Acute Managemnt Of Sickle Cell Crisis And Hypertensive Emergency And Urgency

Question 1

What is SCD and the pathology of SCD

What is the spectrum of SCD

Answer 1

A group of haemoglobin disorders from inheritance of sickle-β-globin gene
The amino acid changed becomes Valine and no more glutamate

➢Hb S gives out oxygen easily
➢Easy breakdown of red blood cells
Red cell rigidity
➢Adhesion to the endothelium
➢haemolysis

Spectrum:
Homozygosity -SS
Heterozygosity-AS(Trait)
Double heterozygote -SC

Question 2

State ten complications of SCD

Answer 2

Vasooclusive crisis including hand-foot syndrome in children
•Hyperhaemolytic crisis
•Acute chest syndrome
•Aplastic crisis
•Sequestration (splenic, liver)
Chronic
•Haemolysis
•Jaundice
•Gallstones
•Anaemia
•Avascular necrosis of the femur head
Other
•Organ thrombosis (e.g mesenteric occlusion, CVA)
•Bone marrow necrosis ± Osteomyelitis
•Chronic ulcers of the lower leg
•Proliferative retinopathy
•Priapism
•Nocturnal enuresis
•Infections
•Growth retardation

Question 3

What will be abnormal in the FBC ,what will you see in sickling test of SCD,
In Hb electrophoresis what will you see
On peripheral blood film what will you see

Answer 3

FBC-low haemoglobin

Sickling test-positive

Hb Electrophoresis
Hb A, S, C, F, D etc: In children with normocytic hemolytic anemia, if results of electrophoresis show only HbS with an HbF concentration of less than 30%, the diagnosis is sickle cell anemia. If HbS and HbC are present in roughly equal amounts, the diagnosis is HbSC disease.
In children with microcytic hemolytic anemia, order quantitative Hb A2 in addition to electrophoresis. If HbS is predominant, Hb F is less than 30% and Hb A2 is elevated, a diagnosis of HbS–beta-0 thalassemia can be inferred. If possible, perform a study of the parents. If the HbA2 level is normal, consider the possibility of concomitant HbSS and iron deficiency. If HbS is greater than A and HbA2 is elevated, a diagnosis of HbS–beta+ thalassemia can be inferred. If HbS and HbC are present in equal amounts, the diagnosis is HbSC disease.
A homozygous patient will have hemoglobin SS (HbSS, 80-90%), hemoglobin F (HbF, 2-20%), and hemoglobin A2 (HbA2, 2-4%). A carrier patient will have HbSS (35-40%) and hemoglobin A (HbA, 60-65%). The test is not accurate in a patient who has recently received blood transfusions

Peripheral blood film:
Sickle cell
Target cell: The target cell is a bell-shaped cell with a relative excess of membrane
Howell-Jolly bodies: basophilic nuclear remnants (clusters of DNA) in circulating erythrocytes.

Question 4

Case Presentation
•24y male, sickle cell disease (SS genotype), 16y
•Generalised pain (7/10): arms, legs, lower back
•No cough, no pain on micturition, no cola-like urine, no vomiting
•No other symptoms
•Similar presentation a month ago
•T – 36.2oC, RR-24cpm

Give differentials

Answer 4

VOC: The sickle cell crisis, also called acute pain crisis or vaso-occlusive crisis, is the most common reason that people with SCD go to the hospital. Episodes are sudden and unpredictable and may be triggered by different unknown risk factors

Question 5

Name three things that precipitate VOC
What investigations will you do for a patient w VOC
What fluids will you give
What medications will you give

If after 12 hours this patient is still in pain
What will you do

Answer 5

Vaso-occlusive crisis

Precipitants
•Dehydration
•Infection
•Extremes of temperature

Vaso-occlusive crisis
•Management
Admit don’t detain
Avoid discharge of an SCD patient within 24 hours 
•Investigations
•Full Blood Count, Urine R/E, Culture, FBC for MPs,
•Targeted lab for the cause
•IV Fluids: 1.5X Maintenance
•Normal Saline
•Dextrose Saline
•Medications
•Pain control: Paracetamol, NSAID’s, Morphine
•Oxygen
•Avoid hypoventilation
•± Antibiotics

12 hours after admission
•Still in severe pain
•O2 sat – 75%, RR -  42cpm
•Cough, bilateral coarse crackles
•High flow O2 by non-rebreather mask given
•After an hour, Sat – 70%
•Patient sent to ICU
•Prone, CPAP started

Question 6

What are the causes of acute chest syndrome

How is acute chest syndrome managed

Answer 6

Causes of ACS
•Infection
•Thrombo-embolism
•Atelectasis

It’s managed as a medical emergency
Put the patient in prone position
Early oxygenation
Analgesia

If no improvement,:

1. exchange transfusion
2. Give nitric oxide
3. Do mechanical ventilation

Antibiotics
Low Molecular Weight Heparin (LMWH

Transfusion with packed cells and furosemide
•RR – 30cpm, still on high flow O2
•24hrs later
•Spleen size from 8cm to 20cm
•Malt-coloured urine
•Hb drop from 8g/dl to 3g/dl

