thalassemia cpc

pediatric department, Sheikh Zayed Medical College/Hospital, Rahim Yar Khan, Punjab, PakistanSaturday, February 8, 2014 1

A young boy known case of “beta Thalassemia Major”

Resham kareemRoll no “354”Final year MBBS


Abdul Manan s/o Jamil Ahmad 6 years Resident of Kott Samaba Presented through Pediatrics Emergency on 1st Feb 2014


Fever

Vomiting

Altered level of consciousness

Yellowish discoloration of eyes and urine 1 day

2 days


Patient is a known case of Beta Thalassemia Major diagnosed at the age of 6 months.

He was in his usual state of health 2 days back when he developed high grade fever which was sudden in onset,, intermittent and not associated with rigors and chills, flu, and cough. Fever was relieved by medication.

Fever was associated with vomiting, 2-3 times per day, vomitus was yellow in color and contained food particles but no h/o blood in vomitus.


He developed altered level of consciousness and irritable behavior. It was associated with drowsiness, confusion, disorientation and mood swings.

My patient developed yellow discoloration of eyes and urine , which was associated with itching and bruises.


There was H/O poor intake of both liquid and solid.

CVS: There is H/O breathlessness on exertion but no Edema feet or cyanosis.

Respiratory system: No H/O cough, wheezing or chest pain

GUS: There is H/O Pain in flank, dysuria, oliguria and yellow colored urine

GIT There is no H/O of diarrhea, constipation, hematemesis or melena, clay

colored stoolpediatric department, Sheikh Zayed Medical

College/Hospital, Rahim Yar Khan, Punjab, PakistanSaturday, February 8, 2014 8

CNS:No H/O of fits, visual loss and weakness.EndocrineNo H/O polydipsia, polyphagia, heat or cold intolerance.Locomoter systemThere is no H/O of joint pain, swelling and stiffness of joint.


Diagnosis At 6 month of age diagnosed as Thalassemia Major in SZH,RYK when he presented with progressive pallor, fever and vomiting.Transfusion HistoryBlood transfusion started from 6 months of life. First 3 years, transfusion at monthly intervalAfterwards, transfusion at 15 days interval.Chelation therapyNever taken yet because of no awareness


Pregnancy: No history of infection, drug intake, trauma or irradiation to mother

Delivery: Birth by uneventful SVD in Private hospital by a doctor


http://images.google.ie/imgres?imgurl=www.lutheransforlife.org/images/people/babies/baby_doctor_01.gif&imgrefurl=http://www.lutheransforlife.org/living/2000/winter/every_child_a_wanted_child.htm&h=284&w=219&prev=/images?q=doctor+++child&start=180&svnum=10&hl=en&lr=&ie=UTF-8&sa=N

Breast feeding •Exclusively breast fed for 6 months

Weaning•Started at 7th month•Contained daliya, cerelac, fruits

Current diet•Home made food


major milestones achieved Smile: 1 monthNeck holding: 3 monthsSitting 7 monthsCrawling: 10 monthsStanding: 1 yearWalking: 15 monthsTalking single word: 1 yearAccurate speech: 1.5 years

“All milestones achieved at normal age”


• BCG at Birth• penta 1, OPV 1, 06 wks• Penta 2, OPV 2, 10 wks• Penta 3, OPV 3, 14 wks• Measles I 09 months• Measles II 15 months

Immunization status complete

according to EPI schedule.


Age of mother: 35 yearsAge of father: 40 yearsBoth are confirmed cases of Thalassemia MinorCousin Marriage4 live siblings

1st sibling: M, 9 yrs., known case of Thalassemia Major

2nd sibling: M, 7 yrs, known case of Thalassemia Minor

3rd & 4th: twin F, 2 yrs, known case of Thalassemia Minorpediatric department, Sheikh Zayed Medical


There is no other history of hereditary or infectious disease

Death:1st immediately after birth(cause unknown)

2nd spontaneous abortion at 4th month of gestation


Education of parents:Mother: Intermediatefather: matriculation

Occupation:father: shopkeeperMonthly Income: 30,000 PKR

House: pukka2 roomslocality: villagesanitary condition: good

Blood is donated by the blood bank of SZH,RYKpediatric department, Sheikh Zayed Medical


water supply: proper and underground Fresh water Good sanitation


Behavior of Child: positiveHabit and interests: positiveClass twoSchool performance: good


Pt. received PARACETAMOL for fever in usual dose Pt. also received FURECIMIDE for oliguria


EXAMINATION,

INVESTIGATIONS AND

MANAGEMENT

MUHAMMAD SALMAN

ROLL NO. 314

FINAL YEAR

21

Examination at the time of admission

6 year old child pale looking having jaundice, not oriented, irritable, drowsy & some dehydrated having vitals

H/R 110/min R/R 24/mintemp 102 FBP 85/60 mm of Hg

22

General physical examination (current)

Abdul Manan, 6 year old boy, average built,

Well cooperative and well oriented in time, place and person lying comfortably in the bed.

