The Beginnings

The Beginnings

  • 2002: Ian, Chubby Ian arrived to a welcoming line of extra loving family in the delivery room. Randy, Lammy, Lacy, Yoko, Kathleen, Auntie Yvonne…2 Uncle Pauls in the waiting room and I think Victor got an eye full on accident!IMG_7011
  • 2003: Micah came so fast his face got bruised up!  It just made him look really tan and he was handsome from day 1!imga0126
  • 2004: At 18 months, Micah was being followed for failure to thrive. We went through a series of different doctors and finally ended up with Doctor Jose Abdenur, the Chief of Pediatric Metabolic Disorders at CHOC. Dr. Abdenur worked hard with many doctors and scientists to find the cause, and genetic diagnosis.  Here are the tests and findings:
    • Blood tests for a year: elevated CPK (these are like muscle tears)
    • Muscle Biopsy in October: showed abnormal muscle tissue, diagnosed with congenital muscular dystrophy. But what type? What is the prognosis? At that time there were 40-50 known causes all with varying prognosis. Ian diagnosed through simple blood draw with abnormally high CPK levels. Both boys now diagnosed with CMD.
    • University of Iowa publishes some new findings just 3 months prior that was pivotal in our diagnostics. Dr. Abdenur finds their research and sends biopsy to University of Iowa for staining. Discover the histology of their muscle weakness, alpha-dystroglycanopathy.  Showed that the muscle tissue was damaged at the cell membrane level because a sugar molecule doesn’t stick to the cell membrane. (I may not have explained that just right, but it was a timely miracle of discoveries so close together with perfect timing. Some people wait years before they know their genotype and cellular degeneration. Dr. Moore said it was serendipitous. That’s when I knew it was Supernatural.)
    • Whole Blood analysis at Cal State Long Beach: Heterozygous for Fukuyama Muscular Dystrophy (two different mutations on the same location, one from Randy, and one from me. Genes code for chains of amino acids, which make proteins, which build cells…tissue…organs…systems… us! Amazing and Complex!)
  • 2005: Both boys diagnosed. Though Micah was not outwardly symptomatic with muscle weakness at this age, Ian was. As a baby he struggled to hold his head up when we played air plane, couldn’t do donkey kicks when on all fours, passed up on the stairs by his toddling friends. When Micah was diagnosed with a muscle disease, I knew Ian had it too. Ian only needed a blood test that showed elevated CPK. (Creatinine Phosphokinase. Not California Pizza Kitchen.)
    • They had their own genotype for FCMD, one never before seen, therefore with unknown prognosis.
    • The founder Fukuyama Muscular Dystrophy is primarily seen in Japan with devastating results on the child. They do not not make the Fukutin protein therefore do NOT walk, or talk, have impaired vision and cognition, with a very compromised life span. Our boys do not have the founder gene.
  • Enrolled in a natural history study at University of Iowa. Participation with Iowa does not benefit us medically speaking with a cure, but it helps them lay the ground work to find one! We are grateful to be in touch with The Wellstone Muscular Dystrophy Cooperative Research Center: Dr. Dr. Kevin Campbell, Director of Iowa Muscular Dystrophy Center. Katherine Mathews, top in the nation for her research on alpha-dystroglycanopathy patients. Dr. Steven Moore, head of pathology who did our cell staining to show the detached muscle cell walls.
  • We try and fly out each year for to provide data for their research and hear about their new discovers. They are vital in kicking MD into oblivion.

2003-2007: The Breaking Years.

  • Diagnostics sucked the wind from our sails, pulled the rug out from under us. Every test was like a gut punch. It was hard to breathe and “hope” for the best when we kept hearing the worst. There was no cure. It was degenerative.  We couldn’t stop it. We had doctor appointments all the time. Micah and Ian were hospitalized for pneumonia. Everything changed, but life continued to spin around us. We got pregnant in 2007 and miscarried in the first trimester.  Ian was 5 then. He said that was the last time he cried.  We stopped feeling invincible, or favored, or spared of bad news.  We didn’t feel like “everyone else.” We couldn’t dream like “everyone else.” We wondered when the boys might grow so weak they couldn’t walk.  We didn’t question or doubt God. But all our dreams of what parenting and family might look like were broken now. We ran to Him with our hurt. We ran deep into His word and His heart for us. It took us a few years to find our new normal and breath again.
  •  2007-2015: The Golden Years!

 

And He has said to me, “My grace is sufficient for you, for power is perfected in weakness.” Most gladly, therefore, I will rather boast [a]about my weaknesses, so that the power of Christ may dwell in me. Therefore I am well content with weaknesses, with[b]insults, with distresses, with persecutions, with difficulties, for Christ’s sake; for when I am weak, then I am strong.” 2 Corinthians 2:9-10

Ian says “We’re all dying. Some read the bible.” It’s amazing how the Bible has had words for all of our joys and sorrows, fears, questions, and hurts. The truth we find in these ancient words are trustworthy, comforting, hopeful, and golden. My boys and I can hope for God’s strength in the times of our greatest weaknesses. And though it may not feel good we can ask for contentment no matter what our circumstances may be.

“So we do not lose heart. Though our outer self is wasting away, our inner self is being renewed day by day. For this light momentary affliction is preparing for us an eternal weight of glory beyond all comparison.” 2 Corinthians 4:16-17.

