Our MMA fighter.
We knew this day could potentially come but we prayed it wouldn't. From day one, this kid has been a fighter!
For those of you that don't know our story, it is told throughout this blog! Feel free to leave this one and head back several posts to see where this journey all began!
Abigail has Methylmalonic Acidemia or MMA. In short, MMA is the byproduct of protein that is not broken down within the body. Abigail was born without the genetic makeup to create enzymes that breakdown protein. From the day of her birth, Abby has been fighting for her life because of this. Because Abby is unable to breakdown protein, she is on a long list of medications and has a special feeding tube called a G-tube where she is fed specialized formula for 18 hours a day.
Abby's short 2 1/2 years of life has consisted of more hospital stays than I can count. We average about one a month with some months having several stays back to back. This is because methylmalonic acid is toxic to the body and when the acidity in the body gets too high, major organs can become permanently damaged and begin shutting down. Thankfully, we have caught every episode before it has gotten to that point.
However, the MMA is beginning to affect Abigail's bone marrow. The bone marrow is where the different components of our blood is made. Abby's bone marrow becomes suppressed with every metabolic episode she has. Because these episodes are becoming more frequent, Abby's bone marrow doesn't have time to fully recover before getting hit again. And after two blood transfusions in the last 8 weeks, the doctors are beginning to worry that we may hit a point that her bone marrow won't recover.
So, why a liver transplant to fix this?? Good question! The liver is where the enzymes are stored that breakdown protein. Unfortunately for Abby, because her body does not have the genetic capability to make those enzymes, her liver has no enzymes in it that breakdown protein. But, when we take out her liver and place a liver from a healthy person without MMA inside her body, that liver begins using its enzyme store to breakdown the protein molecules within Abby's body. This will then decrease the MMA levels in her body and allow the organs that are failing because of the high levels of MMA to begin functioning again.
I know you all have several questions, so let me try and answer a few here.
Is this a cure?
No, this is not a cure. Because this is a genetic issue, Abby will always have MMA. However, with a properly functioning liver, it decreases the chances of major problems to Abby like deafness, blindness, weakening heart muscles, strokes and seizures to greater than half the chance she has now. We should also see a drastic decrease in hospital admissions because the body won't be becoming acidic as often and we should see her desire to eat increase.
How quickly will she get a liver?
We are still in the beginning stages of this process and have many appointments, blood work, and imaging studies to do before she is placed on the transplant list. This process will probably take 4 to 6 weeks. Once we are through this process, she will be placed very high on the transplant list. High enough that the goal would be to have a liver for her within a few months.
Can I be a donor for Abby?
Yes and no. Dave, my husband and her dad, is a possible match due to the fact that they have the same blood type. The process to become a live donor is an extensive one but feasible. The biggest component they look at when matching livers is blood type. But, yes, Abby can receive a partial liver transplant from a live donor.
Where will the transplant take place?
Abby's transplant will be taking place at the Children's Hospital of Philadelphia. She has been followed at this hospital almost her entire life and we know she will be in good hands.
How long is the recovery process?
The initial recovery will be about one to two weeks in the hospital with the first 3 days after transplant being spent in the Pediatric Intensive Care Unit. Abby will be very well monitored to make sure her body does not reject the organ in the first few days. She will be placed on medications to helps her body accept this new organ and will have several follow up appointments post transplant.
This is a long and scary process but one we know must happen in order for Abigail to continue to thrive. She has shown us already in her short little life that she is a fighter and continues to beat the odds. We are asking that you all pray with us as we embark on this journey. My hope is to keep everyone up to date about the process via this blog.
Next step...lots of meetings!!
