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.