this is motherhood {too}: a journey with Harlequin Ichthyosis.

May 13, 2014, This is Motherhood Too, 11 Comments

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By Courtney Westlake.

The air was so cold that I sharply drew in a breath when I opened the car door. There wasn’t a single person outside of the large building, but it was well lit and as inviting as possible. In fact, there was barely another car on the road.

But of course, at 11:00 p.m. on Christmas Eve, most families were at home, children slept fitfully, adults scrambled to create Christmas magic.

My dad ran every red light we encountered. I gingerly slid out of the passenger seat, still experiencing ligament tenderness and sciatic nerve pain from giving birth five days before.

Every step was heavy as the hospital doors automatically opened to a warm lobby where the security guard handed me a visitor pass. 

A life-sized Nativity scene stood in the corner, with an empty manger, waiting expectedly for a baby to be placed comfortably in the straw in just a few hours.

“God,” I begged with an aching heart, “please don’t let my daughter die on your son’s birthday.”

Our daughter Brenna Helen Marie arrived into our lives on December 19, 2011, sending confusion and near panic throughout our hospital room, as doctors and nurses scrambled to figure out what was causing her entire body to be covered with a thick, hard whiteness. Her fingers and toes were contorted in hardened shells, her ears were barely visible and her eyelids were flipped inside out.

Harlequin Ichthyosis.Thanks to the quick actions of the neonatologist on duty and the local pediatric dermatologist, Brenna was cared for immediately and transferred to the nearby NICU, and we soon had a diagnosis: Harlequin Ichthyosis.

Two words that took me days to be able to say, and two words that altered our lives forever. Those two words meant that our daughter’s skin didn’t function properly, and that she may not live more than a few days.

Brenna was kept in an isolated NICU room, in a closed bed with a temperature of 98.6 degrees Fahrenheit, with such high humidity that it looked like a rain forest. The reason for this was because her skin doesn’t regulate her body temperature and doesn’t hold in moisture on its own. Her tiny body, with hard plaques of skin split by deep red fissures, was coated in Aquaphor every 3 hours to keep her skin moist. She was kept on morphine to manage the pain that her skin caused her.

Because it would have been impossible to put an IV in Brenna’s skin, the opening through her umbilical cord was left open with a line to draw blood and run tests. Both this opening and her general skin condition left her very susceptible to infection, and on that Christmas Eve, five days after she was born, my husband Evan was reading her a bedtime story at the hospital when the hospital staff confirmed a very aggressive blood infection. They told him to call me right away.

Harlequin Ichthyosis story of hopeWith my parents present, we held an anguished emergency baptism at 1 a.m. on Christmas Day, and I continued to beg God into the early morning hours to not let my baby die on Christmas. I wondered, sobbing, if I would even get to hold her in my arms before we had to say goodbye. 

We were grief-stricken, exhausted, heart-broken, and fully trusting in God’s plan with all of our beings when numbers began to come back on Brenna’s blood work. A slight improvement. She was fighting. And then later, another test, with more improvement. 

At 6 a.m., we finally relinquished to our exhaustion and to our desire to be home with our two year old son Connor, on Christmas morning. Brenna was stabilized, and through our tears, we were hopeful. 

The lobby was still quiet as I left, but there was sunlight streaming through the glass doors. And there was something new.

The manger was no longer empty; a statue of baby Jesus lay there with his arms gloriously raised up to the heavens. Christmas Day.

Today, two more Christmases have gone by, and each passing year reminds me of God’s presence with our family in that tiny NICU room that night. He taught me to rely on him, regardless of our circumstances.

The years following that night have been full of tests to my faith and trust in God, as we’ve encountered many health challenges for Brenna, from infections to hospitalizations to surgeries. Brenna’s skin disease is a life-long condition, and the daily care to help her be more comfortable, reduce infection risk and stay as healthy as possible can be overwhelming and isolating.

But I’ve found that whenever I need Him most and I turn to Him, I feel that precious presence like I did on Christmas Eve. And when I lose focus on that steady faith, I just have to remember that image of Jesus’s in the manager, with His arms extended up to heaven.

a journey with Harlequin Ichthyosis

Courtney Westlake is a writer and photographer, and author of the blog Blessed by Brenna. She’s a wife and mother to 2 beautiful children, one of whom has a severe skin disease.

This is from the collective writing project: this is motherhood {too}. Do you have a story you’d like to submit?

