Annabelle's Story: Living

"Cherish every moment."

We hear this all the time.  Since I had Makayla I have heard this advice from strangers weekly.  It's a really nice thought.  Of course, we hope to live in such a way that doesn't minimize the simple beauty of the day-to-day, particularly with your children.

Can I be real though?  I wanted to spend a lifetime with Annabelle, not celebrate her weeks and months because I knew there would not be many.

Oh we made the most of it - we threw a family party for her 1 month birthday and Ryan's 31st combined.  And we had a house full of friends for her 2 months.  It was wonderful and I am forever grateful that we made special moments and celebrated the time she was with us.  But part of me hated it.  It was one more reminder that we were losing her.

Annabelle 1 month, Ryan 31.  Great-Grandpa Jack (seated) passed away 2 days after Annabelle did.
This was the last time the family was together.

But what can you do? We settled into routines.  We went on family adventures.  We did our best to live our lives and to love what we had rather than focus on what we were losing.  But we grieved.

We did all the things that we would have done had Annabelle been healthy, and probably even more.  Erin came to visit as often as she could.  We took a billion pictures.

Annabelle didn't do well in the car.  She was the sweetest, calmest baby, but the car seat positioned her in such a way that pressure on her lungs and restriction of her airway made it hard for her to breathe.  Sometimes she was just fine, other times she was not.  It was hard.

A nurse came each week to check on Annabelle - her weight was steady, she seemed the same, so there was no more to be said.  When she was about 9 weeks old, we had a suction machine delivered to the house.  SMA causes respiratory issues and often babies have a difficult time clearing their mouths and throats of normal secretions.  Similar to what dental hygienists use, the machine could help us clear her mouth and throat if necessary.  We were comforted to have something there if we needed it...at first.  It didn't take long for the truth to set in.  The machine is small, hardly intimidating.  But it represented the huge, daunting reality that she was so weak, she could even choke on her own saliva.  We tried using it once.  We thanked God that she didn't need it again.

At home, we did all the things that families of 4 do...I would wrap Annabelle up in the Moby* and we would join Makayla in her many activities.  We colored, read, went for walks...and the girls bonded.  Sometimes I wanted to "protect" Makayla from bonding with her sister.  It might have made things easier in some ways.  But it would have been terribly short sighted and entirely unfair to each one of us.  Annabelle received all the love and attention a little sister could possibly ask for (and probably a lot more).  Makayla got to pour out her love on her sweet sister and forever has those memories...and we have the memories of seeing our girls together.  The social workers, the nurses, the people who are supposed to know - they all told us that Makayla wouldn't remember.  She would have a vague imprint of loss.   She would probably act out at some point in confusion over something missing.  But she wouldn't really remember details.  But she does.  There is no way I can describe to you the simultaneous beauty and heartbreak of watching Makayla take her dolls and act out times she spent with her sister.  She asks about her daily.  And she grieves with a maturity no one could have anticipated.  Our advisers were not misinformed.  They have years of experience to tell them how it should be.  But Makayla is different.

 


*The Moby - you may or may not have any experience with this mile-long piece of unimpressive fabric.  With Makayla, I disliked it - it was hot and awkward and required far too much effort.  I used it here and there because it served its purpose, but my overall impression was "eh."  With Annabelle it was a necessity.  Because Annabelle could not support her head at all, anything I carried her in needed to be entirely supportive of her neck - no structured carriers could accomplish this without significant modification.  In addition, her neck needed to be in a position that didn't block her airway, so even modifications were insufficient.  With the Moby, I could wrap her up in the exact position she liked best, cover just the back of her head to keep it secure, and thus she could go about the daily routines with Mom and Sister.  I can't say enough about how important it was.  In fact, after Annabelle passed, I tried to collect wraps to donate to other families with infants in hospice care - our nurse was glad to pass them on directly to families who needed them.  Unfortunately, the mom sale and trade sites on FB didn't seem to believe that I was really going to donate them, and took down my requests.  I got a couple from friends and cleared the wrap section at the local second-hand store.  It felt good to pass along a tool that I knew would benefit other families in similar situations.

For all the talk of wraps, however, Annabelle was really Daddy's girl.  No matter what her disposition, he could scoop her up and she would be perfectly content.  He carried her in what looked to be the most awkward position: draped over his forearm with his hand supporting her neck and covering her chin.  No matter how awkward it looked, she loved it, and only Daddy could really do it.  He was often a hero, careening around the house with both girls - one content, the other giggling wildly.

Don't let his expression take away from his amazing composure and confidence as hero to 2 princesses.

What more can I say about living while your child is dying?  We did our best.  That is all.

