Standing Up For The Fallen

Yet another international thal day meets us with the theme: 'Thalassaemia past, present and future: Country practices, progress and growing recognition of patients' by popular vote among our thalassaemia communities around the globe. This theme was in hope to reflect and appreciate the progress among our health services to give a new meaning to life. This year’s thalassaemia day how ever comes with a feeling that has fallen heavy in my heart. 

As we grow older, we are only a few in number. I wonder what hope can this country give to the young ones who are born to live this struggle? What hope is there that in the future no child will suffer our pain? What standard of care will ensure to improve our quality of life? When are you all going to realize, the inability to produce blood is not a disability, however we are desperately in need of stability? When will the world accept us as warriors and encourage us with equal opportunities to learn, work, and build families? Do you really think, the services such as transfusion and a free medication is our gateway to live and fulfill our dreams? How about recognising the need for psychosocial support? How about addressing the need to educate societies, families, and patients on the importance of keeping adherence to our treatment? How about simply not bullying us for the way we look? 

Each year, I only hope that everyone recognises the need for a little progress every day. But, for the past couple of years, thalassaemia day greet us with pain. The list of dear lives that we have lost keep on growing. Beautiful moments that had made us stronger are only memories when the warriors who fight for our past, present, and future loses their battle.  My friends, even though they are no longer with us, I can still hear their voice, fighting and standing up for our treatment practices, progress, and empowerment, for our quality of life... and yet only little has changed.

Even more so, this year has been a tough one. Even though we are missing good old friends each year, I was never prepared to lose my beloved soul sister Niuma. She is a role model and a true inspiration. She fulfilled her dreams and gave all of us a precious gift. A little boy who will be the touchstone of her existence, a light of hope for all of us living. I want to keep her legend alive for he needs to know the story of his brave mom, who changed our lives and that she loved him with all her heart and life even way before she knew he was even a possibility. 

I am proud of who she has become and what she has accomplished. My love and respect for her will never be forgotten. She taught me to be calm, to be persistent, to keep on going when the wind seems to be against. She reminds me every time to set my sail and be the storm. I will miss her journey, her guidance, her love, and her patience. I miss her so much. 

There are no words that can express this feeling when you survive so many losses. Everyone is so quick to say how strong and tough we are, but honestly, we do not have a choice. And this is not a choice we would wish for anyone. 

Today, I want to celebrate International Thalassaemia Day, by cherishing the memory of those fallen. The only way I know how to grieve the loss of warriors is to raise voice for our future. Speak up for prevention of new births to our thalassaemia and haemoglobinopathy registry. Educate to prevent iron overload and its complications. Empower our community to prevent negligence and lack of awareness on our treatment and management. Encourage others like me to choose to live a better quality of life.

May Allah bless the departed souls, grant them Paradise, and give strength and courage to live and perceive life the way we all dream to accomplish. Ameen!

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.

Reproductive Health and Sickle Cell Disease by Ssebandeke Ashiraf

It’s my pleasure to introduce my 2^nd guest writer for this blog, my new friend, Warrior Ssebandeke Ashiraf, who joined sickle cell advocacy at the young age of 14 years after realizing the ignorance about sickle cell in his community. In 2012, after his 2^nd year in university, he joined sickle cell association of Uganda that was driven by his passion to learn and understand sickle cell better. With knowledge and better understanding, he managed to joined Sickle Cell Warrior USA as a Uganda Team Leader.

Ashiraf graduated in Bachelor of information and office management at Marakere University Bussiness School in 2013. He is the country reresntative and Kampala Correspondant for Africa Sickle Cell News and World report Nigeria. He is currently working as public Relations Officer of Sickle Cell Network Uganda (SCNU) which he co-founded in 2013.

SCNU is a sickle cell advocacy organization with an aim in improving the quality of lives of Sickle Cell warriors. His bigger vision is to realise a sickle cell free generation. Ashiraf also advocates for Renal Medullary Carcinoma, a rare type of Kidney Cancer that effects majorly sickle cell traits.

Ashiraf is a two-time nominee of WEGO Health Activist of the Year in 2013 and 2014. This year, he was honored the International Sickle Cell Advocate of the Year 2015 by SickleCell101.org to recognize his efforts into raising sickle cell awareness in his community.