No obvious respiratory distress, cyanosis and dysmorphism

Pulse rate: 92/min

Respiratory rate: 18/min

Blood pressure: 100/65 mm of Hg

Temperature: 99 F

Anthropometric measurements

Head circumference: 51cmHeight: 107cm (5th centile)Weight: 17 kg

24

• Pallor • jaundice • Bruises on skin• THALASSEMIC FACIES• clubbing• koilonychias,• leuconychia .• palmer erythema• Lymph node and thyroid are not palpable. • JVP not raised.• Ankle and sacral edema is absent.

POSITIVE

NEGATIVE

25

Orodental hygiene is satisfactory with protrusion of maxillary teeth

ABDOMINAL EXAMINATION INSPECTION abdomen is distended, moving with respiration with no stria,

scar mark, dilated veins. Umbilicus is central and inverted, hernial orifices are intact.

PALPATION there is no tenderness,

liver is palpable 8cm below the Right costal margin in midclavicular line, firm in consistency, sharp edges, upper border is in 5th ICS with a total span of 14.4 cm.

Spleen is also enlarged measuring 10cm below the costal margins , firm in consistency with sharp edges

Kidneys are impalpable bimanually

bladder not distended

GIT 26

•PERCUSSION note is resonant all over except in the region of liver and spleen, where it is dull.

no shifting dullness and fluid thrill

•AUSCULTATION : bowel sounds are audible,

no bruit is audible.

27

CVS

INSPECTION: apex beat is not visible, there are no pulsation, prominent veins or scar

PALPATION: apex beat is palpable in 5th intercostal space medial to mid clavicular line and is of normal character. No other sounds, thrill or left parasternal heave is palpable.

AUSCULTATION: 1st and 2nd heart sounds are of normal character with no added sound

28

CNS Higher mental functions are intact. Speech is normal. Cranial nerves are intact Motor and sensory systems are normal No sign of meningeal irritation are present. Gait is normal

GROSSLY INTACT

29

Respiratory system

There is normal vesicular breathing on auscultation with no added sounds.

30

INVESTIGATIONS

31

Complete Blood Count

Hb: 6.9gm/dl T.L.C: 5500/mm3 Neutophil: 77% Lymphocyte: 18% Eosinophils ; 4% monocytes: 1 % Platelets: 25,000/mm3

32

PERIPHERAL MORPHOLOGY Dimorphic picture ++ Microcytosis ++ Hypochromic, ++ anisocytosis, ++ poikilocytosis, + target cells few Schistiocytosis 1 NRBC/ 100 WBCs Reticulocyte count : 3.8 %

33

Hb electrophoresis

Done at the age of 6 months

Hb F: 98.1 %

Hb A2: 1.9 %

Hb A: 0 %

34

Serum ferritin level: >2000ng/ml (normal-less than 400) Serum LFT’s: Bilirubin=9.5mg/dl

ALT=1261 IU/l

AST=739 IU/l Serum alkaline phosphatase=486U/L Serum albumin = 3 g/dl Serum RFT’s: Blood urea= 203 mg/dl Creatinine= 1.4 mg/dl PT : control 18, APTT : control 39

test 14 test 33

35

Serum electrolyte: Sodium=131mmol/L

Potassium=3.4mmol/L ECG ----- normal Ultrasound of abdomen: hepatospleenomegaly, PV vein size is normal Anti HCV: positive , HbSAg: negative (result for anti HCV by ELISA awaited) Complete urine examination : pus cell 4-6/hpf

albumin +

Blood sugar (random): 90 mg/dl

Thyroid function test T4 1.39ng/dl TSH 4.41µIU/ml

normal

36

Skull X-ray lateral view : thinning of cortex and widening

of dipole bone

37

CXR ------ heart size is normal

38

FINAL DIAGNOSIS

BETA THALLASEMIA MAJOR WITH

complications hepatic encephalopathy (stage II)

Hepatorenal syndrome/ARF

Hyperspleenism

39

TREATMENT GIVEN IN THE HOSPITAL

RIMSHA NAZIRROLL NO.201

FINAL YAER

40

MAINTENANCE OF ABC and monitoring of vitals NG tube and foley catheter passed Hydration I/V antibiotics Lactulose Enema Vit. K Aminoglycoside through NG tube Cimetidine Blood transfusion

41

TREATMENT OUTCOME 07.02.2014

Now pt. is fully conscious, co-operative, oriented in time, space and person with stable vitals.

