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Pathophysiology/etiology of
Statistics of the Disease in Saudi Arabia
SCD stands
for “Sickle Cell Disease” it is inherited the genetic conditions which includes
defect of the shapes as well as haemoglobin functions in the blood. In the
blood vessels, it would increase the likelihood of blockages as well as the
disrupted flow of blood that also produces the serious complication
results. Pathophysiology of SCD occurs
where the insufficient oxygen is present in the vascular system, and the
Hemoglobin sickle becomes more soluble which is also increase the formation of
polymer in the overall viscosity of blood. Pathophysiology of SCD always leads
the tactoids information’s which is gel like the form of the haemoglobin which
exists in the equilibrium by the ordinary soluble states. There are is the
specific proportion for each type which also depends on them;
•
Oxygen presence, because the excess amount of oxygen
supports the prevalence of the liquid states.
•
Concentrations of Sickle haemoglobin have more HbS which
supports gel like states (Smith, 2018)
SCD model Pathophysiology has been proposed which includes
the interactions of the hemolysis as well as the endothelium. Therefore the Hb
is safely packed in the membrane of plasma for RBC, and it is also released the
hemolysis, which set off the inflammatory response. The hallmark of the SCD is
the clinical variability as well as the complexity of the given Pathophysiology
for the different pathways and inflammatory involved. Sickle cell is caused
through the mutations of which is the
chain of the haemoglobin molecules. The result of mutations, which is sickle of
the haemoglobin, has a singular property for the polymerizing when it
deoxygenated, and the perfusion of tissue is interrupted through the sickle
cells.
Prevalence /
Statistics of the Disease in Saudi Arabia
In the following figure, a table is presented
which is extracted from a research article written by Alsultan and research
fellows in 2016. In this table, SCD prevalence, recorded cases of Sickle Cell
Disease, the population of each region, and estimated patients below the age of
14 in all regions of Saudi Arabia
According to a research study
conducted by Alsultan and research fellows (2016), Sickle Cell Disease (SCD)
rate is greater in Eastern regions of Saudi Arabia as compared to western,
southern, and central regions. In the eastern region, the estimated total
number of Sickle Cell Disease (SCD) patients are 43,485 among the total
population of region 2999002. Thus, prevalence is around 145 per 10,000 people.
However, the comparatively northern region has 0 prevalence of Sickle Cell
Disease (SCD) per 10,000 employees. From the population of 1790755, even a
single person does not have Sickle Cell Disease.
Signs and symptoms of Statistics of
the Disease in Saudi Arabia
If any
person has the Sickle cell disease, it is present at birth. But in the newborn
baby, they have no problem form the disease until they reach about 5-6 months
of the age. The Sign and the symptoms of the Sickle cell disease will change
person to persons, and it could also change over time. The major symptoms are
the of Sickle Cell disease has direct results for abnormally-shaped of the
sickle red blood cells which also has the blocking of the flow of blood and
which circulates by body tissues. By impaired circulations, the tissues suffer
the damage from the lack of oxygen. There are the important symptoms of Sickle
diseases include;
•
Anaemia and fatigue
•
Bacterial infections
•
Dactylitis
•
In Spleen sudden pooling of blood
•
Heart and Lungs injury
•
Eye damage
•
Aseptic necrosis
•
Leg ulcers
Some symptoms like the pain crisis,
fatigue, anaemia are occurred at any age, and most of the symptoms occur at a
certain age of groups.
Whereas the Dactyltits is the
painful swelling in feet and hands, which is the first symptoms of young
children and babies by Sickle disease; the Child might have been the sickle
disease, noticed that;
•
Increased fussiness
•
Tenderness, swelling
•
Grabbing avoiding holding items
•
Avoiding standing and walking
Diagnostic procedures/tests of
Statistics of the Disease in Saudi Arabia
The Sickle Cell disease is diagnosed
through the examinations of the blood samples. By the test of haemoglobin electrophoresis that
also evaluates the types of haemoglobin ones. The examining of sample blood is
a method to diagnose sickle cell disease. The test used in the diagnoses is
haemoglobin electrophoresis that determines the type of haemoglobin in the
blood of the patient. In case of abnormal sickle shaped cells, the blood is
identified. The blood test checks for haemoglobin S for the defective form of
haemoglobin. If the screening test is negative, there is no sickle cell gene.
