Precious Gabriel

Hard to find words for this. For now, sharing Aaron's beautiful words posted on Facebook:

Dear friends,

Our precious and Mighty Gabriel changed form on Thursday August 27.

He spent a beautiful day outside with us in the sunshine, and in the evening he passed very peacefully in our loving arms.

Gabriel was with us so briefly, but he will be remembered and loved always.

A memorial event to honor him and celebrate his life will be held on Sunday September 6 at 1pm at the Sorenson-Lockwood Funeral Home in Grayling, Michigan.

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Thank you to those who have reached out to us since Thursday. It means so much to us to have your loving words and support.

Our boy is 2 months old!

Happy birthday our love!

He has been very fussy on and off between sleeping, and periods of more work of breathing, and many episodes of lower oxygen levels with that fussiness. It could be the general decline he is on, or colic, or just feeling blue because of the dreary day. He's an infant, and will have variable moods. And because of the SMA and his respiratory failure, he will have good and bad days. We are hoping for a good night and a little more of play and games tomorrow.

Baby at the Beach

Friday we were blessed with a beautiful day. Our third visitor of the day was a photographer, who generously shared her time to fill a goal of ours, to have photos of Eleanor and Gabriel and we were able to do so outside in our front lawn. The day we went to the ER originally, Gabriel, Eleanor and Joanna had been outside playing on the grass together. It was so nice to be back there making more memories.

We've been cautious about outside time, but Saturday was so beautiful we wanted to chance it by going to our lake park. Gathered Gabriel onto his wagon, and crossed the yard, crossed the road, and stepped out from the circle of fear and anxiety into the sun.

We had a Baby at the Beach!!

Gabriel slept most of that hour and a half, but he was comfortable. His eyes fluttered open a few times to see his parents, the sky, and the dappled light on the leaves above. Aaron, Jo, and Grandma Lucie breathed in the fresh air, felt the breeze, and relaxed a little. Eleanor came home with her Papa and Bubbie, and then she played and jumped in the water as kids are supposed to do in the summer.

Today, Gabriel had a good afternoon. He had quiet awake time and engaged with his eyes and lifted his eyebrows at mom to show he was interested. He moved his arms more than usual and lightly gripped and moved a little ball; it was slight but more play than we've done before. And he smiled, almost laughed, at the nurse who was wiping his lips and it became a game. Also a first. Warms a mother's heart.

One Week Home

Gabriel turned 8 weeks old yesterday, and today marks our 1 week home. We've been thinking about how much can happen in a circle of the sun.

Gabriel had some morning awake time and some smiles, then a good nap. His Papa played piano-- a jazzy version of "Summertime." Mama got him out of bed to hold twice and near the window for sunset. And because he is a normal baby despite the vent, he still had a bit of a grumpy evening, but a little shorter than other days.

We have adjusted to being home slowly but surely, getting more used to his needs and the schedule of his cares. It's a balance between his care, Eleanor's exuberance and need for us, and our own needs. We've had to have some hard conversations lately. It's not easy. We are thankful for help from family, friends, and neighbors.

Mighty Gabriel's New Digs

Sweet Gabriel has the biggest bedroom in the house: our sunroom. He has walls of windows to light his days, and a ceiling fan to watch. There is a constant hum-puff of the air compressor for white noise, and AC for the hot days; when it cools down we'll just keep the windows open. He loves his fox-mirror from CureSMA, raising his eyebrows at it. Today he made a new friend, smiley-balloon and gave it a big smile right back.

We pictured summer as being outside all day, but have been inside much of it. Yesterday we made our first step of being mobile with the baby and the vent, and rolled ourself to the back door and just outside to enjoy the trees and breeze. Gabriel was wide-eyed and rewarded his parents with smiles. He breathed a bit fast, but some of that could have been excitement. He slept well.

Home!

Thursday marked the end of our hospital stay, 30 days. Packed, organized, and feeling ready with the care we can offer Gabriel at home.

We met with many many people in the three days prior to discharge. We recognize how fast it came together and how much effort was made for us.

We said goodbye to many who helped care for Gabriel and our whole family. We are forever grateful for that care and connection.

We rode home in an ambulance without incident, found home support services waiting for us and entered our home. We breathed in home. We breathed in our family. We breathed in life.

Jo's new motto is "be grateful for each breath." We are grateful for the time we have with Gabriel, each breath he has in his life.

