Sickle cell disease

Sickle cell disease ppt

1. SICKLE CELL DISEASE
&
ITS COMPLICATIONS
(COMPLETE INFO. PPT)
Dr. Armaan Singh

2. CASE
A 20 years old male reported with history of
delayed puberty, decreased growth, severe joint
pain, severe weakness and cough. He also have
defective vision
Past history reveals episodes of jaundice, severe
body aches and pains, and gall stones
In his childhood he used to have frequently fever
swelling of the hands and feet and pain in the
chest, abdomen, limbs, and joints and nosebleeds
and frequent upper respiratory infections
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3. CASE
O/E; Decreased growth, delayed signs of puberty
severely anemic, mildly jaundiced, ulcers on right leg,
inflamed gums
Enlarged spleen.
Temp 39.1°C, diaphoretic, and uncomfortable.
HR of 90, BP 116/84 mm Hg, RR 26
O2 Sat 89% and improved to 94% with 6 L/min
via face mask.
Family history: similar problem in one of his cousin
who died at the age of 30, who used to receive blood
transfusions
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4. CASE
WBC of 17 500/μL
62% neutrophils
25% lymphocytes
9% monocytes
2% eosinophils
1% basophils
1% atypical lymphocytes.
Hb was 8 g/dL
reticulocyte 25%
platelet 206 000/μL.
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5. CASE
Which lab test you will advise?
What is most probable cause of patients problem?
What complications the patient may have?
Design therapeutic objectives for this patient?
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7. HEMOGLOBIN: INTRODUCTION
Normal: alpha gene at Chr 16, beta at Chr.11
HbA: 2 Alfa + 2 beta97-98%
Hb A2:2 Alfa + 2 delta 2-3%
Hb F;2 alfa + 2 gamma >1%
Hb S:Glutamic acid at 6 in beta chain replaced with Valine
HbC:
……………………………………………………………………….Lysine
Thalassemia:
Thalassemia describes a group of inherited disorders
characterized by reduced or absent amounts of hemoglobin
Alfa: less alfa chain Chr.16
Beta: Chr.11: less beta, beta thalassemia minor
or no beta, all alfa chain beta thalassemia major :
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8. An autosomal recessive genetic disease of Hb synthesis
Result of a single–amino acid substitution in the β-
globin chain of the Hb molecule, valine for glutamate
at position 6
Sickle cell trait: Pt. with hetrozygous genotype
Epidemiology in KSA:
“The prevalence of SCD in Saudi Arabia varies significantly
in different parts of the country, with the highest prevalence
is in the Eastern province, followed by the southwestern
provinces. The reported prevalence for sickle-cell trait
ranges from 2% to 27%, and up to 2.6% will have SCD in
some areas”

Ann Saudi Med. 2011 May-Jun; 31(3): 289–293.
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SICKLE CELL DISEASE

