Number of Participants: 72
The second meeting of Q1-2015 was conducted for Doctors (undergraduates) under the Haemophilia Training and Awareness Systems (HATS) on 30 March 2016 in Seminar Hall, Ward 32, 4th floor, New Medical Block, Lok Nayak Hospital, New Delhi 110002 under the preview of Dr. Naresh Gupta, Head, Haemophilia Centre. The audience consisted of third and final year undergraduate students from Maulana Azad Medical College (MAMC). Registration process stared at 2:00 PM and awareness folders with information brochure and pre/post assessment sheet were distributed. Soon, after seating the guests, we started the programme with a pre-assessment test consisting of 10 general question relating to haemophilia awareness. Questions were projected on the screen each after another and the sheets were collected back for later analysis.
Dr. Naresh Gupta, Director Professor and Head, Department of Medicine and Head, Haemophilia Centre welcomed the audience and thanked for their valuable presence in the awareness meeting conducted by HDCC. He headed the first session on approach to diagnosis of haemophilia. Correct diagnosis is the first key to any treatment and being a medical practitioner, every medico should be competent and accurate in his/ her diagnosis. Haemophilia is one such disease that is often undiagnosed or misdiagnosed with other bleeding disorder. Sometimes platelet disorder too represent the same manifestation at primary observation though they differ significantly in nature and site. This part of the programme was oriented to provide an insight to distinguish the various bleeding disorder from haemophilia and making the correct diagnosis based on careful observation, extensive familial history collection and studying the laboratory results for bleeding and factor assays. The hallmark of haemophilia bleeds are always associated with deeper tissues i.e. joints, muscles, abdomen, brain etc. compared to ecchymosis and superficial bleeds seen in platelet disorder. Secondly, in laboratory terms haemophilic patients always show a normal PT and an abnormal APTT value where the bleeding occurs without any systemic disorder versus multiple factor deficiencies i.e. DIC, liver diseases, snake bite which shown more than one clotting factor deficiency. However, in 70% of the cases the PWH has a previous history of haemophilia incidence in his familial generation either paternal/maternal, however there is always a 30% chance for new mutations to occur.
Dr. Sunita Aggarwal, Professor, Department of Medicine, MAMC addressed clinical issues in haemophilia. Prolonged bleeding and poor fibrin clot formation leads to serious issues in PWH which can prove to be life threatening at times specially when they are associated with critical organs such as brain or psoas and being a medical practitioner one should be trained and equipped to tackle such emergency situations where immediate treatment is required. She explained the most common musculoskeletal and joints prone to bleed in haemophilia, whose rate entirely depends upon the severity of factor deficiency in the individual. A PWH with less than 1% can bleed spontaneously without any trauma, which is a matter of concern. This severely affects the livelihood with work related chores and overall quality of life. Clinical condition includes pain, swelling and movement restriction of the affected joint/area. On the longer run, repeated bleed in the same site can lead to hematoma or joint arthropathy. An x-ray film was used to show the clinical condition of a PWH suffering bony ankyloses. He also highlighted that simple parenteral modes of infusion especially intramuscular injections or venipuncture access if not performed properly can causes hematoma in PWH. In 1980, when appropriate viral inactivation an viral screening where not routinely practiced the risk for HIV and hepatitis related infection were quiet high as compared to today’s scenario with provide much more safer and reliable AHF products. The risk of inhibitor development in another challenging issue in treating these patients when they stop responding to the drug and bypassing agents have to be supplemented with AHF infusion.
Dr. Sandeep Garg, Professor, Department of Medicine, MAMC spoke on the different aspects of haemophilia management. He covered AHF related infusion for haemophilia type A and B. He also stressed that comprehensive care is very important to manage PWH, as other aspects such as specialist consultation, physiotherapy and counselling were necessary. The session was concluded with a post-assessment test with the same set of questions in which the students out-performed this time as they were well versed with topics. The participants along with the faculty gathered for a group photograph and later refreshments was served. Dr. Gupta thanked the participants for their participation and interest in the awareness programme.
Group Pre-assessment score: 41.1% (234)
Group Post-assessment score: 70.7 (403)
% Improvement: 29.6%