Plight of Sickle Cell Disease in Maldives

I wrote this article back in 2012, when I got the opportunity to speak at Maldivian Thalassaemia Societys’ parent and patients meeting that was organised during a visit of Thalassaemia International Federation delegates.

I used this chance to break the Sickle Silence in Maldives to Dr. Micheal Angastiniotis and Mr. George Constantinou, who were not surprise to find sickle cell in Maldives, but surprised to find me living with sickle cell regardless of the fact that they were told there was no record of sickle cell patients living in this community.

So on the occasion of World Sickle Cell Day 2016, four years later, I post this article that gave me my first opportunity to tell my story Living With Sickle Cell in the 13th International Conference on Thalassaemia and Haemoglobinopathies, and the 15th Conference for Patients and parentsorganised by Thalassaemia International Federation in Abudhabi, 2013.

(As written in 2012)

Sickle cell disease is an inherited blood disorder where the Red blood cells loosed its usual round shape and takes the shape of a ‘sickle’. A C-shape tool that is used in farms. These sickle cells are hard and sticky and die early in comparison to normal red blood cells. These changes in the blood cells can clot together and block small blood vessel and preventing the flow of blood and oxygen to organs and body. This causes severe pain, organ damage, serious infections or even stroke.

A person who has sickle cell disease is born with it. Blood tests such as DNA analysis or electrophoresis can determine if the person is born with the blood defect or not. This blood disease is not contagious. Sickle cell disease is one of the oldest diagnosed blood diseases and it affects millions of people throughout the world and is common among those whose ancestors come from Africa, Saudi Arabia, India and Mediterranean countries. Maldives, a nation where its ancestors are known to have come from various parts of these regions, even though many are not aware, have inherited this life long blood defect along with many other blood disorders, such as thalassemia and hemophilia.

According to Society for Health Education (SHE) (Haveeru, world sickle cell day supplement, June 19^th 2012), among 70,000 people SHE have screened under the thalassemia-screening program, 0.14% of the people have sickle cell trait. Even though this figure may not sound alarming compared to the 18.1% beta thalassemia carriers among the 300,000 Maldivians. This report also mentions that there are certain sickle cell pockets in Maldives. Like 8% sickle carriers in Alif Alif Thoddu, 6% in Gaaf Alif Nilandhoo, 10% in Gaaf Alif Dhiyadhoo, and 3% in Gaaf Dhaal Fare’s. The concern is that the possibility of sickle cell gene meeting with other blood disorder carrier genes, to take in the sickle cell variant disease form is higher compared to world percentages of sickle cell and its variants, which is 5% (WH0). 

The most common form of sickle cell found in Maldives is sickle beta thalassemia.  Screening test done from 2008 to 2011 by National Thalassemia Center reports 46 sickle cell carriers and 10 sickle beta thalassemia patients.  7 sickle beta thalassemia patients and 1 sickle-Hb D patient have been registered at the center. The severity of how this disease affects those with sickle cell and their families is not yet known in Maldives. The lack of awareness and understanding of the excrutiating pain that accompanies a crisis and the overall fatigue and weakness that are the continual companion of those with SCD unfortunately extends to health care practitioners as well.

Sickle cell disease is treated depending on the symptoms, such as to relieve pain, prevent infections, eye damage and strokes. Treatments like receiving blood transfusion, hydration and pain medication are options that can vary depending on the individual and the severity of sickle crisis. A medicine that was used to treat cancer patients, called Hydroxyurea maybe used in severe sickle cell disease patients. Hydroxyurea can reduce the number of crisis and also reduce the number of hospital stays and the need for blood transfusion. Some sickle cell disease patients, including those with sickle beta thalassemia take Hydroxyurea as a form of treatment and usually it has to be taken for life.

Sickle cell disease has been called an ‘invisible’ disease as the effects of sickle cell are not often visible on the physical appearance of the person. Therefore the sickle sufferers are lead to unintentional discrimination due to the lack of knowledge on this disease. Everyday normal life activities are being limited by the fatigue and weakness due to sickle cell disease. In schools and at work place, this is often interpreted as lack effort or will by the sufferer towards their study/work. Usually the family and caretakers of a sickle cell sufferer have to adapt to these limitations and are constantly under stress due to the unpredictable nature of the disease, as it affects all aspects of their lives. Families of sickle cell disease sufferers scrape to meet expenses of treatment like getting to and from medical appointments with the right doctors and specialists, payments of different routine medical tests, medicines and hospital admissions, purchasing assistive devises which are not usually available easily in Maldives. Our community, aren’t yet prepared to cope with the needs of these disabled individuals; many people are unaware that the disease still has no cure and that the excruciating pain of acute episodes of this disease can be severely disabling. However, with right treatment and education, sickle cell patients can adapt to live a life that is close to normal in the society. This is a call to accommodate the needs, rights and treatment for sickle cell patient to ensure that they are not left neglected in this society, by government, medical providers and the public. 

Maldives needs an approach to bring the needs of patients and their families to the forefront. We need a national protocol for treatment for sickle cell disease available to all the islands across the country. This will give the opportunity to lessen the geographic disparities in treatment and to lower the high cost of the disease to families and to the nation as a whole. We need to focus on an approach to empower patients and their families by training them to self-management of the disease. Treat patients’ symptoms as an individual rather than the knowledge of disease itself.

At a national level, many questions must be answered with a clear data in order to shape the appropriate approach to treating sickle cell disease. Such as, how can health care providers and institutions be educated to provide the best and most current treatments, and how can their success and failures be shared as a learning tool to empower patients and their families? How can the government; financially, medically and programmatically best address the needs of those families with sickle cell patients? How can the government/ NGOs reach out to support educational awareness programs on sickle cell disease to the whole community? The most important, is to identify the most pressing and significant concerns of families struggling with sickle cell disease and catering to their needs.

Sickle cell disease has been over looked in Maldives. Even the name of this disease is silent in this community. Over years, sickle cell disease have been misunderstood, under-treated and has not gained the attention that it should deserve given the number of people known to have been effected.

It is time to set a standard care procedure that can serve the patients to an equal chance of living in this society. It is time that we pulled sickle cell disease from the edges of clinical ignorance to the center of their attention, and win the battle against sickle cell disease for the sufferers. It is time to break the sickle silence.