9. COMPARISON WITH USA
African Americans:
SCD: 0.3% Saudia: 2.6%
SCT: 8.0% Saudia: 2-27%
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11. f
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14. PATHOPHYSIOLOGY
Normal Hemoglobin A: two alpha and two beta chains, 96-97%
Glutamic acid is on the 6th position of the Beta chain
Hemoglobin S: Chr.11
Due to a one point mutation, glutamic acid is replaced by valine at
position 6 in beta chain
HbS: during deoxygenation
Polymerize
Crystellize
in RBC’s…………………….leading to………………….. Sickling of Cells:
RBC cell membrane changes: activate coagulation pathways
Rate of polymerization and sickling augmented by:
Hypoxia, deoxygenation
Infections,
Acidosis,
Physical exercise,
Vasoocclusion due to cold as well as hypertonic dehydration
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15. CLINICAL PRESENTATION
Sickle cell trait (SCT) Carrier, recessive
Rare painless hematuria; normal Hgb level; heavy exercise
under extreme conditions may provoke gross hematuria and
complications
Sickle cell anemia (SCA)
Anemia
Chronic hemolytic anemia: jaundice, gall stone,
splenomegaly
Acute Pain crises,
Microvascular disruption of organs (spleen, liver, bone
marrow, kidney, brain, and lung), gallstone, priapism, leg
ulcers, anemia (Hgb 7-10 g/dL)
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16. CLINICAL PRESENTATION
Sickle cell hemoglobin C:
Painless hematuria
Aseptic necrosis of bone: less common
Vaso-occlusive crises less common, occur late in life
Pregnancy-related problems; mild anemia (Hb 10–12 g/dL)
Sickle cell β-thalassemia
Rare crises; milder severity than sickle cell disease because of
production of HbA;
Hb 10–14 g/dL with micro-cytosis
Sickle cell Alfa-thalassemia or β0 Thalassemia
No HbA production; severity similar to sickle cell anemia; Hb
7–10 g/dL with microcytosis
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17. DIAGNOSIS
Laboratory findings
RBC’s: 5-50 % sickled
Low hemoglobin; 7-10%; HbA; 0%; HbS 85-98%
Increased reticulocytes: 10-25%, platelet, and
leukocyte counts; and sickle forms on the peripheral
smear
Routine neonatal screening programs: DNA from
fetal cell for mutation
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18. GOALS OF THERAPY
To reduce
Hospitalizations,
Complications,
Mortality
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19. TREATMENT
GENERAL PRINCIPLES
No Treatment for the primary disease
Lifelong multidisciplinary care
general measures,
preventive strategies,
treatment of complications and acute crises.
Routine immunizations plus influenza, meningococcal,
and pneumococcal vaccinations.
Prophylactic penicillin for children with sickle cell
disease until they are 5 years old.
Penicillin V potassium, 125 mg orallytwice daily until 3 years of age
and then 250 mg twice daily,
Benzathine penicillin, 600,000 units intramuscularly every 4 weeks.
Folic acid, 1 mg daily, is recommended in adult
patients, pregnant women, and patients of all ages
with chronic hemolysis.
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20. FETAL HEMOGLOBIN STIMULATORS AND
OTHER STRATEGIES
Hydroxyurea, a chemotherapeutic agent
Stimulate HbF by stimulating erythropoiesis
In patients with frequent painful episodes, severe symptomatic anemia, acute
chest syndrome, or other severe vasoocclusive complications.
Butyrate and 5-aza-2-deoxycytidine.
Chronic transfusion every 3 to 4 weeks The optimal duration is
unknown
to prevent stroke and stroke recurrence in children.
Maintain HbS of less than 30% of total hemoglobin..
Risks include, hyperviscosity, viral transmission (requiring hepatitis A and B
vaccination), volume and iron overload, and transfusion reactions.
Allogeneic hematopoietic stem cell transplantation
The only therapy that is curative.
Best candidates are
younger than 16 years of age,
With severe complications,
Have HLA-matched donors.
Risks: mortality, graft rejection, and secondary malignancies
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21. STEM CELLS IN THE TREATMENT OF
SCD
Skin stem cells cure mice of sickle cell
anemia
Success is proof that technique has
potential to cure disease
http://www.msnbc.msn.com/id/22136029/
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22. COMPLICATIONS
Acute Chest Syndrome
Septicemia
Stroke or CVA
Acute splenic sequestration crisis (ASSC)
Aplastic Crisis
VasoOcclusive pain: Sickle cell crisis
Severe pain is an emergency called acute sickle
cell crisis
Osteomyelitis
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23. SICKLE CELL CRISIS
Rapid diagnosis and treatment are necessary to
minimize morbidity and mortality.
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24. CASE 1
A 16-year-old boy with a history of SCD
presented to the ED with a 3-day history
of fever, cough, and SOB.
Five days prior, he had been evaluated
and treated for severe pain in his legs and
arms.
He complained of persistent and
worsening pain in both his lower
extremities and pain in his chest, in spite
of oral narcotic therapy.
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25. His medical history included multiple, vasoocclusive,
painful crises, including an episode of priapism, and he
had received multiple blood transfusions over his
lifetime.
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Case 1

26. CASE -1
On examination
Temp 39.1°C, diaphoretic, and uncomfortable.
HR of 80, BP 116/84 mm Hg, RR 26
O2 Sat 89% and improved to 94% with 6 L/min
via face mask.
Conjunctivae were icteric
Mucous membranes were moist
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27. Cardiovascular II/VI systolic ejection
murmur.
labored respiration with suprasternal and
intercostal retractions.
decreased breath sounds in the right
midzone and lower zone, and scattered
crepitations on the right side.
no lower extremity edema
Abdominal examination Normal
CNS Normal
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Case 1

28. WBC of 17 500/μL
62% neutrophils
25% lymphocytes
9% monocytes
2% eosinophils
1% basophils
1% atypical lymphocytes.
Hb was 8 g/dL
reticulocyte 25%
platelet 206 000/μL.
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ABG on room air
•PO2 59 mm Hg
•PCO2 29 mm Hg
•pH 7.32
•HCO3 13 mmol
A chest x-ray right lower-lobe consolidation
with a moderate right pleural effusion.
Case 1