Conquering Dreams

As I get myself ready for the 2nd MEGMA Conference on Thalassaemia and other Haemoglobinopathhies, in Amman, Jordan from 11-12th November 2016, I can't stop thinking how lucky I am for the wonderful and generous friends and family I have. Thank you from the bottom of my heart for being a part of my journey and making it possible for me to participate in this conference. 

What I am most excited about my conquering dream episodes each year that you have made possible is that I get to share my perspective on how medical professional can help empower patients living with sickle cell and other haemoglobinopathies around the world. This is my 4^th presentation at international platform, and the 3^rd year that I will be sharing My personal journey living with sickle cell and hydroxyurea therapy during pregnancy and lactation at the scientific programs.

For those who might ask what is Hydroxyurea (HU), it is a drug that is known to reduce the frequency of sickle crisis episodes and hospitalizations in SCD patients. However, it is believed that if HU is used during pregnancy or if the patient becomes pregnant while taking this drug, the patient should be told of the potential harm to the fetus as animal testing shows that the drug crosses to the placenta and causes embryo toxicity, fetal malformations, growth retardation. Therefore, patients are advised to avoid becoming pregnant while taking HU, or terminating the pregnancy due to its described potential side effects on baby.

This leaves pregnant sickle women with a real dilemma: Take a drug with unknown effects on fetal development or give up a helpful, necessary medication. This I know, because I have been through this dilemma several times since 2004 until 2009. I have been discouraged from ever having a baby, I have been told I should focus on maintaining my health rather than raising a family. My husband had been told that he must be crazy to have kids with me. I have been advised to terminate each of my pregnancy because I conceived on HU. 

Past 11 years, I have had 3 pregnancies each experience different regarding HU therapy. 1^st pregnancy, I had a miscarriage and sever sickle crisis, because I had been taken off hydroxyurea and I didn’t know that transfusion was an option for me. 

My 2^nd pregnancy, I had been off hydroxyurea, transfused every 2 weeks, went in to severe sickle crisis during 2^nd trimester and barely made it to my 37^th week, where he was delivered early. Alhamdhullilah, our handsome lil’ hero Eelaan, gave me a whole new strength to make me realise that anything is possible. I felt like my dream had come true, and no one is going to stop me from dreaming the impossible. The moment he was born our lives changed completely.

Having a child of my own to call me mom was a dream I thought was beyond my capability. And I realized I had more to do and dream a little bigger. My family and friends thought I am crazy to even think for a second baby of all the things that I had gone through, but I had long realized that we never know our possibilities until we try.

I had the courage to go through my last pregnancy while taking HU. Although I was nervous of the unknown, about the effect of Hydroxyurea on baby’s growth, or any abnormality physically and intellectually, I was stressed thinking what if the scans doesn’t detect. I kept mentally preparing myself for the worst. And I was ready to accept whatever may be the outcome of this pregnancy. However anxious I was about not knowing what it might do to baby in the long run, I always knew I had made the best decision to continue with the pregnancy and Hydroxyurea therapy.

I had amazing, healthy and active 40 weeks before welcoming her into our hands.  I kept a close watch on her, at first it felt like such an achievement when she crosses every milestone and she’s establishing them quick. She’s active and a very happy girl. Just like any other 2 yr old would be. My lil’ warrior Eiliyah, Masha Allah, the best decision ever. I believe that my daughter is the hope everyone like me

 is looking for.

Deciding to breastfeed the baby was a hard choice, because I had to go back to HU, and everywhere I read it states that Hydroxyurea is excreted to milk and has the potential of serious adverse reactions and may cause harm to baby. When Eelaan was born, I chose to stop HU and feed him, biggest mistake ever. I went into severe crisis that put me in ICU multiple times and he was not even 2 yrs at that time. I had lost my spleen, was iron overloaded from all the transfusions I had, was diagnosed with avascular necrosis of my right hip, and I had no choice but to go back on HU.

With this experience, I decided I would continue HU even after my lil’ warrior Eiliyah was born. I have taken a huge risk of having a baby while taking Hydroxyurea and I saw no point in stopping the med to breastfeed the baby. I struggle with my doctors to continue Hydroxyurea while breast and bottle-feeding at the same time. So far I have not noticed any negative side effect of HU on her, Alhamdulillah!