If the child is suffering from sickle cell anaemia, then the count of red blood
cell is carried out. In this situation, doctors might suggest an additional
test to check complications of the disease . The genetic counsellor understands
the risk of body. The first investigation to identify sickle cell disease is
listed below,
•
DNA based assays
•
Cellulose acetate electrophoresis
•
Haemoglobin isoelectric focusing (Hb IEF)
•
High-performance liquid chromatography (HPLC) fractionation
•
Chest X-Ray analysis
•
Pulse oximetry
•
Bacterial cultures
•
Plain x-rays of long bones
Medical treatment/management of
Statistics of the Disease in Saudi Arabia
The best method of treatment is a
bone marrow transplant that is also known as stem cell transplant. The process
offers the potential cure for the sickle cell anaemia. This treatment is
reserved for the younger age people of 16 years as the risk of issue increase.
Finding an appropriate donor is difficult and the procedure has serious risk
issues such as death. Treatment for the sickle cell anaemia avoid crises,
prevention of complications, and relieving symptoms. The treatment may include
medications that prevent complication and reduce pain . Another solution is
blood transfusion or bone marrow transplant. The medications that can be used
to treat sickle cell anaemia include,
Antibiotics:
Children
suffering from sickle cell anaemia can start having antibiotic penicillin.
Pain-relieving
medication: The treatment can relieve pain during the sickle cell crisis
and the doctor might prescribe pain medications.
Hydroxyurea
(Droxia, Hydrea)
If hydroxyurea is taken on a daily
basis, it can reduce the frequency of painful crises. It can also reduce the
need for blood transfusions and hospitalizations. The work seems to be done in
stimulating the production of fetal haemoglobin. Use of hydroxyurea can
increase infection risk and if the drug is not supplied it can cause problems
in the prevention of formation of sickle cells. Hydroxyurea can increase risk
of infection and drug usage .
Compare to International Best Practice Guidelines, e.g. NICE / CDC guidelines
Patient Presentation:
Patient information of Statistics of the Disease in Saudi Arabia
HH is a Saudi female patient of 23
years. She is single and not employed therefore she is living with her parents.
The patient is the sister of one brother and two sisters. In the initial
testing, she is a nonsmoker and not have allergies. The patient came to ems on
11/25/19 at 00:20 complaining of severe right upper and lower limb pain. She
had a history of sickle cell disease. Pain medication is taken at home at 21:00
but it was not effective for patient to become better under guarding of pain .
Description of disease/condition
HH
verbalized a pain score of 7/10 using the numeric pain scale. No fever, cough,
chest pain or vomiting. She had no surgical history and past medical issues.
Her vital signs were highly stable as mentioned below,
•
36.3C
•
89 bpm, 18 bpm,
•
119/84
•
100%
EMS attending physician saw the
patient and ordered some blood works BUN/Creatinine/Lytes, CBC with
Differential, Glucose, Random, LDH (Lactate dehydrogenase). HH has a Port A
cath in her right subclavian and was accessed by the EMS nurse. She is on
monthly blood transfusion by haematology and the last one was done three weeks
ago and is due after one week. She was kept in the EMS for 18 hours.
Comparison to a textbook case
of Statistics of the Disease in
Saudi Arabia
The
normal symptoms of sickle cell disease are mentioned below and under these
symptoms, the patient must visit the doctor.
•
fever of 101 F and 38 C
•
chest pain
•
headaches and dizziness
•
extreme tiredness
•
slurred speech
Contrast to a textbook case
of Statistics of the Disease in
Saudi Arabia
The sickle shaped cells are not
flexible, and the shape cannot be changed. The sickle cells last only for 10 to
20 days instead of 90 to 120 days. The body can face trouble in making new
cells and replacing the old ones. The sickle shaped cells can also stick to the
vessel walls and cause a blockage. The lack of oxygen can lead to sudden and severe
pain and the pain is known as crises
Expected prognosis of Statistics of
the Disease in Saudi Arabia
HH
is suffering from sickle cell disease that can reduce life expectancy. With the
proper treatment, quality of life can be improved .
