The Krommenhoek Family & The Puj Tub

I am so excited to introduce you to the Krommenhoek Family of the blog, Trach Ties. Take a visit to their blog for inspiration and about their daughter, Maaike, interesting stories about world travel, and even fun sewing tips! Here is a video that shows this delightful family and the Puj Tub, which helps them bathe their daughter despite her having a trach.

Every Child Deserves Unconditional Acceptance

Children's Craniofacial Association has declared September as Craniofacial Acceptance Month.

HE'S MY SON

Yes, I am aware that September is over. But I can honestly say acceptance is something I fight for every day of the year, on behalf of my son. Born at 36 weeks, Austin was diagnosed with Branchiootorenal Syndrome, a rare genetic disorder that affects 1 in 40,000 people. BOR itself is not a craniofacial disorder it is an autosomal dominant genetic disorder that includes malformations of the ear and cysts in the neck, hearing loss, and malformations of the kidney.





As a result of BOR Syndrome, Austin's list of physical challenges also double as medical dictionary tongue twisters: Gastro-esophageal reflux disease, Microtia, Aural Atresia, Hemifacial Microsomia, Macrocephaly, Hypotonia and Tracheotomy, to name a few. These days I rattle them off with ease, I can explain what each one means to ME and how it affects Austin. BOR contributed to Austin's ears not developing, his jaw being so small that his tongue obstructed his airway, and some facial nerve paralysis.

Although Austin’s life would not be possible without a lot of assistive medical technology; he does have a life, a very full life indeed. He is like any typical 23 month old, he enjoys hanging out with his cousins at Disneyland, watches Blue's Clues, plays with blocks & puzzles and reads his favorite books.

While going for doctor visits or having therapists and nurses in his home are common for him, he remains friendly and (mostly) happy to see them. To explain how he lives his life with a Trach, G-tube or Chronic Lung Disease would require a much lengthier story than we have time and space for here, but in all of his struggles Austin has continued surprise us with his utter resilience. You can read the details on his blog.

HE HAS WHAT?

Microtia is an incompletely formed ear. It may be just a small ear, or other variations including having only a bump of tissue at the location where the ear should normally be found. Microtia may occur as an isolated deformity although it typically presents as part of a spectrum of other defects, either minor or major. It occurs more commonly in males and on the right side (unilateral). Approximately 10% may occur on both sides (bilateral) which is Austin’s case.

Aural Atresia is the closing or absence of an ear canal in the middle ear. Microtia and Atresia can occur alone or together. They can also be associated with Hemifacial Microsomia.

Children born with bilateral Microtia/Atresia often require bone-conduction hearing aids within the first few months of life. Austin got his hearing aid at 4.5 months. Although the surgeries to correct this problem used to be started as early as 4 years of age in the past, most surgeons prefer to start the external ear surgeries at about 6 to 7 years of age.

Hemifacial Microsomia is a condition in which the lower half of one side of the face does not grow normally. The most obvious sign of this condition is a partially formed ear or total absence of an ear and partial facial paralysis. The syndrome varies in severity, but always includes the underdevelopment of the ear and the mandible. This is the second most common facial birth defect after clefts.

The jaw abnormality (micrognathia) in children with hemifacial microsomia may range from a small but normally shaped parts of the jaw bone resulting in a mild asymmetry to complete absence of these structures resulting in a more severe jaw deformity. Because of the jaw abnormality these infants may be at risk for breathing and feeding problems and need to be evaluated by a specialist if there are any indications of airway compromise or failure to gain weight. Occasionally a tracheotomy and/or gastrostomy are needed to help with breathing and feeding.

Branchiootorenal (BOR Syndrome): Just like any other syndrome there is not one clear cut case of BOR, it is actually now classified as a spectrum disorder.

The B in BOR refers to the branchial arches, the area of the embryo that develops into the outer and middle ear, the neck and the lower part of the baby's face. There are several types of malformations of the branchial arches in BOR; Austin has auricular pits, which are very small holes about the size of the hole in a pierced ear, just below his ears. Normally these might be found on the neck area.

Oto refers to the ear and in particular the hearing loss that is part of the syndrome. The hearing loss can be sensorineural, conductive or mixed. It can be stable or progressive and the severity can range from mild to profound. Austin has bilateral, severe conductive hearing impairment (70-90dB).

Renal refers to the kidneys which can be abnormal in size, shape and/or structure. They may be smaller than usual or have a malformation that does not interfere with function or cause any symptoms. Austin thankfully does not have any kidney problems at present.

BEYOND THE FACE IS A HEART

Children with craniofacial differences come in many packages, but the one thing all have in common is that there is a living, breathing, feeling person behind those big named syndromes and disorders. These kids are funny, serious, loving, quirky, accepting and enduring. Each should be acknowledged for their own individual talents, as well as, their challenges.

Keep in mind beyond the face is a heart; the person you are staring, gawking or pointing at is someone's much loved child, it may be my son. Consider instead, a smile and a wave.





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There are many wonderful organizations you can consider supporting. They offer affected families medical treatment or support and resources.

Children's Craniofacial Association (CCAKids)
AmeriFace
Little Baby Face Foundation
FACES: The National Craniofacial Association
Foundation for Faces of Children

A Wink From Heaven

A compilation of thoughts written by April Moody from the blog Wink From Heaven, which is about her son Caleb...

