Sickle Cell - Shape change

Posted by Brianna @sammywinchester, May 15, 2023

My youngest sister was diagnosed at birth with Sickle Cell Anemia. She is currently 5 years old right now. When she went to the hematologist, they said that her cells were changing shape - they were becoming more rounded just as normal cells are. The doctors also recently took her off of the Penicillin. How is this possible? Is this normal?

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@sammywinchester, Hi Brianna. Welcome to Mayo Clinic Connect. The world needs more young women like you who are interested in the medical field. I hope you continue to be fascinated by diseases and blood disorders because they are quite amazing. I had a very aggressive form of leukemia and a bone marrow transplant a few years. Wow, talk about immersion into the world of blood cells and their nuances. I got quite an education and hold a deep appreciation for what our bodies are capable of overcoming.

From my understanding, sickle cell anemia is an inherited trait and it is not reversible. So the likelihood of all of your sister’s cells becoming normal wouldn’t be expected. If a child has one parent with the trait and the other doesn’t have the sickle cell gene, the child will have both sickle cells and normal red blood cells. Sickle cells die off faster than normal red blood cells. Right now her body may be producing more healthy cells, which is great. But from everything I’m reading that wouldn’t reflect a total reversal of the diagnosis of sickle cell anemia.

Mayo Clinic has a good informational article that explains in better detail than what I highlighted. It would be amazing if her condition turned around on it’s own. But the only way for that to happen would be through a total remake of her immune system with a bone marrow/stem cell transplant.

https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876

That actually might be a good question also for one of the nurses or doctors overseeing your medical assistant training. Is your little sister on any treatment for her sickle cell currently?

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@loribmt

@sammywinchester, Hi Brianna. Welcome to Mayo Clinic Connect. The world needs more young women like you who are interested in the medical field. I hope you continue to be fascinated by diseases and blood disorders because they are quite amazing. I had a very aggressive form of leukemia and a bone marrow transplant a few years. Wow, talk about immersion into the world of blood cells and their nuances. I got quite an education and hold a deep appreciation for what our bodies are capable of overcoming.

From my understanding, sickle cell anemia is an inherited trait and it is not reversible. So the likelihood of all of your sister’s cells becoming normal wouldn’t be expected. If a child has one parent with the trait and the other doesn’t have the sickle cell gene, the child will have both sickle cells and normal red blood cells. Sickle cells die off faster than normal red blood cells. Right now her body may be producing more healthy cells, which is great. But from everything I’m reading that wouldn’t reflect a total reversal of the diagnosis of sickle cell anemia.

Mayo Clinic has a good informational article that explains in better detail than what I highlighted. It would be amazing if her condition turned around on it’s own. But the only way for that to happen would be through a total remake of her immune system with a bone marrow/stem cell transplant.

https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc-20355876

That actually might be a good question also for one of the nurses or doctors overseeing your medical assistant training. Is your little sister on any treatment for her sickle cell currently?

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She was taking Penicillin for her Sickle Cell. After she went to the hematologist and they saw what was going on with her blood cells, they took her off of it. So at the moment, she isn't on any Tx for it

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@sammywinchester

She was taking Penicillin for her Sickle Cell. After she went to the hematologist and they saw what was going on with her blood cells, they took her off of it. So at the moment, she isn't on any Tx for it

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Was she on penicillin for a long time? Was this used as a prophylactic to prevent an infection?

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She was on it all her life. I'm not sure what it was used for - I think it might've been used to prevent severe infections as children with sickle cell anemia have a severely compromised immune system.

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@sammywinchester

She was on it all her life. I'm not sure what it was used for - I think it might've been used to prevent severe infections as children with sickle cell anemia have a severely compromised immune system.

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Prophylactic antibiotics are not uncommon for people with compromised immune systems. They can act as a temporary immune system in your sister’s case. I was on two different antibiotics for almost 3 years. They work!
It will be interesting to hear what is changing with your sister. I’m expecting she’ll be having followup appointments to see if this is a positive trend. It’s really sad that little kids have to deal with serious health issues. Will you please keep in touch? I’d like to follow along with your little sister’s diagnosis. Has there been any mention of a bone marrow transplant for her?

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There hasn't been any mention of a bone marrow transplant for her - I honestly think she might be too young unless she's getting put to sleep because she definitely isn't going to stay still.
I will definitely keep and touch and keep updating!

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@sammywinchester

There hasn't been any mention of a bone marrow transplant for her - I honestly think she might be too young unless she's getting put to sleep because she definitely isn't going to stay still.
I will definitely keep and touch and keep updating!

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A stem cell transplant is the only potential cure for sickle cell anemia. This may be a suggestion for your sister in the future. I had a bone marrow transplant and there is no pain involved for either the recipient or the donor. The donor donates cells via a procedure similar to a blood donation except the stem cells are filtered out and the blood is returned to the donor.
The cells are given to the recipient via a blood transfusion. There is no reason to be put to sleep during this infusion and it actually only takes about 15 minutes.
There are actual bone marrow procedures that can be a little more invasive but they are not like the initial transplants. No one is in excruciating pain. Both are a second chance at a normal life.

Being a sibling, if you are not a carrier of the sickle cell mutation and have a matching HLA for your sister, you could be a potential donor for her.
The transplant journey and recovery is slow. There is a preconditioning chemo and the need to recover from the infusion of unrelated stem cells. But it is an opportunity to start with a clean slate and have a normal life span. Children do quite well with this. It’s the same lifesaving treatment children with Leukemia undergo. So if this is offered to your sister, please don’t fear the worst. It is a bit of a rough journey for a year or so, especially the first month. Sickle cell is a life long disease with many complications. If there’s an option for a cure, that should be considered. 🙂

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@loribmt

A stem cell transplant is the only potential cure for sickle cell anemia. This may be a suggestion for your sister in the future. I had a bone marrow transplant and there is no pain involved for either the recipient or the donor. The donor donates cells via a procedure similar to a blood donation except the stem cells are filtered out and the blood is returned to the donor.
The cells are given to the recipient via a blood transfusion. There is no reason to be put to sleep during this infusion and it actually only takes about 15 minutes.
There are actual bone marrow procedures that can be a little more invasive but they are not like the initial transplants. No one is in excruciating pain. Both are a second chance at a normal life.

Being a sibling, if you are not a carrier of the sickle cell mutation and have a matching HLA for your sister, you could be a potential donor for her.
The transplant journey and recovery is slow. There is a preconditioning chemo and the need to recover from the infusion of unrelated stem cells. But it is an opportunity to start with a clean slate and have a normal life span. Children do quite well with this. It’s the same lifesaving treatment children with Leukemia undergo. So if this is offered to your sister, please don’t fear the worst. It is a bit of a rough journey for a year or so, especially the first month. Sickle cell is a life long disease with many complications. If there’s an option for a cure, that should be considered. 🙂

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Thank you!!! I really appreciate it!!

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