11 Comments

  • Reply Margie McCann May 22, 2017 at 6:55 AM

    I can not hold back the tears. Thank you for sharing your story. You found your strength in God as I did on 12/24/1994 when our third son was born perfect but with severe meconium aspirant pneumonia. Basically, my labors are hard and fast, my body does within hours what most women take a full day or more to do, deliver a baby start to finish. My second son had a slight case of it but they got what little he had inhaled out quickly, not so with this little guy. He had breathed in so much of it they could not remove it. He was suffocating from it & I went through the night alone (my husband was at home with our two boys as we had no one to care for them & we were new in town and knew no one) & I didn’t know from one moment to the next if he would live. They didn’t tell me anything all night long & every time I went to the nursery all I could see were doctors surrounding my son. At 8 am the next morning they came into the room (about 12 doctors, interns, residents, medical students, for the first time I cursed a teaching hospital) and told me it was up to him now.
    Well, here is the power of prayer. They told us if he lived he would be in hospital for a few months as the meconium slowly worked it’s way out of his system & the longer it took to clear the more likely the chance of brain damage. He was on a CPAP machine and pure oxygen and they said he could be blind from the high concentration of 02. Nursing my son every 2 hours was a nightmare but I was determined even though the nursing staff thought I was crazy, however, I believe to this day it was the constant skin to skin contact which comforted him and gave him the will to fight! He knew my voice from the whole pregnancy and I didn’t see any reason he wouldn’t recognize it now. God’s plan was different from the doctors. He was happy & home in 5 days. The CPAP was gone in 2 days, they were still giving him chest percussions to help him bring up any of the gunk he had been spewing out for days in a way every doctor had never seen before. It didn’t come out a little at t time like they had always seen but he was bringing it up all at once. He was off the 02 within 3 days and was ready to go home on the 4th day but had to stay one more day for his circumcision.
    Do not ever let anyone tell you God is not all powerful. We had one more son after this who thankfully was healthy, however, when he was not quite 3 weeks old I took my brother & his wife to work, on 9/11. They worked at the WTC building in NYC on the 71st floor. When my son and I were in the courtyard waiting for my brother to find my sister in law after the plane had hit the building next to hers another plane hit her building. They made it to the 6th floor when the building collapsed. My son and I both suffered facial scars from falling debris on that day. My son is 15 years old now and says he will never have the scar removed as it is a reminder.
    Our family grew by 3 as our family adopted my brothers 3 daughters ages: 7 weeks, 18 months & 34 months when their parents were killed. They have brought so much to our lives. When the girls joined our family the damage was on the inside. The night terrors, the screaming, crying tantrums, all the result of having the parents who loved and adored you ripped from your life in a heartbeat. It took years and I know some of the scars will always be there. All we could do is give them all our love and support and be there for them when they needed us. Our youngest daughter had been breastfed by her mother & since I was nursing my then 3-week old son I decided to tandem nurse the two which was a challenge I didn’t know would be as draining as it was but I think the closeness helped our youngest daughter to get past any issues & move on. We truly never know what we are capable of until we meet a problem head on.
    I have a large loving family today & thank God every day for all we have been through with his guidance. We mourn those Satan has taken but we know too God will never allow us to be hit with more than we can bear. He will always make a way for us to deal with it 1 Corinthians 10:13.
    Psalm 46: 1-3
    1 God is our refuge and strength, a very present help in trouble.
    2 Therefore will not we fear, though the earth be removed, and though the mountains be carried into the midst of the sea;
    3 Though the waters thereof roar and be troubled, though the mountains shake with the swelling thereof. Selah.
    Psalm 46:10-11
    10 Be still, and know that I am God: I will be exalted among the heathen, I will be exalted in the earth.
    11 The Lord of hosts is with us; the God of Jacob is our refuge. Selah.
    I love Psalm 46. It is a little long so I just shared the most beautiful parts (in my opinion). May God hold you in His arms and give you comfort knowing all the love Brenna brought to your life here on earth, know it is not the end & she waits for you with her heavenly Father.

  • Reply Peggy Rebeck November 23, 2015 at 1:44 PM

    Beautiful children courageous parents you are truly blessed

  • Reply Jesus July 31, 2014 at 6:15 PM

    Sabine, Just wanted to say a big THANK YOU for the super dance siseson at my husband’s birthday party. You were amazing and completely energized the whole crowd. The party took off from there! THANK YOU!!!!

  • Reply Balee July 30, 2014 at 8:37 PM

    Sabine, Just wanted to say a big THANK YOU for the super dance soeissn at my husband’s birthday party. You were amazing and completely energized the whole crowd. The party took off from there! THANK YOU!!!!

  • Reply Jolie May 14, 2014 at 9:01 PM

    I am so happy you shared this story. I looked up this condition after reading about your precious daughter and it sure sounds serious. I’m thankful you are finding the care she needs and the support you need too. It is inspiring to hear of people knowing God’s presence in times of hard ache. Thanks for being faithful.

    Warm love today from Vancouver, Canada
    jolie

    • Reply Courtney May 15, 2014 at 8:35 AM

      Thank you Jolie! Yes, it is a very critical condition, especially in the first few days of life, but we have been beyond blessed that Brenna is here with us and thriving now.

  • Reply sherry Leeds May 14, 2014 at 5:16 PM

    Brenna Is So Beautiful !! God Bless Your Family !!

  • Reply Sarah May 14, 2014 at 11:52 AM

    I just read the guest post you (Courtney) wrote on Mary Evelyn’s blog…I knew her step mother when I worked in Early Intervention and have gained a lot of insight about life from a parent’s perspective through her…I loved your words there and was glad to read more of your story here! What a small interwebs!

    Thank you for the most adorable reminder of real beauty.

  • Reply Kali May 14, 2014 at 6:54 AM

    Beautiful story, Courtney, of faith and hope. Much blessing and peace to your precious daughter and your whole family. Thanks for sharing with us!

  • Reply Courtney May 14, 2014 at 3:45 AM

    Thank you so much for sharing my story Michaela! Much love to your family.

  • Reply Michaela Evanow May 13, 2014 at 8:34 AM

    Courtney, I’m so glad you shared your story with me. It is so full of hope, trust and yet it’s a scary and tough ride. The isolation, the infections….I’m with you mama, even if we’re not in the same country!
    So much love to you.

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