"If, therefore, thine eye be single, thy whole body shall be full of light." ~Matthew 6:22

I remember the night I found out that our baby Caleb had significant problems. We were faced with the unbearable possibility that our baby might not be born alive. During our first glimpses of him we were filled with worry and deeper heartache than I had ever felt before. The next day I remember telling my mom, "this isn't how I imagined my family picture." I didn't imagine hanging a family picture on my wall that had someone missing. I also hadn't pictured a child whose time may be limited and whose challenges would be severe. It wasn't that I didn't want a disabled child. I wanted Caleb more than anything in the world. I had the opportunity to voice how much we wanted Caleb in many of those early doctor appointments when they presented our options. I pleaded with Heavenly Father for the opportunity to take care of our baby no matter how big his challenges were. We wanted Caleb with all of our hearts, his circumstances just weren't what I had pictured.

I remember that phrase, 'This isn't how I imagined my family picture' every time I put a new photograph in a frame, add a picture to a scrap book, or hang a portrait on my wall. My heart is filled with so much gratitude every time I look at my sweet little family inside those frames. It wasn't what I had pictured, and it is so much sweeter than I could ever have imagined. I would never have pictured how proud I would feel of Caleb for his courage, faith and patience. I never pictured how full my heart would feel as I watch my other little boys lovingly watch over him. I never pictured how my sweet little boy, whose time may be limited and whose challenges are severe, would touch the deepest parts of our hearts and change our lives forever. Just yesterday Matthew told me, 'I just get such a big loving feeling inside whenever I'm around Caleb- and I just have to hug him!'

I took our little family into the studio for some last minute pictures before Caleb went in for surgery [last November]. The pictures aren't fancy, but nothing could be more beautiful to me. I would never have pictured the exquisite delight I would feel in hanging photographs of theses precious souls on my wall...

When I was pregnant with Caleb, we were so excited to find out what we were having for our third child, so our kind friend offered to do an early ultra sound so that we could meet our new little family member! As we were driving to the clinic, I started to feel extremely uneasy. I asked my friend if he would tell us if anything was wrong. He reassured us that he would and that he would probably say something like, "we'll need to talk to a doctor about this."

As soon as the ultra sound began, our first glimpse was of our baby's' head. I immediately asked "is it supposed to look like that?" Instead of seeing any brain tissue, it was completely black. Our friend said, "We'll need to talk to a doctor about this..."

I started to cry and tried to focus on other information he gave us-- we were having a BOY!!! We were counciled to set up appointments with doctors and specialists so that we could learn more about our special little guy. Our hearts were heavy that night as we worried about our new little boy and the challenges that were likely ahead.

Within a few weeks we decided we wanted to name our little boy so that we could pray for him by name. We named him Caleb Joseph! Caleb was a righteous Israelite in the Old Testament who was always faithful and was known for his fearlessness in the face of overwhelming odds.

October 2004:

Dear Caleb, you are not going to be born for another 3 months, but you are such a special part of our family already. You have such a strong and compassionate spirit. I have felt so close to you during this time as we have been uncertain about what your birth will bring. About a month and a half ago, we discovered that you have a great amount of fluid in your brain. Our hearts have been so tender as we contemplate the possibility of only having you for a short time. Our hearts ache knowing that you may have significant problems throughout your life.

In the midst of the uncertainty, there are some things I know for sure. I know that you are my son for eternity. I know that you have an important role to play in our family and that we are privileged to be a part of it. I know that you love us and that you are aware of the deep love we have for you. I know that the Lord hears our prayers and sees our tears....Although I don't fully understand why you have physical hardships, I know that you have a valiant and loving spirit. I really feel you at times comforting me and that you want me to know everything will be okay....

This past week we had an MRI which confirmed the missing corpus collosum, a blockage causing excess fluid, and that you are missing your right eye. Getting that information broke my heart because I don't want you to have one more physical obstacle....I know that your spirit is not limited and that you have a special mission...I have seen hearts change just when people find out about you...

January 6, 2005:

Caleb was born at around 10:30 AM. His little cry was music to my ears! I got a quick glance before he was passed through a little window into the NICU. I got another glimpse of him as I was wheeled into my recovery room, and he was being prepared for transport to Primary Children's hospital. He was beautiful! In spite of the challenges I knew were ahead, I was just tickled so see my new little boy. His spirit is so big, and he just radiates with love.

Dear Caleb, you are here my precious boy! I am so full of love for you, my heart can't contain it all. I thought my heart would burst when I heard you cry after you were born. When they held you up for me to see, it was like looking at an angel! I knew today would be a life changing day for all of us - and it was. My life has been forever changed for the better because you are in it. I only got to see you for a few moments, but those moments will be treasured for my whole life.

January 7, 2005:

Dear Caleb, Today we spoke with your doctor and he explained many things to us. Your brain has not formed. It appears there is little they can do but stabilize you and prepare you to come home with us.... they don't expect you to live for more than a few weeks....at this moment I don't think my heart can bear the thought of letting you go. How can I look into that precious face knowing that my time is limited? I just want to stop this moment in time and hang on to it forever.... I wish I could choose the outcome, but I know your eternal outcome is already known. I pray that your spirit knows how much you are cherished. Each day I have with you is a treasured blessing....

This morning I got to hold you for the first time! It was like being in Heaven! I couldn't hold back the tears as I held your sweet body in my arms. I just rocked you back and forth and tried to memorize every bit of you. Your sweet spirit just seems to heal my soul. It saddens me so much to know that I may spend much of my life missing you. Do you know how dear you are to me?...Do you understand that the hardest thing I will ever do is let you go?

Today was your blessing day. We were able to gather in a hospice room with Dad and I, your grandparents, one of my aunts and our bishop.

Your blessing outfit was huge on you but you still looked like an angel....there was such a special spirit in the room and your Daddy gave you a beautiful blessing....Each of us had a chance to hold you. It was such a special afternoon. That night you weren't breathing very well and we thought you were going to be put on the ventilator again. We were told to be prepared to decide whether or not we would want to do that or just let you go. My heart was in agony as I contemplated letting you go before I was ready. Fortunately, the doctors were able to reposition you and get you breathing again.