Many women are in fear, when it comes to pregnancy and raising a family, since we are reminded of how risky it is to become a mother. In my opinion, doctors who are unaware that HU can improve the health of sickle cell patients during pregnancy is taking a huge risk by stopping it since it would mean the recurrence of sickle complications that could be life threatening for mother and baby.

It’s important that doctors find it in themselves to empower patients and encourage them to be the best they can be. That is why this journey of conquering dreams is so important to me. I hope the story of my personal journey can open the possibility for others like me to have their dreams come true.

Demystifying The Cure! by Marie Ojiambo (part 2)

I shared the article talking about this said cure on my social media channels. From this single post, I received a plethora of questions from my warrior family both in the US and in Kenya. Amongst the inbox messages was one that completely humbled me and affirmed to me that my work in advocacy was not for self but as a responsibility to others. 

Jason wrote to me saying that he had undergone this haploidentical or half match bone marrow transplant procedure. I wrote him back to ask if he would mind sharing the experience with me. Everything, down to the nitty, gritty details… I honestly didn’t expect much from Jason. A paragraph or two I thought would suffice. Then Jason completely opened up to me. I didn’t change anything from his account just so that you read it in its undiluted form, directly from the horse’s mouth. I bombarded Jason with all my questions, all at once, as I felt as though this opportunity would flee from me. In my past experience working in advocacy, it has been extremely difficult to get patients to open up candidly about their condition as well as the daily challenges that having this condition presents them. However, when it came to this gentleman, once he begun to answer my questions I momentarily regretted writing to him in such haste because the account that I received was extremely calm, well put together and detailed although very heart wrenching for me whose sympathetic heart runs deep, much deeper than my empathetic heart, a quality that individuals working in the medical fraternity should posses.

So Jason asked me to be a little patient, two days to be exact, as he needed some time to sit down and respond to all my questions. I was convinced that he’d flaked on me after I didn’t hear back from him two days later. Ten days after I sent him my questions, what felt like a year to me, Jason finally replied in such a personable and patient manner, I thought I was sitting with him while reading this account. It was overwhelming and moved me to tears. When I began my work in advocacy, many don’t know, but I cried a lot when patients wrote to me about the insurmountable health struggles that having this disease presented them. I thought my life was in shambles because of Sickle Cell Disease, and then the gift of advocacy came to me.

Here are my questions followed by ’s Jason’s account:

What are your names? “Jason,

How old are you? 43 years old

What type of sickle cell did you have prior to the transplant procedure? SS or full-blown Sickle Cell Anemia.

How often were you in crisis pain before the procedure? Pretty healthy, major crisis at 15 but not much since.

What led you to exploring the possibility of the transplant procedure?

Problems developed more with age (gout, eye issues, venous stasis, ulcers from lack of proper blood flow/pooling in feet, etc.). Started getting transfusions in mid-30's (less than 10 on my own accord, about 10 as protocol for the NIH)

Would you say that you had, mild or severe manifestations of the disease?

Manifestations of the disease; very mild. Had hospitalizations only related to surgeries not really related to having sickle cell disease. Went to NIH after suffering Acute Chest Syndrome (which was brought upon from a trip to elevation, my fault totally) followed up a year later with Cardiac Arrest (was working out in a gym, lifting weights, passed out, my heart stopped). Received AICD from that (a pacemaker/defibrillator).

How affordable or expensive is this procedure for the regular patient suffering the disease?

The National Institutions of Health is a research facility so it costs nothing but time. You do have to relocate there for the time being and you may be eligible to receive compensation, depends on the protocol you take place in.

Can you outline the process for us?

The process was pretty straightforward but it does take some time. I met with them, ran a baseline of tests to see where I was, if I was a good candidate. Some 8 months later I received a call to see if I wanted to go or would rather wait for them to finish up with some patients to see if any more successes could be achieved. I opted to go right away (I was tired of waiting and a little fearful that my health was deteriorating at a fast pace and waited to see if we could achieve success). I took another trip with my donor (my half sister from my mother who was a match of 4 out of 6 on their DNA matching scale). So the procedure is a Haplo-identical Stem cell transplant. All my siblings were tested and my father as well as my siblings were rated on a scale of 1 to 6. Everyone you are related to is at least a 3 already (your sisters and brothers that is). I have no full blood siblings, I'm the only child from my mother and father and the only one with the disease in my family.

