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T R E A T M E N T O F H E M O P H I L I AJUNE 2004 NO

THE TREATMENT OFHEMOPHILIA BLEEDINGWITH LIMITED RESOURCES

Revised Edition

Shelby L. DietrichHuntington Memorial Hospital Hemophilia CenterCalifornia, U.S.A.


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Published by the World Federation of Hemophilia (WFH); 1996, revised 2004.

World Federation of Hemophilia, 2004

The WFH encourages redistribution of its publications for educational purposes by not-for-profit

hemophilia organizations. In order to obtain permission to reprint, redistribute, or translate thispublication, please contact the Communications Department at the address below.

This publication is accessible from the World Federation of Hemophilias web site at www.wfh.org.Additional copies are also available from the WFH at:

World Federation of Hemophilia1425 Ren Lvesque Boulevard West, Suite 1010Montral, Qubec H3G 1T7CANADATel. : (514) 875-7944Fax : (514) 875-8916

E-mail: [email protected]: www.wfh.org

The Treatment of Hemophilia series is intended to provide general information on the treatment andmanagement of hemophilia. The World Federation of Hemophilia does not engage in the practice ofmedicine and under no circumstances recommends particular treatment for specific individuals. Doseschedules and other treatment regimes are continually revised and new side-effects recognized. WFHmakes no representation, express or implied, that drug doses or other treatment recommendations in thispublication are correct. For these reasons it is strongly recommended that individuals seek the advice of amedical adviser and/or to consult printed instructions provided by the pharmaceutical company beforeadministering any of the drugs referred to in this monograph.

Statements and opinions expressed here do not necessarily represent the opinions, policies, orrecommendations of the World Federation of Hemophilia, its Executive Committee, or its staff.

Treatment of Hemophilia MonographsSeries Editor: Dr. Sam Schulman


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Table of Contents

Introduction................................................................................................................................................................ 1

Clinical Situations with External Blood Loss......................................................................................................... 1

Minor and Recurrent Joint Bleeds (Hemarthroses)............................................................................................... 2

Special Notes .............................................................................................................................................................. 3

In Conclusion ............................................................................................................................................................. 3

Minimal Replacement Therapy ............................................................................................................................... 3

Exercises for the Knee ............................................................................................................................................... 4

Exercises for the Ankle.............................................................................................................................................. 6

Exercises for the Elbow............................................................................................................................................. 9


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The Treatment of Hemophilia Bleedingwith Limited Resources

Shelby L. Dietrich

Introduction

Although the fundamental treatment ofhemophilia bleeding problems is replacementtherapy of the missing coagulation factor,nevertheless in many places and on manyoccasions adequate replacement therapy(clotting factor concentrates, fresh frozenplasma, cryoprecipitate) is not available, butproper and prompt use of conservative

measures that decrease or control the bleedingand prevent future damage may be employed.

This article will describe those measures withspecific application to hemophilia-relatedcommon bleeding problems. It is helpful torecall that bleeding in hemophilia is generallyslow, with continuing ooze because of poor clotformation; this principle enables one to applyconservative measures, stop the bleeding, andpermit tissue repair. In essence one can buy time.

The goals of treatment are identical whetherclotting factor replacement therapy is available ornot, to minimize permanent damage, minimizesuffering and pain, permit tissue healing, andrestore function. If even minimal clotting factorreplacement therapy is available (cryoprecipitatewhich contains Factor VIII only, fresh frozenplasma which contains both factor VIII and factorIX, or specific factor concentrates), then thistherapy should be administered as promptly aspossible when the bleeding episode begins or isrecognized. Early use of replacement therapy,even in minimal amounts, produces better

hemostasis than delayed use.

Clinical Situations with ExternalBlood Loss

External blood loss results from:

lacerations or abrasions of the skin;

oral mucosal bleeding from dentalproblems, from the mucosa of the mouthand from cuts or scrapes to the tongue;

nose bleeds (epistaxes); gastrointestinal bleeding; and kidney bleeding.General measures which can be applied in mostof these situations excepting GI and GU bleedsare the use of firm, constant pressure, elevationof the head when applicable, and application ofice.

a) In general, superficial skin lacerations whichin a normal individual would requiresuturing or use of sterile adhesive stripsmay be treated in the same manner in aperson with hemophilia. Significantlacerations should be immediately sutured,a dose of replacement therapy given (ifavailable), and ice, pressure, and elevationapplied consistently for as long as possible.

In general, skin lacerations which in anormal individual would require suturingor use of sterile gauze strips may be treatedin the same manner in a person withhemophilia. Ice should not be applieddirectly to the skin; ice packs must bewrapped in a thick towel since ice burns andskin damage can occur with prolongedapplication. Ice is effective in stoppingbleeding when used promptly and early butdoes not assist in the resolution of oldhematomas. In toddlers and small children,

small scalp lacerations are often the site offormation of exuberant clots; if such asituation occurs with repeated oozing andenlargement of clots, gentle removal of theclot should be undertaken in order toapproximate the skin edges, permitapplication of pressure, and allow healing.