Question 7

How will you manage hyperhaemolytic crisis or sequestration crisis

Answer 7

• Keep high flow O2
• Serial urine collection
• Serial measurement of spleen size
• Transfuse: be careful
• Choose packed cells
• Hydrate
• Investigation: Ultrasound scan

Stay at ICU one more week
•Became stable
•Transferred to Male Medical Ward

Question 8

How does aplastic crisis present ,what precipitates it,what are you managing in this crisis

Answer 8

Presentation:

Pancytopenia-A condition in which there is a lower-than-normal number of red and white blood cells and platelets in the blood. Pancytopenia occurs when there is a problem with the blood-forming stem cells in the bone marrow.

Precipitated by:

Infection: Parvovirus B19

Anaemia is what you’re managing

Question 9

For chronic management,what will be your counsel

How will you manage this case chronically:

Answer 9

Counsel them to get regular reviews
Avoid dehydration,anoxia,infections,extremes of temperature
Good hygiene and nutrition
Pregnancy

Management:
Folic acid supplementation
Penicillin prophylaxis for children less than 5 years
Pain control
Vaccination against pneumococcus,H influenzae,meningococcal,Hep B
Prophylactic transfusion (to be avoided if necessary)
Malaria prevention
Hydroxyurea
Stem cell transplantation

Question 10

What is hypertension
Name the types and define them

Single Most Important preventable cause of premature death in developed countries
•2010 Health Survey for England
•32% for males
•27% of females
True or false 
What is the rule of halves

Answer 10

Definition
•It is the level of blood pressure which places an individual at an increased risk of cardiovascular events and, when treated, results in more benefit than harm

• Essential Hypertension
• Heterogenous genetic and environmental condition
• Secondary Hypertension
• Increased blood pressure due to an underlying disorder
• Accounts 10 – 75% of cases

True

Rule of halves
•½ of those with increased Blood pressures have not been diagnosed
•½ of those who have been diagnosed are not on treatment
•½ of those receiving treatment do not have adequate control

Question 11

What can increased blood pressure cause

Answer 11

Loss of auto regulation leading to endothelial damage leading to microangiopathic haemolytic anemia ,platelet and fibrin deposition
Ending in ischaemia

The increase can cause pressure natriuresis leading to sodium and volume depletion which leads to RAS activation leading to increased local and systemic AII (this can also cause increased blood pressure ) causing increased sympathetic activity leading to increased blood pressure

Question 12

What is the ESC = European Society of Cardiology; ESH = European Society of Hypertension. Classification of optimal,normal,high normal,grade 1-3 hypertension,isolated systolic hypertension
Under ambulatory blood pressure monitoring what are the normal and abnormal values for daytime,night time and 24 hours

Answer 12

Optimal: systolic <120 And diastolic <80

Normal: systolic <130 And / or diastolic 80 – 84

High Normal: systolic 130 – 139 And / or
85 – 89

Grade 1 Hypertension (mild):
140 – 159
And / or
90 – 99

Grade 2 Hypertension (moderate)
160 – 179
And / or
100 – 109

Grade 3 Hypertension (severe)
>180
And / or
>110

Isolated Systolic Hypertension
>140
And / or
<90

DAYTIME
Normal: < 135/85
Abnormal: >140/90

NIGHT-TIME
<120/85
>125/75

24 HOURS
<130/80
>135/85

Question 13

What is the effect of hypertension on the body (name five)

Name four fundamental questions to consider during clinical assessment

Answer 13

Kidney disease
Erectile dysfunction
Heart failure
Stroke
Vision loss
Heart attack
Arteriosclerosis 
Brittle bones
Hypertensive encephalopathy 
Elevated sugar levels 

Is this sustained Hypertension?

2. Is this Primary or Secondary Hypertension?
3. Are there other cardiovascular risk factors?
4. Is there target organ disease??

Question 14

Under clinical assessment history and physical exam what things should you consider
Name some secondary causes of hypertension

Answer 14

Clinical Assessment (History and Examination)
•Duration of elevated Bp
•Previous drug treatment and side effects
•Contraindication to specific drugs
•Family history
•Previous history of pre-eclampsia or hypertension in pregnancy

Secondary Cause???

1. Young Age
2. Sudden – onset hypertension
3. Presenting as Malignant Hypertension
4. Acute Rise with previously stable BP control
5. Severe or ‘resistant’ hypertension

Question 15

What’s the difference between hypertensive emergency and urgency

Answer 15

Hypertensive Emergency
•Severe increased blood pressure
•Acute or progressive organ dysfunction
•Cardiac ischaemia
•Encephalopathy
•Stroke
•Pulmonary Oedema
•Renal Failure

Hypertensive Urgency
•Severe increase in blood pressure

•WITHOUT EVIDENCE OF ACUTE OR PROGRESSIVE TARGET ORGAN DYSFUNCTION

Question 16

In malignant hypertension what signs are seen

What kind of prognosis is there?

