A 12-year-old male child presented with pain in both legs and left shoulder due to sickle beta thalassemia. He has a history of recurrent sickle cell crises. He was treated with antibiotics, hydration, pain medications, and blood transfusion. He was discharged with instructions to follow up in 3 months and start hydroxyurea if another crisis occurs within 6 months to help reduce future crises.

1. CASE PRESENTATION ON
SICKLE PAINFUL CRISES
R.Anusha
Pharm D VI Year
Roll No:170514882007.

2. Patient name: Master BK
Gender: Male
Age: 12 Years
IP No: 331290
DOA: 7/2/20
DOD: 12/2/20

3. SUBJECTIVE
• Chief Complaints: Pain in both legs (R>L), Left shoulder
pain.
• Present Illness History: Pain in both legs (R>L), Left
shoulder pain aggravated since 1 day. H/o low grade fever on
and off.
• Past Medical History: Sickle beta thalassemia with HbS
79%, A2 5.9. Sickle painful crises 2 episodes in the last 3
months and Dec 2019 now in Feb 2020.
• Past Medication History:
T.FOLVITE 5 mg PO OD

4. OBJECTIVE
PHYSICAL APPEARANCE:
Height: 147 cms
Weight: 35kgs
B.M.I: 16.2 kg/m2
CVS
RS
Abd WNL
CNS
MS:C/o pain in right hip,thigh,
left shoulder.
VITALS:
B.P: 90/60 mm of Hg
RR: 18 breaths/min
P.R: 104 beats/min
Temperature: 980 F
SpO2:100% on RA

5. Lab test:
Hb: 9.9 g/dL
TLC:10,700 cells/mm3
Platelet: 4 lakh/micro liter
Sr.Cr: 0.4 mg/dL
CRP:169 mg/L (upto 10 mg/L)
Blood culture and Urine culture: Negative
C-X Ray: Normal

6. FINAL DIAGNOSIS:
K/c/o Sickle Beta Cell Thalassemia
C/o Recurrent episodes of Sickle Painful Crises

7. DRUG CHART
BRAND GENERIC DOSE ROA FREQ Start date Stop date
Inj.ZOSTUM Cefaperazone
1000 mg
Sulbactum 500mg
1.5 g IV BD for 5
days
7/2/20 11/2/20
Inj.TRAMADOL Tramadol 50 mg IV STAT/
SOS
7/2/20 -
IVF FLUIDS 0.9% Normal
saline
75
ml/hr
IV STAT 7/2/20 -
Inj.PAN Pantoprazole 20 mg IV STAT 7/2/20 -
Inj.ZOFER Ondansetron 4 mg IV STAT 7/2/20 -
T.FOLVITE Folic acid 5 mg PO OD 7/2/20 Continued
Inj.PCM Paracetamol 500 mg PO STAT 7/2/20(100F) 8/2/20
T.CROCIN Paracetamol 500 mg PO SOS 8/2/20 11/2/20
T.VOVERAN Diclofenac 50 mg PO BD 8/2/20 11/2/20

8. Goals of the Treatment
• Management of vaso-occlusive crisis
• Management of chronic pain syndromes
• Management of chronic hemolytic anemia
• Prevention and treatment of infections

9. ASSESSMENT
Day 1(7/2/20):
12 year old male child k/c/o sickle cell disease with sickling
cell crises presented to ER with a case of B/L lower limb pain,
Left upper limb and shoulder pain since 1 day.
T.Bil: 1.26 PLAN:
SGOT: 40 Medications as per chart
SGPT:18 Continue low flow O2
ALP: 229
T.Proteins: 7.3

10. STAT MEDICATIONS GIVEN IN ER
• Inj.ZOSTUM 1.5 mg IV
• Inj.TRAMADOL 50 mg IV
• Inj.IVF FLUIDS 75 ml/hr IV
• Inj.PAN 20 mg IV
• Inj.ZOFER 4 mg IV
He was started on broad spectrum antibiotic, hydration and
fluids, pain killer.

