Hemophilic arthropathy

hemophilic arthropathy physical medicine and rehabilitation

1. HEMOPHILIC ARTHROPATHY SKNAYAK DNB PMR 2 ND YR

2. HEMOPHILIA X LINKED RECESSIVE BLEEDING DISORDER. CAUSED BY DEFICIENCY OF CLOTTING FACTORS- FACTOR VIII (HEMOPHILIA A) FACTOR IX (HEMOPHILIA B) PATIENTS WITH HEMOPHILIA (PWH)- 85%- HEM-A, 15%- HEM-B

3. OTHER TYPES OF BLEEDING DISORDERS VON WILLEBRAND DISEASE OTHER FACTOR DEFICIENCIES PREVALENCE OF HEM-A- 1 IN 5000 BIRTHS HEM-B- 1 IN 30000 BIRTHS.

4. SEVERITY GRADING DEPENDING UPON THE BASAL LEVEL OF FACTORS. MILD- 6-40% MODERATE- 1-5% SEVERE- LESS THAN 1%

5. COAGULATION CASCADE

6. CLINICAL MENIFESTATIONS

7. SITES OF BLEEDING

8. DIAGNOSIS APPROPRIATE HISTORY AND CLINICAL FEATURES WITH DIFFERENTIAL DIAGNOSIS. SCREENING TESTS- PLATELET COUNT, BT, PT, APTT. PREFERRED TEST IS PLATELET AGGREGOMETRY. CONFIRMATION BY FACTOR ASSAYS.

9. MANAGEMENT ACUTE BLEED SHOULD BE MANAGED WITHIN 2HRS. DESMPRESSIN (DDAVP) RISES FACTOR VIII BY SIX TO EIGHT TIMES FACTOR REPLACEMENT THERAPY

10. JOINT HEMORRHAGES CHARACTERISED BY RAPID LOSS OF ROM AS COMPARED TO THE BASELINE THAT IS ASSOCIATED WITH ANY OF THE FOLLOWING PAIN OR UNUSUAL SENSATION IN THE JOINT AT THE END OF THE RANGE PLAPABLE SWELLING, AND WARMTH OVER THE SKIN OVER JOINT.

11. REBLEEDING- WORSENING OF THE CONDITION EITHER ON TREATMENT OR WITHIN 72HRS AFTER STOPPING THE TRETMENT. TARGET JOINT- A JOINT WITH 3 OR MORE SPONTANEOUS BLEEDS HAVE OCCURED WITHIN A CONSECUTIVE 6 MONTHS

12. THREE STAGES OF HEMARTHROSIS- ACUTE- NO CLINICALLY DETECTABLE SEQUELAE SUBCUTE- DETECTABLE JOINT DAMAGE FOLLOWING REPEATED EPISODES OF HEMARTHROSIS CHRONIC ARTHROPATHY- SIGNIFICANT LOSS OF MUSCLE FUNCTION AND MUSCLE TENDON CONTRACTURE FOLLOWING REPEATED BLEEDING EPISODES

13. ACUTE HEMARTHROSIS PAIN WITH RESTRICTED ROM WITH SWOLLEN AND TENDER JOINT RELIEVED ON REPLACEMENT THERAPY. NO CLINICALLY DETECTED SEQUELAE OF DAMAGE TO BONE OR MUSCLE.

14. SUBACUTE HEMARTHROSIS FORMATION OF TARGET JOINT CLINICAL SIGNS OF JOINT DAMAGE BETWEEN THE BLEEDING EPISODES.

15. CHRONIC ARTHROPATHY ALSO KNOWN AS HEMOPHIILIC ARTHROPATHY. DESTRUCTIVE AND PROLIFERATIVE SYNOVITIS. IT MAY BE KNEE, ANKLE AND ELBOW.

