Management of Patients with Sickle Cell Disease

Sickle cell is a term used to describe the most common inherited blood condition in America. The word sickle comes from the Latin word meaning, a handheld agricultural tool. Sickle cell was first discovered by Dr. James B. Herrick in 1910. Dr. Herrick noted sickle shaped erythrocytes upon microscopic examination of the blood of an African American male. The term sickle cell was coined as a phrase in the early 1920’s. Since these early observations, much has been learned about the origins, genetics, pathophysiology, and management of this disease.

There are about 70,000 people in the United States affected with Sickle cell disease (SCD). The disease mainly affects African Americans. Sickle cell occurs in about 1 out of every 500 African American births. Sickle cell disease also affects Hispanic Americans. The disease occurs in 1 out of every 36,000 Hispanic American births. There are about 2 million Americans that have the sickle cell trait. This occurs in about 1 in 12 African Americans. SCD can result in severe complications which can be prevented or delayed with proper knowledge, understanding and treatment.

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To provide the highest standard of care, nurses caring for people with SCD should understand the pathophysiology of the disease, nursing interventions, and anticipatory guidance strategies for the common complications of sickle cell disease (Jakubik et al. , 2000). All these factors will be addressed in this paper. Definition of Sickle cell disease The sickle cell disease refers to a blood disorder that is inherited by an individual and it affects the red blood cells. Individuals who suffer from the sickle cell disease usually have their red blood cells containing an abnormal type of hemoglobin.

This results to difficulties when the red blood cells due to the sickle-shaped red blood cells are unable to pass adequate amount of blood to reach all the body parts appropriately. This is because the body’s small blood vessels are blocked by the sickle-shaped red blood cells (Quinn et al, 2004). For a body to carry out its functions well, all the body tissues must be supplied with blood. Failure to this, the tissues becomes damaged. This situation results to the sickle cell disease complications. In our body systems, haemoglobin is the red blood cells’ component that is essential in carrying oxygen from the lungs to the body tissues.

Under normal circumstances, the haemoglobin A should be present in the red blood cells. However, other abnormal types of haemoglobin exist. The red blood cells containing the haemoglobin A are soft and round, which makes it easy for the cells to squeeze through the body’s tiny blood cells. The sickle cell disease results from an individual’s red blood cells containing the haemoglobin S. The normal red blood cells live for about 120 days before being replaced, while the haemoglobin S-containing red blood cells live for a very short period(about 16 days). The sickle-shape of the red blood cells decreases the flexibility of the cells.

The sickle cell disease is a lifelong and chronic disease which is characterized by periodic painful attacks together with some health complications in patients. There are different types of the sickle cell disease which include; the Sickle Beta-Zero Thalassemia, Sickle-Haemoglobin C Disease (SC), Sickle Beta-Plus Thalassemia, and the Sickle Cell Anaemia (SS). The sickle cell conditions in individuals are often inherited from the parents, where the type of haemoglobin that an individual inherits from the parents determines the type of haemoglobin made in an individuals red blood cells. Pathophysiology of the Sickle Cell Disease

When it comes to the sickle cell disease pathophysiology, the elasticity of the red blood cells is put into consideration. In an individual’s body, the normal red blood cells show a great level of elasticity. With this elasticity, the red blood cells are able to deform in order to pass through the blood capillaries. The red blood cell sickling is attributed to low oxygen tension, where the cell elasticity is decreased and the cell membrane damaged. When the normal oxygen tension is restored in a person’s body, sickle-shaped red blood cells fail to go back to their normal shape (Quinn et al, 2004).

Ischaemia and vessel occlusion are some of the complications that result from the rigid blood cells’ inability to deform so as to pass through the blood capillaries. Haemolytic anaemia and the sickle haemoglobin properties gives rise to the sickle cell disease symptoms. The deformation of the red blood cells emanates from the formation of rigid polymers by the sickle haemoglobin if there is deoxygenation. The beta globin gene HBB mutations give rise to the sickle cell disease, and the mutation results to the sickling of the haemoglobin. The sickle cell disease can be inherited from the parents in form of an autosomal recessive disorder.

For instance, the risk of a child getting the disease increases when the two parents have the sickle cell gene. The point mutation that occurs in the ? globin chain of haemoglobin results to the replacement of the amino acidic glutamic acid at the sixth position with amino acid valine (less polar). The ? – globin gene is located on the chromosome 11 short arm and therefore, haemoglobin S (HbS) is formed by the association of two mutant ? – globin and two wild type ? globin subunits. A polar amino acid at the ? -globin chain sixth position is absent when the body cells experience low oxygen conditions.

Consequently, the aggregation of haemoglobin, which is also referred to as the non- covalent polymerization of the haemoglobin occurs. It is then that the red blood cells are distorted into a sickle cell shape due to decreased elasticity. Clinical manifestation and the complications of the sickle cell disease Severe cases of sickle cell disease can be attributed to the vaso- occlusive sickle cell crisis. This is one of the major ways through which the sickle cell disease is clinically manifested (Shull, E. , et al. 2007). Vaso- occlusion is a condition that the body experiences when blood flows in the body.