...as I was alone in my hospital room that night I was overcome with sadness. I just can't bear the thought of losing you...I want you to know how much I treasure you and although I don't understand why things need to be this way, I know that the Lord knows our needs and will comfort my breaking heart...

January 13, 2005:

One of my favorite days was Tuesday. Our doctor let Dallan, the boys and I take Caleb into a hospice room, and Josh and Matthew were able to see Caleb for the first time!

Normally no children under age 18 are allowed, but our sweet doctor has been so kind to consider the needs of our little family during this time. Josh and Matthew were so excited and I was so touched as I watched them hold and interact with their little brother. Josh was so tender with Caleb and just couldn't stop hugging him and rubbing his face against Caleb's face. His little spirit was so full of Joy to be with his baby brother. He didn't want to put him down. Matthew was so excited not to be "the littlest" anymore and just loved holding Caleb! He couldn't wipe the smile off of his face, and he loved playing with Caleb's little fingers. The boys were able to hold and snuggle their baby for two whole hours. As parents, our hearts were so touched to have such a special time together, because even when he's home, Caleb will have to spend most of his time under an oxygen mask.

The past few days have been really full as we've been learning how to use all of the hospice equipment so that we can take care of Caleb at our home.... We are so excited to get to be near Caleb everyday! We know that even though we are overwhelmed with how we will be able to keep up with 24 hour care, our family is so blessed to have this little miracle in our lives.

Caleb was in the NICU at primary children's hospital for 8 days. He was so sweet and patient with all of us. He only needed a ventilator for a few days, then was able to breathe with supplemental oxygen. It became apparent to all of us that there was nothing more that could be done for him in the hospital, so we prepared to take him home.

It was overwhelming at first to bring Caleb home. We were surrounded by a host of hospice helpers, learned how to use a kangaroo pump, run the oxygen equipment, and tried to keep Caleb as comfortable as we could. We were counciled to pick out a burial spot, and we did. We worried if each day would be our last.... We had a birthday party for Caleb every week to celebrate his time with us, and we realized we wanted our time with Caleb to be JOYFUL. We decided not to let the medical community take our hope away. We decided that we could celebrate each day and have hope for the future instead of grieving.

January 18, 2005:

Josh loves to hold Caleb's hands and we often find him just quietly watching over him. Matty likes to sing Caleb the ABC's over and over again. We're trying not to look too far into the future and are just enjoying each day with our little miracle - even diaper changes are just sweet and fun!

Caleb has brought an overpowering spirit of love into our home. We feel so honored to be with him. Every time we change his diaper, rock him in the rocking chair, kiss his soft little cheeks, or even just stare at him through tear filled eyes -- we are reminded how much Heavenly Father must love us to bless us with this time with our little miracle. Our hearts are wrapped so tightly around our little baby. We are just so in love with him!

He responds so much to love....

and as we hug him, sing to him, or stroke his little face - his oxygen levels and heart rate always seem to improve.

Caleb has taught us what perfect love feels like. I have watched the way people linger at his crib side and didn't want to leave his presence...

April 2008:

If I knew I had to say goodbye to any of my sweet boys, what words would I want them to hear from their mom? I've asked myself that question numerous times especially during tender times with Caleb. It seems like there should be a bigger way to say "I love you"- Where are right words to say that I love them so much that it takes my breath away- that I love them so much that sometimes it even hurts- that I love them so much that my heart wants to beat for them? I know that before they were born just the "hope" of them filled my heart with joy- that when I was expecting I loved knowing that they were growing right next to my heart- and now that they are here, my heart is wrapped so tightly around them that I can't breathe.



I took Caleb to the doctor today and spent the afternoon having chest x rays. He did aspirate and as a result has Pneumonia. He isn't moving air through his lungs very well. He is on a big dose of antibiotics and is in need of a lot of supplemental oxygen. My heart has been tender as I've wondered what I should say to Caleb today. How do I tell him how dear he is to me and how proud I am of him?

But then when I wrap my arms around him, and when I'm really still, I can feel his heart telling me that he knows- and that...

"I love you" is enough.

January 2009:

I have sweet moments with Caleb when I know that I live with an angel. Even when Caleb is so sick that he can hardly move...he still has a miraculous effect on people. During his last hospital stay, I was so touched by the visitors Caleb had...visitors from within the hospital. Many doctors, residents and nurses who have met Caleb before, would come by day after day to check on him, stand by his bed for a moment, and feel his sweet little spirit. Caleb couldn't walk or talk with them - He touched their hearts... and they felt it. I felt it. One told me she had never been so touched and inspired before. I know. Me too.


This last visit was 13 days long. Nine IV's later...and Caleb still shines. I live with an angel, whose feet have never left the ground.

My 2nd little boy, Matthew, would often tell us that Jesus peeked in our windows...I know that the Lord has kept a close watch on our little family as we have been on this journey together...

Caleb is currently 4 years old!

He continues to remind us to find JOY in our journey every day!

We aren't sure how much he can see out of his eye, but we know he responds to light. We are also unsure of his hearing abilities, but I see him respond to my voice, to music, and sometimes to loud sounds. Caleb has a trach to help him breath. He also has a shunt to help drain the fluid from his brain.

Caleb is currently on Phenobarbital (seizures), Levothyroxine (thyroid) Previcid (reflux) Baclofen (muscle relaxant) and atrovent (airway) We have had him on topamax, zantac, and erythromycin, but have since weaned him off of them. We also tried Helmet therapy to help with the shape of his skull, but decided it wasn't worth making him uncomfortable when only minimal results were expected. We have also opted not to have surgeries for his cleft palate at this time.

