Guidelines for the management of
part of the management of acute joint bleeding
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part of the management of acute joint bleeding
episodes. (Level 2) [4,6,7] ■ As soon as the pain and swelling begin to subside, the patient should be encouraged to change the position of the affected joint from a position of comfort to a position of function, gradually decreasing the flexion of the joint and striving for complete extension. ■ This should be done as much as possible with active muscle contractions. Gentle passive assistance may be used initially and with caution if muscle inhibition is present. ■ Early active muscle control must be encour- aged to minimize muscle atrophy and prevent chronic loss of joint motion. ■ Active exercises and proprioceptive training must be continued until complete pre-bleed joint range of motion and functioning are restored and signs of acute synovitis have dissi- pated [8]. ■ If exercises are progressed judiciously, factor replacement is not necessarily required before exercising. TABLE 5-1: DEFINITION OF RESPONSE TO TREATMENT OF ACUTE HEMARTHROSIS [1] Excellent Complete pain relief within 8 hours and/or complete resolution of signs of bleeding after the initial injection and not requiring any further replacement therapy within 72 hours. Good Significant pain relief and/or improvement in signs of bleeding within approximately 8 hours after a single injection, but requiring more than one dose of replacement therapy within 72 hours for complete resolution. Moderate Modest pain relief and/or improvement in signs of bleeding within approximately 8 hours after the initial injection and requiring more than one injection within 72 hours but without complete resolution. None
No or minimal improvement, or condition worsens, within approximately 8 hours after the initial injection. Note: The above definitions of response to treatment of an acute hemarthrosis relate to inhibitor negative individuals with hemophilia. These definitions may require modification for inhibitor positive patients receiving bypassing agents as hemostatic cover or patients who receive factor concentrates with extended half-lives. Arthrocentesis 1. Arthrocentesis (removal of blood from a joint) may be considered in the following situations: ■ a bleeding, tense, and painful joint which shows no improvement 24 hours after conser- vative treatment ■ joint pain that cannot be alleviated ■ evidence of neurovascular compromise of
■ unusual increase in local or systemic temper- ature and other evidence of infection (septic arthritis) (Level 3) [4,9,10] 2. Inhibitors should be considered as a reason for persistent bleeding despite adequate factor replacement. The presence of inhibitors must be ruled out before arthrocentesis is attempted.
TREATMENT OF SPECIFIC HEMORRHAGES 49 3. The early removal of blood should theoretically reduce its damaging effects on the articular carti- lage [10]. If there is a large accumulation of blood, it will also decrease pain. 4. Arthrocentesis is best done soon after a bleed under strictly aseptic conditions. 5. When necessary, arthrocentesis should be performed under factor levels of at least 30–50 IU/dl for 48–72 hours. Arthrocentesis should not be done in circumstances where such factor replacement is not available. In the presence of inhibitors, other appropriate hemostatic agents should be used for the procedure, as needed. (Level 3) [4] 6. A large bore needle, at least 16-gauge, should be used. 7. The joint should be immobilized with mild compression. 8. Weight-bearing should be avoided for 24–48 hours. 9. Physiotherapy should be initiated as described above. 5.2 Muscle hemorrhage 1. Muscle bleeds can occur in any muscle of the body, usually from a direct blow or a sudden stretch. 2. A muscle bleed is defined as an episode of bleeding into a muscle, determined clinically and/or by imaging studies, generally associated with pain and/or swelling and functional impair- ment e.g. a limp associated with a calf bleed [1]. 3. Early identification and proper management of muscle bleeds are important to prevent perma- nent contracture, re-bleeding, and formation of pseudotumours. 4. Sites of muscle bleeding that are associated with neurovascular compromise, such as the deep flexor muscle groups of the limbs, require imme- diate management to prevent permanent damage and loss of function. These groups include: ■ the iliopsoas muscle (risk of femorocutaneous, crural, and femoral nerve palsy) ■ the superior-posterior and deep posterior compartments of the lower leg (risk of poste- rior tibial and deep peroneal nerve injury) ■ the flexor group of forearm muscles (risk of Volkmann’s ischemic contracture) 5. Bleeding can also occur in more superficial muscles such as the biceps brachii, hamstrings (triceps surae), gastrocnemius, quadriceps, and the gluteal muscles. 6. Symptoms of muscle bleeds are: ■ aching in the muscle ■ maintenance of the limb in a position of comfort
■ severe pain if the muscle is stretched ■ pain if the muscle is made to actively contract ■ tension and tenderness upon palpation and possible swelling 7. Raise the patient’s factor level as soon as
8. Rest the injured part and elevate the limb. 9. Splint the muscle in a position of comfort and adjust to a position of function as pain allows. 10. Ice/cold packs may be applied around the muscle for 15-20 minutes every four to six hours for pain relief if found beneficial. Do not apply ice in direct contact with skin. 11. Repeat infusions are often required for two to
GUIDELINES FOR THE MANAGEMENT OF HEMOPHILIA 50 12. The patient should be monitored continuously for neurovascular compromise; fasciotomy may be required in some such cases. (Level 5) [16,17] 13. Hemoglobin level should be checked and corrected if needed as muscle bleeds can result in significant blood loss. 14. Physiotherapy should begin as soon as pain
15. Factor coverage during this process is prudent, unless the physiotherapist is experienced with hemophilia management. Serial casting or splinting may be required. Supportive bracing will be required if there has been nerve damage. 16. Increasing pain during physical therapy can suggest re-bleeding and should be regularly eval- uated [19].