29. In the ED, he received
antipyretics
supplemental oxygen
cefotaxime 2 g IV
packed red blood cell transfusion was initiated after 20 mL/kg of
normal saline was infused
Over the next hour, while waiting for a bed to become
available in the intensive care unit, the nurse noticed
that the patient's oxygen saturation continued to
worsen, and he was hypoxic even on supplemental
oxygen of 12 L/min via nonrebreather mask.
He underwent emergency intubation
A diagnostic pleural tap was performed which
demonstrated an exudative fluid.
The resulting Gram stain and culture were negative.
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Case-1

30. CASE 1
What is it
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31. 1.ACUTE CHEST SYNDROME
1 of the most serious and life-
threatening complications of SCD
Leading cause of mortality and
morbidity in affected patients, since the
impact of more effective antimicrobials
and the pneumococcal vaccine
Caused by a vasoocclusive crisis
involving the pulmonary vasculature.
Not distinguishable from pneumonia
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32. New infiltrate on chest radiograph in combination
with at least 1 clinical sign or symptom
Chest pain
Cough
Wheezing
Tachypnea
Fever
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•Fever and cough are the most common in
children
•chest pain, sob, and chills are common in
adults.
1.Acute Chest Syndrome
Dx

33. Common causes
Pulmonary infection:
Mycoplasma pneumoniae more
commonly associated with acute chest
syndrome
Thromboemboli
Fat emboli
Rib infarction
Infection and fat emboli were the most common
identifiable causes.
Vichinsky EP, Neumayr LD, Earles AN, et al. Causesand outcomes of the acute chest syndrome in sickle cell disease.
National Acute Chest Syndrome Study Group [published erratum appears in N Engl J Med 2000; 343:824]. N Engl J Med
2000;342:1855–65.
1.Acute Chest Syndrome
Possible causes
Iatrogenic:
excessive hydration
or
narcotic use
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33

34. Therapeutic Modalities
Supportive measures
 Oxygen for hypoxia
 Appropriate hydration
 Appropriate pain control
Antibiotics: third-generation cephalosporin +
macrolides
Transfusion therapy:
Reports of dramatic improvement in clinical condition
after initiation of transfusion
Simple transfusion
Exchange transfusion
Experimental therapy
Nitric oxide
Corticosteroids
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1.Acute Chest Syndrome
Bodo I, Khoury H, Blinder M. Rapid resolution of the acute chest syndrome of sickle cell disease after automated red cell exchange.
Blood 1997;90 Suppl 1:23b

35. 2.SEPTICEMIA
SCD pts have impaired immunologic function that is caused
by splenic dysfunction.
Impairment of splenic function can occur in infants as young
as 3 months.
High risk for encapsulated organisms such as S pneumoniae
and H influenzae.
Recommended antibiotic
Third-generation cephalosporin; ceftriaxone, or cefotaxime
Vancomycin should be added to protect against penicillin-
resistant strains of S pneumoniae if suspected until culture
results become available
All SCD patients with fever must be managed with extreme caution
because of the risk of overwhelming bacteremia which can rapidly
lead to septic shock

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36. 3.STROKE OR CVA
Major complication of SCD
Is a leading cause of death in both and disability
children and adults
The most common is blockage of the intracranial
internal carotid and middle cerebral arteries.
 Patients with stroke usually present with obvious
signs such as acute hemiparesis, aphasia or dysphasia,
seizures, severe headaches, cranial nerve palsy,
altered mental status, or coma.
The most common tends to be hemiparesis.
Can be very subtle, such as a slight limp
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37. TREATMENT: STROKE OR CVA
Initial therapy is
exchange transfusion in an ICU setting to reduce Hb
S to less than 30% of total Hb.
After acute clearance of symptoms should be started
on a long-term transfusion therapy.
If not on a long-term transfusion program have an 80%
chance of recurrent stroke within 3 years of the initial
event
Long-term transfusion involves regularly scheduled
blood transfusions aimed at reducing the percentage of
Hb S and not at normalizing the Hb level.

