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 Table of Contents  
CASE REPORT
Year : 2022  |  Volume : 9  |  Issue : 1  |  Page : 46-50

Homeopathic Management of Afibrinogenemia along with Beta-Thalassemia


1 Communication Consultant, Homoeopathy in Haemophilia, C/o Sakha the Centre of Research and Charity, Upnagar, Nashik, India
2 Principal Investigator, Homoeopathy in Haemophilia, C/o Sakha the Centre of Research and Charity, Upnagar; Professor and HOD of Medicine, Motiwala (National) Homoeopathic, Medical College, Nashik, India
3 Former Director, National Institute of Immunohaematology, KEM Hospital, Mumbai, Maharashtra, India
4 Research Associate, Homoeopathy in Haemophilia, C/o Sakha the Centre of Research and Charity, Upnagar, Nashik, India
5 Professor and HOD of Physiology, Motiwala (National) Homoeopathic, Medical College, Nashik, India

Date of Submission17-May-2022
Date of Decision14-Jun-2022
Date of Acceptance02-Jul-2022
Date of Web Publication15-Sep-2022

Correspondence Address:
Kundu Tapas
Homoeopathy in Haemophilia, C/o Sakha the Centre of Research and Charity, Upnagar, Nashik - 422 006, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/AYUHOM.AYUHOM_27_22

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  Abstract 


Afibrinogenemia is an extremely rare inherited bleeding disorder. Less than 100 such cases have been described from India. Beta-thalassemia carrier status (trait) is common in many Indian casts and religious groups; hence, a rare combination of these two conditions can be expected. Here, we report one such case treated solely using homeopathic medicine. The male patient was borne out of a nonconsanguineous marriage. He had delayed separation of the umbilical cord after 14 days and bleeding from the site. In addition, he had multiple bleeding episodes treated successfully by homeopathy since 11 months of age. His thalassemia trait needed no treatment. As fibrinogen concentrate is not easily available, this line of management could also be considered if the concentrate or safe cryoprecipitate is not immediately available.

Keywords: Afibrinogenemia, beta-thalassemia, bleeding disorder, homeopathy


How to cite this article:
Omkar K, Tapas K, Kanjaksha G, Gulfisha M, Rita K. Homeopathic Management of Afibrinogenemia along with Beta-Thalassemia. AYUHOM 2022;9:46-50

How to cite this URL:
Omkar K, Tapas K, Kanjaksha G, Gulfisha M, Rita K. Homeopathic Management of Afibrinogenemia along with Beta-Thalassemia. AYUHOM [serial online] 2022 [cited 2022 Oct 7];9:46-50. Available from: http://www.ayuhom.com/text.asp?2022/9/1/46/356168




  Introduction Top


Afibrinogenemia is a rare inherited bleeding recessive condition.[1],[2],[3] Few cases have been reported from India[2] where beta-thalassemia and other hemoglobinopathy genes are common. Hence, rarely a carrier state of beta-thalassemia with afibrinogenemia may be possible. Here, we report one such patient treated using classical homeopathy.


  Case Report Top


A 4-year-old male child was born out of nonconsanguineous marriage with a full-term normal delivery. The mother was a beta-thalassemia carrier, and the father was normal. The boy had delayed separation and prolonged bleeding from the umbilical cord. Subsequently as the boy grew up, he started having epistaxis, gum bleed, soft tissue bleed and prolonged bleeding from minimal trauma. He had a history of 12–17 bleeding episodes per month. There was no family history of bleeding, and there was no history of blood transfusion. On examination, the patient showed ecchymotic patches and bleeding from the gum. He was irritable and was probably in pain because of elevated bruises which came up spontaneously. He was nonfebrile and completed all immunization schedules, but injectable vaccines caused hematoma. No scar is seen. No organomegaly was noted. The rest of the clinical examination was normal.