My 2 sisters from my mother were 4 out of 6. My sister, brother, and father were all 3 out of 6. They chose one sister over the other because we have the same blood type. My sister was given a drug for 5 days to release fetal stem cells from the marrow which were then harvested through an 8 hour long blood draw.

Once there, I received Chemotherapy and Radiation therapy. Going in, I expected to be dog sick from Chemo like how we see in movies (vomit, crawling on my knees, etc.). It was nothing like that. I walked 5 miles the day I had chemo and radiation. I was on a boatload of medication (stuff to keep my immune system down), the most meds I've ever taken in my life. So chemo and radiation wiped my immunity away, I was given the donor cells (like a long transfusion) and then we had to wait…..

I did blood draws everyday to keep an eye on my immunity, to watch the white cell count, to check the chimerism (amount of donor cells sticking around). Slowly your immunity starts to come back (white cell counts go up, but you're still susceptible to infections) so you're still on a bunch of medications to keep you free of infection but at the same time keep your immunity suppressed enough so that the donor cells can take root and not get rejected by your own cells

What was the recovery period like?

I spent 50+ days in the hospital where typical time is 30 days. I was getting fevers and fevers as most know, usually equals infection, so that held me up. I finally got out then I had to spend 100 days in the area so that they can monitor me. Hospital visits 3 times a week with blood draws, medical reviews, the whole gambit.

Would you recommend the procedure to other warriors?

Yes. My thinking is/was that you got a shot to get rid of this disease, why wouldn't you take it? The NIH told me that people went there and declined the transplant procedure because of various reasons (bone marrow biopsy, which is invasive etc.), huh? I can't understand that, but it's not for me to judge. My whole life I've dreamed about what I could be, physically (mentally too, because this and any disease can take a toll on you mentally) if I did not have this disease. That's just me though. So if you have a chance to do it, I would hope that all who can, would do it.

How are you now?

It's safe to say I did not get "cured." My donor cells dropped to less than 8% after being over 69% after the transplant. Consider that full-blooded siblings have had 100% chimerism resulting in the "cure" of the disease. I have pretty much retuned to my old self pre-transplant, which is no better nor worse. So what did I gain? Nothing…. But, what did I lose? Nothing…. Nothing ventured, nothing gained. I had a shot, I took it! I missed, but if I had to do it again, I would in a heartbeat for a sliver of a chance that it could work. I'm still under the observation of the NIH. Still on a boatload of medication.

How has this procedure changed your life?

I found out that despite being as healthy as I am and have been, I'm not that tough. Being in the hospital 50+ days drove me insane! Away from my family, I've never felt so alone. Health wise, I'm not any better, not any worse. I'm not allowed to do a whole lot because they want me to take it easy so that is boring when I'm ready to go back to the gym, go back to work, etc. But like I said, I'd do it all over again to try and be rid of this.

Jason then went on to say

‘I'm sorry it took so long to get back to you, got busy with appointments (looking into doing another transplant in the future possibly). If I forgot anything or you want more specifics of something I may have glossed over don't hesitate to ask. Warriors can message me questions that they may have regarding anything.’

I thanked Jason.

And to this he said,

“You're welcome. I love talking about it to inform people because this is the forgotten disease. I've never known anyone with SCD my whole life so I dealt with this alone. Luckily I was pretty healthy but I want those that aren't as lucky as I was to know that their future can change and they can hopefully look forward to getting rid of this disease. So it's my pleasure. Thank you for having a forum where I can do that.’

So now that you know a little more about ‘the cure’, would you consider it?

I know I would ☺

Stay Positive Warriors. 

Love and Light,

Marie.

**Jason can be reached on mrjwwright2001@gmail.com or Fb inbox Jason Wright.

Press the link below if you have not read the part 1 of Demystifying The Cure! by Marie Ojiambo :

https://shifneez.blogspot.com/2016/10/demystifying-cure-by-marie-ojiambo_10.html

Demystifying The Cure! by Marie Ojiambo

I am super excited to feature my very first guest blogger, my very dear sister warrior Marie Ojiambo on this page. She is also a Sickle Cell warrior and a global advocate for sickle cell disease awareness. She was diagnosed at the age of 1 and has since been living with the condition.

In the year 2013, she founded the Sickle Strong Initiative (SSI) a not for profit whose mandate is to raise awareness around sickle cell disease and to advocate for better healthcare opportunities for patients suffering the condition in Kenya.