Caleb is a peaceful and patient little boy. His whole countenance shines! He loves to be LOVED. He loves it when I rub his feet, sing to him, and snuggle him. He is so patient with us and he is patient with the challenges he faces in his little body. Even when he has hard days with his respiratory system, seizures or feedings, his little spirit still shines through. He loves to be surrounded by his family and he loves to hear our voices. He also loves it when he is well enough to be outside to feel the sunshine on his face.

For Caleb, touch is largely how he experiences the world. I've helped him splash the water in the bath tub. I've put fall leaves in his hands and helped him crunch them. I've brought snow inside and helped him build a little snowman on a cookie sheet....I've wrapped his arms around my neck and held them there when we've needed a hug...I've put his hands on my cheeks and let them melt to my skin. He loves it when I rub his feet, rub my face in his hair, and rub his chubby tummy. Caleb loves to be touched!

Our biggest goal is to simply surround Caleb with our love...

We have a vision specialist/preschool teacher who visits him twice a week. I have a consult with a physical therapist and Occupational therapist once a month so that I can continue to keep his body stretched and comfortable. Overall, though, I know that Caleb grows and responds the most to LOVE! I know that he has a happy spirit and that he wants our time with him to be joyful!

We are so thankful for our little miracle. The overwhelming love we feel for him gives us just a glimpse of the way that our Heavenly Father loves us. The tenderness of knowing our time with him will be limited is also surrounded by so much sweetness. I wish there were a "heart language" that could portray the sweetness that surrounds us as we watch our 3 boys love each other, as we feel Caleb's sweet little body in our arms, and as we feel the love that has filled our home to overflowing.

My biggest sources of inspiration are prayer, other parents, the scriptures, and music.

Two of my favorites songs are:

"I Feel My Savior's Love"

and "Consider the Lillies."

The lyrics:
Consider the lilies of the field,
How they grow, how they grow.
Consider the birds in the sky,
How they fly, how they fly.

He clothes the lilies of the field.
He feeds the birds in the sky.
And He will feed those who trust Him,
And guide them with His eye.

Consider the sheep of His fold,
How they follow where He leads.
Though the path may wind across the mountains,
He knows the meadows where they feed.

He clothes the lilies of the field.
He feeds the birds in the sky,
And He will feed those who trust Him,
And guide them with His eye.

Consider the sweet, tender children
Who must suffer on this earth.
The pains of all of them He carried
From the day of His birth.

He clothes the lilies of the field,
He feeds the lambs in His fold,
And He will heal those who trust Him,
And make their hearts as gold.

Some talks that have recently inspired me are: The Ministry of Angels by Jeffrey R. Holland, Come What May, and Love It by Joseph B. Wirthlin, and The Infinite Power of Hope by Dieter F. Uchtdorf.




***Caleb is having surgery today to get a G-tube to help him eat, and a nissin to help with reflux. Please keep your thoughts and prayers with Caleb and his family. Visit their blog, Wink From Heaven, to send them well-wishes and to thank them for sharing their special story.

Child of Love

A compilation of writings by Tammy Hodson at Praying for Parker.

A mom.

A medically fragile little boy with Down syndrome.

A story to tell.

We consider Parker to be an exceptional blessing in our lives. We are constantly amazed at how such a little guy has inspired so many people. But we believe that one of his callings in this life is to touch the hearts of others. Parker still has at least two extensive surgeries in his future.


His health issues include:

1. Pulmonary Hypertension - One of Parker’s doctors describes Parker’s PH as being caused by upper airway obstruction due to severe obstructive sleep apnea, combined with chronic lung disease due to Parker’s underdeveloped pulmonary system. This could cause heart failure, which requires Parker being on supplemental oxygen and medication. If we can keep Parker healthy enough for his lungs to grow, then new tissue will be generated to slowly heal his lungs. This will lead to his PH numbers receding.

2. Tethered Spinal Cord - Parker’s spinal cord was attached to his sacrum, which could cause paralysis as he grows. His cord was de-tethered in May of 2005, but vigilance is required to make sure that the cord does not re-tether.

3. Failure to Thrive - because Parker is constantly so sick, he hasn’t been growing very well. He weighs 23 pounds and is 2.5 years old.

What can I say? The kid is teeny. Really, really teeny.

But he’s HUGE in courage, smiles, spunk and love.

4. Bowel Obstruction - Parker will need two surgeries to create a functional tush, aka, his Teeny Tiny Designer Heiny. As of now has a colostomy until he becomes healthy enough to undergo such extensive surgeries.

5. To help with Parker’s severely obstructive sleep apnea, and his severe pulmonary hypertension, Parker was trached in October of 2007.


6. To help Parker get enough nutrition he received a g-tube in November of 2007.

Because of these issues, Parker is on several expensive medications as well as using several items of durable medical equipment. Parker has already had two surgeries and many stays in the PICU at Primary Children’s Hospital in SLC, Utah.

To help us keep Parker healthy we have him in what we call our “Safe Room” This is a Parker and Mom and/or Dad room only, the kids can only come in one at a time after they have washed, sanitized and masked up.

Parker receives the following therapies and services:

Occupational Therapy
Through Alpine School District Parker receives one hour of OT a month. Parker is working on reaching across his baseline on a consistent basis and increasing his hand strength. We work with Parker to hold a crayon with the proper grip. We are teaching Parker how to insert small objects into even smaller openings. Parker is learning how to twist things on and off and flip switches. He has successfully mastered both throwing and catching a ball. Any ball. The kid loves balls. Parker has also mastered the fine art of stacking. They kid can stack anything…anywhere.

Physical Therapy
Through Alpine School District Parker receives physical therapy twice a week. He has mastered crawling (when he wants to), and pulling himself up to a stand. He is still learning the best way to get out of a stand. And we keep the faith that Parker will, indeed, walk. And run. And skip. And jump.

