Influenza in the Maldives: A Clear and Present Danger

Thalassaemia and other haemoglobinopathies, including sickle cell disease (SCD), are among the most prevalence of all chronic diseases in Maldives. Thalassaemia has become a household name, and there are now over 840 registered patients in the Maldives, with this number growing almost daily.

Thalassaemia and other haemoglobinopathies are long considered a childhood disease by the community, given their early onset and poor prognosis. However, an age distribution graph of registered patients at Maldivian Blood Services 2014 reporting shows many of our patients have overcome the challenging childhood phase and defied the odds by transitioning to young adulthood and even beyond. A greater challenge then, given the age distribution of thalassaemia and haemoglobinopathy patients, is to improve their survival rates, especially given the precipitous decline in survivability once patients reach adulthood. 

Source: Dr. Michael Anagastinosis and Maldivian Blood Services

One crucial factor for the patients fare well in adulthood may be the reoccurrence of splenic problems. A major proportion of adult patients suffer from hypersplenism, a condition marked by an extremely large and overactive spleen. As part of these diseases, the spleen commences removing blood cells too early and too quickly. This forces patients to have frequent blood transfusions and prevents from controlling iron overload through chelation therapies. Hypersplenism also causes enlargement of the spleen, in many cases requiring removal of the spleen to prevent further health complications. Furthermore, patients also experience hyposplenism, a condition where the spleen is inactive or they don’t even have a spleen, a condition known as “asplenia”. This condition is mainly noted with patients who have sickle cell disease. Patients that are suffering from complications with hypersplenism usually end up having a surgical splenectomy, which has been shown to improve the frequency of transfusion and control of iron overload. The spleen serves an important health “regulatory” function and frequently, patients without a spleen suffer from serious and life-threatening infections

Individuals diagnosed with either thalassaemia and other haemoglobinopathies are said to be “immunocompromised,” with fewer body defenses to fight against infections. Because of this condition, patients that are immunocompromised, it is easier to get more severe infections and require extra “protections” including receiving all of the recommended childhood vaccinations. Many patients with these two medical conditions have had their spleen removed (

splenectomised) or are asplenic, making it very important for them to follow medical recommendations for vaccinations to avoid infection related complications and also to boost their immune system. As an illustration, the terrible swine flu epidemic of 2009-2010 resulted in several published medical articles documenting the incredible risk faced by both children and adults with thalassaemia. For instance, a Malaysian study[1] documented that 58.3% of splenectomised thalassaemia patients were hospitalized for upper respiratory tract infections. The same study also emphasized the importance of influenza vaccination for such patients, along with comprehensive counselling, and prompt initiation of treatment to prevent potentially fatal complications from infections. Another article[2] pointed out that patients with thalassaemia can present with classical clinical signs and symptoms of influenza. These include being febrile, showing malaise, having a runny nose, sneezing, and coughing. Left untreated, will develop more severe, if not life-threatening infections. The author went on to state, “Autoimmune haemolytic anaemia may occur during the course of influenza A infection”, which can pose a major health concern for individuals diagnosed thalassaemia and who have not been vaccinated against influenza. A U.S. study[3] documented serious illness that accompanies the flu, and an Italian[4] study reported a high frequency of influenza complications that could be avoided with proper vaccination. This includes acute chest syndrome and in some cases mechanical ventilation to assist breathing. Taken together, these studies confirm what we already know: flu is dangerous, especially for SCD and thalassaemia patients, and must be avoided with annual influenza vaccinations and proper hygiene, whenever possible.

Thalassaemia International Federation, WHO mission to the Maldives, August 2014, Dr. Michael Angastiniotis (TIF Medical Advisor) NICOSIA – CYPRUS compiled a report on the situation analysis of thalassaemia patients in Maldives and it states that infection-related mortality is the second leading cause of death after heart disease.

With the current outbreak of influenza and H1N1, and limited availability of vaccine, Maldives have come to the realization of the number of people at high risk for the infection. While the debate goes on how to prioritise the high-risk categories, the lives of our thalassaemia and other haemoglobinopathies patients are hanging on a line. With the maximum number of deaths this year compared to the past couple of years, it is only natural for the rest of us to be in fear for our lives.

Influenza vaccination is annually recommended for all thalassaemia and sickle cell patients. It is somewhat unnerving to know that these clear medical guidelines are not being followed and many patients / parents are unaware of their health importance.

DIRECT CALLS FOR ACTION 

Those of us ‘active’ in the thalassaemia and SCD community are concerned about this new healthcare crisis. We want to see more being done to protect our immune-compromised peers. Both influenza and Swine flu pose a tremendous health threat to the whole country. Diseases like the “flu” are easily communicated and affect children, the elderly, chronically ill and sickle cell patients more virulently than stronger and healthier people. We have already lost far too many of our young peers, their lives cut short too quickly, often by a disease that can be prevented through proper education and vaccination. The question, from a medical and public health standpoint, is have we done everything we can to prevent further loss of our beloved peers to the flu?

There are several easy and very cost effective steps that we can take to limit exposure of sickle cell and thalassaemia patients to the flu. First, we can mount a public health campaign that reminds people to get their influenza vaccinations. Doctors that work with sickle cell and thalassaemia patients (as well as other chronic diseases) can talk to their patient as flu season approaches, reminding them of the importance of vaccinations. Prevention works and reduces the risks of suffering from the complications of flu or any communicable disease for that matter. We can also take concerted measures to ensure there is sufficient vaccines in our medical stores so that everyone can be vaccinated. Finally, we can use the news and media (including social media) to remind people of the importance of vaccinations and time this media campaigns around flu season. Social media is an effective tool to communicate health information.

We can take exposure precaution measures but that doesn’t ensure our safety. The truth is that we can’t ignore the fact that this flu outbreak is posing a major life-or-death health crisis for the thalassaemia and haemoglobinopathies community. I thus urge our health system to implement an annual influenza vaccination program and to follow treatment guidelines for mandatory use for all genetic blood disorder patients in Maldives.

ACKNOWLEDGEMENTS

I extend my humble and deepest gratitude with utmost admiration to my mentors Russell E. Ware, M.D, Ph.D, Director, Division of Haematology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA and Lawrence M. Scheier, Ph.D.President, LARS Research Institute, Inc. Scottsdale, Arizona, USA (www.larsri.org), for your continuous support and encouragement. I am so grateful for your feedback and guidance in shaping up this article. Thank you so much!

REFERENCES

[1]Zarina, A. L., Norazlin, K. N., Hamidah, A., Aziz, D. A., Zulkifli, S. Z., & Jamal, R.  (2010). Spectrum of infections in splenectomised thalassaemia patients. The Medical Journal of Malaysia, 65(4), 283-285.

[2]Wiwanitkit, V. (2010). Thalassaemia and influenza: A consideration. Hong Kong Medical Journal (Letter), 16(1), 75.

[3]Strouse, J. J., Reller, M. E., Bundy, D. G., Amoako, M., Cancio. M., Han …, Casella J. F. (2010). Severe pandemic H1N1 and seasonal influenza in children and young adults with sickle cell disease. Blood, 116, 3431-3434.

[4]Colombatti, R., Perrotta, S., Masera, M., Palazzi, G., Dora, L., Notarangelo, A., P. …, Samperi, P. (2011). Lessons learned from the H1N1 pandemic: The need to improve systematic vaccination in Sickle Cell Disease children. A multi-center survey in Italy. Vaccine, 29(6), 1126-1128.