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38. CASE 2
A 44-year-old diabetic presented to the ED complaining of nonexertional
dyspnea and severe back pain for 12 hours before presentation.
The patient reported malaise, fatigue, weakness that started 3 days before,
chronic blurred vision, insomnia, and anxiety.
The remainder of the review of systems was unremarkable.
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38

39. O/E
HR 101 bpm
RR 31/min
Temp 37C
BP 148/62 mm Hg
o2 sat 99%.
The patient was awake, alert, and oriented
He was motionless to avoid back pain.
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Case 2

40. Normal S1 and S2
Chest Normal
Strength was 4/5 in all 4 extremities.
Deep tendon reflexes were normoactive.
Normal flexor plantar response was obtained, and no
meningismus
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Case 2
O/E

41. WBC 11.2 × 109/L (with no abnormalities in
differential count)
Hg of 9.4 g/dL
HCT of 26.3%
MCVof 76.7 Femtoliters (fL)
MCH 27.3 pg
Platelets of 144 × 109/L.
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Case 2

42. Total bilirubin level of 2.3 mg/dl
Direct bilirubin level of 0.8 mg/dL
ESR 54 mm/h
C-reactive protein level of 2.3 mg/dL.
ECG Normal
MRI of the lumbar spine was Normal
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• Blood glucose 267 mg/dL
• AST 79 U/L
• ALT 30 of U/L
• ALK Ph 475 U/L
Case 2

43. Despite aggressive narcotic treatment of back
pain, the pain continued to increase
CT abdomen: an enlarged spleen
1 hour later hypotension of 90/50 mm Hg.
The new CT scan of the abdomen revealed an
increasing splenomegaly compared with the
previous one
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Case 2

44. Despite transfusion therapy, the patient's Hb
progressively dropped to a level of less than 4 mg/dL
over the course of 3 hours, with thrombocytopenia (<50
× 109/L).
immediately transferred to an ICU.
altered mental status.
Airway protection with intubation and mechanical
ventilation were initiated.
As the patient was rapidly deteriorating, an emergent
splenectomy was performed
The patient recovered every organ function and, 6
months later, has resumed his normal activities
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Case 2

45. 4.ACUTE SPLENIC SEQUESTRATION
CRISIS (ASSC)
Clinical Presentation:
Sudden impounding of red blood cells by the spleen
Characterized by the rapid fall in hemoglobin concentration, rise in
reticulocyte count, and splenomegaly
Requires prompt recognition and treatment.
In the adult patient, ASSC is extremely rare.
Hypotension caused by large volumes of blood (mainly sickled
cells) entrapped in the spleen.
Hb levels may fall acutely more than 2 g/dL less than the patient's
normal value, causing circulatory compromise
Treatment:
Prompt diagnosis and therapy with RBC transfusions
Surgical splenectomy may be indicated in certain patients to prevent
recurrences
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46. 5.APLASTIC CRISIS
Temporary cessation of red cell production with a
corresponding decrease in the reticulocyte count.
Approximately 80%, are thought to be caused by human
parvovirus B19 infection
Diagnosis is made by comparing baseline blood and
reticulocyte counts to those obtained during the acute
illness.
Sign Symptoms: , tachypnea, tachycardia, or hypoxia
Treatment:
Simple blood transfusion to raise serum Hb back to the patient's
baseline and to prevent heart failure secondary to severe anemia.
Parvovirus B19 is contagious, affected persons should be isolated
from pregnant women, who are at risk for miscarriage with
infection, and from immuno-compromised patients and those
with chronic illness
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47. 6.OSTEOMYE
LITIS
Most commonly caused by Salmonella species
or Staphylococcus aureus
Bone pain or joint pain with localized swelling
and decreased range of motion, along with
fever, should alert the physician to the
possibility of osteomyelitis.
Increased white blood cell count and elevated
ESR
Broad-spectrum antibiotic:
Ceftriaxone:
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48. 7.PRIAPISM
Painful prolonged erection of the penis
Caused by sickling of the red blood cells producing venous
stasis in the erectile tissue of the penis.
The resulting stasis causes ischemia, hypoxia, and pain.
Treatment:
Initial treatment involves intravenous hydration and analgesia.
Antianxiety agents
Vasoconstrictors to force blood out of corpus cavernosum:
Phenyl ephedrine
Epinephrine
Vasodilators: to relax smooth muscles:
Terbutaline
Hydrallazine
Episodes refractory to this initial management include direct
irrigation of the corporeal bodies of the penis
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49. VASO-OCCLUSIVE PAIN
CRISIS
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49

