the adult must live in an area where he or she can have access to medical facilities fairly frequently, for regular follow-up. Psychometric implications (such as the development of a scale or questionnaire) The use of a pain scale can be helpful for both children and adults who suffer from the illness, as well as their parents, and can be used to monitor levels of pain over an extended period of time.
(Hendricks-Ferguson & Nelson, 2003) Rural areas Patients in rural areas without regular hospital or health care access may need transportation or at-home care if unable to travel. This is not simply true for the care needed to mitigate the illness or pain, but also for such aspects of care as flu injections, given the sensitivity of patient’s immune systems. (Miller, et. al, 2005) Bibliography : “Diagnosis. ” (2003) National Institute of Health: NIH. Retrieved 4 Dec 2005 at http://www. nhlbi. nih. gov/health/dci/Diseases/Sca/SCA_Diagnosis. html “How is Sickle Cell Anemia Treated? ” (2003) National Institute of Health: NIH.
Retrieved 4 Dec 2005 at http://www. nhlbi. nih. gov/health/dci/Diseases/Sca/SCA_Treatments. html Hendricks-Ferguson, Verna ; Martha A. Nelson. (Jun 2003) “Treatment of Children with Sickle Cell Disease. ” AORN Journal. Retrieved 4 Dec 2005 at http://www. findarticles. com/p/articles/mi_m0FSL/is_6_77/ai_103379524/pg_2 Miller, Martha L. , Caroline A. Obert, Geli Gao, Najat C. Daw, Patricia Flynn, Elaine Tuomanen. (Aug 2005) “Cephalosporin-resistant pneumococci and sickle cell disease. “Emerging Infectious Diseases. Retrieved 4 Dec 2005 at Retrieved 4 Dec 2005 http://www. findarticles.
com/p/articles/mi_m0GVK/is_8_11/ai_n14893784 “What is Sickle Cell Anemia? ” (2003) National Institute of Health: NIH. Retrieved 4 Dec 2005 at http://www. nhlbi. nih. gov/health/dci/Diseases/Sca/SCA_WhatIs. html “What Causes Sickle Cell Anemia? ” (2003) National Institute of Health: NIH. Retrieved 4 Dec 2005 at http://www. nhlbi. nih. gov/health/dci/Diseases/Sca/SCA_Causes. html Wellbery, Caroline (1 Dec 2003) “Hydroxyurea improves mortality rates in sickle cell anemia. “American Family Physician, Retrieved 4 Dec 2005 at Retrieved 4 Dec 2005 at http://www. findarticles. com/p/articles/mi_m3225/is_11_68/ai_111036455