Speech Therapy
Through Alpine School District Parker receives one hour of speech therapy a week. Parker is learning how to express his needs through sign. He is working on two word sentences such as ‘Want Car.” Although “No! Mom!” still seems to be his all time favorite.

Feeding Therapist
Our school district will not provide any feeding therapies for Parker. So we have found an independent therapist to help Parker. It is fairly common for kids with g-tubes to no longer have a desire to eat by mouth. But since eating helps prepare a child for speaking, we feel that this therapy is something we need to provide for Parker. Plus, we hope that one day Parker will no longer need special feeds via a g-tube. When that time comes we don’t want him not to understand such basics as chewing, moving food around in his mouth, drinking from both a cup and straw and swallowing.

The whole eating thing is made even harder by Parker’s trach. Kids that have been trached can’t smell or taste. In order for Parker to smell or taste he has to be wearing his ’speaking valve’ on his trach. But because of Parker chronic lung disease he can’t wear the speaking valve for more than about an hour a week.



Pre-school
Because of Parker’s health issues he is unable to attend school. And after lots of discussion and prayer both Reed and I feel that Parker needs would best be met in a home schooling setting. In a few years we may re-evaluate this decision, but I doubt it. Through Alpine School District Parker is visited once a week by a special needs pre-school teacher.

This year Parker will continue to work on colors and sorting and identifying animals. I have also created almost 25 lesson plans that will cover topics from bugs to trucks to gingerbread men. I want Parker to explore the world around him to the best of his ability.

I am so grateful for the degrees I have in Early Childhood and Elementary Education. These have served me well with all of my kids. But they are even more of a blessing with Parker.

Because insurance only partially covers Parker’s numerous medical bills, a special account has been set up at all Zion’s Banks in the name of Parker Reed Hodson. These funds will help to ensure that Parker is able to get the care that is absolutely necessary to his overall health, well being, and quality of life. All donations will benefit the Uniform Gifts to Minors Act in the name of Parker Reed Hodson. Contributions to this fund may be made by clicking on the 'donations' link in the sidebar of Parker's site, or at any Zion's bank,or by emailing Tammy at: hods5mom[at]aol.com.

We absolutely believe that with the love, prayers, and support of family and friends Parker will be able to overcome his health setbacks and will be a much loved and needed member of our family.

Sincerely,

Reed, Tammy, Bailey, Brant, McCall, Rigel, Kensley, and Parker Hodson.

Parker's current status: He had surgery just two days ago for a bowel obstruction.

If you could keep our Brave Hero in your prayers for his pain to be manageable and that he can heal and return home, that would be so very appreciated.

Child of Love

by Ronald S. Cole-Turner

Child of love, our love's expression

loves creation, loved indeed!

Fresh from God, refresh our spirits,

into joy and laughter lead.

Silent Angel

About Jophie
by Jophie's mom Trina

In 1993 I had just completed my foster parent training. Little did I know that just a few minutes from my home a sweet baby was being born. That baby would soon become my son.

Jophie was born April 9, 1993. I received my foster parent license in the mail on May the 8th and on May the 9th just one month after his birth I received that call.

At 26 I was very nervous to be going to "just peek" at this sweet but very ill baby boy. This would be my first foster child and a baby at that! The next 30 minutes driving towards the hospital seemed like an eternity.

Brain damaged

Vegetable

Medically Fragile

CT Scans show sporadic damage

Guarded Life Expectancy

That was just a few of the words I would hear that day.

Did I hear those words?

Yes.....Sort of.

My eyes could not get past that beautiful baby lying there. His eyes and that hair....Oh my! There was so much more to him than all those big words and tests. He was small at just under 5 pounds. Small but mighty would soon outline the life of one baby boy who many thought had a grim future.

Yes! He was indeed about to show the world just how much more there was to him!

As we began our journey together, it became apparent to me that we were on no ordinary road to say the least. In the beginning the road was full of many bumps as we battled seizures, severe spasticity, feeding issues, many screaming sleepless nights and days as he battled drug and alcohol withdraws.

On February 11, 1997 just 2 months before his 5th birthday, I made this perfect little boy my son and so began our journey as mother and son with a much bumpier road full of its own share of curves, twists, and turns.

His health began to fail slowly but steadily as even more problems began to tumble into the mix. He began having numerous infections, pneumonia's, and rashes of unknown origin. The severity of his cerebral palsy and brain damage were becoming more evident with his age as he appeared to "stand still" in time cognitively. His motor skills were almost non-existent.

At this time it was determined he portrayed skills that of a newborn to 6 month old. Many tests and feeding studies were performed to check for reflux and aspiration as we felt he was doing both based on the chronic lung infections. The tests continued to prove non-conclusive.

By the age of 5 he became infected with varicella pneumonia from the chicken pox vaccine. YES it is unusual but it was what it was. I still thank God every day for my dear friend and sons pediatrician for listening to my heartfelt plea from his hospital room that early 3:00 a.m. morning. Even when the attending doctors felt I was wrong she believed me when I said, "He's dying before my very eyes". Sherries love for Jophie would prove to save his life that day. Thank you my dear friend!

Less than 6 hours later he was being rushed to the PICU in DIC(Disseminating Inter vascular Coagulation), shock as well as a severe case of chicken pox outside and within his lungs. Every breath would prove to rupture those blisters spreading it even more. His respiration's were in excess of 80, his heart rate was zooming. My baby was slipping away fast.

Papers were being shoved in my face. Sign this

Sign that

"Do you want us to save him?

"Is he a full code?"

Extraordinary measures taken?

Can we give blood if we need to?

Those were the questions being tossed at me as they gently guided me from his room.

One last kiss and one last "I love you" were what I thought would be my last.

