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Thalasseamia |
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Mediterranean Anaemia A Laymans guide
Thalasseamia is a genetically inherited blood disorder, giving rise to a very severe form of anaemia, specifically affecting people around the Mediterranean region. If two carriers of the affected gene marry and have children then they have a one in four chance of giving birth to an affected child every pregnancy, hence the Turkish governments requirements for blood tests being taken before people marry should a couple both prove to be carriers then they are given full medical advice to assist their decision making regarding having children.
This strategy has greatly diminished the numbers of babies being born with this condition. It is estimated that about 8% of Turkish people living around the Mediterranean could be carriers; this relatively high number is due to the fact that carrying this gene gave some protection against Malaria, a disease that used to be rife in the area.
If a child is born with Thalasseamia Major undetected they will start to show symptoms by the age of 6 months. Only 16 years ago it took one local mother many trips to Istanbul and several years to have her daughters condition correctly diagnosed; nowadays, with much greater awareness of the condition there is a major treatment centre for the disease in the University Hospital in Antalya and another treatment centre in Muğla. The local hospital in Fethiye can now carry out blood transfusions for these patients, but it lacks a dedicated doctor and nurse and therefore adequate monitoring of the patients does not appear to be available in Fethiye. Families that can afford to do so prefer to travel to Mugla for treatment for more adequate monitoring and to escape the risk of an inexperienced person searching for a suitable vein for the transfusion!
The genetic defect prevents the proper absorption and use of iron to form haemoglobin in the red blood cells and hence severely limits the capacity of the blood to carry oxygen. This inability to utilise iron from the diet also leads to a toxic build up of iron in the body which if not dealt with leads to complications for organs such as the spleen, the liver and the kidneys, whilst the bodys efforts to manufacture sufficient red blood cells also leads to bone deformities as the bone marrow capacity endeavours to enlarge itself. The anaemia itself leads to lethargy, irritability and failure to thrive and complications that can lead to the need to remove the spleen.
Treatment for this condition is on two main fronts; firstly sufferers require regular transfusions of red blood cells, often every 3-4 weeks. Secondly they require drugs to help dissipate the build up of iron in their bodies. This latter treatment used to require almost daily infusions of drugs under the skin, for example over a period of 8-9 hours for 5-6 days a week, but recently advances have been made so that these drugs that can be taken orally. Both these treatments have to be backed up with great care that the sufferer has sufficient, exercise, rest and a good healthy diet.
IF a sufferer has been medically well maintained and IF there is a close relative of a suitable blood group then bone marrow transplant surgery can lead to a cure of the condition. However the patient continues to need monitoring and treatment as a transplant recipient. Donated blood has to be specially treated before it can be transfused to these patients. The overall cost to the health service to treat each patient with the iron level controlling drug is around 4,500TL per month.
The Turkish Health service provides all the medical treatment required and bone marrow transplants are carried out in Antalya, but the families affected cannot all afford the extra expenses regular hospital visits incur. They also suffer from the stress involved in having to make their small child undergo such regular and invasive treatment. In order to qualify for the treatment, the child/patient has to be examined at the University hospital every 2 years to obtain a special report. If they are lucky enough to be suitable for a bone marrow transplant they have to live in Antalya for about a year so that the hospital can properly monitor the treatment even after they have left hospital.
FIG aims initially to try to help the local families who need assistance by providing travel and subsistence monies and in the case of some very poor families, the provision of furniture, clothes and the means to provide a suitable diet. We have already visited and provided some money to a 16 year old boy living near Bayir Koy and a 13 year old girl living in the Kara Muar/Menteseoglu area. Both of these can barely afford to get to the local hospital and apparently are not being monitored sufficiently. Therefore FIG also hopes to encourage talks about better facilities being made available in Fethiye for ALL the 24 or so families in this area. With the information we have to date, we consider that the local hospital requires at least one doctor, part of whose duties are dedicated to treatment of this condition and one dedicated nurse and preferably a dedicated reception room/ area for treatment of these special patients.