This is a dance, we are not in charge. Gabriel is leading.

Thank you to my brother for support: "you two are listening close. This is what bravery and courage and love is."

Bravery is not the absence of fear. It's being afraid and doing it anyway. Gabriel is doing his part.
We will do our best.

Another Stable Day

Another stable day for Mighty Gabriel. Joy.


We learned more about the machines that help support Gabriel. We also met with our care manager who is moving mountains to get all of this arranged so quickly. She is so skilled, and so caring.
We are so eager to go home, but we want to do it right. We will stay until Thursday to practice then hopefully get home. It's a short time to get all the practice, but we will also have help at home.
Over the past 4 weeks we have had so many kind, helpful, and caring brilliant people help take care of Gabriel. We are so grateful and touched. And in the past week we have also had many of them stop by our room again for social visits, to check in on this little person who has touched their hearts.
We will remember.

Breathing More Comfortably

Gabriel did well on his new vent and mask overnight. His blood carbon dioxide level actually improved (lower) compared to the old ventilator. And more importantly, he looks to be breathing more comfortably.
We are hopeful that we will be able to go home soon. Our care team sprang into action to make calls and arrangements. It was a busy Monday for us, this time with learning about all our new machines and practicing care for Gabriel's needs.

Meanwhile, we talked to Gabriel, held him, and he spent time with Uncle Chris and Aunt Dena. Gabriel will miss them, as they travel back home soon. We will miss them too.

A Week in Review

Thursday Gabriel mostly rested, making 3 stable days on his same settings.
We met with discharge planning folks, and about Home nursing help.

Friday started hopeful. We had at least 8 smart people put their heads together about a tubing interface from the Trilogy vent to Gabriel's current nasal mask which fits. He seemed comfortable, with a few adjustments. But later he had some lower oxygen readings while he was breathing comfortably (we know that working harder can cause a desat, this wasn't it) and he had too much carbon dioxide in his blood, so we had to go back to the old vent.

Saturday was a day of rest for Gabriel and worry for the parents. He had an acute low oxygen episode, due to mucus but he got past it. We worried the rest of the day. We will start chest percussion to help him keep that stuff thin and out of his lungs.

Eleanor came to visit again. She has been visiting her cousins and loves them! They went to the lake cabin and she played in the sun and water, now looking tan and older than a few days ago. Their dog plays fetch with her. We are glad she is having fun.

Today we are working on the plan to get home, and breathing is the number one. Gabriel today got a new mask, which fit better than thought last week. He switched to it and tolerated it well. He seems comfortable. It fits over his nose and between his eyes, so he looks a bit like Darth Vader. But all along I've compared his looks to Spock (eyebrow raise), Professor X (bald head) and the Bionic Man, so this is just another step for our sci-fi baby.

6 Weeks Old

Happy 6 week birthday, Gabriel!! We love you!!

Gabriel was stable, better than the last two days. Gabriel had a lot of awake time, 2 hours with mama holding him. Then Uncle Chris and Aunt Dena visited and Gabriel LOVED Uncle Chris. They made eyes at each other; Chris bounced his legs and played bicycle kick. Gabriel was fascinated. He smiled quite a few times! We are glad to be reminded that Gabriel is a cute kid with a normal mind that is growing and developing with movement and play and sounds. Thankful to have noise and play re-enter our room.

Today is 3 weeks here.
Planning for home: Gabriel is unique in that he is smaller than the usual child on a ventilator, and there are few choices of masks for the planned home ventilator machine. Many people are putting their minds together to come up with a solution. It's looking like next week. At least power to our home has been restored (after the storm).

A Real Smile

Today was special. Gabriel and mama were talking, when mama laughed and Gabriel smiled, a real smile.

Our friends at Child Life helped us make some memories to hold onto. Eleanor visited and we made "art projects." Ink hand prints with the four of us; plaster molds of Gabriel's feet; sculpey-clay beads with his hand prints. And Eleanor sang "Twinkle Twinkle" for a musical memory.

Hard Day

Today was a hard day. Gabriel tried a sprint, but immediately had a severe episode of low oxygen. It was scary, but we had a nurse and respiratory therapist in the room with us and they acted quickly. But we will likely not try any sprints. There was a second issue in the evening. We were almost too anxious to leave, but the night went well and we slept too.