16. CHRONIC KNEE ARTHROPATHY HYPERTROPHY DUE TO SYNOVITIS AND EFFUSION RESTRICTED ROM GENU VALGUM AND LATERAL AND POSTERIOR SUBLUXATION

17. CHRONIC ANKLE ARTHROPATHY TIBIOTLAR AND OR SUBTALAR JOINT. RESTRICTED ROM AT THE ANKLE AND FOOT. WEIGHT BEARING IS PAINFUL. ON MRI- ASEPTIC TALAR DOME NECROSIS

18. CHRONIC ELBOW ARTHROPATHY RESTRICTED ROM AT THE ELBOW AND RUJ. NEUROLOGICAL SYMPTOMS DUE TO ULNAR NERVE ENTRAPMENT

19. PATHOPHYSIOLOGY

20. RADIOLOGICAL FEATURES OF CHRONIC ARTHROPATHY ARNOLD HILL GARTNER STAGING XRAY- STAGE 0- NORMAL STAGE I- NO SKELETAL ABNORMALITY, SOFT TISSUE SWELLING STAGE II- OSTEOPOROSIS AND OVERGROWTH OF EPIPHYSIS, NO CYST, NO NARROWING OF THE CARTILAGE SPACE STAGE III- EARLY SUBCHONDRAL BONE CYST, SQUARING OF PATELLA, PRESERVATION OF CARTILAGE SPACE STAGE IV- MORE ADVANCED FINDINGS OF STAGE III. STAGE V- FIBROUS JOINT, CONTRACTURE, DISORGANISED JOINT

21. PETTERSSON SCORE

22. ACUTE HEMARTHROSIS FACTOR REPLACEMENT SHOULD BE STARTED WITHIN TWO HOURS. FACTOR VIII AND IX- 25-40 IU/KG RICE REGIME IF BLEEDING DOESN’T STOP- REPEAT HALF THE DOSE OF LOADING DOSE IN 12 HRS IN HEM-A & IN 24HRS IN HEM B.

23. AFTER THE RELIEF OF PAIN AND SWELLING- CHANGING THE POSITION OF THE LIMB FROM COMFORT TO FUNCTIONAL POSITION WITH GENTLE PASSIVE ASSISTANCE. EARLY ACTIVE MUSCLE CONTROL. LOW INTENSITY STRETCHING EXERCISES PROPRIOCEPTION AND ACTIVE EXERCISES. USE OF CRUTCHES IN DIFFICULTY IN WEIGHT BEARING (ILIOPSOAS HEMATOMA)

24. INDICATION OF ARTHROCENTESIS- NO IMPROVEMENT AFTER 24 HRS JOINT PAIN THAT CAN NOT BE ALLEVIATED EVIDENCE OF NEUROVASCULAR COMPROMISE UNUSUAL INCREASE IN TEMP. OR SYSTEMIC EVIDENCE OF INFECTION(SEPTIC ARTHRITIS)

25. CHRONIC ARTHROPATHY CONSERVATIVE – PHYSIOTHERAPY, ORTHOTICS SLOW AND STEADY ISOMETRICS NO GAIN WITH PAIN- REANALYSE THE STATUS OF MUSCLE, LOOK FOR NEURO DEFICITS IF ANY. EXERCISES ARE TO BE INDIVIDUALISED. USING THE BODY WEIGHT AND GRAVITY AS RESISTANCE

26. EVERYDAY WALKING, CYCLING, CLIMBING ARE HELPFUL. SWIMMING AND OTHER NONCONTACT SPORTS. HYDROTHERAPY IS USEFUL IN MULTIPLE JOINT INVOLVEMENT.

27. SPLINTING AND ORTHOSES TO IMMOBILISE THE JOINT PREVENT DEFORMITY MAINTAINING THE ROM IMPROVE JOINT FUNCTION SUPPORT THE WEAK STRUCTURE CORRECTION OF CONTRACTURE

28. QUENGEL CAST DROP OUT CAST TRACTION WITH THOMAS SPLINT WITH SPLIT RING

29. QUENGEL CAST

30. DROP OUT CAST

31. ASSISTIVE DEVICES- CRUTCHES, CANES OR WALKER AFTER EVALUATION OF WEIGHT BEARING STATUS OF THE PATIENT.

32. SYNOVECTOMY RADIONUCLIDE SYNOVECTOMY CHEMICAL SYNOVECTOMY ARTHROSCOPIC OR OPEN

33. OTHER INTERVETIONS- HYALURONIC ACID, ARTHRODESIS TOTAL JOINT REPLACEMENT

34. REFERENCES WORLD FEDERATION OF HEMOPHILIA, JOURNAL OF BLOOD MEDICINE

35. THANK YOU