11. Day 2(8/2/20):
Previously painful crises once a year.
Last admission in Dec 2019.
Same side pain every time in right leg.
HbS 79% , A2 - 5.9
In between episodes absolutely no pain.
IMP: Painful Crises
PLAN: Add regular PCM+Diclofenac; Tramadol PRN
If pain does not settle will plan transfusion
Hydration 1-1.5 L atleast
O2 2 lit
CBC tomorrow

12. Day 3(9/2/20):
• Hb:8/ PCV:25/ TLC:8000/Platelets:2.4lakh
• Pain decreased
• Child: Conscious, Oriented
• On 2 lit O2
• Vitals stable
PLAN
Rx as per chart
Monitor vitals

13. Day 4 (10/2/20):
• If one more episode occurs, plan for Hydroxy urea after 2nd
episode.
Day 5 (11/2/20):
• No fever episodes since yesterday
• No pain
• Hb:7.7/ PCV:23/ TLC:5000/Platelets:2.4 lakh
• Temperature:98 F, RR:20, BP: 110/80
PLAN:1 UNIT PRBC transfusion today.

14. Day 6 (12/2/20):
• No fresh complaints
• 1 unit PRBC transfusion done yesterday
• BP:110/80, PR: 100, RR:22
PLAN:
Discussed the role of Hydroxycarbamide to reduce the number
of painful episodes, so far he has not any severe crises like
chest crises or CNS complications, hence there is no role of
prophylactic blood transfusion.
If another episode of sickle cell crises occurs with in 6 months,
plan to add Hydroxyurea.

15. HYDROXYUREA
• In 2017, the FDA approved it to treat children with sickle cell
disease. Hydroxyurea is also used to treat cancer. But doctors
use a lower dose (amount) to treat sickle cell disease than to
treat cancer.
• Hydroxyurea reduces the problems that sickle cell disease
causes. People with sickle cell disease who take hydroxyurea
have fewer:
Pain crises
Episodes of acute chest syndrome
Blood transfusions
Hospital stays
Hydroxyurea can also prevent or slow down damage to organs

16. • Hydroxyurea makes the red blood cells bigger. It helps them
stay rounder and more flexible — and makes them less likely
to turn into a sickle shape.
• The medicine does this by increasing a special kind of
hemoglobin called hemoglobin F. Hemoglobin F is also
called fetal hemoglobin because newborn babies have it.
When there is higher levels of hemoglobin F, red blood cells
are less likely to cause problems.
• Plan discharge today.

17. PLAN
MONITORING PARAMETERS:
Monitor Hb levels
Monitor temperature
Monitor vitals

18. PATIENT COUNSELLING
Disease related
Sickle cell disease is an inherited blood disorder affecting red
blood cells. Normal red blood cells contain hemoglobin A. People
with sickle cell disease have red blood cells containing mostly
hemoglobin S, an abnormal type of hemoglobin. These red blood
cells become sickle-shaped (crescent-shaped).
Sickle cell disease can cause periods of intense pain (called “pain
crises”) and other problems(Aneima,Acute chest syndrome,Organ
damage).
Preventing Crises
• Get enough oxygen
• Drink plenty of fluids
• Avoid getting over-heated or getting very cold
• Avoid getting infections, and quickly treat infections when you
do get them

19. • To prevent oxygen loss, avoid:
• Demanding physical activity (especially if the spleen is
enlarged)
• Emotional stress
• Environments with low oxygen (high altitudes, non
pressurized airplane flights)
• Smoking
• Known sources of infection
• To make sure you're getting enough fluids:
• Avoid too much exposure to the sun
• Have fluids on hand, both at home and away
• Recognize signs of dehydration

20. • Seek emergency medical attention for any temperature of
101.50 F or higher
• Practice good hand hygiene, especially washing hands
frequently when coughing or sneezing or caring for others
who have colds.

21. Drug related:
T.FOLVITE 5 mg 1 tab PO OD After food to continue till
review.
T.CROCIN 500 mg 1 tab PO TID After food for 5 days.(Pain)
T.VOVERAN 50 mg 1 tab PO BD after food for 3 days.(Pain)
REVIEW AFTER 3 MONTHS.