Child and adolescent health issues require extra concentration due to the fact that an early disease can lead to an improper growth which may impact the entire life of a person and therefore it is important to ensure that childhood and adolescence diseases are looked after with extreme care in order to improve the quality of life and the growth of the patients. Sickle cell anemia is one of the widely studied subjects today, considering that it can cause acute chronic conditions which include severe pain and organ dysfunctional ties and has a high fatality rate. This disease is also known as sickle cell disorder and is basically a genetic condition which is caused due to hemoglobin disorder, and due to inheritance of mutant hemoglobin genes coming from both the parents. This disease is classified under the group of hemomoglobinopathies mainly known as thalassemia and sickle cell anemia and the intensity can be understood by the fact that approximately 5% of the population in the world is expected to carry the genes that are responsible for hemomoglobinopathies.
One of the major reasons why this study becomes important is that, as of today every year about 300,000 infants are expected to be born with major disorders relating to hemoglobin, and almost 66% of these cases are dealing with sickle cell anemia. Another important factor which makes this disease important as a study is that there are millions of healthy carriers of this disease who only have inherited one mutant gene from their parent, and therefore in case if their partner also carries the other gene there is a strong a possibility of the newborn being affected with the sickle cell disease.
Considering the high mortality rate it is important that the significant symptoms of sickle cell anemia are studied and observed. One of the major issues relating to it is that, the importance of it being assessed against the yardsticks of infant, is important to manage the mortality rate which can be one in five if not diagnosed at the right time. With various interventions and disease awareness the survival rate above five years has definitely increased in the past few years, but there's still is a huge chance of premature deaths as even after surviving with that age, the risk related to the disease remains the same. According to the World Health
It is a common issue that most of the people were suffering from sickle cell anemia suffer with chronic anemia, where the concentration of hemoglobin in the blood can drop down to 8 g per DL .. The Thalassemia Society of Australia was formed in the year 1979 in order to provide awareness and control of the disease and all the number of patients has risen by more than 13%, it is still being controlled through various measures. There is a reason website which was launched ‘http://www.thalnsw.org.au’, in order to support awareness of the disease and the latest treatments and hospitals that are working on further improvement in the quality of the life of patients and other areas of management of the disease.
Children between 6 to 18 months are often seen to have painful swelling in hands or feet due to the Hanford syndrome which is caused by sickle cell disease. The children will survive after five years of a very difficult phase that they have to face due to the acute pain caused by various conditions like acute chest syndrome, pneumonia, priapism, bone or joint necklaces or renal failure and the mortality after this age is mostly caused due to unpredictable painful crisis condition which may lead to a sudden hospitalization and in uncontrollable situations leads to death.Administration of penicillin during the childhood and taking precautions like avoiding excessive heat and cold to prevent dehydration and to be kept in close observation of a specialist centre dealing with chronic sickle cell conditions.
The reason behind the importance which is given to neonatal screening of sickle cell disease is that all the above mentioned precautions can only be effective in case if they are taken during the first few months of the child's birth. There are few patients who suffer with severe conditions in which the general precautions and interventions are not applicable, and in that case blood transfusions and iron-chelating therapy are commonly taken actions. In areas where secure disease is commonly found, it is important to prepare special care centers so that the prevention and awareness program can be run effectively.
Sickle cell anemia is a genetic disease and it was the first of its kind which was initially characterized at the molecular level. The effectiveness of the sickle cell DNA can be understood by the fact that it is so small that it's like one nucleotide of DNA in almost 3,000,000 human cells, however still it is able to alter the chemical properties of the protein complex, Iron and hemoglobin which carry the oxygen within red blood cells. .The disease is associated with acute pain and chronic conditions which may prove fatal at any point of time in life, although there are various interventions available in order to increase the life of the patients and improve the quality, the average life that a patient suffering from sickle cell disease is expected to be between 20 to 40 years.
It becomes important that the nurses are aware of the basic symptoms of sickle cell disease and they must be aware of the right method to refer the children to the special care centers which can provide comprehensive care to the children during the early stages, including the interventions and should be able to provide requisite awareness to the parents so that they are also able to understand and cater to the requirements of their children. There are various researches that are under process at the moment and in case if they reveal good results in future it can be expected that the rate at which children are tracked this disease from their parents can be decreased drastically.