His current investigations LFTs bilirubin 3.7 mg/dl

ALT 272 IU/L

AST 129 IU/L

Al.phos 326 IU/L CBC Hb. 7.7 g/dl blood transfusion advised RFTs urea 32 mg/dl

Creatinine 0.8mg/dl

42

THALASSEMIA

Dr. ZAHID MEHMOOD

FCPS-II Resident

Pediatric Dept.

SZMC/H, R.Y.K.

INTRODUCTION

BASIC 3 TYPES

•Hb A 2α + 2β

•Hb A2 2α + 2δ

•Hb F 2α + 2γ

1.The α-globin genes are encoded on chromosome 16 ,

2. γ, δ, and β-globin genes are encoded on chromosome 11

PEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 44

fetal period 1ST TRIMESTER HB F ,2nd trimester Hb A, 3rd trimester Hb A2

At birth appears Hb F 98% gradually decline till 6months

At 6 months Hb A >95%, Hb A2 ≤3.5 %, Hb F <1.5%.

•If synthesis of α chain is suppressed – level of all 3 normal Hb A (2α ,2β), A2 (2α ,2 δ),F(2α ,2γ) are reduced

•If β chain is suppressed - Hb A (2α ,2β), is suppressed

compensatory rise in Hb F & Hb A2PEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 45

DEFINITION• Thalassemia syndromes are a heterogeneous group of inherited

anemias characterized by reduced or absent synthesis of either alpha or Beta globin chains.

•One of the most common single gene disorders

ETIOLOGY

• Autosomal recessive

•MutationsPEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 46

CLASSIFICATION OF THALASSEMIAS

•β Thalassemia

• α Thalassemia

• γ Thalassemia

• δ Thalassemia

• δ β Thalassemia

• Hemoglobin Lepore syndrome

• Hb C Thalassemia

• Hb D Thalassemia (Punjab)

• Hb E ThalassemiaPEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 47

Αlpha-THALASSEMIANO. OF GENES PRESENT

GENOTYPE CLINICAL CLASSIFICATION

4 genes αα/αα Normal3 genes αα/- α Silent carrier2 genes - α/- α or

αα/- - α thalassemia trait

1 gene - α/- - Hb H Disease0 genes - -/- - Hb Barts / Hydrops

fetalisPEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 48

ALPHA THALASSEMIA•Highest prevalence in Thailand

• α chains shared by fetal as well as adult life. Hence manifests in both

• These thalassemias don’t have ineffective erythropoiesis because β and γ tetramers are soluble chains and hence not destroyed always

• Silent carrier – not identified hematologically,

•Diagnosed when progeny has Hb Barts/ Hb HPEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 49

BETA THALASSEMIA


HISTOTY

Dates back over 50,000 year ago in a valley of Italy and Greece now covered by the Mediterranean.

Thalassemia is a Greek word Thalassa which means the sea (Refer to the Mediterranean) emia which means “related to blood”


EPIDEMIOLOGY

Worldwide ,15million have clinically apparent thalassemic disorders

About 100,000 babies worldwide are born with thalassemia each year.

Globally in 2010 it resulted in about 18,000 deaths

Genetic disorder of hemoglobin are the commonest single gene disorder in Pakistan. PEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 52

THALASSEMIA IN PAKISTAN

In Pakistan the disease is seen in almost all the parts of country, with an estimated carrier rate of 5-8% ( About 9.8 million carriers in total population)

Approximately 4000-5000 beta thalassemia children are born each year.

Average life expectancy in Pakistan is 10 years.

At present disease load is around 100,000 patients throughout the country.


THALASSEMIA IN RAHIM YAR KHAN (SZH)

2013 Total admissions 14433

Thalassemia 1335(9%)

Total thalassemic patients registered in SZH,RYK are 160


CLASSIFICATION OF Β THALASSEMIA

CLASSIFICATION

GENOTYPE CLINICAL SEVERITY

thal βminor/trait

/ +, β β/ 0β β

Silent

thal βintermedia

+ / +, β β+/ 0β β

Moderate

thal majorβ 0/ 0β β Severe

/β β Normal


PATHOPHYSIOLOGY

•Since chain synthesis reduced -ẞ

1. gamma 2ץ and delta δ2 chain combines with normally produced α chains ( Hb F (α2 2ץ ) , Hb A2 (α2 δ2) - Increased production of Hb F and Hb A2