Nursing Assessment of Statistics of the Disease in Saudi Arabia
|
Safe
environment
The environmental factors that can
affect the sickle cell anaemia are listed below, •
Air quality •
Climatic variable,
temperature •
High altitude |
Communication Communication is carried between
patient HH and expert doctor. |
Breathing The patient suffering from sickle cell
anaemia can develop high blood pressure in their lungs that is pulmonary
hypertension. |
|
Eating & drinking eggs, fish, fat-free milk, yoghurt,
chicken, lean meat, cheese, leafy green vegetables, beans or tofu,
calcium-fortified foods such as soymilk, tofu and orange juice
|
Elimination HH was not suffering from any disease
of blood discharge and swelling. |
Washing & dressing During treatment, HH was wearing clean
hospital and good hygiene dress. In the case of washing, the family members
refused nurses from any services.
|
|
Thermoregulation The skin of HH was not dry and warm but
it was smooth at a constant temperature of 36.3. The blood pressure of the
patient was normal and there was no pressure injury except scars.
|
Mobilization HH was having a history of sickle cell
disease that is a disease of the blood. The muscles of the patient became
weak due to lung disease. The patient was not able to have pressure on
anything and she was becoming very weak. She was not even able to squeeze the
hand of the nurse fellow. |
Work & play/social HH is single and not employed therefore
she is living with her parents. HH is the sister of one brother and two
sisters. In the initial testing, she is a nonsmoker and not have allergies.
|
|
Expressing sexuality HH is single.
|
Sleeping HH is suffering from pain and her
sleeping pattern is disturbed. Most of the time she prefers to sleep, and she
sleeps on her right side. |
Dying
/ Pain In the beginning, she was suffering
from pain, she also had a history of sickle cell anaemia.
|
|
Normal findings |
Abnormal findings |
|
normal valine |
Normal valine is replaced by glutamate
at position 6 |
|
Normal surface |
Deoxygenated and have a small patch of
hydrophobic with double white spot |
|
No special chains |
Hydrophobic spots are stick to each
other that result in deoxygenated haemoglobin to produce chains |
|
Simple crystal haemoglobin |
6 beta chain that binds alanine 70 and
leucine 88 |
Nursing Care
Plan
Three nursing diagnosis of
Statistics of the Disease in Saudi Arabia
|
Diagnosis |
Goal |
Implementations |
Evaluation |
|
1 |
Relief
of pain |
Managing
pain •
HH will be subjected
to the description of pain and pain rating for the guide of analgesic agents. •
Elevation and support
of the acutely swollen joint. •
Teach patient
relaxation techniques, a distraction to ease pain, and breathing exercises. •
Implement aggressive
measures that preserve function . |
Relief
from the pain |
|
2
|
Decrease
the incidence of crisis |
Prevention
and management of infection •
Proper monitoring of
symptoms and signs of infection •
Use of antibiotics
promptly •
Teach the patients to
have oral antibiotics and indicate emphasize on the need for antibiotic
therapy. •
Check all signs of
dehydration. |
The
decrease in the incidence of crises in the patient |
|
3
|
Enhancement
in a sense of power and self-esteem |
Promoting
skills •
Development of a
strategy that will enhance pain management for the promotion of a therapeutic
relationship. The process is mainly based on the mutual trust of HH patient
of sickle cell disease. •
Provide HH with other
opportunities to have proper decisions for the daily care of the patient and
to increase the feelings of control. •
The implementation
plan must focus on the strength of the patient and it can deficit to improve
effective treatment . |
Increase
in the sense of power in the patient. |
|
4 |
Complications
absence |
Increasing
knowledge •
If the
doctor-prescribed hydroxyurea as she is a single female it could teach
patients about different situations and how she can participate in the
development of sickle cell crisis. The
patient is single, but the doctor will discuss with her about the current
situation because she is going through childbearing age. The doctors will
tell the patient about the effect of the drug as it can harm unborn
children. •
The proper steps will
be taken to prevent the diminish such crises including stressful situations,
adequate hydration, and keep warm. •
The medical and
paramedical staff will teach the patient about the current state of health.