Due to tissue infarction and the intravascular sickling, acute cases of the vaso- occlusive may occur where painful episodes are experienced. The vaso- occlusion in sickle cell disease may result to a variety of clinical situations, an example being the vaso- occlusive crisis (VOC). In children with sickle cell disease, this clinical situation is the most frequent symptom (children below 2 years). Areas of the body that are affected by the vaso- occlusive crisis include the lungs, bones, spleen, brain, abdomen and the penis. Throbbing pain and deep gnawing can be seen in patients.

In addition, swelling and erythema can accompany the pain. Pain crisis in children commonly affects the shoulders, elbows, and femurs. Other areas that are infected include ribs and the iliac crest. The clinical manifestation of the sickle cell disease also involves pulmonary problems such as the acute chest problem and the oxygen saturation. Acute chest problem is characterized by respiration distress as well as some episodes of acute chest pains. As compared with the adults, the children are more susceptible to acute chest syndrome (Yallop et al, 2007).

Acute chest problem can cause deaths in patients, and it results to serious complications. Another major clinical manifestation of the sickle cell disease is priapism. In priapism, males experience a prolonged painful erection. There are two different clinical presentations of priapism. One presentation is whereby shuttering priapism is experienced together with episodes of priapism (multiple). In the second clinical presentation, there is more than two hours prolonged episode. Two forms of priapism exist; low- flow priapism and normal- flow priapism.

The low- flow priapism is more common in adults, where the normal- flow priapism is common in children. In adult patients, priapism results to severe clinical conditions that may be seen in an individual by having restrictive lung disease, cerebral vasculopathy, and end stage renal disease. Cerebral vascular accident has been recorded in children. The sickle cell disease manifestation and complications can be seen in pregnant mothers with the sickle cell disease. This is characterized by vascular stasis, increased metabolic demands, as well as hypercoagulable state.

Some of the fetal complications that may due to the manifestation of the sickle cell disease in pregnancy include low birth weight, pre-term delivery, intra-uterine growth restriction, spontaneous abortion, and increased rate of fetal death in utero. There are various infections that are common in patients with the sickle cell disease. The infections in individuals vary, with some resulting to more severe complications than the others. Some of the infections include;Osteomyelitis,Bacteremia,Meningitis,and Bacterial Pneumonia(Vichinsksy, 2008).

One of the leading causes of death in children with the sickle cell disease is the acute sequestration crisis, where young children who fail to undergo a spleen auto-infarction and have homozygous SS disease are likely to be affected. In older children, the acute sequestration crisis can develop if they have the Sickle-Beta thalassemia syndrome or the haemoglobin SC. This may be characterized by increased reticulocytosis, massive splenomegaly, and decreased hematocrit. The rapid occurrence of cardiac decompensation may occur in children. The liver sequestration may also be seen in patients, where the liver is tender and enlarged.

There is anaemia, hyperbilirubinemia, and reticulocytosis. However, the cardiac compensation does not occur in this case because the liver is less distensible than the spleen. Cerebral vascular event may be another form of manifestation of the sickle cell disease. For instance, some events may result to a stroke. The cerebral vascular accidents lead to very devastating sickle cell complications. The most affected parts of the body due to cerebral vascular accidents are, the anterior cerebral arteries, middle cerebral arteries, and the internal carotid arteries.

Some of the clinical symptoms of children experiencing cerebral vascular accidents include; seizures, gait disturbances, hemiparesis, and aphasia. A stroke that may develop in this condition is a medical emergency. The manifestation of the sickle cell disease is also seen in the growth and development of individuals and in the psychosocial issues (Anie, 2005) . It is also very important to note that, there are other complications of the sickle cell disease in an individual. These complications include; hepatobiliary complications, renal complications, dermatologic complications, bone complications, and retinopathy.

The dermatological complications can be seen in leg ulcers. The first clinical manifestation of the sickle cell disease in an individual is the painful hand –foot syndrome, where a unique painful crisis in feet and hands is experienced. This is referred to as dachylitis. The young children who experience the condition often cry due to the pain, have tender hands and feet, and also fail to bear weight as required. The Medical Management of the Sickle Cell Disease Medication The treatment of the sickle cell disease can be done using oral analgesics and good oral hydration.

This is very essential in the treatment of mild episodes in patients. This is because, the majority of the sickle cell disease painful episodes are mild, and a situation that makes it easy for the episode to be successfully treated at home. For instance, in the treatment of the vaso-occlusive crisis. However,the painful episodes may be severe in patients which requires the patients to be admitted in the hospitals for treatment through vaso-occlusive pain management . Painful crisis in patients needs to be treated by maintaining good tissue oxygenation,analgesics,and intravenous fluids hydration.