2. Relative excess of α chains → α tetramers forms aggregates → precipitate in red cells → inclusion bodies → premature destruction of maturing erythroblasts within the marrow (Ineffective erythropoiesis) or in the periphery (Hemolysis)→ destroyed in spleenPEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN,

PUNJAB, PAKISTAN Saturday, February 8, 2014 56

PATHOPHYSIOLOGY….. continued

Anemia result from lack of adequate Hb A → tissue hypoxia→ ↑EPO production → ↑ erythropoiesis in the marrow and sometimes extramedullary → expansion of medullary cavity of various bones

Liver spleen enlarge → extramedullay hematopoiesisPEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN,


IRON OVER LOAD

Causes

•Hemolysis

•Increased hematopoiesis

•Increased absorption from GIT

•Repeated blood transfusion


ACCUMULATION OF IRON•Deposition in pituitary - endocrine disturbance - short

stature, delayed puberty, poor sec. sexual characteristics•Hemochromatosis - cirrhosis of liver•Cardiomyopathy (cardiac hemosiderosis) -cardiac failure•Deposition in pancreas -diabetes mellitus•Lungs: restrictive lung defects•Adrenal insufficiency•Hypothyroidism, hyperparathyroidism• Increased susceptibity to infections (iron favours bacterial

growth) espc : Ye rs inia infectionsPEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 59

CLINICAL FEATURES

INFANTS:

•Age of presentation: 6-9 mo (Hb F replaced by Hb A)

•Progressive pallor and jaundice

•Cardiac failure

•Fever

•Hepatomegaly

•Splenomegaly PEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 60

CLINICAL FEATURES

BY CHILDHOOD:

Severe anemia-cardiac dilatationJaundiceTransfusion dependent Changes in skeletal system Growth retardation


SKELETAL CHANGESTHALASSEMIC FACIES

Frontal bossing, maxillary hypertrophy, depression of nasal bridge , Malocclusion of teeth

PARAVERTEBRAL MASSES:

• Broadening of ribs at costo-vertebral attachment

• Paraparesis

• PATHOLOGICAL FRACTURES:

• Cortical thinning

• Increased porosity of long bones DELAYED PNEUMATISATION OF SINUSES - sinusitis

PREMATURE FUSION OF EPIPHYSES - Short staturePEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 62

CLINICAL FEATURES (THALASSEMIA INTERMEDIA)

•Moderate pallor, usually maintains Hb >6gm%

• Anemia worsens with pregnancy and infections (erythroid stress)

• Less transfusion dependant

• Skeletal changes present, progressive splenomegaly

•Growth retardation

• Longer survival than Thalassemia majorPEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 63

CLINICAL FEATURES (THALASSEMIA MINOR)

• Usually ASYMPTOMATIC

• Mild pallor, no jaundice• No growth retardation, no skeletal abnormalities, no splenomegaly•MAY PRESENT AS REFRACTORY IRON DEFICIENCY ANEMIA

(Hypochromic microcytic anemia)• Unresponsive/ refractory to Fe therapy• Normal life expectancyPEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 64

DIAGNOSIS

BLOOD PICTURE

•Hb – reduced (3-9mg/dl)

•RBC count – increased

•WBC, platelets – normal,


PERIPHERAL BLOOD PICTURE

•RBC indices – MCV & MCH,MCHC reduced

•microcytic hypochromic anemia, anisopoikilocytosis, target cells, nucleated RBC, leptocytes, basophilic stippling, tear drop cells

•Cytoplasmic incl bodies in α thal

• Post splenectomy : Howell-Jolly and Heinz bodies

•Reticulocyte count increased (upto 10%) but Relative Reticulocytopenia PEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN,


•Total. Bilirubin & Indirect. bilirubin – increased

•B.M. study: hyperplastic erythropoiesis


Hb ELECTROPHORESIS

Beta Thalassemia Major

Hb F: 98 %

Hb A2: 2 %

Hb A: 0 %


X ray skull:

“ hair on end” appearance

or“crew-cut” appearance


IRON OVERLOAD ASSESSMENT

•S.Ferritin

•Urinary Fe excretion

•Liver biopsy - standard

•Myocardial-liver MRI indexes


TREATMENT• Supportive Treatment

•Psychological/ social support

•Blood Transfusion

•Chelation Therapy ( For iron overload)

• Surgical

•Bone marrow transplant

•Newer therapyPEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 71

SUPPORTIVE TREATMENT

•Vitamin C – increases iron excretion

•Restrict Fe intake – decrease meat, liver, spinach

•Folate – 1 mg/day

•Genetic counselling

•Psychological support

•Hormonal therapy – GH, estrogen, testosterone, thyroxinPEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN,