LEARN A LITTLE ABOUT 2 LOCAL PATIENTS With our volunteer Aysema and with Soner who is the father of a child with Thalasseamia (Mediterranean anaemia) we visited two young people suffering for this serious genetic disease. We eventually found Suleyman and his family, about 2 kilometres off the Antalya road in the area of the village of Bayır Köy. Suleyman is sixteen and is in his final year of compulsory education; he does well at school. To get there he has to walk the two kilometres of forest road to pick up the school transport and of course he has to walk the same distance to get home. Every 3-4 weeks he has to come down to Fethiye to obtain a blood transfusion, he generally travels on his own, partly because he is old enough, but also I suspect because the family can only raise enough money for one fare. He is managing, but because he cannot regularly get to Muğla, where there is a centre dedicated to the treatment of his condition, it does not appear that his medical management is good enough. Immediately we have stepped in to help with travel expenses and are formulating plans whereby we can help his family see he also has an adequate diet etc. In the longer term we are getting involved with others to encourage the setting up of some dedicated facilities in Fethiye. The second child we saw was a 13 year old girl, who lives much nearer Fethiye, but whose family have very little regular income and who struggle to get her to the hospital every 15 days or so, as she needs transfusions more frequently than most. Again we are helping this family with some regular monthly money to help with travel expenses. As we gradually come to understand the dilemmas of families in the area with this problem we aim to provide funds for travelling especially when they have to go to Muğla or Antalya and make efforts to ensure that dietary needs can be met. We shall also partake in discussions, applying some pressure by our interest, towards providing better facilities locally, so that even the families who choose to travel to Muğla every month or so eventually do not have to.
From news on 28 02 2011 Two weeks ago two of us accompanied young Suleyman, a Thalasseamia sufferer, on one of his 3 weekly visits to the local hospital for a blood transfusion. He normally makes this visit alone, but really he needs accompaniment, there are so many things to fetch and carry. We sent him home with a new back pack, some clothes and a variety of food supplies. Importantly we learned that he needs to see a specialist to upgrade the treatment he currently receives. As a result we made the funds available and he and his father should have made it to Muğla last Friday. Suleyman lives over 20 kilometres from Fethiye in a small home some 2 kilometres into the forest off the main road to Antalya, it takes him some time to get to the hospital in Fethiye let alone get to Muğla! Also during the last 2 weeks Dr İsmail Hakkı Timür finally made his promised visit to see us, because of our interest in helping Thalasseamia sufferers. Dr İsmail Hakkı Timür is a top Thalasseamia specialist and a deputy health director for the province of Muğla. He told us about the plans to develop a special centre for Thalasseamia sufferers in Muğla including plans to obtain and provide a house where patients can stay overnight, especially now that it is expected they will have 2 units of specially prepared blood on consecutive days, rather than one unit on one day. And the fact that Muğla will acquire a Haematologist, a specific requirement now for the treatment of this disease and one of the many recent changes to the health system, the largest of which you may have noticed being the institution of family doctors, in what used to be local health centres. The plans are excellent but we felt that more effort needed to be made for local patients to have the bulk of their treatment here and Mustafa Şıkman, Honarary British consulate and a FETAV founder and committee member, really helped us to make a strong case. We look forward to continuing negotiations to achieve this aim. On Tues 15th March there will be a general meeting for the Thalasseamia families, when we can plan with them how best we can help.
On Tuesday 15th March in the council meeting room we met with ten families who have a member suffering from Thalasseamia. The aim was to introduce them to FIG, listen to their opinions and together develop productive ways to move forward. Mustafa Şıkman chaired the meeting; Aysema introduced FIG, our aims and the coupon system we have devised for them to be able to purchase items in our shop. Dr Çiğdem, now a family doctor, but one who has taken a great interest in the sufferers of this disease, assisted us by giving a medical overview when needed and a nurse attended on behalf of local health management. For the time being FIG will continue to give monetary help with fares to distant treatment centres, and indeed the help we provided has meant one young patient, having travelled to both Muğla and Antalya, is now receiving the treatment he really needs and one other family has monthly financial help with transport costs. We facilitated the families to give some details of their experiences and they confirmed that current treatment facilities for blood transfusion - in Fethiye could be a lot better. We discussed at length the feasibility of better facilities being made available in Fethiye as undoubtedly the local patients would benefit enormously. The current upheavals in the health system, with the re-organisation and introduction of the family doctor system, in some ways makes this an inopportune time however we agreed to continue to try to generate the will to make the necessary changes for what is a very feasible project. It was good to meet so many families and good to begin to generate a group that will work together to obtain better facilities
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Fethiye International Group |