For the adolescents and much older children who suffer from the sickle cell disease, Patient Controlled Analgesia (PCA) may be used as treatment. The analgesics standard doses may be effective in treating some patients, while it may fail in others. These doses when administered may lead to adequate pain control when considerable dosage escalations are used. When this is done, an oral dosing schedule can be implemented to manage the sickle cell disease. Dactylitis in children especially those who are under five years of age can also be treated using analgesics and hydration.

In treating priapism, an initial evaluation of the manifestation needs to be done. For treatment, analgesic therapy and hydration can be used. There is need to give oxygen therapy to patients as one way of treating the sickle cell disease. Penile aspirations can be done when prolonged priapism fails to be treated using the conservative therapy. In the past, priapism has been treated using varying degrees of effectiveness using pharmacologic agents. In order to evaluate the best method of treating priapism, evaluation of the patient’s condition will require to be done by the medical experts, pediatric hermatologists, and an urologist.

In treating the cerebral vascular accidents, medical interventions may include a neurological and physical examination. The first step would be to undertake a CT scan. Exchange transfusions can be used to treat acute chest syndrome. The exchange transfusion need to be both prompt and also aggressive (Ballas et al, 2006). In the treatment of the sickle cell disease by handling priapism simple transfusion therapy can be done. The cerebral vascular accidents which are devastating to patients need to be treated by first conducting a complete neurological and physical examination.

For example, a CT scan can be done in order to rule out the intracranial haemorrhage. The diagnosis of stroke can then be followed by an exchange transfusion. The sickling of the red blood cells becomes possible when the haemoglobin S is reduced to less than 30% through a double volume. Due to the decrease of haemoglobin S, the brain’s poor perfused areas have the amount of sickling reduced. The mortality and morbidity of stroke can be affected by the exchange transfusion. Preventing the sickle cell complications can be done effectively using transfusion therapy.

Finding compatible blood may become difficult due to the development of antibodies to the red blood cells antigens. Re-stroking in children can be done through the transfusion therapy. Treating the sickle cell disease by using transfusion therapy is very expensive. The treatment of cerebral vascular accident can also be done through allogeneic bone marrow transplantation. This requires one to have a matching sibling donor, where there is a HLA identical match. This treatment is very effective for patients with care. Education in sickle cell disease treatment

Education is very essential in the treatment of the sickle cell disease. One through which education contributes to the treatment of the disease is by education forums such as workshops. Through the workshops, various issues that relate to the sickle cell disease can be discussed. The forum brings together, social workers, medical experts, physicians, nurses, family members, sickle cell patients, and researchers from the medical fields. The needs of the patients who suffer from the sickle cell disease are addressed comprehensively. This promotes effective treatment of the sickle cell disease.

By looking at the current problems faced by the sickle cell disease patients, the researchers, physicians, and patients can work together to prevent and treat the disease. Pilot and model programs that are good in the treatment can be developed. The formulation of recommendations leads to the solving of problems experienced in the research of the sickle cell disease. Personal experiences from sickle cell patients educate people on how and why they need to treat the sickle cell disease effectively. The recommendations made for Action Plans developed in the education programs aim at addressing the needs of the sickle cell patients.

The community based education programs embrace psychosocial programs and pain management. The barriers that hinder effective care and compliance treatment of the sickle cell disease can be identified, studied, and dealt with through the education program. Educational initiatives improve health care practice patterns, decrease the stigmatization of patients, and promote comprehensive care and family education, as well as counseling. The role of nurses in the treatment and management of the sickle cell disease Nurses in the current medical word, play a vital role in the prevention, treatment and management of the sickle cell disease.

In order to provide the best care to sickle cell patients, the nurses are required to take a multidisciplinary approach in treatment due to the chronic and complex nature of the disease (Platt, 2008). The nurses have the responsibility to coordinate patients care in sickle cell disease treatment which leads to effective treatment and management of the disease in addition, the nurses work very hard to assist patients and their family member s to learn and manage how well the daily needs of patients can be provided for.

Some of the needs that the nurses assist the patients and the family members include; patient education transfusion, chelation therapy compliance, pain management, and the preventive primary care issues. The nurses multidisciplinary approach enables them to make appointments for the patients, ensure that they keep the appointments, make health care follow ups, and encourage the implementation of the recommendations made to them. Another role of the nurses in the treatment and management of the disease is in their participation in providing preventive health care.

Summary Sickle cell disease is a blood disorder and one of the worlds most inherited blood condition. The rate of occurrence of the sickle cell disease differs, with the disorder being more common in African Americans and the Hispanics as compared to the whites. Since it was discovered, a lot of research work has been done to learn more about the disorder. The disorder is manifested in different ways, and the complications being either mild or severe.

The methods of treating and managing the sickle cell diseases also vary depending on the type of complications that an individual suffering from the disease experiences. Education is very vital in the treatment and management of the sickle cell diseases and the resultant complications. The medical practitioners have great responsibility to play in the sickle cell disease treatment and management. The nurses have shown their commitment and dedication to assist patients to effectively manage the disease and the complications that characterize the disease.