Hours later by the grace of God AGAIN......Jophie was stable but very critical
He would remain critical for several days which led into weeks and then months. This was by far one of the worst emotional roller coaster rides we had been on thus far.

Over the course of time he would prove to not just me but to many doctors, nurses, and onlookers just how strong a fighter he was and indeed how powerful our God really is. God moved mountains for Jophie and changed many lives during that hospital stay including mine.

The next few months things settled down a bit. He continued to battle chronic pneumonias, infections, and sometimes rare/odd super bugs however, we made the best of it and were able to make some wonderful memories!

By the time he was nearing 6 the pneumonias and infections were pretty much constant. Feeding was becoming more of an issue. Don't get me wrong Jophie LOVED food! His favorites were cotton candy yogurt, pintos and cheese from taco bell....(TONS of sour cream please!) :O) In fact I had just introduced him to chicken nuggets, cheese burgers, chili, and spaghetti! I had found a way to grind up just about anything with a combination of bread and apple sauce making it possible for him to eat.

Just beyond 6 years he was literally bombarded again with not one but 2 super bugs. Staph E. and enterobactor....UGH! By this time in his life, infectious disease had become our closest friends. Jophie continued to be named in their medical books for oddities and super bugs.(I would have rather he been anonymous).

Regardless, this hospital stay lasted nearly 6 months and once again he would meet face to face with death. The super bugs that he had were not only found in his blood but also in the cultures from his lungs. The only way for those to be there would be from aspiration. It was determined that testing be done again for the reflux and aspiration. The testing did indeed prove he was and most likely had been since the age of 2 ("Silently aspirating and refluxing") It had went undetected until that moment.

My world so I thought stopped. The one and only thing that Jophie and I could do together that was "normal" was eating and they were about to take that from us. I fought and wrestled with God. NO! I did not want this to happen. Don't take away the only "normal" thing we can do together. He enjoys eating so much!.

Finally, after tons of praying, struggling, fighting and arguing with God literally, I realized it was either this or he was going to die. My focus switched very quickly to being thankful for his life. I also knew that even though we had been dealt another blow, with my help Jophie would learn to adapt and we both would come out better and stronger than before!

All food at that time was stopped forever, a central line was placed, TONS of steroids were put on board for healing his lungs, many big gun antibiotics to battle these 2 bugs were being used AND TPN and Lipids were started to sustain him until which time he could recover enough for surgery.

The plan was to perform a fundoplication(Wrap the esophagus around itself to stop the refluxing and aspirating), place a feeding tube and cut the muscle where the intestines meet the stomach to create quicker dumping.

Without all the food and liquid that his little lungs had grown so accustomed to, his body slowly began to heal. When he showed what we felt was the best improvement, his surgery was scheduled. That 2 hour surgery turned into 12 because of many complications. The surgeon was able to perform all but one of the procedures. She was unable to cut the muscle for quicker dumping due to time spent under anesthesia, lack of access due to his misshapen back/chest, and scar tissue from damage sustained over the years.

Jophie healed slowly after surgery due to his puny state to begin with and by the first of the year we were able to return home. At home IV therapy was continued via his central line by me for the next 6 weeks. We had to continue for 6 more weeks after that because his blood was still testing positive for the staph E. Finally, all blood work came back negative and his central line was pulled.

During the summer/fall of 2004, Jophie would spend nearly 5 months hospitalized facing death toe to toe once again.

During this stay he would be infected with Proteus Morabilis TWICE, Pseudomonas, Stenotrophomonis Maltophilia, A pleural effusion and thank God it resolved itself, An AVM(Arterial Vascular Malformation) which caused a GI bleed and surgery was required to repair it, Paraflu(Which we both had for 2 weeks), Psueodomonis became active AGAIN, many allergic reactions to antibiotics, Tons of side effects, withdrawals, rashes, severe diarrhea, Neutropenia(Low white count), Pancytopenia(Low white count, red count, and platelets), blood transfusions, adrenal failure, AND finally home! Phew! What a Ride!

One year later he was still trying to recover. His body was battered, much weaker, and we continued to control break through seizures that at one time were mostly controlled and were unable to fully wean him from the very steroids that saved his life. We now fear his adrenals have failed because of it. We're hopeful the adrenals will begin working on their own again but, until that time the steroids that have caused this problem are once again saving his life.

Two years later in 2006 he would be hospitalized with Mycoplasma and C-Diff. Fortunately, I was able to bring him home after only a weeks stay. I along with Jophie's nurses would continue a 6 week long treatment regimen to kill those nasty bugs. It was during this time that I finally accepted the fact that I just couldn't keep track of all his medications. The many medications he was taking multiple times during the day made that abundantly clear. I usually manage just fine however, when he is ill you have to add the "extras" on TOP of what he already is taking and that's when my eyes begin to cross. The solution. A dry erase board mounted on my living room wall.:0)

March 2008 would prove to be another life altering hospital stay. After nearly going into respiratory failure, Jophie was transported via ambulance to the hospital where he landed in the PICU for the next 3 weeks in very critical condition.

Over the years Jophie's back/shape has gotten much worse making it next to impossible to treat him during a crisis. Fortunately, the wonderful PICU docs and staff were able to stabilize him on CPAP rather than placing him on the vent. He responded very well to this treatment and it would prove to save his life.

Tests would reveal an active Pseudomonas again. For those of you who haven't followed Jophie, he is colonized with Pseudomonas meaning it never goes away but rather goes into an inactive state. The plan is to keep those evil bug colonies down to a small number all the while trying to build up Jophie's good army who are in a constant battle to keep those evil ones at bay! I like to think of it as dragon slaying. Now if I could just find me a knight to slay these dragons we might be in business ;)

Because of Jophie's misshapen body and the poor state of his overall health it was determined that a trach was in order to prolong his life, give him a better quality as well as make it possible to effectively treat him during a crisis all of which I felt were very important if not life sustaining. What I didn't know was how long it was going to take to get to this point nor what he/we would have to endure along the way.