They can reduce the depression formed on the mind of the patient . |
Reduce
the complications and overcome the issues faced by HH. |
|
5
|
Prevention
of nosocomial infections |
It is important to have proper
management of potential complications that measure additional precautions. |
Reduce
the possibility of infection |
|
6 |
Risk
of fluid volume deficit that is related to hydration |
•
Calculate the
requirement of fluid daily. •
Monitoring fluid
consumption and adjust. •
Observation of signs
dehydration •
Record of output and
intake. |
Optimization
of fluid intake Dehydration
exacerbation crises |
|
7 |
HH
and her family will verbalize the risk factors associated with sickle cell
crises and how it can be minimized |
•
Use NIC intervention to teach the
process of disease and assisting the patient to understand all the
information and process to overcome the issue. •
Review of basic sickle cell disease and
teaching the patient and her family about the symptoms and signs of the
disease. •
Arrangement of genetic counselling and
diagnosis of all family members to measure if they are suffering from the
disease. |
The
knowledge of disease will ensure treatment alignment and adherence to
preventive measures. Questions and concerns will be related
to the knowledge of the disease, preventive skills, monitoring process, and
transmission of disease to others in the family of HH. |
Conclusion of Statistics of the Disease in Saudi Arabia:
The
aim of the present report was to analyze the sickle cell disease and how it can
be prevented by having significant policies.
Summarize of the current condition
of Statistics of the Disease in
Saudi Arabia.
HH is suffering from sickle cell
disease. EMS attending physician saw the patient and ordered some blood works
BUN/Creatinine/Lytes, CBC with Differential, Glucose, Random, LDH (Lactate
dehydrogenase). HH has a Port A cath in her right subclavian and was accessed
by the EMS nurse. She is on monthly blood transfusion by haematology and the
last one was done three weeks ago and is due after one week. She was kept in
EMS for 18 hours .
Patient & family teaching
of Statistics of the Disease in
Saudi Arabia:
The role of the family in overcoming
the disease is very important. In the present situation, HH is a single female.
She is the sister of one brother and two sisters. They will be provided with
the basic information about controlling blood pressure and sugar level of blood
in the healthy diet of the patient. The family members will ensure the
maintenance of healthy weight and food. They will be aware of the consequences
if they will not follow the instructions of doctors. The doctors will be giving
all the information to the family of the patient .
Patient
& family response to teachings of
Statistics of the Disease in Saudi Arabia:
The whole family members of HH were
cooperative and they showed a complete understanding of the disease and how to
prevent it from spreading more in the body of HH. After having educational and
informational sessions with the members of the HH family, I thought them about
the issues and health considerations that must be considered to overcome the
issues .
Expectations after discharge
of Statistics of the Disease in
Saudi Arabia:
HH
will have regular treatment of 6 months at least.
References of Statistics of the Disease in Saudi Arabia
Alsultan,
A., Jastaniah, W., Afghani, S. A., Bagshi, M. H., Nasserullah, Z., Al-Suliman,
A. M., & Alabdulaali, M. K. (2016). Demands and challenges for patients
with sickle-cell disease requiring hematopoietic stem cell transplantation in
Saudi Arabia. Pediatr Transplantation, 20(6), 831-835.
Hemoglobin,
H. (2017). Collecting Samples for
Laboratory Testing. Retrieved from labtestsonline.org: https://labtestsonline.org/tests/sickle-cell-tests
Kidshealth. org. (2019). Sickle Cell Disease. Retrieved from
kidshealth.org: https://kidshealth.org/en/teens/sickle-cell-anemia.html
Maakaron, J. E., Taher, A. T., Yu,
J., & Besa, E. C. (2019). Sickle Cell
Anemia. Retrieved from emedicine.medscape.com: https://emedicine.medscape.com/article/205926-overview#a1
Saraf, S. L., Molokie, R. E.,
Nouraie, M., & Sable, C. A. (2015). Differences in the clinical and
genotypic presentation of sickle cell disease around the world. Paediatr Respir Rev., 15(01), 04-12.
Shiel Jr, W. C. (2019). Sickle Cell Disease (Sickle Cell Anemia).
Retrieved from https://www.medicinenet.com/sickle_cell/article.htm
Smith, Y. (2018, August 23). Sickle-Cell Disease Pathophysiology.
Retrieved from https://www.news-medical.net/health/Sickle-Cell-Disease-Pathophysiology.aspx
Sundd, P., & al, e. (2018). Pathophysiology of Sickle Cell Disease. Annual Review of Pathology Mechanisms of Disease, 14(1), 261–90. Retrieved from https://sci-hub.tw/https://www.ncbi.nlm.nih.gov/pubmed/30332562
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