1. This type of muscle hemorrhage has a unique presentation. Signs may include pain in the lower abdomen, groin, and/or lower back and pain on extension, but not on rotation, of the hip joint. There may be paresthesia in the medial aspect of the thigh or other signs of femoral nerve compres- sion such as loss of patellar reflex and quadriceps weakness. The symptoms may mimic acute appendicitis, including a positive Blumberg’s sign. 2. Immediately raise the patient’s factor level.
3. Hospitalize the patient for observation and control of pain. Maintain strict bed rest. Ambulation with crutches is not permitted, as ambulation requires contraction of the muscle. (Level 4) [20-22] 4. It is useful to confirm the diagnosis and monitor recovery with an imaging study (ultrasonog- raphy, CT scan, or MRI). (Level 4) [20-22] 5. Limit the patient’s activity until pain resolves and hip extension improves. A carefully supervised program of physiotherapy is key to restoring full activity and function and preventing re-bleeding. Restoration of complete hip extension before returning to full activity is recommended. (Level 4) [20-22] 6. If residual neuromuscular deficits persist, further orthotic support may be necessary. 5.3 Central nervous system hemorrhage/head trauma 1. This is a medical emergency. Treat first before evaluating. 2. All post-traumatic head injuries, confirmed or suspected, and significant headaches must be treated as intracranial bleeds. Sudden severe pain in the back may be associated with bleeding around the spinal cord. Do not wait for further symptoms to develop or for laboratory or radio- logic evaluation. 3. Immediately raise the patient’s factor level when significant trauma or early symptoms occur. Further doses will depend on imaging results. Maintain factor level until etiology is defined. If a bleed is confirmed, maintain the appropriate factor level for 10-14 days (refer to Tables 7-1 and 7-2). (Level 4) [23,24] 4. Intracranial hemorrhage may be an indication for prolonged secondary prophylaxis (three to six months), especially where a relatively high risk of recurrence has been observed (e.g. in the presence of HIV infection). (Level 3) [23,25,26] 5. Immediate medical evaluation and hospitaliza- tion is required. A CT scan or MRI of the brain should be performed. Neurological consulta- tion should be sought early. (Level 4) [27,28] 6. Severe headache may also be a manifestation of meningitis in immunocompromised patients.
TREATMENT OF SPECIFIC HEMORRHAGES 51 5.4 Throat and neck hemorrhage 1. This is a medical emergency because it can lead to airway obstruction. Treat first before evaluating. 2. Immediately raise the patient’s factor level
3. Hospitalization and evaluation by a specialist is essential. (Level 5) [15] 4. To prevent hemorrhage in patients with severe tonsillitis, treatment with factor may be indicated, in addition to bacterial culture and treatment with appropriate antibiotics. 5.5 Acute gastrointestinal (GI) hemorrhage 1. Immediately raise the patient’s factor levels.