To say the last 7 months have been horrendous would be an understatement of great magnitude. If fact, 6 weeks into this new trach lifestyle I began questioning my decision to even allowing it.

The last 6 months Jophie has struggled with severe pain. He was coughing up blood on a regular basis. He was unable to sit up or to vocalize nor were we able to explain to him why. He's dealt with fear and frustration over that inability to vocalize. There has been lots of crying and gnashing of teeth. We both have had many sleepless nights and days as well as many trips to the ENT who was overseeing his progress as well as troubleshooting the problems. My battles with his insurance over yet more medical supplies related to this new trach continue as they still are limiting or refusing to cover needed/life sustaining items. The battles are never ending. Like I said we need a knight to slay these dragons who seem to come in many shapes and forms ;)

During the course of all this mess we were able to determine that Jophie was unable to wear the size or brand of trach that was originally placed. In fact it caused a huge ulcer and nearly bore a hole through his windpipe thus the cause of all the bleeding/pain/infections which led to a 2nd trip via ambulance due to his airway nearly closing off while Jophie's nurse and I watched helplessly. Ugh! A smaller pediatric trach was placed in the ER then he was admitted for surgery number 2 where the new pediatric trach would be evaluated as to whether or not to keep it and repair any damage sustained by the old trach as well as remove any granulation tissue that may have formed. To make matters worse, were having to boil/reuse these trachs which was causing the reoccurring infections not to mention warping the trachs which also caused more damage/injury and could have easily broken off in his airway.
Fast forward to now October 2008.

Jophie is a much happier little guy! After many trials and lots of tears, we've found the brand of trach Jophie can use. Of course its custom and pricier than the rest but, its working and I for one am NOT going to muck with it! Would you? :0) His anxiety/fear has lessened considerably if not completely during daily trach cleaning as well as his weekly trach changes. He's been able to sit up for short periods of time and often for a couple hours. We've really seen improvements just in the last 3 to 4 weeks and have actually been able to do some "fun" things with him for the first time in sooooo long! I can't tell you how healing that has been for not only Jophie but me as well. He's still swollen quite a bit from the massive steroids but we are successfully weaning trying ever so hard to get him back to his maintenance dose. If all goes well barring any complications he should be back to maintenance by the second week of December! Phew! It's been a long road on this wean starting way back in march but so far so good!

Daily we continue to juggle steroid dosages by increasing and decreasing based on illness, stress, infections etc...Infections and super bugs continue to plague him. He now sees a pediatric endocrinologist at Columbus Childrens Hospital for his failing/failed adrenals. 5 years ago I changed his formula to Novasource pulmonary which is geared towards children with breathing and respiratory problems. This formula has absolutely been miraculous unfortunately we've just found out they will no longer be making it so the hunt is on for something comparable. I've also incorporated 3 different types of yogurt including an immunity booster as well as crystal light immunity which we add daily to his water infusions. Our hope is these things will help his compromised immune system as well as the candida that plagues him off and on. He now weighs around 100 pounds and daily we are slowly seeing the sparkle return to his eyes. I know without a doubt the new things we are trying are helping but the real credit goes to our heavenly father who continues to keep Jophie wrapped in his loving/healing arms.

The years have indeed been hard on Jophie. He has endured more than most do in an adult life thats for certain. He's faced death on more occasions than I can count. He's battled the ventilator on more than one occasion and he's had his share of surgeries and surgical procedures. He also has endured and continues to endure more tests, painful and unpleasant procedures, braces and corrective equipment, treatments, surgeries, rare and deadly super bugs and faces many monsters beyond what any of our imaginations could fathom. Jophie accepts all these daily routines that help him to survive and yes....he does this with a SMILE!

As you can see Jophie is MY LIFE. He is in every single breath I breathe. I love him so much sometimes it hurts which only fuels me further to fight for what he needs. He is indeed a special little guy who continues to touch lives everywhere he goes. I've been witness to miracles beyond measure and watched Doctors and Nurses stand with mouths gaping open as they try to comprehend what has just occurred. Time and Time again he survives, proclaiming loudly for all who see...

There is a God, I am loved, and above all he is in control. I am not a mistake nor am I damaged goods. I'm perfectly made and worthy of saving.

A masterpiece.

If he does nothing else in this world except for changing hearts and making others see beyond his disabilities and really SEE HIM as the masterpiece that he is, THEN he's accomplished everything.

It's been a long, scary road. We continue to travel this road together. Jophie and I with Jesus holding our hands.

A hero is said to be someone who is distinguished by exceptional courage and strength.

My life has forever been changed by such a person.

He's my hero

He's my son.

A Day in our Life

While most people have long since settled for a good nights sleep, Jophie and I are just beginning our day bright and early at 1 a.m.

1 a.m.--Reposition Jophie/Change diaper and bed pad and/or sheets that are usually wet/Give Carafate/Sterlize water/Add Immunity booster to his water infusion/Begin water infusion which takes 2 hours/Set Timer to alarm every 20 minutes/Albuterol Breathing Treatment

3 a.m.--Prepare Novasource Pulmonary for tube feeding/Crush Medications=Klonopin/Tegretol/Cortef/Bendaryl/Zofran/Mix crushed meds with sterile water and leave to soak/Repostion Jophie/Change diaper and bed pad and/or sheets that are usually wet/Give meds via Mic-key and begin Tube feeding/Set Timer to alarm every 20 minutes to give slow bolus till all feeding is done which takes around 3 hours

6 a.m.--Tube feeding is finished/Reposition Jophie/Change diaper and bed pad and/or sheets that are usually wet/Aluterol Breathing Treatment/Flovent/Begin water infusion which takes 2 hours/Set Timer to alarm every 20 minutes

7 a.m.--Carafate

7 a.m. to 9 a.m./Try to catch a few Zzzzzs!