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2 The Treatment of Hemophilia Bleeding with Limited Resources

b) A retained friable berry-like clot in themouth impedes healing and allows slowbleeding to continue. Continuing ooze fromloose deciduous (baby) teeth is a nuisanceand occasionally the cause of significantblood loss. The loose tooth should be gently

but expeditiously extracted and pressureand ice applied to the bleeding site forseveral hours. The loose tooth acts as aforeign body and irritant to the gingiva.

c) Continued oozing from tongue lacerationscan ultimately result in significant bloodloss, and if such bleeding continues, thechild's hemoglobin should be checked, andif significant anemia has occurred,transfusion of blood (as fresh as possible) isindicated. This situation is particularly

dangerous in infants and small childrenwho may suffer considerable blood loss inproportion to their total circulating bloodvolume with progressive anemia resultingin cardiovascular compromise. Diet forchildren with bleeding from the oral cavityshould consist of cold or chilled and softfoods. Use of straws should be prohibited toavoid dislodging the formed clot. Excessiveuse of the nursing bottle is also discouraged,since the nipple of the bottle may dislodge aclot.

d) Significant epistaxis (nose bleed) is rare inthe person with hemophilia compared to theperson with von Willebrand Disease andcan usually be successfully treated with restwith the head slightly elevated (to 30%), iceto the nose, and if necessary, packing of theanterior nasal cavities; packing, however,must be done with care. Gentle pressureshould be applied to the nares. Childrenoften swallow blood from nose bleeds andlater vomit the blood; they can havesignificant blood loss in this fashion.

Ingested and digested blood may causeblack (tarry) stools. In small children,hemoglobin and hematocrit should bemeasured frequently in the face of ongoingooze or bleeding.

e) Bleeding from the gastrointestinal tract,whether upper or lower, indicates the needfor medical consultation; blood in the urineor hematuria, on the other hand, can be

treated with watchful waiting and a highfluid intake (150-200 ml/hr) to prevent clotobstruction in the ureters. Even withoutreplacement therapy a daily dose ofprednisone for three to five days is oftenhelpful in stopping GU bleeding.

f) Local/topical treatment: hemostasis ofminor lacerations and abrasions in themouth, including the tongue and gingiva,may be aided by application of topicalthrombin preparations or gauze soaked indilute epinephrine solutions (1-10,000aqueous epinephrine). Topical hemostaticpreparations can also be applied to skinabrasions.

Minor and Recurrent Joint Bleeds(Hemarthroses)

Minor and recurrent joint bleeds are thehallmark of hemophilia A and B and ultimatelylead to significant morbidity, impairment offunction, and joint damage. Prompt recognitionand treatment of joint bleeds is essential toprevent these long-term complications. Basicconservative principles of treatment are: rest inthe position of least pain, application of ice, andelevation and immobilization. In the upperextremity, bleeds in the wrist and elbow should

be treated with a sling. If cryoprecipitate or freshfrozen plasma is available, administer one dosewhen the bleed is recognized and a second dosein 24 hours. Whenever necessary, analgesiashould be used, acetaminophen or paracetamol,avoiding the use of aspirin-containingcompounds. When pain, stiffness, and limitationof range of motion subsequent to knee bleedshave subsided, it is critical to begin or resumestrengthening exercises to the musclesprotecting the knee. Strong muscles around theknee can prevent recurrent hemorrhages and

chronic synovitis. Active isometric resistiveexercise is possible without the use ofreplacement therapy. Similarly, gentle activeexercise and range of motion should beundertaken when elbow bleeds subside. The useof high-top boots or tennis shoes for recurrentankle bleeds, particularly in toddlers andchildren, is helpful.


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The Treatment of Hemophilia Bleeding with Limited Resources 3

Special Notes

a) Bleeding after inadvertent circumcision:Continue the use of topical pressure as longas feasible. Do not suture or cauterize!

b)

Sore throat and tonsillitis with or withoutcough: Tonsils and adenoids reach theirmaximum size during early childhood, andrespiratory infections are frequent. Inflamedtonsils can precipitate peritonsillar bleedingwith serious results. Similarly, continuedcoughing can cause pharyngeal and trachealbleeding. Antibiotics should be employedfreely together with a cool, liquid diet untilthe child recovers. If necessary to suppresscough, a codeine-containing cough medicineis efficacious. If a child has increasingdifficulty speaking or swallowing, suspector rule out pharyngeal, laryngeal, ortracheal bleeding.

c) Awareness of possibility of significant bloodloss within the retroperitoneum and thoracicand abdominal musculature: A significantamount of blood leading to anemia andeven shock can be lost within the largemuscles of the thorax and back and in theretroperitoneal space. Unexplained pallor,weakness, or lethargy and lower abdominalpain may lead to the diagnosis of such

problems and medical intervention.

d) Intramuscular deep hematomas of the calfand forearm: These are potentiallydangerous because of pressure on nervesand blood vessels, and must be treated withbed rest and elevation until the swellingsubsides.

In Conclusion

a) The dos of conservative treatment are:

For extremity bleeds, do rest in aposition of elevation where feasible,apply ice, and resume normal activitywhen pain and stiffness disappear withspecial attention to restoration of musclestrength.

Do be aware of the possibility andpotential of significant blood loss whereoozing is continuous over several daysin small children.

Do be extremely observant of a toddler'slevel of activity and do encourage

children and adolescents to report thefirst subjective symptom or objectivesign of joint bleeding in order topromptly administer conservativemeasure.

Do observe the child's body appearanceclosely during daily bath; look forasymmetry and swelling.

Do suture lacerations. Do exercise gradually after joint bleeds

subside; learn isometric exercises.Strong muscles protect joints.

b) Be aware of deep muscle hemorrhages inthe forearm and calf which can cause nerveentrapment and neurovascular compromiseand ultimate tissue death.

c) The donts of conservative treatment are:

Do not cauterize! Do not apply constricting circular

bandages or wraps.

Do not scold a child for having a bleed. Do not circumcise if a family history of

bleeding is known. Do not apply ice to the skin without a

protective towel or cloth.

Minimal Replacement Therapy

a) Fresh frozen plasma contains factor VIIIand factor IX, 250 units in 250 ml of plasma.To avoid fluid overload, administer one bagevery 8 to 12 hours for hemophilia A. Onebag every 24 hours may be adequate forhemophilia B. Reduce volume for infants

and children.

b) Cryoprecipitate: one bag usually contains80-100 units of factor VIII only.


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