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Haemophilia is a haemorrhagic diathesis caused by the congenital deficiency in the blood of Factor VIII (von Willebrand factor/ antihaemophilic globulin). It is a sex linked characteristic, transmitted by the asymptomatic female carriers, and manifest only in males.1 If a carrier has a son, he has a 50% chance of having haemophilia, and a daughter has a 50% chance of being a carrier.2

Although haemophilia is a congenital disorder it is unusual for excessive bleeding to be noticed until babies are about 6 months old, when superficial bleeding or haemoarthrosis may occur. This apparent delay in presentation is due to the relative inactivity of babies in the first few months of life and it is only when they begin to move about that more trauma results in bleeding.2

Symptomatic patients usually have factor VIII levels <5%, with a close correlation between the clinical severity of hemophilia and plasma AHF (anti haemophilic factor) level. Patients with <1% factor VIII activity have severe disease; they bleed frequently even without discernible trauma. Patients with levels of 1 to 5% have moderate disease with less frequent bleeding episodes. Those with levels >5% have mild disease with infrequent bleeding that is usually secondary to trauma. Occasional patients with factor VIII levels as high as 25% are discovered when they bleed after major trauma or surgery. The majority of patients with hemophilia A have factor VIII levels below <5%.3

Hemophilic bleeding occurs hours or days after injury, can involve any organ, and, if untreated, may continue for days or weeks. This can result in large collections of partially clotted blood putting pressure on adjacent normal tissues and can cause necrosis of muscle (compartment syndromes), venous congestion (pseudophlebitis), or ischemic damage to nerves. Patients with severe hemophilia are usually diagnosed shortly after birth because of an extensive cephalhematoma or profuse bleeding at circumcision.

Typically, a hemophilia patient presents with pain followed by swelling in a weight-bearing joint, such as the hip, knee, or ankle. The presence of blood in the joint (hemarthrosis) causes synovial inflammation, and repetitive bleeding erodes articular cartilage and causes osteoarthritis, articular fibrosis, joint ankylosis, and eventually muscle atrophy. Bleeding may occur into any joint, but after a joint has been damaged, it may become a site for subsequent bleeding episodes.

Patients suspected of having hemophilia should have a platelet count, bleeding time,prothrombin time(PT), and partial thromboplastin time(PTT). Typically, the patient will have a prolonged PTT with all other tests normal. Because of the clinical similarity of factor VIII deficiency and factor IX deficiency, any male with an appropriate bleeding history and a prolonged PTT should have specific assays for factor VIII and factor IX.

Management: Tenets regarding the treatment of bleeding in hemophilia patients include the following: (1) Symptoms often precede objective evidence of bleeding. (2) Signs of bleeding may not appear until several days after well-documented trauma. Early treatment is more effective, less costly, and can be lifesaving. (3) Avoid the use of aspirin or aspirin-containing drugs, which impair platelet function and may cause severe hemorrhage.

Plasma products enriched in factor VIII have revolutionized the care of hemophilia patients, reduced the degree of orthopedic deformity, and permitted virtually any form of elective and emergency surgery. The widespread use of factor VIII concentrates has also produced serious complications, including viral hepatitis, chronic liver disease, and AIDS.

Homoeopathic Management: This includes confidence in physician himself and taking the challenge of treating a hereditary disease because so many homoeopaths leaves hope upon hearing a genetic disorder. Our literature is full of antihaemorrhagic remedies. Syntheis 9.2 contains following 65 medicines.

GENERALS - HEMORRHAGE - blood - non-coagulable4

Adren. ail. am-c. ancis-p. anthraci. Apis aran. Arn. ars. BOTH. both-ax. bov. calc. calc-lac. calc-p. carb-an. Carb-v. carc. cench. chin. chlol. chloram. cloth. cortico. croc. CROT-C. Crot-h. dig. dor. Elaps Erig. FERR. ferr-m. gal-ac. Ham. HIR. ip. Kali-p. kreos. lac-c. LACH. LAT-M. led. merc. mill. nat-m. nat-n. Nat-s. Nat-sil. NIT-AC. op. ph-ac. PHOS. puls. rad-br. Sec. sil. staphytox. stront-c. Sul-ac. sulph. ter. vip. visc. x-ray.

Case Report: A patient diagnosed severe haemophilic was shown to me on 09/05/2006. Pathological report reveals Factor VIII activity 02 %, Clotting time more than one hour, aPTT 155 sec. Parents were nearly hopeless because they were advised lifelong transfusion of  Factor VIII per month which is too costly and two mama( mother’s brother)  died of haemorrhage at age of 11 and 13 years (Haemophilia?).

I took the case as a challenge and repertorised the case as follows with following symptoms 

MIND - ACTIVITY - desires activity

MIND - ANGER - children; in

MIND - CHEERFUL - waking, on

HEAD - PERSPIRATION of scalp

STOMACH - APPETITE - wanting

SLEEP - POSITION - side; on

SKIN - ECCHYMOSES

SKIN - INDURATIONS, nodules, etc.

SKIN - INDURATIONS, nodules, etc. - bluish - spots

GENERALS - HEAT - sensation of

GENERALS - HEMORRHAGE - blood - non-coagulable

GENERALS - HEMORRHAGE - Internally

I have chosen Phosphorus as most appropriate remedy after studying its materia medica.

At that time there occurs small bluish nodule under the skin which is due to haemorrhage in muscle and skin due to pressure.

I used the method of hair transmission (Sahani Effect vide Transmission of Homoeo drug energy from a distance- B.Jain Publishers)6 .Phos 0/1 was transmitted with patients hair on 09/05/2006.Sac lac also given. Nash says “Under Phosphorus the blood becomes so broken down that it will not clot any more, and we have purpura haemorrhagica”. Hering Guiding symptom says about Phos as “Hemorrhages : profuse; frequent and profuse, pouring out freely, then ceasing for a time; from free surface and from tissues; from various parts of body, haemoptysis, bleeding of gums, hemorrhoids; metrorrhagia, etc.; blood very fluid and difficult to coagulate; vicarious, from nose, stomach, anus, urethra; associated with Bright's disease; from internal organs”.5

Follow Up: Induration in bluish nodule began to decrease from first day and disappeared in few days. But they continue to recur from time to time till date.  As I have discussed above the problem arises when child began to walk. Pressure cause bleeding in weight bearing joints resulting in haemoarthrosis. This child suffer from haemarthrosis of left ankle causing swelling and pain in joint. He does not put feet on the ground.

Plate 1              Plate2

Arnica 0/3 was transmitted on 08/08/2006, swelling and pain decreased within 3 days as evident in plate2.

After few days again Phos 0/1 was transmitted which is continued till date. There occurred minor cut, sometime by own nail of child but bleeding stooped in few minutes. Advised for lab investigation of Factor VIII and BT, CT.

Report of 12/09/2007 shows marked improvement in Factor VIII from 02% to 17% and Clotting Time from more than 1hours to 10minutes. (Reports enclosed)

Interpretation : After one and half year of treatment patient improved from almost severe haemophilic to mild haemophilic. This case also demonstrates efficacy of hair transmission and role of homoeopathy in genetic disorders.

REFERENCES

1-Short Practice of Surgery – Baily & Love
2-Principle and Practice of Medicine Davidson
3-Harrison’s Principle And Practice Of Medicine 15th edition
4-RADAR homoeopathic Software 9.2
5-Encyclopedia Homoeopathica Ver 2.2
6-Transmission of Homoeo Drug energy from a distance Dr. B. Sahani

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