What is SMA?

Spinal Muscular Atrophy

We had never heard of SMA before Annabelle was diagnosed.  The research is unpleasant to put it nicely, but I've listed some basic information that will help explain her condition.

SMA is a motor neuron disease which causes weakness and atrophy of the muscles.  Those with SMA are grouped into four types (I, II, III, IV) based on the highest level of motor function they express at the time of diagnosis. 

Outwardly, it affects muscle tone of the neck, arms, and legs.  Breathing and swallowing are also accomplished with muscles which puts SMA patients at huge risk for respiratory problems.

Annabelle's hypotonia (low muscle tone; weakness) was what concerned the doctors.  Very few conditions cause distinct, prolonged hypotonia in infants, so there was a short list of disorders to test for.  The fact that she ate so well confused the doctors - in patients her age, weak suck and swallow reflex is one of the key indicators.

SMA does not affect cognitive functions or touch or pain receptors.  

Annabelle could feel our caresses and cuddles as much as any other baby.  She was a very happy girl which is good indication that she didn't experience excessive pain.

It is a rare disorder occurring in approximately 8/100,000 live births.  It is one of the most common rare diseases and the #1 genetic killer of infants.  It is caused by a missing or mutated SMN1 gene.

Surprisingly, 1/40 people are genetic carriers of the recessive trait that causes SMA.  When 2 carriers of the recessive trait conceive a child, there is a 25% chance of that child expressing SMA, and a 50% chance of that child being a carrier.  Carriers are not physically affected by the trait.

Ryan and I are both carriers of the recessive gene mutation.  We have no family history of SMA and there was no way we could have known that we were carriers until we were genetically tested.  Even amniocentesis done during pregnancy does not routinely screen for SMA (although we didn't have this testing done) so nothing could have warned us about what was to come.   Now, however, we are aware and this knowledge decisively impacts our decisions regarding future children (you can read about Our Options soon when I reestablish that link).

Let me take this moment to say that, despite the pain and sorrow we have experienced, I am so grateful we walked into parenthood ignorant of our genetic propensity to pass on SMA.  Had we been tested prior to having children (some advocate this), we may never have taken the risk which blessed us with 2 beautiful daughters.  Yes, knowing has changed us, but I would not advocate diving into your genetic makeup unless you have reason to believe there could be a significant risk.

Find More Information:
Families of SMA
Fight SMA

Annabelle's Story: Home at Last

The relief of being released from the hospital was quickly diminished by the terror of sharing horrible news with our families.  My parents were home with Makayla, excited to hear that Annabelle was coming home.  They had made a Welcome Home sign for the door.

It is impossible to detail the heartbreak we brought with us.  "She has a terrible disease" we repeated to those who needed to know.

The onset of grief is much quieter and far less cathartic than how it is portrayed in the movies.  There was no yelling. There was no throwing or crashing or outbursts of any kind - these all provide release; we experienced none.  There were tears and there were questions; the kind without answers.  Oh God, have mercy on us.

But Annabelle was there with us.  She was alive and beautiful, and so we lived.  We had a family party for her 1-week birthday.  We didn't talk about her like someone already gone.  We took pictures and everyone smiled.  And the smiles were genuine.

Gay Walker from Trinity Kids Care - who had heard my desperate rejection of hospice care the day before - came to see that we were settled in.  She would take our case and provide the support we needed in order to continue living even in the shadow of death.

What we never got was the answers to the questions that plagued us - when? how? You can pretend it doesn't matter.  You can say that you're just going to enjoy every moment and live in the now.  But you can't; you always want to know.  I'm sure knowing would not have made it any easier, and since there was nothing anyone could tell us, we just did the best we could.

When Makayla came home from the hospital, she slept next to my bed for a month before "graduating" into her own room.  Annabelle spent the first night home in her own bed, in her own room - not because I didn't want to be beside her every moment (I so desperately did, and spent many nights in her room), but because we wanted her to get to use her room for as long as she could.

Annabelle’s condition caused muscle weakness, so she didn't move very much, but then most newborns don’t. In so many ways, she was a typical baby, and if you didn't know, you wouldn't guess that she was dying.

We went to the beach, Disneyland, and Oak Glen.

Makayla was over the moon to have a sister.  She held her hands, taught her signs, traced her feet with crayons, sang to her when she cried, sat with us while she nursed, and helped me change her diapers.  The girls bonded instantly, and I feared the day I would have to explain why they couldn't play anymore.

Between visits from hospice nurses, we played at the park, did crafts, and spent time with family. From the outside, things looked wonderful. Our hearts were breaking, but Annabelle deserved a full life.