Gabriel's First Concert

Gabriel was stable. He had his second visit with Uncle Peter. He also had his very first house concert with a famous singer songwriter; we were grateful for the gift, and Gabriel had open eyes. We also showed Gabriel a sparkly pinwheel and mirror toys sent from CureSMA.

We are amazed and grateful to CureSMA. Jo emailed them and within 2 hours they had sent out an information packet by overnight delivery, and then a care package of age- and ability-appropriate toys. Soft blankets, pillows, lightweight toys (balloon, feather), sensory stimulation toys (the pinwheel, crackly paper), and even a Fisher Price ocean music/projector. We appreciate the support from people who don't know us, but know the pain we are going through.

Time with Eleanor

Gabriel seemed to be working harder to breathe at times, so the doctor increased the breath support (number of breaths per minute he gets more air pressure in). The nurse said he was fussy again at night (he poops and is fussy every night between 5 and 7pm) but this is normal baby behavior.

Aaron and Jo and Eleanor celebrated life by having a family day outside in the sunshine at the beach. It was a beautiful day. After two weeks of stress and air conditioning (which felt to Jo like March), we were happy to be out in the summer.

Busy Day

Today we talked to our specialists, social work, and care management about discharge planning: how can we all get home for quality family time with Gabriel. We also decided to celebrate his life, and focus on living well for all of us. Sunshine, music, outdoors. Eleanor went with grandma to the Meijer Garden again.

Gabriel had a one-hour sprint off the mask. A volunteer photographer visited and took beautiful photos of Gabriel with mama and dada; Gabriel had open eyes and seemed to enjoy it. He made a few gurgles that may have been coos.

After this, we had a family time and sang "Circle of the Sun" and he watched with wide eyes. A music therapist also visited and recorded his and our heartbeats; she will use his heartbeat as the rhythm and play a song of our choosing over it. How special!

Following Gabriel's Lead

Gabriel did a short time off the mask ventilator, but not a long sprint. We want him to be comfortable. We are following his lead-- he will tell us what he can do.

We got sad news from the two studies that he doesn't qualify based on how early his symptoms started, and the support he needs now. As I said above, Gabriel is leading.

A Day for Rest

Gabriel saw the occupational therapist, who wanted to re-make his wrist splints. It's a little larger and deeper, and looks like a one-sided boxing glove. Our Mighty Gabriel.

The PICU doctor and we decided that the sprints were causing him to work hard to breathe, and made him more tired; so today he rested.

Longer Sprint Trial

Gabriel's goal was a 5-hour sprint on the nasal cannula. He didn't do as well, with a little saliva in his airway and he needed to get that sucked out. He was breathing harder and we ended the sprint at 4 hours; he needed rest after. His blood gas showed that he wasn't exchanging oxygen and carbon dioxide well during that time. This made us sad.

Two friends stopped by to share some time with Gabriel, and he opened his eyes a little at the end of their visit. He is beautiful.

Waiting

We saw all our teams again. Mama was focused. The teams were going to talk to the two treatment studies about Gabriel, and what our breathing goals needed to be for him to be able to participate. We are just waiting.

Gabriel did one 4-hour long sprint and did well. At the end, he was mad and silent- crying, but that was about a dirty diaper (he hates poop) and we were reassured that this is normal baby behavior.

Grand Rapids

We returned, bringing Eleanor with us to live in Grand Rapids as a family, albeit with one of us in the hospital. Gabriel was stable, and did two 2-hour sprints like a champ. The goal of the sprints is weaning him off the ventilator so we could possibly get out of the hospital and maybe go to the study.
He had eyeball time with mama holding him.

Possible Drug Trials

Gabriel continued his sprint trials. We talked to a doctor about a possible treatment in a drug trial in Columbus; it actually inserts a new SMN1 gene into DNA; however he is not ready because of his breathing issues; he needs to wean off the ventilator. We were told to also look into a study in Chicago.

After Gabriel's sprint we wrapped him up and kissed him and went to Portage to see Eleanor and reconnect. So hard to be away from either of them.

The Diagnosis

Gabriel was stable, and we planned more "sprints" on a nasal cannula to see if his muscles are strong enough to wean off the ventilator support.