The boy was investigated with normal biochemistry of renal, liver, and thyroid function. Routine hematological investigation showed hemoglobin (Hb) of 7.7 g/dl, total leukocyte count of 19.4 109/l, and platelet count – 606 109/l. Smear showed microcytosis and hypochromia. High-performance liquid chromatography for Hb showed HbA2 of 5.8%, and the rest is adult Hb (11 months of age).

Coagulation screen showed whole blood clotting time using Lee and White method of more than 30 min (normal up to 20 min), activated partial thromboplastin time of 690 s (normal 34 s.), prothrombin time of 296 s (normal 15 s), and thrombin time of 70 s (normal 14 s), and factor assay showed Factor II – 60% (normal 50%–150%), Factor V – 95% (normal 50%–150%), Factor VII – 80% (normal 50%–150%), Factor VIII – 65% (normal 50%–150%), Factor IX – 51% (normal 50%–150%), Factor XIII – 100% (normal 50%–150%), vWF: Ag – 73% (normal 50%–150%), and vWF: RCoF – 62% (normal 50%–150%). Mixing studies showed no inhibitor. Plasma fibrinogen assay using Clauss technique is <20 mg/dl (150–300 mg/dl). The present case clearly shows that plasma fibrinogen assay using clauss technique is suggestive of underlying Iron deficiency. The values were, Serum iron (42 μg/dl), transferring (2.4 g/L), ferritin (09 ng/ml) and total iron binding capacity (360 μg/dl) and percentage saturation of transferrin was 11.6% suggesting underlying iron deficiency where sTfR level is 7.2 mg/L (normal up to 4.0 mg/L). Ultrasound of the abdomen showed mild splenomegaly.

Hence, the final diagnosis of the patient was beta-thalassemia trait with congenital afibrinogenemia and iron-deficiency anemia (chronic blood loss).

The following mental symptoms were also noted for the selection of homeopathic remedy for the case. They were:

  • Always wants to go out
  • Obstinate
  • Loves to play with water
  • Clings to mother during bleeding episodes
  • Weeping during pains
  • Revengeful.



  Results Top


Homeopathic medicines were started as per the rules of classic homeopathy.[4],[5],[6],[7],[8] Mental and physical symptoms were used as the criteria for selection of remedy. As homeopathic medicines have an impact on mental condition as well as on physical bleeding and anemia. It was found that at the end of homeopathic treatment, his Hb levels increased significantly to 10.7 g/dl. His bleeding episodes per month reduced from 12 to 17 to only 1–2. Few episodes of drug selection and management are shown in [Table 1]. The treatment outcomes were measured on patient-reported outcome measures such as the Adaptive and Maladaptive (Modified) Behavior Scale[9] [Table 2] and the Wong–Baker Pain Rating Scale[10] [Table 3]. The outcome of the patient was assessed using the Modified Naranjo Criteria in Homeopathy.[11] This tool helped in mapping the outcome achieved in the patient on 10 domains, as shown in [Table 4].
Table 1: Few episodes managed homeopathically

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Table 2: Assessment of Scale - Adaptive/Maladaptive (modified) Behavioral Scale

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Table 3: Assessment of Scale - Wong–Baker Pain Rating Scale

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Table 4: Patient outcome assessment on modified Naranjo criteria in homeopathy