When we dedicated her at church, we fretted over what to say - how could we share such news with our church family without casting a shadow over the sweet life she was here to live?  So after much thought and prayer, we kept it very short and very simple.  And we shared her life verse from 1 Peter 2:

Therefore, rid yourselves of all malice and all deceit, hypocrisy, envy, and slander of every kind. 2 Like newborn babies, crave pure spiritual milk, so that by it you may grow up in your salvation, 3 now that you have tasted that the Lord is good.

The Lord is good.  He MUST be good.  I do not say this lightly, or thoughtlessly.  I do not say this because I've always gone to church so I guess I better believe in God.  We have seen what it means to fear the Lord.  He is mighty and powerful and capable of anything.  We have looked into the face of death and know that Christ overcame it.  God did not give us the miracle we hoped for.  I don't know if I'll ever know why.  There are all sorts of canned answers, but none of them are helpful.  He chose not to.  That's the only answer that makes sense.  That doesn't mean He wasn't merciful.

Annabelle's Service

We knew Annabelle's story needed to be heard, so we invited everyone to hear it.  We wanted to honor Annabelle and we wanted to share the Gospel and I truly hope we accomplished both at her service.  

Ryan's created a beautiful slide show tribute that allowed others into the 2 months and 8 days we had with Annabelle.

Sitting down to consider what we would say was enormously difficult.  Many nights were spent crying in front of the computer attempting to put into words the life and love we shared with our beautiful daughter.  In reality, it was an impossible task so I decided to use what I had written to her already even though I had only intended those notes for her.

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Thank you all for gathering to honor Annabelle’s life.  How can we convey the duality of our lives - the truest happiness of having two beautiful daughters; the deepest sorrow of losing one?  April began writing to her early in the pregnancy, and excerpts from those notes help to reveal how Annabelle’s story is our story.

12/27 We got to see you in your first ultrasound today!  Daddy and I are so excited about you joining our family.  I’ve been feeling very sick, but since I was sick with your sister, it’s almost reassuring that things are going well…when I think about all the joys of having you, the nausea fades.  I am so blessed to be your mommy.

1/1 I crave apples.

2/9 Dear baby girl, we got to see your sweet face in the ultrasound.  You were dancing around the whole time.

We had never heard of SMA.  Throughout the pregnancy, we prayed for the health and safety of our new baby with no doubt that everything would work out.  We had done it once, and we knew what to expect.

4/1 Daddy felt you kick today!

5/11 I’m sitting on the couch watching you do somersaults in my belly.  You are very active and I love it…You are going to steal everyone’s hearts – mostly your sister’s!  She loves you already and can’t wait to have a new playmate in the house.  I pray that you will become very close.

6/10 On Sunday a woman prayed over us at church and I wanted to share her words with you.  She prayed that I would have no fear; that I would trust the Lord with this pregnancy, and that you would be safe and healthy.  You are loved and you are safe because I trust God.  He is giving you to us for a great purpose. As much as Daddy and I love you, He loves you more.

Spinal Muscular Atrophy (SMA) is a genetic disorder that prevents muscle nerves from regenerating.  Annabelle lacked a simple protein that made the difference between life and death.    Routine genetic screening does not check for SMA.  Few people know about it because those with the SMA do not live long enough to be noticed.   Annabelle had Type 1, the most severe form, which can be present before birth.

7/20 I had gotten used to you moving a lot – big movements that would make my tummy dance.  Now, I only feel hiccups and smaller movements like you’re running out of space.  It certainly is different than what I remember with your sister and I am anxious to meet you and learn your personality. 

8/14 I thank God for being able to carry you in my tummy all these months, and I am eager to see your beautiful face and kiss your sweet cheeks…for now I will try to enjoy each little hiccup I feel.

On August 16^th at 4pm, Annabelle Faith was born over 8 lbs, and beautiful.  The doctors noted that she wasn’t moving much, but it was just considered trauma during birth.  Annabelle ate well and slept well, and we were expecting to go home a day early – we knew what we were doing this time.  But the doctors delayed our plans – Annabelle still wasn’t moving enough to be released. 

Days passed and she was transferred to CHOC for further examination. I thought it was extreme, but “better safe than sorry” I told myself.  Frustration grew as we strained to hear any hints from the doctors.  We were not getting answers.  No one wanted to reveal their thoughts until the genetic test results, and then everything changed.  In a crowded conference room with plastic chairs, the doctors told us methodically that our little girl was not getting better; she was going to die.  We were discharged with all expediency to come home to a family expecting good news. 

And so began our double life: living, working, making beautiful family memories; crying out for mercy, wandering in the dark, fearing what was to come.