We were told the the lab test would take 2 weeks, maybe longer; the idea of waiting was agonizing. But today the test result came, confirming Spinal Muscle Atrophy (SMA). We had a long meeting with our team, to talk about SMA symptoms, course, and prognosis. Briefly, SMA happens when we are missing two copies of a gene that makes a protein to keep peripheral nerves (ones from the spine to the muscles) alive; without these nerves, the muscles get weaker. It affects all muscles, starting with arms and legs and later affecting breathing muscles. The brain and senses are normal.

You can read more about SMA at http://www.curesma.org or https://www.mda.org/disease/spinal-muscular-atrophy

Papa and Bubby visited to see Gabriel. We parents fell asleep early and hard.

Sprinting

Gabriel had some time with Aaron, who held him. Gabriel wore a nasal cannula, to take off the bionic mask and give his nose a rest from the mask; they call this a "sprint." Jo took Eleanor to the Meijer Garden to run around; the Children's Garden had a beaver dam to climb in, sandbox, and sculptures. Eleanor asked a few questions about Gabriel; that was tough.

Skin 2 Skin

We talked to Early Care, which from our understanding focuses on keeping the family intact in the setting of chronic illness. Just what we need. Gabriel was stable and Jo held him skin to skin for a while.

Conversations Continue

Aaron held Gabriel in a chair during the time off the ventilator. It went well. There were more conversations.

Our Brains Hurt

It was a big day, a tough day. We talked to six groups (pretty much our whole team). There is so much we have to think about for Gabriel's comfort and care. We talked more about SMA. Our brains hurt after.

Bath Time

Gabriel got a bath with a really nice nurse. He had just a little time off the ventilator on nasal cannula with high flow oxygen and he slept on his tummy a while, then on mama. Nice. But he had an episode and needed to be back on the ventilator for rest.

Visiting with Uncle Peter

Gabriel's uncle Peter visited. We touched him and changed diapers and Peter showed us his magic baby soothing skills (rubbing the forehead/third eye area).

Explaining it to Sister

Gabriel was more awake, and he started a temporary feeding tube into his intestine (jejunum), but still getting breastmilk. Eleanor visited her brother today. We missed her! Mr Russ, a new friend from Child Life, helped us introduce her to the whole situation and assess how she would handle it; she was good, and was more interested in the machines in the room and the big windows.

Possible Diagnosis

Aaron and Jo drove in the early morning hours to meet Gabriel in the PICU. He seemed to be doing better and was able to breast-feed and swallow ok, so we went upstairs to the Step down Floor. We continued feeding and nursing and touching and talking to him on the bed. He was weaned off oxygen and IV fluids stopped. But the next day he looked so tired.

We talked to a hospitalist, neurologist, and geneticist. There was a possible diagnosis of Spinal Muscle Atrophy, and that was best to get first before anything else.
He had another episode and we moved back to the PICU for rest on a noninvasive ventilator. He got a mask and a bionic helmet. He gets a constant flow of air to keep his airway open (CPAP) and occasional breaths of more pressure (IPAP) to reduce his work of breathing. We hoped he had more energy to then get off the ventilator and maybe go back to eating.

Going to the ER

Jo was holding Gabriel, when his lips turned blue, we brought him to the ER, and dropped Eleanor off at Aaron's parents house. We half expected (hoped) that the ER staff would send us away as overly nervous parents. They didn't. With retractions (using extra muscles to breathe), he needed to be watched by specialists at DeVos Children's Hospital in Grand Rapids.

Instead, Gabriel had another episode just as the transport team arrived; he flew by helicopter to the pediatric intensive care unit (PCCU). We followed by car and arrived just as the sun was rising.

The Story Begins

Our beloved son Gabriel Anthony was born on June 24 of this year.

While he was indescribably beautiful at birth, and in most ways very healthy, we did notice almost right away that something was unusual. He was hypotonic, a fancy word for floppy. He had very low muscle movement tone and very weak grip.

Mysteriously, he displayed none of the other symptoms that usually accompany such hypotonia. He "scored" well on all the scales, and nursed well. His bowels were more than functional.

As a precaution, the doctors kept us in the hospital for a couple extra days as they performed blood tests, urine tests, and even an MRI. Everything came back normal.

Aaron's mom recalled that he too was a "floppy baby", but grew out of it. We hoped that was all it was. The doctors sent away for some more obscure tests, but sent us home rather than wait in hospital.

For a couple weeks, all was fine. We snuggled, and nursed, and went outside to walk and to our lake beach. He was growing well, an ounce a day.