Hannah’s Story

My Hannah-Banana was born almost 4 weeks early.  She was born on May 5th ~ Cinco de Mayo!  I NEVER need an excuse for a margarita but I sure do love  THIS excuse 🙂

Hannah & Sophia meeting for the first time!

Hannah & Sophia meeting for the first time!

Our lives changed immediately; we went from a family of 3 to 4 and while it doesn’t seem like that big of a deal, IT’S A BIG DEAL!!!  The funny thing is I always wanted a “tribe of kids” – had to be an even number though.  I didn’t want anyone left by themselves on an amusement park ride!  So, if we were to have more kids, we would have to bypass 3 and go straight to 4 ~ AND that’s not going to happen!  I can barely get through the weeks with my new 10-AGER and Sophia hasn’t even been 10 for a month yet!

Sophia, THE 10-AGER!

Sophia, THE 10-AGER!

So here she was, my new little Hannah-Banana.  My sweet little baby who I wanted to smell all the time ~ that new baby scent would be gone before I knew it and I just couldn’t get enough.  You all know what I am talking about…

Little Hannah-Banana

Little Hannah-Banana

Our first month was without havoc – Sophia was only 15 months old so it was just a crazy balancing act but we embraced it with open arms.  Sophia was starting to talk in little sentences and I didn’t want to miss out on that.  Our favorite was when she learned what a chicken does…BA-COCK!

What does a chicken do?

What does a chicken do?

Then things started to change.  My little Hannah-Banana wasn’t feeling well.  She got conjunctivitis somehow and it wouldn’t go away.  She got a yeast infection on her bum that wouldn’t go away.  She seemed so agitated and that wouldn’t go away.  She couldn’t keep my milk down.  She wasn’t sleeping.  I walked into my pediatrician’s office and just cried.  Did I do something wrong?  Was I an unfit mother?  How did all this happen?????  Dr. Paul looked at me and looked at Hannah and sent us off to the hospital – I don’t know what’s wrong, she said.  But I do know I’m NOT sending you home until we find out.  Hannah tested positive for RSV – OK, we’ve got a diagnosis.  They loaded us up with antibiotics and sent us on our way.  I had a brand new baby…for 10 days.

Happy 4th of July

Happy 4th of July

We finished the antibiotics.  And then she turned pink again.  And then she started to get the rash on her bum again. And then the gook in her eyes came back again.  And then the respiratory distress started.  My baby was having a hard time breathing.  I’ll never forget when I noticed her chest was con caving so deeply – I just cried.  And cried.

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I walked back into my pediatrician’s office and she knew.  She picked up the phone and called the ambulance.  We were on our way back to the hospital.  We saw way too many doctors.   Oncology.  Infectious disease.  Hematology.  Pulmonary.  Liver doc.  Kidney doc.  You name them – we saw them.  Then after being there for over a week, the Pulmonologist walked into our room and said, I know you guys are overwhelmed.  I know you’ve seen way too many doctors.  I just stuck a big spoon in this pot and we’re about to see what comes of it and then he left.  A few days later, they told us we’re sending you home.  We don’t know what happened to Hannah but we’re sending you home soon.  And then they left.  But then they came back a couple hours later…WITH THE BIG SPOON.

Doug had just left to grab a couple of things from the house.  I just remembering hearing, Mrs. Smith, it seems as though Hannah may not have a functioning immune system…she may need a bone marrow transplant…we’re sending you to Children’s Hospital tomorrow…

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It all sounded like Charlie Brown’s teacher…I just remember thinking, someone call Doug.  PLEASE someone just call Doug.  I was screaming…SOMEONE CALL DOUG.  Doug was back in 15 minutes or so.  I remember him walking in the room…and that’s it.  That’s all I remember from that day.  That day changed our lives forever.  It changed my Hannah-Banana’s life forever.

Side note:  This pulmonologist with the BIG SPOON?  He had a patient a few years before with the same disease.  Something in her blood work made him think to run these additional tests…honestly, right place, right time, and RIGHT DOCTOR.  I’m also pretty convinced if my pediatrician wasn’t “on it” to begin with, I wouldn’t be writing my story about Hannah right now.  They saved my baby’s life.  They saved MY LIFE.

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Hannah was diagnosed with ADA SCID (Adenosine deaminase  – severe combined immunodefiency).  This is a genetic disorder whereby her body does not produce the enzyme Addenosine Deminase and without that enzyme her T cells die prematurely.  Our immune systems are ineffective at fighting off infections without T cells.  The most common treatment for SCID, brought on by many different types of mutations which disable the immune system, is a bone marrow transplant. However, with the type of SCID Hannah has, there is a non-curative treatment called PEG-ADA. Similar to treatment for diabetes where the enzyme insulin is injected, Hannah receives a form of the missing enzyme in a shot she receives twice a week. She’s been on this medication since August 2, 2004 and has been remarkably healthy since then.  For those who are lucky enough to be around her, SHE’S A HOT MESS!  Apple doesn’t fall far from the tree is what I hear people say – not sure what they are implying 😉

Hannah is missing a little teeny-weeny gene from her DNA.  We always knew gene therapy would be an option one day.  We’ve debated about this for a long time.  Why fix it, when it ain’t broke, right?  She’s doing well on the PEG-ADA so no reason to mess with it.  Well, she’s getting older.  As kids get older, they don’t respond as well to the gene therapy.  Hannah’s at that cusp; she turns 9 in May.  So we think, why not give it a try.  If it doesn’t work, she can go back on the PEG…BUT what if it does work????  WHAT IF HANNAH RESPONDS TO THE TREATMENT??  How GREAT would that be??