8/22 Beautiful girl, you are so sweet and easy-going, even when Makayla steals your pacifier and claps your hands together…she is also teaching you to sign and wave and she loves changing your diaper…Auntie Erin came out just to see you and is extending her stay so we can celebrate your one-week birthday…I don’t know how much time we have with you, but I know that forever you will be well-loved, and forever you will be my daughter, my beautiful Annabelle.  I pray for strength to be a good mom, for peace and clarity, and for a miracle.

Annabelle was so perfect and precious.  It was easy to forget the medical truth.  There were daily reminders – lack of movement, occasional struggles to catch her breath – but in between, she was just our perfect daughter.  What else could we do?

9/10 My sweet girl you are so loved.  I discovered that when your sister takes your paci, it’s so she can give you a big kiss, then she gives it back.  Grandma and Grandpa argue over who will get to hold you next, even after you pooped on Grandpa!  Yaya and Papa love you and request pictures every day.  We are already planning your 1 month celebration and the whole family will be there…We have chosen your life verse – a verse we hope will share a message with our friends, and family, and even strangers; one that will point to the beauty and purity of your life and encourage us to strive for the same purity.

We got Disneyland passes, visited the beach, went to the park, and tried our best to live normal lives.  We kept the news close, mostly so we could continue to live in the fantasy that Annabelle would always be with us.

We grew accustomed to visits from the nurses, but there were so many questions that never seemed to have answers.  When? How? We wanted to know, we wanted to keep building those expectations. 

9/11 I have taken to calling you “sister.”  It’s something I never thought I’d do, but I love how Makayla says “my sister” as she runs to your crib in the morning.  Today we had our weekly visit from your nurse, Miss Gay.  She is so tender with you and Makayla, and I am very thankful that she is here to help us watch over you.  You have opened doors to new relationships that may never have been possible without you.  I think it’s all part of the story you have to share with the world, and I don’t think I’ll ever know just how many people you will impact or how deeply you will affect them. 

9/13 You smiled at me!

9/14 Daddy loves you so much.  He is the only one who can calm you down without fail – he flips you onto your tummy on his arm and it calms you immediately…I am not brave.  I am not strong.  I am just a mom trying to love her babies and fighting the very present knowledge that I cannot protect them.  I am lost and I am weak.  But I have Jesus, I have Ryan, I have Makayla, and right now I have Annabelle.

Friends rallied around us in a way we never expected.  Most of the time we just felt un-deserving, like it was happening to someone else or we were just crazy and it wasn’t really happening at all.

9/28 Many days, I grieve the loss of the things we’ll never get to do, things I’ll never get to say.  I try so hard to appreciate the time we have…many have told me to enjoy every moment.  And it’s true – of course that is what we want.  But no one knows what that really means.  To go about our lives, doing fun things and making sweet memories, all while reality nags.  Still, we do our best…some days are very hard, my sweet girl.  I have to be honest about that.  I worry about you and desperately want to rescue you.  I am trying, and often failing, to trust God every day. 

We walked through the orchards of Oak Glen.  We even bought the girls matching Christmas dresses.

10/16 We celebrated your 2 month birthday with a house full of friends.  It was wonderful, but there have been many emotions this week.  The reality is I don’t want to celebrate each moment.  I want an entire lifetime with you.  You are my beautiful daughter, always.

10/23 You smell like apples today.  I love every bit of you.

Although Annabelle struggled from time to time, we were adapting. October 23^rd was the first time we realized that we were completely out of control.  After a strange breathing incident Annabelle momentarily passed out then came back seemingly unaffected.  We were hesitant to leave Annabelle alone that night, even though she was sleeping sweetly.  The next day, I lingered before going to work, reading to the girls and holding Annabelle.  After I left, the girls played in the kitchen.  Makayla traced Annabelle’s feet and drew pictures of the family.  The call came at 10 – come home.  At 10:36 Annabelle gave her last heartbeat cradled in my lap.  It was if she knew her time had come.  She passed without pain or drugs and with the love of her parents surrounding her. 

10/24 Oh my beautiful Annabelle, today we had to say goodbye to you…I held you and sang to you, and you waited just long enough for Daddy to get home.  The whole time you stared peacefully at us, as though you knew everything would be ok.  You had a full tummy and a clean diaper.  You were showered with kisses and held tightly…please forgive me for any moment I took for granted…you are perfect and strong in Jesus’ arms. 

11/1 You lived your whole life.  God knew your days before you were born, and your life was exactly as long as it was supposed to be.  Even still, as your mommy, I long for more time with you.  Thank you for being my daughter.  

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