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  Discussion Top


A rare case of afibrinogenemia with beta-thalassemia carrier state managed homeopathically has been described here. The patient's anemia was contributed by his thalassemia carrier state combined with continued bleeding tendency. Congenital afibrinogenemia is a very rare recessive congenital bleeding disorder. In such disorders bleeding manifestations vary, some bleed heavily including fatal intracerebral bleed others do not bleed at all and some other present with disfiguring scars and even thrombosis. Our patient from early childhood showed moderate to heavy bleeding tendency. Beta-thalassemia mutation is not rare in India,[12] and regular screenings for this condition in India and in many countries have been suggested.[13] The varied presentation of afibrinogenemia has not been satisfactorily explained though an extensive clinical study of this condition as well as an experimental study of this condition in zebrafish model has been described.[14] One question could be legitimately asked why homeopathy for a condition for which concentrates replacement of fibrinogen is available?[15] Because of the rarity of the condition and cost involved, this concentrate is not widely available in developing countries. Hence, usually cryoprecipitate is used for this deficiency. Cryoprecipitate has many safety concerns and that too is not widely available in remote areas of the country, but good homeopathic physicians as well as cheap homeopathic medicines are available. Our study clearly shows that such a case can be managed well homeopathically, and our previous study using homeopathic medicines in congenital bleeding disorders such as severe hemophilia had been extremely positive.[16] The case history is taken and acute totality including the mental generals, the physical generals, and the particulars is considered while prescribing for each hemorrhagic episode emphasizing on the peculiar characteristic of the patient. The patient is provided with an emergency homeopathic kit preloaded with 50 predefined homeopathic medicines for telephonic management in the case of emergency. The patient started treatment at the age of 11 months and is of 4 years now. All the hemorrhagic episodes were managed with acutely indicated homeopathic medicines using the software RADAR 10.0-Schroyens F., synthesis 9.0 (English). The medicines were repeated according to the intensity and severity of the bleeding. A combination of congenital bleeding disorders with hemoglobinopathy is extremely rare but has been reported on several occasions.[17],[18],[19],[20],[21],[22],[23] The reasons to study these cases are several folds, i.e., one disease may influence the severity of other diseases, as in our case, an underlying beta-thalassemia trait has caused a higher degree of fall of Hb than could be expected. Many families suffering with haemoglobinopathies and congenital bleeding disorder family undergo prenatal diagnosis, so if one is missed in the family or remain recessive in prenatal diagnosis, this may not be taken into account and missed, as is seen in one of the authors' case.[18]

The present case clearly shows that homeopathic medicine having holistic action has helped not only in controlling bleeding, improved Hemoglobin level but also improves mental condition associated with chronic disability though the level of factor concentrates remains the same which is the limitation of the study. There is a need for more extensive evaluation of this mode of therapy in rare bleeding disorders and families where consanguinity is common and factor concentrates are not easily available.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given consent for images and other clinical information to be reported in the journal. The guardian understands that name and initials will not be published and due efforts will be made to conceal the identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Bolton-Maggs PH, Perry DJ, Chalmers EA, Parapia LA, Wilde JT, Williams MD, et al. The rare coagulation disorders – Review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation. Haemophilia 2004;10:593-628.  Back to cited text no. 1
    
2.
Mukaddam A, Kulkarni B, Jadli A, Ghosh K, Shetty S. Spectrum of mutations in Indian patients with fibrinogen disorders and its application in genetic diagnosis of the affected families. Haemophilia 2015;21:e519-23.  Back to cited text no. 2
    
3.
Stanciakova L, Kubisz P, Dobrotova M, Stasko J. Congenital afibrinogenemia: From etiopathogenesis to challenging clinical management. Expert Rev Hematol 2016;9:639-48.  Back to cited text no. 3
    
4.
Hahnemann S. Organon of Medicine. 6th ed. New Delhi: B. Jain Publications; 1990. Aphorism 40.  Back to cited text no. 4
    
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Crompton B. Best of Burnett. New delhi: B. Jain publisher Pvt. Ltd.; 1992.  Back to cited text no. 5
    
6.
Miller G. Remedy Relationship Chart. New Delhi: B Jain Publishers; 2002.  Back to cited text no. 6
    
7.
RADAR-homeopathic software. Marketed in India by B. Jain publishers, New Delhi. Available from: www.radar-uk.co.uk. [Last accessed on 2021 Jan 15].  Back to cited text no. 7
    
8.
Robert HA. The Principles and Art of Cure by Homeopathy. Chapter 17, Susceptibility. 3rd ed. New Delhi, India: Indian Books & Periodicals Publisher; 2005.  Back to cited text no. 8
    