2. Acute gastrointestinal hemorrhage may present as hematemesis, hematochezia, or malena. 3. For signs of GI bleeding and/or acute hemor- rhage in the abdomen, medical evaluation and possibly hospitalization are required. 4. Hemoglobin levels should be regularly moni- tored. Treat anemia or shock, as needed. 5. Treat origin of hemorrhage as indicated. 6. EACA or tranexamic acid may be used as adjunc- tive therapy for patients with FVIII deficiency and those with FIX deficiency who are not being treated with prothrombin complex concentrates. 5.6 Acute abdominal hemorrhage 1. An acute abdominal (including retroperitoneal) hemorrhage can present with abdominal pain and distension and can be mistaken for a number of infectious or surgical conditions. It may also present as a paralytic ileus. Appropriate radio- logic studies may be necessary. 2. Immediately raise the patient’s factor levels.
5.7 Ophthalmic hemorrhage 1. This is uncommon unless associated with trauma or infection. 2. Immediately raise the patient’s factor level.
3. Have the patient evaluated by an ophthalmolo- gist as soon as possible.
GUIDELINES FOR THE MANAGEMENT OF HEMOPHILIA 52 5.8 Renal hemorrhage 1. Treat painless hematuria with complete bed rest and vigorous hydration (3 litres/m 2 body surface area) for 48 hours. Avoid DDAVP when hydrating intensively. (Level 4) [33] 2. Raise the patient’s factor levels (refer to Tables 7-1 and 7-2) if there is pain or persistent gross hematuria and watch for clots and urinary obstruction. (Level 4) [33,34] 3. Do not use antifibrinolytic agents. (Level 4) [33] 4. Evaluation by an urologist is essential for eval- uation of a local cause if hematuria (gross or microscopic) persists or if there are repeated episodes. 5.9 Oral hemorrhage 1. Early consultation with a dentist or oral and maxillofacial surgeon is essential to determine the source of bleeding. The most common causes are: ■ dental extraction ■ gingival bleeding often due to poor oral hygiene ■ trauma 2. Local treatments must be considered to treat the hemorrhage. These may include: ■ direct pressure on the area using a damp gauze swab, maintained for at least 15 minutes ■ sutures to close the wound ■ application of local hemostatic agents ■ antibiotics, especially in gingival bleeding due to poor oral hygiene ■ use of EACA or tranexamic acid as a mouth- wash
3. An appropriate dose of regular paracetamol/acet- aminophen will help control the pain. 4. Antifibrinolytic agents should not be used
5. Factor replacement may be required as directed by the hemophilia centre. 6. Oral EACA or tranexamic acid should be used if appropriate. (Level 4) [37,38] 7. Advise the patient to avoid swallowing blood. 8. Advise the patient to avoid using mouthwashes until the day after the bleeding has stopped. 9. Advise the patient to eat a soft diet for a few days. 10. Evaluate and treat for anemia as indicated. 5.10 Epistaxis 1. Place the patient’s head in a forward position to avoid swallowing blood and ask him to gently blow out weak clots. Firm pressure with gauze soaked in ice water should be applied to the ante- rior softer part of the nose for 10-20 minutes. 2. Factor replacement therapy is often not neces- sary unless bleeding is severe or recurrent [15,29]. 3. Antihistamines and decongestant drugs are useful for bleeds specifically related to allergies, upper respiratory infections, or seasonal changes. 4. If bleeding is prolonged or occurs frequently, eval- uate for anemia and treat appropriately. 5. EACA or tranexamic acid applied locally in a soaked gauze is helpful.