 We don’t get to hope in Glorious Heaven because we are good, or because we are so pitied from our affliction.  Our afflictions do make us hunger for healing, health, and heaven. But, we get to hope in Heaven because Jesus made a way for the broken, imperfect, and sinful people like you and me. He died for our sickness, pain, hurt, injustice, and sins to make a clear path for us to be in His weighty Glorious Home.  We are being transformed to put our HOPE in Christ and our eternal home in heaven.

“Count it all joy, my brothers, when you meet trials of various kinds,  for you know that the testing of your faith produces steadfastness. And let steadfastness have its full effect, that you may be perfect and complete, lacking in nothing.” James 1:2-4

Words like these help me not be surprised for the hardships all around me. They are not attacks on us. But God has growth for us, not because I can pull up my boot straps, but because He’s going to make us steadfast, complete, and not lacking in anything. I couldn’t do any of this on my own

“You make known to me the path of life;    in your presence there is fullness of joy;    at your right hand are pleasures forevermore.” Psalm 16:11

I remember HATING having this degenerative diagnosis without a cure. But what helped me get up the next morning was knowing I didn’t want to be without Jesus. If this was the path He was going to walk with me, then I had to stick with Him, right here. And he promised me Joy in His presence. Why would I choose another path? What were my other options? Denial, Avoidance, Bargaining, Manipulation, Divorce, Drugs, Anger… Acceptance with Joy sounds way better. We do not want to mourn our losses so much that we miss out on the JOY of the moment. The boys were still with us. They were gifted, talented, and witty. They sinned, wrestled and fought like regular brothers. They earned their black belts, became amazing musicians, and had lots of faithful friends.  We are closer as a family, a little wiser, less tempted by the shiny worldly promises, more patient with each other, and more able to enjoy this fragile life. IMG_1255

  • 2009: God blessed us with another baby sister, Kaiya Rayne. She is an abundant blessing from above and blesses us all with Joy every day.  We were not afraid of having three kids with muscular dystrophy. We had enjoyed our new life and fallen in love with our boys and what God was doing. Raynie has brought more sunshine and blessing than we could imagine!IMG_7076
    • Boys published in the Journal of Neuromuscular Disorders. May Issue.

      “Further evidence of Fukutin mutations as a cause of childhood onset limb-girdle muscular dystrophy without mental retardation” Definitely not mentally retarded.  They are both so smart.

  • 2014: Ian showed abnormal EKG and Echocardiograms. Followed every six months. Began oral medications, and no more rollercoaster rides.
  • 2015: August: Ian began Milrinone, a 4-5 day IV treatment to increase the force of each contraction and open the rest of the vessels to let the blood flow against less resistance.

2016: Ian’s Heart Beat Adventure

  • March: Ian’s second Milrinone treatment, a little earlier than expected.
  • June: Third Milirnone Treatment, a little earlier than expected.
  • Last week of June, enjoyed his second year of Mt. Hermon Inter High Camp in Northern California. Difficulty breathing on first night. Told to drink water and elevate his head. He was uncomfortable, weak, and short of breath all week but just wanted to make it through the week with all his friends. He loved the late nights of worship and cabin times, feeling that it might be his last camp. He told a camp friend that he might not be able to come next year and his friend cried for him. Deep.
  • July 3rd: Back together as a family in Mill Valley visiting my cousins. Short of Breath and Active Crackling in his chest. Rushed to local ER that night, Diagnosed with Heart Failure.
  • July 4:5 am transported by ambulance to UCSF Benioff Children’s Hospital for heart failure with moderate to severe mitral valve regurgitation. (Leaking Left Ventricle)… (Felt like my world was Crashing and my son was fading away…) Stabilized and began heart transplant discussion. Even though we were so far from home we had Awesome care for the whole person and the whole family! The Body of Christ and Bennioff Hospital were outstanding supports.  Food came every day. Friends for everyone. Meaningful activities. Blessed by worship and amazing support from the body of Christ! WOW! Discharged and and sent home so we could meet with our own doctors. Though weak and nauseous, he had a little over one week to enjoy family life at home.
  • July 10-18: home but nauseous with shortness of breath and vomiting. 2 ER visits and doctor visits. Adjusted oral meds but it didn’t help.
  • Tayvin & Trevor
    Tayvin has been in the hospital 10 days longer than us. He will be flown to Children’s Hospital of Philadelphia soon!

    July 19: Admitted to CHOC Cardiac Intensive Care Unit. Sustained on Milrinone but could not wean him of medication. Met Tayvin who had his third heart surgery. 103 visitors in 12 days!

    • July 29: Started a blog: iansheartbeat.wordpress.org
    • August 1: Ian Airlifted by helicopter to UCLA Mattel Children’s Hospital PICU for Heart Transplant Evaluation and Education. Stabilized Ian and ran tests from multiple specialties: Cardiology, Neuromuscular, Pulmonary, Psychiatry, Social Work, with tons of tests and blood work!
    • August 6: Moved to less critical floor. Began weaning Milrinone to .5.  Became symptomatic again. Short of Breath, Nauseous, Cold, Emesis. Adjusting all other medications to help him walk, breathe and eat without nausea and chills. We take these things for granted and don’t realized how much our hearts work to make this possible!
    • August 9th, approved by UCLA Team!
    • August 11th, Insurance approval! Waiting!!
  • To Be Continued!!

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