Save the 'Bleeders'
Genesis of a solution from sorrow: healthcare provision for hemophilia patients in Thrissur, Kerala, India
By Sujata Devadas, October 03, 2016
Kannan, E. Raghunandanan’s hemophilic son, passed away 5 years ago, age 24. In 1803, Philadelphia physician John Conrad Otto, was the first to recognise the bleeding disorder hemophilia that primarily affects men. He called these men ‘bleeders’.
Coping with Kannan’s dire malady in Thrissur district of Kerala, India, his parents realised the absence of medical facilities to ameliorate their son's health condition in their own vicinity. Kerala’s Hemophilia Society is in its capital Thiruvananthapuram, Established by Dr K.N. Pai, it is more than 250 km away - about 6 hours by road.
In 1995, Raghunandanan and Dr Jacob Mathew therefore registered the Kunnamkulam Chapter of the Hemophilia Society (KHS) - a fellowship of Persons With Hemophilia (PWH), their family members, physicians, paramedics, social workers and activists, under the Charitable Societies Act in Thrissur district. Dr Jacob Mathew was the founder president. Raghunandanan, Kannan’s father, continues to be its founder secretary.
Losing blood copiously
When an injury or bruise occurs, several proteins help the blood to clot. When some of these essential proteins are absent, that person suffers hemophilia, profuse blood loss.
Kannan, Raghunandanan’s son, often bled twice a week. Regardless of the expense, his parents ensured that he received a Factor VIII protein infusion to help the blood to clot. Kannan required painkillers, but soon, he grew immune to them. After continuous bleeding, Kannan died of excruciating pain.
This devastating inherited blood disorder can cripple, cause severe pain and death if left untreated. The World Federation of Hemophilia, headquartered in Montreal, Quebec, Canada, surveyed 106 countries in 2014 to estimate hemophilics in 91% per cent of the world population. Statistics show 178,500 hemophilics.
Hemophilics can have both internal and external uncontrolled spontaneous bleeding. The extent of blood loss varies with how severe the deficiency is. Internal bleeding can result in pain, swelling and permanent damage.
A safe replacement therapy
In the early 1900s, hemophilics received blood infusion as treatment. In 1937, 2 Harvard physicians discovered clotting factors in blood plasma, making infusion of plasma alone sufficient. But 'the sufficient amount' required infusion of a huge volume to ensure the patient’s recovery.
The discovery of cryoprecipitate - an extract rich in a blood-clotting factor obtained from frozen blood plasma - helped control serious bleeding in 1965. Emergency surgery and elective procedures for hemophilics became more manageable because of this discovery.
Treatment advances continue. Keeping everyone’s convenience in mind, the international agreement is to name and distinguish blood clotting factors with a suffix of Roman numerals.
At present, companies such as Baxter and Nordisk manufacture clotting factors VII, VIII and IX, using recombinant DNA technology. These are pure and free from contaminants.
Weaving a safety net
In 10 years, the Kunnamkulam Chapter of the Hemophilia Society (KHS) has instituted many measures to help hemophilics and others with bleeding disorders having Factor I, V, VII, X, XIII deficiencies and Von Willebrand disease.
Professor N N Gokuldas, a postgraduate in zoology with a special interest in human genetics says “we are morally obligated to help these people. A call for help may come at any time. So, KHS makes imported clotting factors manufactured by recombinant DNA technology available at the lowest possible cost to protect the afflicted from contracting infections transmitted by transfusions. 382 hemophilics and patients with other bleeding disorders are in our care. KHS offers financial assistance, regular physiotherapy and educational scholarships.”
Hemophilics are generally male. Women become carriers if they inherit the hemophilia gene from their fathers. Once identified as a carrier, that woman may opt not to marry or conceive. Others undergo prenatal diagnosis 12 weeks after conception. If the foetus has the genetic deficiency, the pregnancy can be terminated.
Thrissur's Jubilee Mission Hospital, Unity Hospital and Kunnamkulam's St. Antony’s Hospital are 3 medical centres that treat hemophilics regularly. In an emergency,
doctors Jacob Mathew, Ashok Verma, Sr. Merly, Suseela Innah, Sr. Mercy, Varghese and dental surgeon Dr Poulose Kutty attend to hemophilics free of charge. Thrissur's Pain & Palliative Care Society offers pain management care. Qualified physiotherapists help hemophilics strengthen their muscles and joints.
Professor Gokuldas confirms that the Indian Medical Association and the Indian Dental Association always cooperate, offering medical education programmes to manage patients with such disorders.
Necessary immediate care
Professor Gokuldas, Raghunandanan and his wife Rema raise awareness on the importance of carrier detection and prenatal diagnosis regularly. KHS distributes pamphlets on hemophilia, the associated problems and treatment options.
Instructions to family members of hemophilics in First Aid and how to manage hemophilia, particularly before the sufferer can reach a doctor are given in 3-day camps by doctors, nurses, physiotherapists and trained non-medical people.
All hemophilics are advised to immunize themselves from Hepatitis B. Registered nurses give subcutaneous vaccines free of cost. KHS holds such immunization drives for other bleeding disorders as well. Only a few balk at immunization.
The dismal cost of ignorance
Despite all the blood safety measures at blood banks in India, many contract infections transmitted by transfusion -- HIV, Hepatitis B, Hepatitis C (HCV), cytomegalovirus and malaria, to name a few. In consequence, three hemophilics in KHS are HIV+, three are HCV+.
The HIV+ people are receiving antiviral therapy (ART) at the Government Medical College, Thrissur. Measures have been taken to help the HCV+ patients obtain treatment from a well-known hepatologist practising at a private hospital in the neighbouring Kochi district.
More pertinently, medical professionals and trained volunteers sensitize hemophilics about transfusion-transmitted infections as a very real possibility. “I use PowerPoint presentations to explain the scientific aspects of bleeding disorders, stressing the importance of SAFE blood, and why clotting factors made by recombinant DNA technology is free of contamination,” says Professor Gokuldas. Raghunandanan discusses the socio-economic dimensions of the condition while his wife Rema, a law graduate with qualifications in psychotherapy, provides free counselling services.
Hemophilics & families, members of KHS, meet every three months. Children, youth, mothers and parents organize camps. On average, 30 children attend their Children’s Meet.
Dr Ashok Kumar, Dr Ajith, Professor Gokuldas, Raghunandanan, Radhakrishnan,
Rema, Jimmy and other members from various hemophilia chapters help the state and local bodies understand bleeding disorders.
This yielded an encouraging outcome. The Social Security Mission of Kerala Government now gives Rs. 1000/-per month as assistance to each hemophilic. In limited amounts, the government now also provides AHF (antihemophilic factor, Factor VIII) free of cost under the Karunya Benevolent Fund.
KHS receives a small amount of funds from philanthropists who wish to remain anonymous. The American non-profit hemophilia sponsorship organization, Save One Life, has offered help to hemophilics under KHS care. Their website http://www.saveonelife.net states that caring for a hemophilic in a developing country can be done for under a dollar a day.
On October 15 2016, KHS and the Kannan Memorial Trust inaugurated a 6,500 sq.ft. shelter for mothers who carry the hemophilia gene and are often harassed by their in-laws. Although these women may wish to escape victimization and stay in the shelter, this is generally not tolerated in the local community. So, in case of such disputes and harassment, the shelter provides short-term protection to the mother and her child, giving counselling services round the clock to bring about reconciliation.
KHS aims to establish a physiotherapy unit soon. Hemophilics need to strengthen their joints and muscles to reduce the likelihood of becoming physically handicapped.