Leah's Story

On July 4th, 2008; our lives changed forever as I showed Jeff a positive home pregnancy test I had taken just hours before.  We had decided we were ready to start a family, and after just 1 month of being off the pill, I 'knew' I was pregnant.  I almost fainted at work one day .... I just felt .... different.  Sure enough, we were pregnant! We had a miscarriage scare, I had bouts of morning sickness, and in mid-January of 09 a pre-term labor scare sent me to the hospital for an extended stay.  My blood pressure was rising, Leah's weight gain was not great, and by Feb 8th, an ultrasound showed that the doppler reading on the umbilical cord indicated that Leah was not getting sufficient blood flow.  In Labor and Delivery the docs tried to induce -- with every contraction Leah's heart rate was dropping.  It was time for plan B: a C-section was scheduled for 9 AM the following morning.  At 9:36 AM I saw my angel Leah for the first time. 

She. was. perfect.

Within hours the doctors were confused as to what was going on.  Her blood sugar was off, she threw up the first bottle they gave her, she had an odd spot of sloughed off skin at the very top of her chest, and 2 strange blistery looking spots: one on a thumb, and one a big toe.  As the days passed more blisters popped up.  No doctor knew what was happening.  The NICU nurses treated her like she had some strange baby disease --- with inpervious blue gowns and gloves.  They warned me to be sure I washed my hands really well after I held her for fear of transmitting whatever she had to others.  It killed me. 

We went home with the diagnosis: Epidermolysis Bullosa.  We saw an EB specialist who assured us Leah's case was a modest case of Simplex.  As the months passed, Leah's skin began to break down more .... and to our horror, her breathing worsened .....

On the evening of July 10th, 2009, we all sat in a PICU room inside DC's Children's National Hospital.  An episode of respiratory distress had landed us in the ER the night before.  Around 6 PM Leah started screaming .... like we had never heard her scream.  She was now in severe respiratory distress.  Within minutes Leah had an emergency tracheostomy performed. 

When she was back in her room recovering .... I stood at her bedside with my head in my hands, crying.  I knew that was the beginning of the end, and nothing would ever be the same again. 

Almost a full month later we all came home: Leah, her trach, and her feeding tube.  The amount of care she required went from 60 to 600 mph.  But, those days were some of the "happiest Leah" days ever - and for that, I would've done anything to keep her happy.

While in the hospital healing from the trach, our new dermatologist performed a biopsy. The results that came back were not what we wanted, but were somehow what we expected: Leah had Junctional EB, most likely the Herlitz form. Junctional EB involves both the external (skin) and internal (airway, GI tract, etc).

A child with JEB-H is not expected to reach their 1st birthday ..... my little Leah almost beat those odds................

 

I wrote this the morning after Leah passed away.  I'm sure now, after months of thinking about that day, I could write so much more about little details I remember ... how it was a cold, rainy, miserable day, how the sound of the monitors beeping was making me insane .... how 2 of nurses from Leah's floor came up to see us in the PICU and I hugged them as if I would never let them go.  But so much of it still has that numb feeling; so much so that I think this post has to stand as-is.....  

On the 31st, after we came home from the hospital; we stared at each other for a few hours and then went to bed.   I woke up about 14 hours later, wandered around my eerily empty house, looked in a crib that Leah would never sleep in again, and then opened my laptop and wrote this:

I know most people have found out by now in various ways but, yesterday morning around 9:45 my beautiful little baby Leah got her much earned butterfly wings.

I'll try to write more later about everything, including how we are holding up (which for right now is as well as could be expected).

We still don't really know what happened. Somewhere around 5:30 AM her heart stopped and she stopped breathing - she was resuscitated and sent to the PICU. From there it all went down hill. She was given blood, epinephrine, steroids ............ nothing was helping. Around 9:30 the nurse couldn't get a blood pressure on her and we knew it was time to stop. The Leah that was laying on that table was not MY Leah. It looked nothing like her - we knew the real Leah was already gone. She was given 2 doses of morphine to ensure she was pain-free.

So please know that she was not on an uncomfortable hospital bed with strangers around her pumping foreign things into her little body during her last minutes. We had them take the ventilator off and disconnect the IVs and she was placed in my arms. My little fighter stayed with me for a few minutes - battling until the very end. I just rocked her and told her: "No more pain, no more blood, no more hospitals, no more EB". All I ever wanted for her was to be pain free and happy - and this is the only way that could've happened. I know she's in heaven - EB free and beautifully perfect. I know all my grandmothers and great-grandmothers are passing her around, loving her. I know she's smiling that Leah smile with those big blue eyes. She will never be alone and will never be in pain again. I know she'll always be with me, watching over me - and I know I will see her again some day and I'll be able to hug her as I never could here.

Golden slumbers fill your eyes

Smiles awake you when you rise

Sleep pretty darling do not cry

And I will sing a lullaby