sickle-cell disease

John Joseph.

What happened to him and what happened now? John is, is Sickle Cell.

Okay.

Uh, we're in Kaduna State, uh, Polytechnic.

[cheering] [applause]

John Joseph.

What happened to him and what happened now? John is a sickle cell.

Okay.

Bis zum Jahr 2525 könnten Wissenschaftler herausgefunden haben, wie man noch mehr damit erreichen kann, mit erstaunlichen Ergebnissen, wie etwa die Veränderung des gesamten Körpers.

Heutzutage nutzen Wissenschaftler die CRISPR-Technologie zur Behandlung von Sichelzellenanämie.

Allerdings sind wir derzeit nur darin geübt, Krankheiten zu beheben, die durch eine einzelne fehlerhafte Mutation verursacht werden.

In der Zukunft werden wir immer besser darin, Dinge zu reparieren, die mehrere Mutationen betreffen, sozusagen wie der Schritt von der Behandlung eines gebrochenen Beins hin zur Heilung eines komplett verletzten Körpers.

Next month, November 2nd, 3rd, 4th, 5th, and 6th will be Prison Jail Felon Talk.

Also coming up, we got Sickle Cell Talk October the 14th.

October the 19th is Mental Health Talk.

October 21st is gonna be Stop the Violence.

My name is Hadiza Ismail Rufaai, and I am your host.

Today, I am honored to be joined by Samira Haruna Sanusi, an author, a humanitarian, and the founder of the Samira Sanusi Sickle Cell Foundation.

Samira's story is one of extraordinary resilience.

After spending years battling sickle cell disease and enduring numerous surgeries, she eventually underwent a life-changing bone marrow transplant.

Choosing to not let her journey end with survival, she transformed her experience into a mission to advocate for people living with sickle cell disease.

She is the author of S is for Survivor, a deeply personal memoir.

Samira, welcome to the Boomin Boomer Podcast.

Thank you for having me.

Hello.

For a two-part miniseries about the recent Cochrane overview of systematic reviews she has led, entitled "Overview of the Effectiveness and Safety of Red Blood Cell Transfusions versus No or Restrictive versus Liberal Transfusion for Treating or Preventing Complications Experienced in Sickle Cell Disease." So, an overview of systematic reviews looking at all different types of red cell transfusion interventions for sickle cell disease.

It's an important topic and also maybe an unfamiliar methodology.

So we thought it might be interesting to get Lisa to take us through, firstly, how the study was done, which we'll cover in this episode, and in the second episode, we'll focus on what the team found.

An example here is looking at use of inhalers in, uh, pediatrics for asthma.

And finally, assessing the same intervention for the same condition or population, where different outcomes or time per- points are addressed in different systematic reviews.

An example here is the review that we've conducted, where it's looking at red cell transfusion to treat or prevent different conditions associated with sickle cell disease.

Thank you.

October the 1st is going to be autoimmune disease awareness.

October 2nd is going to be sickle cell talk.

October 5th, 6th, 7th, 8th, and 9th is going to be prison jail felon talk.

October 19th is going to be the next mental health talk.

I just talk things out about my life, but I wouldn't release them to the public.

So, this particular night, um, I was in this group called the FTM Men's Room, and I had started making friends with a couple dudes in the group, and I found out one of the guys had sickle cell.

So I'm talking to him, and he's telling me, "Well, I don't know if you can get on testosterone because they said I couldn't get on it." And I was like, "Word?" This was like 2008.

I had started testosterone 2009.

Y'all remember back in the day, 2008? I'm scrolling, and I found out the dude died.

His name was Jay.

He had sickle cell, and what he didn't tell me was he decided to go on the black market to go get certain things to enhance his masculinity, and he didn't know what he was doing, and he basically end up un-aliving himself because he didn't know exactly what he was taking.

Maybe his blood pressure went up and it made him have a stroke or heart attack.

So if we could just first, and this is open to the floor for everyone, what is sickle cell anemia?

Sickle cell disease is a genetic disorder.

People can have sickle trait, which means they have one gene.

But in sickle cell disease, they've inherited two genes, one from mom and one from dad.

And the sickle cell gene affects hemoglobin.

And when you have sickle cell disease, Your hemoglobin molecules stick together in your red cells and it changes the shape of your red cell, giving it that sickle form.

And that causes a lot of downstream problems, the major one being hemolysis or breakdown of those red cells.

But because of the breakdown of those red cells and the stiff red cells and increased reticulocyte count, those cells can get stuck in the microvasculature and cause significant amount of pain, which is the most common complication of the disease.

Very nice to be here.

It's a pleasure to have you on, and just to kick things off, for anyone sort of unfamiliar with sickle cell, what is it, and, uh, why do patients need regular tr- uh, blood transfusions?

Sure.

So sickle cell disease is a inherited blood disorder.

Uh, so it's where the red blood cells, which should normally be round and flexible-

Mm

Uh, it can lead to organ damage and eventually be fatal.

Wow.