9.
van Duijn G, Dijkxhoorn Y, Noens I, Scholte E, van Berckelaer-Onnes I. Vineland Screener 0-12 years research version (NL). Constructing a screening instrument to assess adaptive behaviour. Int J Methods Psychiatr Res 2009;18:110-7.  Back to cited text no. 9
    
10.
Wong DL, Hockenberry-Eaton M, Wilson D, Winkelstein ML, Schwartz P. Wong's Essential of Pediatric Nursing. 6th ed. St. Louis: Mosby Elsevier; 2001. p. 1301.  Back to cited text no. 10
    
11.
Lamba C, Gupta V, Haselen R, Rutten L, Mahajan N, Molla AM, et al. Evaluation of the Modified Naranjo Criteria for assessing causal attribution of clinical outcome to Homeopathic intervention as presented in case reports. Homeopathy 2020;109:191-7.  Back to cited text no. 11
    
12.
Colah R, Gorakshakar A, Phanasgaonkar S, D'Souza E, Nadkarni A, Surve R, et al. Epidemiology of beta-thalassaemia in Western India: Mapping the frequencies and mutations in sub-regions of Maharashtra and Gujarat. Br J Haematol 2010;149:739-47.  Back to cited text no. 12
    
13.
Cao A, Rosatelli MC, Monni G, Galanello R. Screening for thalassemia: A model of success. Obstet Gynecol Clin North Am 2002;29:305-28, vi-vii.  Back to cited text no. 13
    
14.
Fish R, Di Sanza C, Neerman-Arbez M. Targeted mutation of zebrafish fga models human congenital afibrinogenemia. Blood 2014;123:2278-81.  Back to cited text no. 14
    
15.
Casini A, de Moerloose P. Fibrinogen concentrates in hereditary fibrinogen disorders: Past, present and future. Haemophilia 2020;26:25-32.  Back to cited text no. 15
    
16.
Kundu T, Shaikh A, Kutty A, Nalvade A, Kulkarni S, Kulkarni R, et al. Homeopathic medicines substantially reduce the need for clotting factor concentrates in haemophilia patients: Results of a blinded placebo controlled cross over trial. Homeopathy 2012;101:38-43.  Back to cited text no. 16
    
17.
Dhiman P, Chaudhary R, Sudha K. Sickle cell-β thalassemia with concomitant hemophilia A: A rare presentation. Blood Res 2015;50:264-7.  Back to cited text no. 17
    
18.
Colah RB, Shetty SD, Surve RR, Phanasgaonkar SP, Nadkarni AH, Gorakshakar AC, et al. Prenatal diagnosis in a family at risk for beta-thalassemia and hemophilia A: An uncommon association. Hemoglobin 2004;28:343-6.  Back to cited text no. 18
    
19.
Capra R, Mattioli F, Kalman B, Marcianò N, Berenzi A, Benetti A. Two sisters with multiple sclerosis, lamellar ichthyosis, beta thalassaemia minor and a deficiency of factor VIII. J Neurol 1993;240:336-8.  Back to cited text no. 19
    
20.
Giannini E, Fasoli A, Botta F, Testa R. Wilson's disease with concomitant beta thalassaemia and factor V deficiency. Ital J Gastroenterol Hepatol 1998;30:633-5.  Back to cited text no. 20
    
21.
Eldor A, Rachmilewitz EA. The hypercoagulable state in thalassemia. Blood 2002;99:36-43.  Back to cited text no. 21
    
22.
Colah R, Shetty S, Nadkarni A, Ghosh K, Mohanty D. Masking of the clinical severity of severe haemophilia A in a patient with beta-thalassemia major – Its implications in prenatal diagnosis. Prenat Diagn 2004;24:925-6.  Back to cited text no. 22
    
23.
Abou Mourad Y, Shamseddine A, Hamdan A, Koussa S, Taher A. Report of a rare co-incidence of congenital factor V deficiency and thalassemia intermedia in a family. Ann Saudi Med 2004;24:301-2.  Back to cited text no. 23
    



 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4]



 

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