50. 8.VASO-OCCLUSIVE PAIN CRISES:
SUMMARY
Most common symptoms of SCD
Severe pain
Caused by sickle-shaped red blood cells trapped in small blood
vessels causing localized ischemia.
Triggered by
Dehydration, fever, cold exposure, and emotional stress
Therapy
Intravenous/Oral hydration
Pain management
It is useful to assess pain in a standard manner using pain
measurement scales ……………..See next
Causal Treatment: (treatment of the cause)
Poloxamer 188 (Flocor) a surfactant returns RBCs to a non adhesive
state and blocks RBC aggregation to enhance blood flow in ischemic
areas
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51. PAIN SCALE
0  –  Pain free.
Mild Pain  – Nagging, annoying, but doesn't really interfere with daily
living activities.
1  –  Pain is very mild, barely noticeable.  Most of the time you don't
think about it.
2  –  Minor pain.  Annoying and may have occasional stronger
twinges. 
3  –  Pain is noticeable and distracting, however, you can get used to
it and adapt.
Moderate– Interferes significantly with daily living activities.
4 – Moderate pain. If you are deeply involved in an activity, it can
be ignored for a period of time, but is still distracting.
 – Moderately strong pain. It can't be ignored for more than a few
minutes, but with effort you still can manage to work or participate in
some social activities.
6 – Moderately strong pain that interferes with normal daily
activities. Difficulty concentrating.
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52. PAIN SCALE
Severe Pain – Disabling; unable to perform daily living
activities.
7 – Severe pain that dominates your senses and significantly
limits your ability to perform normal daily activities or
maintain social relationships. Interferes with sleep.
8 – Intense pain. Physical activity is severely limited.
Conversing requires great effort.
9-Excruciating pain. Unable to converse. Crying out and/or
moaning uncontrollably.
10 – Unspeakable pain. Bedridden and possibly delirious.
Very few people will ever experience this level of pain
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53. PAIN MANAGEMENT
Mild to moderate pain
NSAID’s or acetaminophen.
Moderate pain
Weak opioid, such as codeine or hydrocodone.
Severe pain
IV opioid morphine, hydro-morphone, fentanyl, and methadone.
Titrate to pain relief and then administer on a scheduled basis with
as-needed dosing for breakthrough pain.
Patient-controlled analgesia can be used
Avoid
Meperidine should be avoided because accumulation of the normeperidine
metabolite can cause neurotoxicity, especially in patients with impaired renal
function
Minimize dependence /addiction by :
Aggressive pain control,
Frequent monitoring,
Tapering medication according to response
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54. Principles of pain management (WHO guidelines)
Morphine is the preferred agent in treatment of sickle cell pain.
Start by the mouth
By the Clock:
Regular analgesia (4-6 hourly) with breakthrough doses when needed
By the ladder:
Patients move up the ladder or may also move down the ladder if pain decreases.
Individualized Therapy:
Start with higher step for Patients presenting with moderate to severe pain.
Some don’t tolerate oral medication, plan for alternative route.
Consider non drug therapies. as well
No standard dose of opioid - morphine from 5mg to 1000mg every four hours.
With attention to detail:
Total analgesia usage should be monitored every 24 hours,
Breakthrough doses should be adjusted in line with changes to regular medication.
New pain should be assessed promptly
Patients should be informed of possible adverse drug effects.
feb, 17, 2015 Dr. Armaan Singh 54

55. feb, 17, 2015 Dr. Armaan Singh 55

56. When and how IV
REASSESS before starting IV opioids and ADJUST dose
frequently, but not before 8 hours
Scheduled IV Narcotic Dosing for 24 hours, round the
clock
Morphine sulfate: 0.1 mg/kg, 5 - 10mg, IV scheduled
every 3-4 hours.
Hydromorphone: 0.015 mg/kg, 0.75 - 2mg, IV scheduled
every 3-4 hours.
Monitor vital signs and pain level, using the pain scale,
before and after every dose
feb, 17, 2015 Dr. Armaan Singh 56

57. IV dosing: cares
Doses should be based on level of tolerance to
opioids. Most SCD patients have some opioid
tolerance.
Maximum analgesic effect within 10-15 minutes and
will usually last 2-3 hours.
 Consider around-the –clock (ATC) (patient may
refuse) to ensure the patient is offered the medication
consistently at the preferred interval.
feb, 17, 2015 Dr. Armaan Singh 57

