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.

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. 

#missionironcontrol

My Journey To Iron Chelation

Being sickle- beta Thalassaemic, I have been lucky to avoid regular transfusions until the age of 25yrs (22 was my first transfusion). My pregnancy with Eelaan in 2008, I was advised to stop the medication (a drug called Hydroxyurea

that was helping me maintain my Hb and reduce sickle pain episodes) and continue blood transfusions to maintain Hb above 8. I had to do blood transfusions every 2wks. Eelaan was

delivered at 36wks; by that time I had more than 13 transfusions done. Also I had multiple episodes of sickle crisis post pregnancy that required me to go for more transfusions. By 2012, I was admitted in ICU in Bangalore 3 times and had lost count of the number of blood transfusions I had done.

I give a lot of commendation to my parents for teaching me how to be observant of my body changes. Because of them, I am always on self-diagnosis mode. But all these years I have been learning and experimenting on ways to avoid sickle complications with all I knew and have experienced. I had already lost my spleen, and was diagnosed with avascular necrosis. I was being very careful to avoid infections and putting stress on my right hip to slow down the

progression of AVN. When I realised my serum ferritin level was rocket high during the severe crisis I went through in 2012, I had a lot of questions that were answered by the docs but not once iron chelation was a part of the discussion.

After recovering from my crisis, I was determined to volunteer with Maldivian Thalassaemia Society to raise awareness on sickle cell and thalassaemia. This decision, I believe was my gateway to heal.

Many times we are made to believe that thalassaemia is a disease of the young and they do not pass the age to become adolescents. Here I met extraordinary #thalwarriors who welcomed me into their thal family. Most importantly, I am so grateful for all the things I learnt about thalassaemia from them. I learnt that I needed to be checked for iron overload after 20 or so red blood cell transfusions and that accumulation of iron in the organs can lead to its function failure and eventually my death. The seriousness of iron overload complications cannot be physically seen until it was too late. But at that time, I had put my best effort to live healthy, exercise daily and eat properly. Surprisingly my serum ferritin was slowly dropping. And I didn’t 8 any blood transfusions. It amazed some of the doctors that I had discussed it with, that without chelation my ferritin was slowly coming down. I was told to keep them in loop of the updates. I believed my body was removing the iron from my body by itself.

Last year, I was having some upper abdominal discomfort. Went to a crisis due to an infection. My #thalfriends convinced me to do a t2* MRI, a scan that can measure the iron concentration in the organs. Alhamdhullilah my heart was clear but measured my liver iron concentration to be severe. Since then there has been lots of discussion to decide on what iron chelator would be best for me, and yet we never came to a conclusion as all of them had serious side effects. I have excused myself for a year to choose the best therapy, weighing at the end of all the cons than the pros.

3wks back, I had a wake up call. My upper tummy hurts; friends and acquaintances thought I am pregnant again. No, I was not pregnant, but my liver is enlarged, and my liver profile was no longer normal. I realized I might be in risk of permanent liver damage. If I keep waiting and denying the fact that it was possible to happen to me, I might be too late. 3 days ago, for the first time in my life I started my first iron chelation therapy. Desferal. I am determined to save my liver, my life. I have come too far achieving all my dreams to loose everything.

Today I am on a mission. #missionironcontrol with my #thalwarriors who have inspired me and motivated me to become a better person. I have found a new respect to those warriors living with thalasaemia major and complying with iron

chelation especially through daily desferal. Insha Allah, I am determined enough not to stop until all the extra iron is out of my system and practice healthy living to avoid further transfusions and be healthy for my babies, loved ones, families, friends and stand strong for others like me. I begin my iron chelation journey thanking everyone for making me a stronger #sicklecellwarrior!!

Bismillah! May Allah give me the strength to carry on!