Trained at the University of Nairobi, School of Pharmacy, Marie Ojiambo is a Pharmacist by profession. Marie recently graduated with her Master’s degree in Industrial and Physical Pharmacy from St. John’s University, College of Pharmacy and Health Sciences in Queens, New York. She is specializing in drug research and development, with a focus in formulation sciences.

Through SSI, Marie has been able to host medical training and awareness campaigns for patients suffering the disease in Kenya. She is also the proprietor of the annual sickle cell public awareness forum, Ongea (which means speak out in Swahili). It is a platform that gives sickle cell warriors an opportunity to speak out about their condition, share and interact with each other. It also provides an opportunity for warriors to engage with and challenge the government, the pharmaceutical industry, insurance companies, other stakeholders and the public on what they are doing to improve the health care and environment that these patients are exposed to. Her initiative hosted the third edition of Ongea on the 18^th of June this year that brought together 300 warriors and their families. 

Marie is also former Miss Kenya USA (2013), the People’s Princess Miss Africa USA (2014), first runners up and Face of Kenya USA (2016) platforms that she has used to raise public awareness on the disease.

Loss In The Sickle Battle Field Is A Lesson To Be Learnt

Few years ago, I gave hope to a girl, told her she can grow up, study as high as she can, have a normal life, have family on her own. I made her to dream big, and told her that she can have anything she wanted, as long as she took care of herself. That one year, she went to school, did her best to excel. She took care of herself, for a moment she owned her disease. And then, the bomb started ticking. I never thought that day would come. My heart sinks every time I think of her. How brave she was even to the last moment. The story of her life is worth a thousand lessons. And one of those lessons included Sickle-beta thalassaemia.

If you google up sickle-beta thalassaemia you’d find it listed among rare diseases. Sickle beta thalassemia is inherited from parents that can be diagnosed as early as when the baby is in your tummy or usually during early childhood.

Its symptoms and complications may vary from mild to severe, depending on the beta thalassaemia gene that is carried by the person. Problems include moderate to severe anaemia (low blood count); episodes of pain in the arms, legs, stomach, and back (pain crisis); increased risk of serious (blood) infections in younger children; gallstones; enlarged spleen; and risk of sudden drops in blood count and many more other complications that may lead to organ failure that may be fatal. 

Now you must be wondering what a crisis is? The sickle-shaped red blood cells break down faster than normal cells. The sickle shape cell have a tendency to stick to each other and also can get stuck in your blood vessels, preventing flow of blood (oxygen) to areas of the body. This causes immense pain and can damage body organs, such as kidneys, lungs and even bones.  Stroke and other medical problems can be caused by blocked blood flow. When these problems happen, it is called a crisis.

I hope u all realise now that sickle cell is a ticking time bomb. With 16.3% Beta Thalassaemia and 0.14% sickle carriers in Maldives (source: Society for Health Education) the possibility of these two genes to meet in one child is so high here, According to the recent analysis of the sickle variants diagnosed at the SHE lab, from 1998 to 2015, there were 108 sickle cell trait, 9 Sickle-Beta Thalassaemia, and 6 homozygous, fully blown Sickle Cell Aneamia cases diagnosed. Sickle Cell Disease is not such a rare disease in Maldives. But it’s an invisible disease. Get yourself screened. Know your status. It’s your responsibility.  Prevent a child being born with Sickle cell Disease cause I wouldn't wish this pain on my worst enemy.

Her story also tells us that we need sickle cell specialists here, in order to guide and treat patients like us. A person with expert knowledge whom may actually be able to diffuse this sickle bomb and prevent any fatality.  But since we don’t have one at the moment, its important that we have access to appropriate and reasonable medical care. wipe away clinical ignorance by raising sickle awareness among the medical professionals, parents and caregivers. we need to win this battle against Sickle Cell Disease.

The treatment and research plans so far have been weak. The lives of individuals and their families struggling with the diseases to the most part have been ignored. Our experiences as a patient needs to be listened to and learnt. We may not have a certificate that says we are sickle experts but we have survived a history of trial and error.

Take our lives, our experiences as an open book. Each one us, has a story to tell. If you read and look into it, you’d find that our fight is the same, however, our struggle to win this battle differs in each individual. The different roads that we took to beat the unbearable pain are worth a try on someone who’s fighting the battle in your hands.