TH

BY
PARVATHY NAIR
15Q0421
PHARM D IV
THALASSEMIA
ALPHA THALASSEMIA
BETA THALASSEMIA

• They are a group of heriditary disorders characterized by a

genetic deficiency in the synthesis of beta- globin chains.
• In the homozygous state, beta thalassemia causes severe ,
transfusion-dependent anemia.
TYPES
ETIOLOGY

• Beta globin gene mutations

Beta thalassemia affects 1 or both of the beta- globin genes. The defect can be a
complete absence of the beta-globin protein ( ie, beta zero thalassemia) or a
severely reduced synthesis of the beta- globin protein
• Intracellular fetal Hb concentrations
• The level of expression of fetal Hb (ie, the expression level of the
gamma globin gene) in red blood cells determines, in part, the severity of the
disease. Pts with high fetal Hb have milder disease.
• Coinheritance of alpha thalassemia
• Pts with coinheritance of alpha thalassemia have a milder clinical course
because they have a less severe alpha beta chain imbalance.
• Coexsitence of the sickle cell trait
The coexistence of sickle cell trait and beta thalassemia is a major
and symptomatic hemoglobinopathy with most of the symptoms
and complications of sickle cell disease.
CLINICAL PRESENTATION
• SIGNS AND SYMPTOMS
• Laboratory Parameters
• Hb:- unstable HB LEVELS
• Beta chain synthesis ratio or performing genetic tests of the
alpha-globin cluster(Southern blot or polymerase chain reaction
assay tests
DIAGNOSIS
• Most children with moderate to severe thalassemia show signs and symptoms within their first two
years of life. If your doctor suspects your child has thalassemia, he or she may confirm a diagnosis
using blood tests.
• If your child has thalassemia, blood tests may reveal:
• A low level of red blood cells
• Smaller than expected red blood cells
• Pale red blood cells
• Red blood cells that are varied in size and shape
• Red blood cells with uneven hemoglobin distribution, which gives the cells a bull's-eye appearance
under the microscope
• Blood tests may also be used to:
• Measure the amount of iron in your child's blood
• Evaluate his or her haemoglobin
• Perform DNA analysis to diagnose thalassemia or to determine if a person is carrying mutated
haemoglobin genes
• Prenatal testing
• Testing can be done before a baby is born to find out if he or she has thalassemia and determine
how severe it may be. Tests used to diagnose thalassemia in foetuses include:
• Chorionic villus sampling. This test is usually done around the 11th week of pregnancy and
involves removing a tiny piece of the placenta for evaluation.
• Amniocentesis. This test is usually done around the 16th week of pregnancy and involves taking a
sample of the fluid that surrounds the foetus.
 TREATMENT
• Treatments for mild thalassemia
• Signs and symptoms are usually mild with thalassemia minor and little, if any, treatment is needed. Occasionally,
you may need a blood transfusion, particularly after surgery, after having a baby or to help manage thalassemia
complications.
• People with severe beta-thalassemia will need blood transfusions. And because this treatment can cause iron
overload, they will also need treatment to remove excess iron. An oral medication called deferasirox (Exjade,
Jadenu) can help remove the excess iron.
• Treatments for moderate to severe thalassemia
• Treatments for moderate to severe thalassemia may include:
• Frequent blood transfusions. More-severe forms of thalassemia often require frequent blood transfusions,
possibly every few weeks. Over time, blood transfusions cause a buildup of iron in your blood, which can
damage your heart, liver and other organs. To help your body get rid of the extra iron, you may need to take
medications that rid your body of extra iron.
• Stem cell transplant. Also called a bone marrow transplant, a stem cell transplant may be an option in select
cases, including children born with severe thalassemia. It can eliminate the need for lifelong blood transfusions
and drugs to control iron overload.
• During this procedure, you receive infusions of stem cells from a compatible donor, usually a sibling.
SEPTIC ARTHRITIS
`
PATIENT PROFILE FORM