TREATMENT OF SPECIFIC HEMORRHAGES 53 6. Consult with an otolaryngologist if the bleed is persistent or recurrent. Anterior or posterior nasal packing may be needed to control bleeding. 7. Epistaxis can often be prevented by increasing the humidity of the environment, applying gels (e.g. petroleum jelly or saline drops/gel) to the nasal mucosa to preserve moisture, or adminis- tering saline spray. 5.11 Soft tissue hemorrhage 1. Symptoms will depend on the site of hemorrhage. 2. Factor replacement therapy is not necessary for most superficial soft tissue bleeding. The appli- cation of firm pressure and ice may be helpful [15,29]. 3. Evaluate the patient for severity of hemor- rhage and possible muscular or neurovascular involvement. Rule out possible trauma to spaces containing vital organs, such as the head or abdomen. 4. Open compartmental hemorrhage, such as in the retroperitoneal space, scrotum, buttocks, or thighs, can result in extensive blood loss. Treat with factor immediately if this situation is suspected. 5. Hemoglobin levels and vital signs should be regu- larly monitored. 5.12 Lacerations and abrasions 1. Treat superficial lacerations by cleaning the wound, then applying pressure and steri-strips. 2. For deep lacerations, raise the factor level (refer
3. Sutures may be removed under cover of factor concentrate. References 1. Definitions in hemophilia. Recommendations of the scientific subcommittee on factor VIII and factor IX of the scientific and standardization committee of the International Society on Thrombosis and Haemostasis. JTH 2012 (in press). 2. Aronstam A, Wassef M, Choudhury DP, Turk PM, McLellan DS. Double-blind controlled trial of three dosage regimens in treatment of haemarthroses in haemophilia A. Lancet 1980 Jan 26;1(8161):169-71. 3. Aronstam A, Wassef M, Hamad Z, Cartlidge J, McLellan D. A double-blind controlled trial of two dose levels of factor VIII in the treatment of high risk haemarthroses in haemophilia A. Clin Lab Haematol 1983;5(2):157-63. 4. Hermans C, de Moerloose P, Fischer K, Holstein K, Klamroth R, Lambert T, et al; European Haemophilia Therapy Standardisation Board. Management of acute haemarthrosis in haemophilia A without inhibitors: literature review, European survey and recommendations. Haemophilia 2011;17(3):383-92. 5. Mathews V, Viswabandya A, Baidya S, George B, Nair S, Chandy M, Srivastava A. Surgery for hemophilia in developing countries. Semin Thromb Hemost 2005 Nov;31(5):538-43. 6. Gomis M, Querol F, Gallach JE, Gonzalez LM, Aznar JA. Exercise and sport in the treatment of haemophilic patients: a systematic review. Haemophilia 2009;15(1):43-54. 7. Mulder K. Exercises for People with Hemophilia. Montreal: World Federation of Hemophilia 2006. 8. Heijnen L, Buzzard BB. The role of physical therapy and rehabilitation in the management of hemophilia in developing countries. Semin Thromb Hemost 2005;31(5):513-7.
GUIDELINES FOR THE MANAGEMENT OF HEMOPHILIA 54 9. Ingram GI, Mathews JA, Bennett AE. Controlled trial of joint aspiration in acute haemophilic haemarthrosis. Ann Rheum Dis 1972;31:423. 10. Rodriguez-Merchan EC. Aspects of current management: orthopaedic surgery in haemophilia. Haemophilia 2012;18(1):8-16. 11. Aronstam A, Browne RS, Wassef M, Hamad Z. The clinical features of early bleeding into the muscles of the lower limb in severe haemophiliacs. J Bone Joint Surgery 1983;65-B(1):19-23. 12. Beyer R, Ingerslev J, Sørensen B. Current practice in the management of muscle haematomas in patients with severe haemophilia. Haemophilia 2010;16(6):926-31. 13. Railton GT, Aronstam A. Early bleeding into upper limb muscles in severe haemophilia clinical features and treatment. J Bone Joint Surgery 1987;69-B(1):100-102. 14. Rodriguez-Merchan EC. Musculoskeletal complications of hemophilia. HSSJ 2010;6:37–42. 15. Singleton T, Kruse-Jarres R, Leissinger C. Emergency department care for patients with hemophilia and von Willebrand disease. J Emerg Med 2010 Aug;39(2):158-65. 16. Llinás A, Silva M, Pasta G, Luck JV, et al. Controversial subjects in musculoskeletal care of haemophilia: cross fire. Haemophilia 2010;16(Suppl 5):132-5. 17. Rodriguez-Merchan EC. Orthopedic management in hemophilia: a Spanish outlook. Semin Hematol 2008;45(2 Suppl 1):S58-63. 18. Blamey G, Forsyth A, Zourikian N, et al. Comprehensive elements of a physiotherapy exercise programme in haemophilia—a global perspective. Haemophilia 2010;16(Suppl 5):136-45. 