Sickle Cell Anemia: Nursing Perspective
For nurses pain is the most discussed part of sickle cell disease and sickle cell anemia, this is one area where major care is taken at the level of pain is extreme and is very difficult to handle as considering the average age of the patients they are unable to bear it for too long. The most common areas that suffer from sickle disease pain in the abdomen, the back or the chest and the pain may appear in four different phases and is impacted by various other factors which will be reviewed from the literature. The type of pain is heterogeneous and the severity, duration, location, frequency and character are different for different patients, hence the interventions that have to be applicable also have a very wide range. The pain may be refused, migratory, may involve several areas or may just be localized and the intensity of it varies from mild to extreme and is usually depicted to be more than the pain caused post-operative procedures. There are various side effects of the sickle cell disease is well at due to the various chronic conditions that the patient says it is common to develop low self-esteem, dissatisfaction, depression, anxiety, cool and social isolation and decreased participation in daily activities of life, there are patients who even report poor relationship with the family.
Conclusion
The most important point and inference that can be drawn from the essay is related to the awareness of this disease; as inferred from the various discussions that this disease primarily affects patients since birth and most of the children remained unscreened due to lack of information and awareness in parents and nurses. Moreover, there are lot of misunderstandings related to the diagnosis due to which there is a delay in screening the patients at the right time and the same results in adverse clinical situations. It becomes important that the nurses are aware of the basic symptoms of sickle cell disease and they must be aware of the right method to refer the children to the special care centers which can provide comprehensive care to the children during the early stages, including the interventions and should be able to provide requisite awareness to the parents so that they are also able to understand and cater to the requirements of their children. There are various researches which are under process at the moment and in case if they reveal good results in future it can be expected that the rate at which children are tracked this disease from their parents can be decreased drastically. Another important factor which came out of the review of literature is that the quality of life of the patients is greatly impacted by the kind of nutrition and interventions available to them, hence it is required that the patients are made aware of the basic treatment programs related to sickle cell patients and the conditions that they may face in future so that they may be aware of the same and can handle it. Depression and emotional breakdown was also one of the primary complications that were the result of sickle cell diseases, hence a regular and effective counseling should also be provided to the patients so that they may lead a positive life.
REFERENCES
Creswell, J. (2009) Research design: Qualitative, quantitative, and mixed methods approaches, 3 edn, Los Angeles: Sage Publications.
Frankel, R., Naslund, D. and Bolumole, Y. (2005) 'The white space of logistics research: A look at the role of methods usage', Journal of Business logistics, pp. 185-208.
Green, C., Anderson, K.O., Baker, T.A., Campbell, L.C., Decker, S., Fillingim, R.B. and Kaloukalani, D.A. (2003) 'The Unequal Burden of Pain: Confronting Racial and Ethnic Disparities in Pain', American Academy of Pain Medicine Vol.4 No.3, p. 277–294.
Kalpan, B. and Maxwell, J. (2005) ''Qualitative Research Methods for Evaluating Computer Information Systems'', Evaluating the Organizational Impact of Healthcare Information Systems, pp. 30-55.
Lavin, J., Sim, M. and Gill, V. (2010) 'Managing sickle cell disease', Nursing Made Incredibly Easy Vol.8 No.6, November, pp. 24-33.
Lenfant, C. (2002) The management of sickle cell disease, New York: National Heart, Lung and Blood Institute Division of Blood Diseases and Resources.
Leticia, S.A., Myla, E. and Olivia, T.M. (2011) 'Changes in Platelet Count as a Predictive Tool in Sickle Cell Acute Vaso-Occlusive Crises: A Pediatric Study.', Journal of Clinical Pediatrics , pp. 657-661.
Maxwell, J. (2005) Qualitative research design: An interactive approach, New York: Sage Publications, Inc.
Rees, D., Williams, T. and Gladwin, M. (2010 ) 'Sickle-cell disease', Journal of Paediatric Haematology, pp. 2018-2031.
Yin, R. (2009) Case study research: Design and methods, 4 edn, vol. 5, Sage Publications.
Yontz, C.J. and Waller, K.V. (2011) 'Homozygous Sickle Cell Anemia and Secondary Complications: A Case Study', Journal of Clinical Labortary science Vol.24 No.2, pp. 79-84.
Zack-Williams, D. (2007) 'Sickle Cell Anemia in pregnancy and the neonates: ethical issues', British Journal of Midwifery Vol.15 No.4, pp. 205-209.