There have been some successful results over the past few years.  The clinical trial she’ll be participating in is a new protocol that docs seem excited about.  She’s already begun the journey with her week’s stay at NIH in early February.  She went in for a procedure call Apheresis ~ the procedure involves collecting specific stem cells to be harvested for a bone marrow transplant.  The missing gene is introduced into the stem cells they collect and then those cells are injected back into her blood stream in a manner similar to other bone marrow transplants.  It’s a little more complex than that, but that’s the best I can do in my own layman’s terms.

AND LET ME TELL YOU, the science behind this is amazing.  As Doug puts it, “It’s incredible to learn how much scientists understand.  But also a little scary as it is clear there is still a lot which is not fully understood.”  I should also make note, that Doug did give me the verbiage to write for the explanation of ADA-SCID.  He’s good like that ~ a man of little words but when he speaks, it’s very intentional.  That’s my D.U.G…

Without getting into more long drawn out medical detail, Hannah has responded incredibly well to the whole process so far.  Not only was she BRAVE, STRONG, COURAGEOUS and still had all her wits about her, she gave the docs more blood cells then they could have ever imagined!  She was like a little super star in the hospital with docs, interns, and fellows stopping by her room to meet her congratulating her on her BIG stem cell collection – she looked at them with her one-eyebrow look and said, I don’t even know what you’re talking about!?!?

What the...???

What the…???

Brave Girl!!

Brave Girl!!

Now, we are waiting for the next phone call letting us know when to come in for our extended stay.  This will be a doozy…when Hannah goes back into NIH, she’ll prepare for her bone marrow transplant using the cells extracted from Aphersis as well as her actually bone marrow.  This little vial of newly “infected” stem cells with the ADA gene will be pushed through her port line…and then we wait.  We wait to see if her immune system can function on its own without the need for the PEG-ADA.  The thing about waiting is we’ll be waiting in isolation.  At the hospital.  In a hospital room.  My Hannah will be in her own little bubble until we see signs of a working immune system.  She could be there for 3 months.  She could be there for 6 months.  We just don’t know.  What we do know IS this is NOT an event with a beginning and an end.  This is a process…a journey.  I hope, a new beginning…

Doug, Sophia, Hannah and I will be in the same room but for a handful of times.  Thank God for FaceTime!!  We will take turns staying with Hannah at NIH and tending to Sophia and keeping up with her life back in B’ville.  It will be challenge for all of us.  Even with the good, the bad and the F-UGLY,  I know we will get through it.  Thankfully, we have family and friends who support us on so many different levels.  As I said in my Valentine’s letter, it will take many lifetimes of gratitude to make up for all the generosity and love that has been shown to us.

My cup runneth over ~

Photo by Purple Fern Photography

Photo by Purple Fern Photography

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15 thoughts on “Hannah’s Story

  1. Thank you for sharing your stories and your family, the courage strength and LOVE you guys are made of is nothing short of Magnifabutastic!!! Count us in on the homefront, we even do windows :)!!

  2. Thanks for sharing Monica. Hannah will be in our prayers. God is so amazing always working things out I am convinced you met the MD who you needed to because of him he just works like that. Blessing us in ways we don’t even know or could ever imagine.

  3. Monica
    What a beautifully written story. My thoughts and prayers go out to you and yours. I have a ton of respect for you and Doug, what amazing parents you are. Your family is an inspiration
    Xoxo

  4. Well said, Monica! You know we’ll be praying for Hannah and the rest of the Smith fam! Love you all to pieces Xoxo PS tell Hannah if she wants to chat, we can be penpals while she’s in isolation. 😉

  5. Such a lucky family to have such a beautiful girl. Just beautiful! Doctors who take their time and love their work are a Godsend…whe others are confusing as hell!

  6. Oh my goodness… tears and smiles of joy. We love ya guys. That Hannah Banana sure is one tough cookie. Hmmm, she sure does remind me of someone. Ha, now that I think about it, she’s a mini version of that “someone” 🙂 Hugs and Blessings!

  7. Thank you so much for sharing and for letting us be a part of your life! Hannah and your family continue to bring us joy and many blessings!!! God has given you a precious and strong gift, Hannah, who has an incredible way of touching the lives of those she meets. We love you guys!!!

  8. Our thoughts and prayers are with you and your family. Having traveled the same difficult road…rest assured you are in the best healthcare facility in the world! Our Cindy started going to NIH at the age of 9 for the same disease. She was the 2nd gene therapy recipient in the world in January. 1991. She continues to go to NIH for regular checkups and has done very well. Update on our Cindy: Healthy, happy, and recently started a new page in her life as a newlywed!

  9. You are such a wonderful family! Hannah is soo lucky to have you! Thank you for sharing your beautiful story and family with us…priceless! We are praying for you 🙂 XOXOXOXOXO

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