19. Beeton K, Cornwell J, Alltree J. Rehabilitation of muscle dysfunction in hemophilia, 2 nd edn. World Federation of Hemophilia Treatment of Hemophlia monograph 24. Montreal: World Federation of Hemophilia, 2012. 20. Ashrani AA, Osip J, Christie B, Key NS. Iliopsoas haemorrhage in patients with bleeding disorders- -experience from one centre. Haemophilia 2003;9(6):721-6. 21. Balkan C, Kavakli K, Karapinar D. Iliopsoas haemorrhage in patients with haemophilia: results from one centre. Haemophilia 2005;11(5):463-7. 22. Fernandez-Palazzi F, Hernandez SR, De Bosch NB, De Saez AR. Hematomas within the iliopsoas muscles in hemophilic patients: the Latin American experience. Clin Orthop Relat Res 1996;(328):19-24. 23. Ljung RC. Intracranial haemorrhage in haemophilia A and B. Br J Haematol 2008;140(4):378-84. 24. Nakar C, Cooper DL, DiMichele D. Recombinant activated factor VII safety and efficacy in the treatment of cranial haemorrhage in patients with congenital haemophilia with inhibitors: an analysis of the Hemophilia and Thrombosis Research Society Registry (2004-2008). Haemophilia 2010;16(4):625-31. 25. Patiroglu T, Ozdemir MA, Unal E, Altuner Torun Y, Coskun A, Menku A, Mutlu FT, Karakukcu M. Intracranial hemorrhage in children with congenital factor deficiencies. Childs Nerv Syst 2011;27(11):1963-6. 26. Zanon E, Iorio A, Rocino A, Artoni A, Santoro R, Tagliaferri A, Coppola A, Castaman G, Mannucci PM; the Italian Association of Hemophilia Centers. Intracranial haemorrhage in the Italian population of haemophilia patients with and without inhibitors. Haemophilia 2012;18(1):39-45. 27. Traivaree C, Blanchette V, Armstrong D, et al. Intracranial bleeding in haemophilia beyond the neonatal period--the role of CT imageing in suspected intracranial bleeding. Haemophilia 2007;13(5):552-9. 28. Witmer CM, Manno CS, Butler RB, Raffini LJ. The clinical management of hemophilia and head trauma: a survey of current clinical practice among pediatric hematology/oncology physicians. Pediatr Blood Cancer 2009;53(3):406-10. 29. Bush MT, Roy N. Hemophilia emergencies. J Emerg Nurs 1995 Dec;21(6):531-8. 30. Guthrie TH Jr, Sacra JC. Emergency care of the hemophiliac patient. Ann Emerg Med 1980 Sep;9(9):476-9. 31. Kouides PA, Fogarty PF. How do we treat upper gastrointestinal bleeding in adults with haemophilia. Haemophilia 2010;16(2):360-2. 32. Mittal R, Spero JA, Lewis JH, Taylor F, Ragni MV, Bontempo FA, Van Thiel DH. Patterns of gastrointestinal hemorrhage in hemophilia. Gastroenterology 1985;88(2):515-22. 33. Quon DV, Konkle BA. How we treat haematuria in adults with haemophilia. Haemophilia 2010;16(4):683-5. 34. Ghosh K, Jijina F, Mohanty D. Haematuria and urolithiasis in patients with haemophilia. Eur J Haematol 2003;70(6):410-2. 35. Kane MJ, Silverman LR, Rand JH, Paciucci PA, Holland JF. Myonecrosis as a complication of the use of epsilon amino-caproic acid: a case report and review of the literature. Am J Med 1988 Dec;85(6):861-3. 36. Mannucci PM. Hemostatic drugs. N Engl J Med 1998 Jul 23;339(4):245-53. 37. Franchini M, Rossetti G, Tagliaferri A, et al. Dental procedures in adult patients with hereditary bleeding disorders: 10 years experience in three Italian Hemophilia Centers. Haemophilia 2005;11:504–9. 38. Vinall C, Stassen LF. The dental patient with a congenital bleeding disorder. J Ir Dent Assoc 2008 Feb-Mar;54(1):24-8. 39. D’Young AI. Domiciliary application of CryoCuff in severe hemophilia: qualitative questionnaire and clinical audit. Haemophilia 2008; 14:823-7.
55 6.1 Musculoskeletal complications 1. The most common sites of bleeding are the joints and muscles of the extremities. 2. Depending on the severity of the disease, bleeding episodes may be frequent and without apparent cause (see Table 1-1). 3. In the child with severe hemophilia, the first hemarthrosis typically occurs when the child begins to crawl and walk: usually before two years of age, but occasionally later. 4. If inadequately treated, repeated bleeding will lead to progressive deterioration of the joints and muscles, severe loss of function due to loss of motion, muscle atrophy, pain, joint deformity, and contractures within the first one to two decades of life [1,2]. Yüklə 0,65 Mb. Dostları ilə paylaş:
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