58. Scheduled IV Narcotic Dosing -- Opiate Tolerant patients
Convert the patients usual oral dose to IV:
Morphine IV/PO ratio: 1:3
Hydromorphone IV/PO ratio: 1:5
Example: Patient is taking morphine SR 60mg PO
q12h and is now in pain crisis, requiring an additional
10mg PO q4 hours. 10mg X 6 = 60 mg + (60 mg x 2) =
180 mg PO morphine/day.
Convert PO to IV: 180mg PO / 3 = 60mg IV over 24h =
10mg IV q4h
Start with 50-75% of the calculated equianalgesic dose
if changing / converting to a different opioid to allow
for incomplete cross-tolerance between opioids.
Dose adjustment for taper
Decrease dose by 25% per day once the patient’s pain is
under control for 24 hours
feb, 17, 2015 Dr. Armaan Singh 58

59. Monitoring the patient
Chest X-ray: Order for any patient with cardiopulmonary
complaints, hypoxia, know chronic lung disease, fever,
tachycardia, or tachypnea.
Complete blood count q24 hours
Comprehensive metabolic panel, magnesium,
phosphorous q48 hours
Keep magnesium level > 2 mg/dL:
Magnesium < 1.8 mg/dL, replace with IV magnesium
May need to follow with daily oral supplementation
Magnesium > 1.8 mg/dL, replace with oral product
Lactic dehydrogenase (LDH) q72 hours
feb, 17, 2015 Dr. Armaan Singh 59

60. PCA

Patient-controlled analgesia (PCA) is a method of
pain control that gives patients the power to control
their pain. In PCA, a computerized pump called the
patient-controlled analgesia pump, which contains a
syringe of pain medication as prescribed by a doctor,
is connected directly to a patient's intravenous (IV)
line.
feb, 17, 2015 Dr. Armaan Singh 60

61. Patient Controlled Analgesia (PCA)
For setting where scheduled IV dosing is not controlling the patient’s pain.
There is no “PCA protocol.”
Continuous opioid infusion
should not be used in opioid naive patients until assessed the needs over a
given period of time (i.e. after 12 hrs of demand/bolus doses)
Only use a in patients with a known opioid requirement.
Those patients taking daily opioids: calculate an equianalgesic dose of
currently used opioids over past 24 hrs and then convert to an
equianalgesic basal rate
Example: Patient taking 120 mg extended release morphine Q 12 hrs now
in crisis taking an additional 15 mg immediate release morphine q 4 hrs. 15
mg X 6 = 90 mg + (120 mg X 2)= 330 mg PO morphine/day. Convert to IV
equivalent 330/3= 110 mg IV morphine/24 hrs = 4-5 mg/hr.
If changing/converting to a different opioid, start with 50-75% of the
calculated equianalgesic dose to allow for incomplete cross-tolerance
between opioids.
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62. Titration of Dose
Basal infusions will take at least 8 hours to reach steady state.
Do not titrate the basal rate more frequently than every 8
hours.
Never increase basal rate by more than 100% at any one time.
Demand Doses: Adjust demand dose size every 30-60 minutes
to quickly reach adequate analgesia.
For mild-moderate pain increase dose by 25-50%.
For moderate-severe pain increase dose by 50-100%.
feb, 17, 2015 Dr. Armaan Singh 62

63. Converting IV to Oral Pain Management
Once the IV dose has been tapered to 50% of the
initial dose, start oral morphine or hydromorphone:
Morphine & Hydromorphone: Add total daily dose of
IV morphine received; multiply by 2-3 to determine
total daily dose.
Immediate release formulations should be
administered on a scheduled basis, every 4 hours.
Sustained release formulations should be
administered every 12 hours.
Morphine to oral Oxycodone:
Convert morphine 10mg IV q4h to oxycodone 30 mg PO
q6h.
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64. Adjunct therapies
Bowel regimen: All patients on opioids must also be
on a bowel regimen of stool softener and a cathartic.
May administer Hydroxyzine 25-50 mg PO with each
narcotic dose.
Itching:
Diphenhydramine 50mg IV/PO can be given with the
initial dose of morphine and PRN
Diphenhydramine may be given in conjunction with
opiates for additive effect.
 Nausea: administer prochlorperazine 10mg PO PRN
nausea.
feb, 17, 2015 Dr. Armaan Singh 64

65. EVALUATION OF THERAPEUTIC
OUTCOMES
All patients should be evaluated regularly to establish
change in baseline, parameters
Laboratory evaluations
complete blood cell and reticulocyte counts
HbF level.
Kidney and Liver function tests and pulmonary function
Patients should be screened for retinopathy.
The efficacy of hydroxyurea can be assessed by
monitoring the number, severity, and duration of
sickle cell crises.
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