• Pt Name: XYZ Age: 6 yrs Sex: female

• IP No: 27647 Dept : Paed Unit: B
• Height : 104 cm Weight : 15.45 kg DOA: 31/7/18
• Reason for admission: K/C/O Thalassemia Major
C/o swelling over the right knee joint since 6 days
• Past medical History: K/C/O Thalassemia major since 6 months of
age. Blood transfusions is going on. every month rejuated blood
transfused.
• History of present illness
Pt was apparently alright 6 days back then she developed swelling
over the right knee joint gradually in onset, progressive in nature. Pain is
present at knee joint pricing type of pain. No radiating of pain,
restriction of movement of knee joint. Pain aggregates on walking. No
H/O vomiting
No H/O fever
No H/O burning micturition
No H/O fall
• General Physical Examinations Systemic Examinations
Well built, conscious • CNS: conscious, oriented with
Vital signs – stable TPP, all cranial nerves intact
Pulse – 82 bpm • RS: NVBS +ve B/L AE +ve
BP – 94/60 mmHg No added sound
• CVS: S1S2 heard, no murmur
RR-32 cpm
• P/A: soft non tender, moderate
Temp – afebrile spleenomegaly
PROVISINAL DIAGNOSIS

K/C/O thalassemia major

with septic arthritis
LABORATORY DATA
HB Hb(g/dl) 10.8 gm%
CBC WBC (4,500-10,500 CELLS/µl) 10,800
POLYMORPHS(40-74 %) 71%
BASOPHILS(0-1%) 00
EOSINOPHILS(0-5%) 05
LYMPHOCYTES(20-40%) 19
MONOCYTES(0-7%) 05
RBC(4.6-48 MILLION/µL) 3.86
PLT(150,000-450,000 cells/mm3) 2,83,000
ESR (<15 mm/hr) 105
CRP(10-6 mg/L) 28.5
 TREATMENT CHART
BRAND NAME GENERIC NAME DOSE ROUT FREQUEN 1 2 3 4 5 6
E CY
IVF DNS @30ml IV Y
/hr
INJ. VANCOMYCIN 20ml IV TID Y Y Y Y Y Y
VANCOMYCIN NS
over 1
hr
INJ, PIPZO PIPERACILLIN 2g IV TID Y Y Y Y Y Y
TAZOBACTAM 250 mg
SYP. RANTOP RANITIDINE ORAL SOLN 5ml P/O BID Y
SYP.IBUGESIC IBUPROFEN 100mg P/O TID Y Y Y Y Y Y
PLUS PARACETAMOL 162.5m
g/5ml
TAB.TRAMADOL TRAMADOL 10mg P/O SOS Y Y Y
INJ. AVIL PHENARAMINE MALATE 25mg IV STAT Y
FOLLOW UP
DATE BP PULSE O/E TREATMENT ADVICE

31/7/18 94/60 82 c/o itching and rashes over the body As per the chart
Afebrile
S/E:
RS: B/L AE +VE, CLEAR
CVS: S1 S2 HEARD
P/A: SOFT, NON TENDER,
SPLEENOMEGALY(+)

1/8/18 104/70 98 c/o pain As Per the Chart

Antalgic hard knee joint IVF stopped
S/E:
RS: B/L AE(+) CLEAR
CVS: S1S2(+)
P/A: SOFT NON TENDER,
SPLEENOMEGALY(+)
2/8/18 114/70 92 c/o pain As Per the Chart
Antalgic hard Continue IV Abs
knee joint
S/E:
RS: B/L AE(+)
CLEAR
CVS: S1S2(+)
P/A: SOFT
NON TENDER,
SPLEENOMEGA
LY(+)
3/8/18 110/70 100 Afebrile C ST
NFC
4/8/18 115/70 98 NFC C ST
Knee semi flexed Started
swelling(+) TAB TRAMADOL
SOS
5/8/18 120/80 96 NFC C ST
6/8/18 118/80 98 NFC C ST
7/8/18 118/70 82 NFC C ST
Tab Tramadol 10
mg(1/2)
DIAGNOSIS