Answer 16

Malignant Hypertension
•Increased BP
•SBP -  150 – 290mmHg
•DBP – 100 – 180mmHg
•Progressive target organ damage
•Papilloedema
•Pathologically
•Arteriolar fibrinoid necrosis
•Poor Prognosis
•1-year mortality about 90%

Question 17

Under accelerated hypertension and resistant hypertension what signs are seen

Answer 17

Accelerated Hypertension
•Increased Blood pressure
•Retinal haemorrhage
•Without Papilloedema
•Not Important these days

Resistant Hypertension
•Failure to reach target Bp despite multiple antihypertensive agents including a diuretic (at least 3 antihypertensives at a maximum dose)

Question 18

How do you assess emergency and urgency clinically (name five signs) and using blood pressures

Answer 18

Assessing Emergency and Urgency
•How does the Bp compare to previous readings?
•>160/100 mmHg sufficient to cause problems in a patient with previously normal bp
•A patient with long standing hypertension may tolerate higher bps

Clinical Assessment
•Headache
•Dizziness
•Neurological deficit
•Visual disturbances
•Breathlessness
•Chest pain
•Leg pain

Question 19

In assessing emergency and urgency if there’s evidence of target organ damage how will you manage the person

In assessing emergency and urgency what do you find out concerning the drugs the person has been on

Answer 19

Is there evidence of Target Organ Damage??
•MANAGE AS AN EMERGENCY UNTIL PROVEN OTHERWISE
•Neurological
•Left Ventricular Failure
•Acute Kidney Injury
•Myocardial Infarction (GET AN ECG)

What drug has the patient been on until now??
•CONSULT
•Continue current medication
•Add further treatment as appropriate
•Make modification
•Check adherence to medication

Question 20

What investigations are done in assessing emergency and urgency

Answer 20

Investigations
•Urinalysis:
Proteinuria
Hematuria

•Blood Urea Electrolytes, Creatinine (RFT):
Creatinine
Potassium
•FBC

ECG:
•MI
•LVH
•Ischaemia

•Chest X-ray:
Pulmonary Oedema
Cardiomegaly

• Echocardiogram
• CT Brain

Question 21

How will you manage hypertensive emergency

Answer 21

Management (Hypertensive Emergency)
•Treat in a High Dependency environment
•Continuous BP monitoring
•There may be volume depletion
•Resuscitate with Normal Saline (0.9%)
•Initial aim of treatment
•Decrease DBP to 110 – 115mmHg (Aim to achieve this in 2-6hours)
•Rapid fall may            clinical consequences with decrease cerebral, spinal cord or myocardial perfusion or AKI

Management (Hypertensive Emergency)
•Parenteral options are often required
•Ideally in an infusion
•Drugs
•Sodium Nitroprusside
•Drug of Choice
•0.5ug/kg/min every 5minutes until adequate response
•Labetalol (combined α- and ß-blocker)
•Particularly in IHD and Aortic Dissection
Labetalol is also used in pre eclampsia 
•IV 20mg initially over 1min
•Followed by infusion 0.5 – 2mg/min
•Safe in pregnancy

Management (Hypertensive Emergency)
•Drugs
•GTN
•2 – 10mg/h
•Useful in Symptomatic ACS and Acute LVF
•Caution in volume deletion
•Tachyphylaxis after 24-48 hrs
•Once Bp within target range, transfer to oral agent, and wean IV infusion

Question 22

How will you manage hypertensive urgency

Name some lifestyle interventions for BP reduction as a management of hypertension

Answer 22

Management (Hypertensive Urgency)
•If there is no Target Organ Damage, it does not necessarily require hospital admission
•Repeat Bp after 1-2 hours to confirm
•Oral agents preferred
•Aim for a Diastolic Bp 100 – 110mmHg at first. Recheck Bp after 24 – 48 hrs
•Treat according to laid down Protocol (JNC 8 guideline/BHS guideline)

BHs guidelines:
Step 1:If person is less than 55 give ACE inhibitors or low cost ARB (angiotensin II receptor blockers)
If person is less than 55 years or black patients of any age give calcium channel blocker
Step 2: give both groups ACE inhibitors or low cost ARB (angiotensin II receptor blockers) and calcium channel blocker
Step 3: give both groups ACE inhibitors or low cost ARB (angiotensin II receptor blockers) and calcium channel blocker and thiazide like diuretics
Step 4: in resistant hypertension give both groups ACE inhibitors or low cost ARB (angiotensin II receptor blockers) and calcium channel blocker and thiazide like diuretics and consider future diuretics or alpha blockers or beta blockers
Consider specialist advice

Intervention:
Weight reduction
Diet(consume diet rich in fruit and veggies,low fat diary products )
Dietary sodium restriction
Physical activity
Alcohol : males less than 21 units a week
Females: less than 14 units a week
No smoking
Reduce total fat intake
Increase consumption of oily fish
Replace dietary saturated fats with monounsaturated family