9 a.m.--Nurse arrives/Prepare Novasource Pulmonary for tube feeding/Crush Medications=Klonopin/Tegretol/Cortef/Zofran/Prevacid/Mix crushed meds with sterile water and leave to soak/Reposition Jophie/Change Diaper and bed pad and/or sheets that are usually wet/Give Albuterol and Intal Breathing treatment/Give Flovent/Set Timer to alarm every 20 minutes to give slow bolus till all feeding is done which takes around 3 hours/I lie down to try and catch a couple more hours of sleep

12 p.m.--Reposition Jophie/Change diaper and bed pad and/or sheets that are usually wet

1 p.m.--Begin water infusion which takes 2 hours/Set Timer to alarm every 20 minutes/Give Carafate

3 p.m.--Prepare Novasource Pulmonary for tube feeding/Crush Medications=Klonopin/Tegretol/Cortef/Mix crushed meds with sterile water and leave to soak/Reposition Jophie/Change Diaper and bed pad and/or sheets that are usually wet/Give Abuterol Breathing Treatment/Give meds via Mic-key and begin Tube feeding/Set Timer to alarm every 20 minutes to give slow bolus till all feeding is done which takes around 3 hours/Eeek! I haven't eaten yet! Make a mad dash to grab some food!/Grab any meds at pharmacy/groceries/pay any bills that needs paying while I'm out and about

5 p.m. on Monday/Tuesday/Thursday Nurse leaves/3 p.m. on Wednesday/Friday Nurse leaves

6 p.m.--Reposition Jophie/Change Diaper and bed pad and/or sheets that are usually wet

7 p.m.--Give Albuterol and Intal Breathing Treatment/Give Carafate/Begin water infusion which takes 2 hours/Set Timer to alarm every 20 minutes

Try to snatch a few more Zzzzzs here!

9 p.m.--Prepare Novasource Pulmonary for tube feeding/Crush Medications=Klonopin/Tegretol/Cortef/Mix crushed meds with sterile water and leave to soak/Reposition Jophie/Change Diaper and bed pad and/or sheets that are usually wet/Give Abuterol Breathing Treatment/Give meds via Mic-key and begin Tube feeding/Set Timer to alarm every 20 minutes to give slow bolus till all feeding is done which takes around 3 hours

1 a.m. and we begin all over again!

This breakdown of our schedule doesn't even include the many other things that have to be done daily with no set time. I've only included the necessary things that HAVE to be done and HAVE to be on a schedule.

Now for the "other" things that also need tending to but we have to just work in between everything else

1. Tubing changes on his Oxygen Concentrator/Nebulizer/Air Compressor and Tubing to his trach

2. Equipment check on all of his equipment to make sure everything is in working order

3. Answering any alarms from those machines

4. Suctioning

5. Range of Motion/Stretching

6. Bathing/Grooming

7. Cleaning Filters to all machines

8. Sterlizing water and/or any equipment supplies that need it

9. Laundry and just keeping up with his Bed pads/sheets/clothes that tend to be
soggy from just bout every body secretion you can imagine is a chore in itself and then theres mine!

10. Dishes

11. Sterlize/wash all his tubes/syringes/feeding bolus sets/Trach mask/Nebulizers masks/Trachs/bottles

12. Pooey Patrol! Every 3 days we have to give prophylatic medications to keep up with that little sluggish system of his! ;0)

13. Phone calls daily to Medical supply companies/Insurance

14. Restocking Medical Supplies/Putting Medical supplies away as they are delivered

15. Bills that have to be paid

14. Trips to the pharmacy for medications which seems to be neverending

16. The house and outside yard comes very last but still houses need cleaned and yards need mowing

Now try slipping in there an "outing" or something "fun" for Jophie besides all the medical stuff or how bout a doctors appointment or worse still he gets sick and then we have a whole nother set of meds and treatments!

Phew! Jophie sure is a busy kid and keeps his momma and nurses busy too! It definitely takes a team to care for him and meet all his needs on a daily basis. I'm very thankful for the nurses we have and wouldn't trade them for anything. In fact, they are more like family than "hired help". I like it that way especially for Jophie. Everything in his life is so "clinical/medical" and his home is one place I won't have that. He needs to feel comfortably in his home and know this is a fun/safe place to be! Now, if I could just find another nurse to fill these gaps we are having on the weekends I'd be one happy camper. We are still winging it alone every other Saturday and are limited 3 out of 4 of our Sundays. This weekend is one of those weekends and I for one can tell you....I am one tired momma! No regrets and no complaints from this corner though! I'd go to the ends of the earth to meet Jophies needs no matter how tired it makes me!

Meals 4 Wheels is a collaborative project inspired by Jophies need for transportation and medical supplies. Due to Jophies progressive illness, he is no longer able to ride in our current vehicle safely or comfortably. Our goal is not to get a new car, but to supply Jophie with a safe means of transportation. As medicaid continues to cut costs and pay for less of his medical supplies and equipment, Jophies needs have become greater. This is how the idea of the cookbook came about.

The actual cookbook was created from our success with weight loss. Since May of 2007, six of us collectively have lost a total of over 200 pounds. This was done exclusively by using the types of recipes in our cookbook, exercise, helpful tips as well as an overall lifestyle change. Through a lot of experimenting we were able to create healthful recipes without compromising what we all love most. The Taste! We believe that this cookbook can help you successfully lose weight and improve your own quality of life, just as by purchasing one you will help us to improve Jophie's quality of life.



Please support Trina and Jophie by leaving comments and checking out the cookbook site here.