milan

Keira’s Leukapheresis

One day when we were back home in Arizona, we were being interviewed by a reporter who asked to describe the gene therapy treatment we were hoping to get for Keira. Dave said it best when he began to answer with “It’s something out of a science fiction novel…”

The process by which they do all of this is remarkable. But simply put, they extract stem cells, put them into vectors, take out the bad genes, replace them with good ones and then reinsert the stem cells into the body via a virus (without the bad parts of said virus) so that it quickly spreads throughout the body. 😳

Step 1 is leukapheresis, the removal of stem cells. In order to do that, Keira had to have a central line surgically placed into her femoral artery. That is where they would hook up the machine that would process her blood and remove stem cells.

It was a quick surgery and as with all IVs she gets, once they are placed she doesn’t even care they are there and just wants to continue playing like normal (fun for Mom who gets to watch her every move and make sure nothing gets tugged on or twisted).

Straight from surgery she went to the leukapheresis room to get hooked up to the machine. Each session would require her laying (optimal position for extraction) as still as possible for 4 hours. No easy feat for an almost 8 month old who is on the move.

The cords you see coming from her central line go to the machine behind her. That one machine does it all! And that is Paola who was absolutely wonderful! Keira would light up when she saw her and she was so kind to bring me espresso each day.

The first session was a little easier as Keira was coming out of sedation from the surgery and was tired. The second day I had to pull out some distractions. Thankfully, we did not need to do a third session as they got the maximum amount of stem cells needed for the procedure.

They generally hope to get around 30 million stem cells per kilo (which I hadn’t known prior). So when they told me on the first day they got 10 million stem cells per kilo I thought ‘Wow, great! We’re done in one session.’ Not so much. Thankfully they were able to double her dose of the medication used to stimulate the stem cells’ movement throughout the body so on the second day they were able to extract 32 million stem cells per kilo! Of the 42 million total, 5 million will be used as back up and the rest will be used in the gene therapy, giving her the maximum dose for the procedure.

A closer look at the leukapheresis machine. The bag on the upper right is the collection of her stem cells.

It was really crazy to see all of this happening in one machine and Keira laying there happy as a clam like nothing was going on.

Around us were three other beds with people also receiving stem cell removal. They were all older men and women who got a kick out of Keira smiling at them, and blabbing on and on. “Bella!” or “Bellisima” they would all say. 🥰 Hopefully she brightened their days a little as they brightened ours!

Next step for Keira: surgery on Monday to have a permanent central line placed for the upcoming gene therapy.

  • Our happy girl!
  • Dancing to the Bealtes in the hospital room.
  • Waiting to go into surgery with Dad
  • Taking a nap while they begin treatment.
  • Her naps made the 4-hour process of laying down so much easier that first day!
  • Wires or not, she is on the move.
  • Pulling an all-nighter… 🤦‍♀️😴
  • Kisses for Mama!
  • “Bellisima!”
  • Happy we’re done and ready to go home!

The Beginning of Keira’s Treatment

The day finally arrived! On Friday, September 4th we took the first step in Keira’s gene therapy treatment!

After being admitted to the pediatric floor of the hospital they put in an IV (which needed to be taken out and redone the day after), took blood samples (which they do daily) and every 12 hours inject a medication that will prompt her stem cells to be released from the bone marrow and into the bloodstream.

On Monday morning, she will have surgery to place a central venous catheter followed by the leukapheresis procedure for collection of stem cells.

So far, she’s been handling the hospital stay great and gets over any pokes fairly quickly. The only thing that really holds her back is the IV on her foot and the bandage protecting it.

That aside, she’s having no trouble keeping me up at night.

One thing I came prepared for (for her anyway) was bringing food from home (bananas, avocados and berries). I’d heard stories from other gene therapy families about the difficulty of them understanding that a baby this age eats normal foods (in addition to formula) and not pureed or processed foods. I’ve also heard the food at the hospital is just plain bad. They were right.

I’ve told them multiple times about her diet and they still bring the pureed, processed and soupy foods. For me, here’s an example…

  • I don’t know what this was but it smelled , and tasted, like black licorice. 😝
  • Better this time…chicken thighs (I think), steamed cut carrots and not the best risotto.
  • This time I asked for pasta al pomodoro (spaghetti basically) for dinner and got an egg frittata with green beans and soup. 🤔
  • This morning I was served tea in a bowl. 😳 With biscuits. This is also the only thing they offer children for breakfast.

Food aside, the rest of our stay so far has gone well and the staff is amazing.

To say we are excited about this treatment and the life it will allow for Keira (and all of us) is an understatement.

What’s next? After this, we go home – to our new apartment instead of the hotel we were staying at – for a few weeks before Keira is admitted to the hospital for 40-60 days for the remainder of the treatment. That will include chemotherapy, the gene therapy itself (administration of corrected cells), followed by ongoing bloodwork, scans and other tests to ensure her body is safely accepting treatments and all levels are returning to normal.

Thank you everyone for the outpouring of love and support throughout this process. It means the world and we will continue to keep you updated on our journey! ❤

The view from our room.

We Made It!

It’s officially happening! Today, we landed in Milan, Italy to begin Keira’s gene therapy treatment!

Every step of this journey has been truly unreal. But to be here now, it has sunk in. We made it!

And we made it thanks to all of you who have supported, prayed, donated and sent us all the good vibes.

I know the next 5 months will not be easy for Keira (or all of us) but it will all be worth it when we can see her run, jump, spin in her dresses and talk in sentences. I hope she talks our ears off for the rest of our lives!

Every Mom knows the relentless “Mom! Mom! Ma! Mommy! MOM!” when kiddos are trying to get your attention and I am so excited for that!

Eva (our oldest who is 5 years old) of course does that daily (and yes, it can sometimes drive me crazy) but MLD has given us an entirely new perspective and appreciation for literally everything in life. Especially in relation to our girls. They are our world and every moment is precious.

So, what’s next? Hopefully we can sleep a little bit while we quarantine for 2 weeks! And on Monday just Dave, Keira and I go to the hospital to begin the process by discussing the treatment plan with doctors and doing a bunch of testing (bloodwork, EKG, xray, MRI, you name it). It’ll be a busy week. And we can’t wait!