K/C/O Thalassemia major with

septic arthritis
 DISCHARGE MEDICATION

BRAND NAMET100 GENERIC NAME DOSE ROUTE FREQUENCY DURATION

SYP RANTAC RANITIDINE 5 ml P/O BID 10 DAYS
SYP IBUGESIC PLUS IBUPROFEN 100mg P/O TID 10 DAYS
PARACETMOL 162.5mg
/5 ml
TAB TRAMADOL TRAMADOL 50 mg P/O SOS
TAB CIPLOX CIPROFLOXACIN 500mg p/o BID 10DAYS

Review: Come after 10 days

PHARMACEUTICAL CARE PLAN

SUBJECTIVE EVIDENCES OBJECTIVE EVIDENCES

K/C/O Thalassemia Major Hb : 8.6 g/dl
RBC : 3.86 millions/µl
C/O swelling over the right knee joint ESR : 105 mm/hr
since 6 days CRP: 28.5 mg/L
ASSESMENT

Based on the subjective and objective evidences the patient is

diagnosed to have K/C/O Thalassemia with Septic arthritis.
MONITORING PARAMETERS

• HAEMOGLOBIN
• RBC
• WBC
• ESR
• CRP
GOALS OF THERAPY

• To relieve the signs and symptoms

• To reduce the swelling and pain
• To improve the walking style of the patient
• To improve the quality of the life of the patient
GENERAL APPROACH

• Medical management of infective arthritis focuses on adequate and timely drainage of the
infected synovial fluid, administration of appropriate antimicrobial therapy and immobilization
of the joint to control pain
• Acute prosthetic infection can be cured medically if it is of the early type or secondary to
hematogenus spread without any evidence of periatricular soft-tissue involvement or joint
instability
NON PHARMACOTHERAPY

• Joint immobilization
• Physiotherapy
• Synovial fluid drainage
• Surgical intervention in Prosthetic Joint Infection
PHARMACIST INTERVENTION

• DRUG INTERACTION
• Piperacillin + Vancomycin
Piperacillin increases toxicity of vancomycin by unspecified
interaction mechanism. Monitor kidney function in patients concomitantly
administered with piperacillin and vancomycin.
• Ibuprofen + Vancomycin
Ibuprofen increases levels of vancomycin by decreasing renal
clearance . Interaction mainly occurs in neonates.
PATIENT EDUCATION

• Instruct patients with a prosthetic joint in place to recognize bac

infections in other parts of their bodies to prevent associated
bacteremias.
• About disease
• β Thalassemia Major: They are a group of heriditary disorders
characterized by a genetic deficiency in the synthesis of beta- globin
chains.
• Septic arthritis : It is an infectious arthritis, may represent a direct
invasion of joint space by various microorganisms, most commonly cause
by bacteria
• About drugs:
• Syp Ranitidine: Ranitidine is a H2RA. It can be given twice in a day or
orally once a after the evening meal or at bedtime.
• Ibuprofen: It is a NSAIDs which will exert its effect by inhibits both
COX1 and COX2
• Paracetamol: It is also an NSAIDS. It is a weak inhibitor of
the synthesis of PGs
• Tramadol: It is a synthetic opioid analgesic used to relieve
pain
• Ciprofoxacin: It is a fluroquinolones and will inhibit DNA
gyrase and topoisomerase IV, which are required for bac
DNA replication
LIFESTYLE MODIFICATIONS

• Excercise
• Eat a healthy diet
• Get Vitamin C
• Keep track of your weight
• Avoid Alcohol
• Manage treatment and monitoring schedules so as to minimise any
unnecessary impact on normal daily activity
THANKYOU