In: Nursing
Sickle cell disease usually begins at early childhood.Most common form is sickle cell anemia.Pain is the most coomon feature in sickle cell crisis.So the top priority for the patient with Sickle cell crisis is pain medications.
1.what is the top priority for patient with sickle cell crisis?
a. Anticoagulant therapy can be used in Sickle cell crisis. But it is not the top management measure. Venous thrombo embolism can be seen in patients with Sickle cell crisis. Warfarin, Heparin can be used to prevent the complication like vaso occlusive crisis in Sickle cell crisis.
b.Pain medications
Pain is the most coomon feature in sickle cell crisis.Normally healthy RBC's are smmoth, disc shaped and flexible. In sickle cell diseases RBC's become curved and stiff. Due to this condition of RBC, it does not easily flow through the blood vessel. This blockage causes severe pain. This pain can occur anywhere in th body such as legs, arms, hip, back ,chest,joints.The pain starts suddenly and may last for few hours or days or even longer.So the top priority for the patient with Sickle cell crisis is pain medications.
c. Vaccinations
Vaccinations directly does not have any role in prevention of Sickle cell crisis. Folic acid supplimentation in early childhood can prevent the sickle cell diseases
d. Fluid restrictions
No restriction of fluids in Sickle cell crisis. Instead extra fluids are recommended routinely.The extra fluids administration aims at slowing or stopping of sickling process and therby reducing pain.
2.What is the overall pathophysiolofy of cerebral palsy ?
Cerebral palsy is a congenital disorder characterised by person's ability to move and maintain the balance.It ocurs due to abnormal development of brain mainly during pregnancy or with 3 years of life.
a.The overall pathophysiology of cerebral palsy is damage that occurs during brain development during pregnancy.The main factors are interuption of oxygen supply to the fetus during pregnancy and birth asphyxia.
b. spinal cord damages and perpheral nerve interruption
About 80% of children with cerebral palsy will develop spinal cord deformity in their life. Spinal cord deformity is mainly due to imbalances of muscles
c. Oxidative stress
Oxidative stress occurs due to lack of antioxidants or imbalnce between free radical activity and antioxidant activity.Lack of antioxidants can be present in cerebral palsy
d. Chromosome mutations
Environmental exposure to certain toxins (eg.cigarette smoke) can cause gene mutations and genetic defects.Some genetic defects cause brain malformations which can lead to cerebral palsy.
3.Patient diagnosed with sickle cell crisis and acute bronchitis. what causes this?
The main cause for this is low oxygen level
a. Low o2 level
Sickle cell disease cause defective Hb, which inhibits the ability of RBC to carry oxygen. his results in lack of oxygen in tissues(hypoxemia).Most of the sickle cell crisis patients develop abnormal pulmonary fuctions. This mainly includes airway obstruction, hypoxemia and restrictive lung disease.In acute bronchitis chest tightness, breathlessness and cough are the main features.In both conditions oxygen levels are low.
b. Injury to the kidney
Damage to kidneys and loss of fluid through urine can be seen as a complication of sickle cell crisis.
c. Increase in hemoglobin
In sickle cell disease less RBCs are present and this results in anemia(low hemoglobin)
d. Increased airway phlegm
Patients with sickle cell disease may frequently develop respiratory complications.Chest syndome in sickle cell patients cause lung injury, breathing difficulty and decreased oxygen in the body.Increased airway phlegm may be present in acute bronchitis.