PSYCHOLOGICAL/ SOCIAL SUPPORT

• WISH

• BLOOD DONATION

• MORAL SUPPORT

• CHELATION THERAPY


BLOOD TRANSFUSION

•BT at 4-6 wks interval

Packed RBC, leucocyte-poor•Hb to be maintained – (Hb >10.5 gm/dl)• If regular transfusions and chelation - no hepatomegaly, no

facies•10-15ml/kg RBC raises Hb by 3-5gm/dl –


CHELATION THERAPY

• ( 1 unit of blood contains 250 mg iron)

•DESFERRIOXAMINE

•DEFERIPRONE

•DEFERASIROX


BONE MARROW TRANSPLANTATION

• Severely affected child• Minimum transfusions • No iron toxicity • Age usually < 5 years• HLA identical donor (sib)

• Risk factors:

Hepatomegaly >2cm

Portal fibrosis

Iron overload

Older age

• Success rate In low risk young patients, the 5yr thalassemia free survival rate is 73 -94% at National Institute of Blood Disease & Bone Marrow Transplantation, Karach


SURGICAL TREATMENT - SPLENECTOMY

•Deferred as long as possible. At least till 5-6 yrs age

•Spleenectomy (indications):

•Hyperspleenism (spleenomegaly+mono/bi/pancytopenia)

•Massive splenomegaly causing mechanical discomfort

•Progressively increasing blood transfusion requirements (>180-200 ml/kg/yr) packed RBC


NEWER THERAPIES

•GENE MANIPULATION AND REPLACEMENT

• Hb F AUGEMENTATION

• Hydroxyurea

• Myelaran

• Butyrate derivatives

•


PRENATAL DIAGNOSIS

• β/α ratio: <0.025 in fetal blood – Thal. major

•Chorionic villous biopsy at 10-12 wks

• amniocentesis at 15-18th wk gestation Analysis of fetal DNA

• PCR to detect β globin gene


PREVENTION:

•Preventing marriage b/w traits

•Antenatal diagnosis

•Termination of pregnancy if Thalassemia major


COMPLICATIONS

•Hypersplenism ------- surgery• Bleeding Diathesis--- FFP•Hemosiderosis ------- chelation therapy•Hepatitis and hepatic encephalopathy• Pathological Fractures •Growth Retardation •Diabetes Mellitus• Failure to attain Puberty•Complications of Repeated Blood Transfusions PEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 81

COMPLICATIONS….. continued

• Repeated infections

• Cardiac complications (Anemia , Hemosiderosis)

• Pericarditis

• Arrythmias

• Heart Block • CCF


MANAGEMENT OF HEPATIC ENCEPHALOPATHY

• MAINTENANCE OF ABC and monitoring of vitals and protein restriction

• NG tube and foley catheter passed

• Gastric lavage if malena or haemetemesis

• I/V antibiotics for infection

• Lactulose to change gut flora

• Enema for constipation

• Hydration

• Vit. K

• Oral Aminoglycoside / Metronidazole

• H2 blocker/ PPI

• Blood transfusion if there is anemiaPEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 83

HEPATORENAL SYNDROME

• Hepatic failure, hypernatremia, hypokalemia, oliguria, deranged RFTs

• Two types

type 1 – acute

type II -- chronic

• Withdrawal of nephrotoxic drugs

• Renal function recovers if hepatic function improves


WHAT WE CAN DO FOR THIS PATIENT FURTHER• Chelation therapy

• Hb F augmentation

• Spleenectomy and vaccination

• Bone marrow transplantation

• Prevent other complications of thalassemia


CAUSES OF DEATH IN THALASSEMICS• The most common cause of death in older children was

heart disease(cardiomyopathy)

followed by infection &liver disease.

•While in younger children

infections outnumber the cardiac complications.


KEY MESSAGE

•Prevention is better than cure

• Better to prevent thalassemia.

• IF not, Better to prevent complications of thalassemia.

• How:

By monitoring patients clinically & by investigations in

order to detect earlier ,so intervention can be done.PEDIATRIC DEPARTMENT, SHEIKH ZAYED MEDICAL COLLEGE/HOSPITAL, RAHIM YAR KHAN, PUNJAB, PAKISTAN Saturday, February 8, 2014 87

WHAT WE AIM FOR THALASSEMIA


.

It was like a dream come true for 12-year old Naima Gul, resident of Mingora, Swat, when she became the first female pilot of the Pakistan.


thalassemia cpc

Health & Medicine