Prader willi, Uncategorized

Dark Rooms, Part 1

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Memories exude a color in my mind. Darkness is the only way to describe a week I did not expect in our journey to a diagnosis with Elsa.

I frequented our pediatrician weekly for weight checks. I pulled myself together for each visit and thoughtfully placed Elsa in her car seat with all the towel propping for our 15 minute drive. I pretended I was brave and non-emotional for each visit. I put my clinical hat on for the hour to discuss every little minute, hour, day of Elsa from week to week. I did this until my pediatrician said she suspected seizures. I couldn’t hold it in anymore. I broke down in tears and bewilderment. Was I not catching seizures? Was I a terrible parent?

It only took a day to schedule a flight to Anchorage and see the pediatric neurologist for an EEG. This would monitor her brain activity, asleep and awake, for signs of petite mal seizures. My husband and I arranged our schedules for our two boys, his work, and my travels to Seattle. My friend would pick me up from the airport and drop me off at my hotel. My aunt would meet me the next day at Seattle Children’s Hospital for support.

I was relieved we would see a neurologist quickly but scared there was something more serious going on with Elsa. I had bittersweet moments throughout the day. Thoughts were constantly on her feeding schedule, her wakefulness, her facial expressions, just looking for signs of seizures. It was all my brain could focus on.

This was my first time at Seattle Children’s, a place where I would call home for a week. It was a place where the twisting hallways become my walking route for exercise. I was tired but anxious for her EEG. It was the first appointment of the day so it was quiet in the hospital. We were lead into the exam room and the technician explained each step of the procedure. 20180627_092441Her limp arms did not protest all the wires and goop placed on her head. It would take 30 minutes where we sat and watched her drift from sleep to gentle arousal. My heart was full, heavy, and anxious. I sat in a rocker next to the table staring at her face. She slept peacefully with minimal facial expression. I finally took my book out to calm my mind and preach to my soul.

Kiss the Wave: Embracing God in Your Trials has pointed me back to Jesus in my dark rooms. I was only on the second or third chapter at this point but it reminded me through suffering and anxiety, through heart wrenching moments, Jesus is still in loving care of our hearts. Pages are filled with scripture that I prayed over Elsa, prayed into my heart, and pleaded with Jesus for peace. It was a reminder that God did not leave me, I was not alone, and I had Him to call upon in the darkness of night.

I prayed these over Elsa as she lay on the treatment table.

The Lord has established his throne in the heavens, and his kingdom rules over all. (Ps 103:19)

I know that you can do all things, and that no purpose of yours can be thwarted. (Job 42:2)

God is our refuge and our strength, a very present help in trouble. (Ps. 46:1)

 

~**~

 

Goop was still in her long curly red hair when we left the room. We had to wait another two hours to see the neurologist for the results. My aunt and I ate in the cafeteria where parents and children gathered to escape their hospital beds. Families loomed over the television screen that displayed if their child was in surgery or recovery. You could feel the heaviness that fell on individual tables. You could palpate the anxiety of uncertain outcomes of a daughter or son. And yet, there was light streaming through the big windows and smiles on the children’s faces as they talked with their siblings.

It was time to see the neurologist. Maybe it was on my face or maybe I put on a façade of composure but I was wrecked with anxiety. The nurse measured her head, took her height and took her weight. I was keeping track of all three on my phone and I knew this was not good. She had plateaued. My façade was gone. Tears had already filled me eyes as I met the neurologist for the first time. She gently looked over Elsa. The good news was no seizure activity. This was my small light in the darkness. The bad news, she wanted us to stay and be admitted to inpatient for further testing. Even though this was not what I had planned, it would become a blessing. It’s funny how a moment entrenches us and not long after it becomes a glimmer of hope.

Tears came quickly for half an hour as she explained Elsa was lethargic, plateaued in her weight and head circumference, needed an MRI, and additional blood work. I knew Elsa was sleepy. We lived 6 weeks of her small life feeding her around the clock. We worked endlessly to keep her awake and stimulated. I felt like my ability to mother my child was taken from my hands. My attempts had failed and I didn’t know how to fix it.

The next statement she said gripped my heart even more. I knew it to be true but I wanted something else. “I want to do a FISH blood test specific for prader-willi syndrome.” This is a fluorescent in-suto hybridization that detects errors in the genome by deletion. She also wanted to test for imprinting errors. If you haven’t read my previous blog posts, I had suspected prader-willi day 1. My physical therapy training had me over thinking everything, this included. My gut was right if the neurologist suspected it as well. Clinically I could see all the signs and physical manifestations of prader-willi, but deep down I just wanted to see my daughter. I didn’t want to see Elsa just as a clinical presentation.

One of the longest walks was taken to her hospital room. I was crying through the hallways. Holding Elsa in my arms, two case managers lead me to her room. I’m sure people were staring, but that was not on my mind. Failure was on my mind. Sadness and grief, pain and exhaustion filled my body.

It took hours to get settled into the room as nurses, doctors, and case managers rotated in and out. I hadn’t eaten but a small bite since 11am and it was now evening. I hadn’t stopped crying since we left the neurologist’s office. Once the doctors left for the day I was able to speak more with my husband back in Alaska. He calmed me down. His optimistic words filled my ears and his care for me reminded me to eat, sleep, and look after myself as much as I could. His heart always pointed me back to Jesus too, reminding me to lean on Him. Again I was reminded of a hopefulness that was buried deep in my heart. There would be light again. It might have been buried but it was still there.

God is in the midst of her; she shall not be moved; God will help her when morning dawns. (Ps 46:5)

 

 

 

 

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Prader willi, Uncategorized

Survive or Thrive

The first time you meet your child is one of the best moments in life. So new, so naive, vulnerable, and reliant on innate instincts of the human condition. You would think motherhood would come naturally. You would think breastfeeding and understanding your babies every little noise would be instinct. If you’re a mother, you know this to be false.

Every child is different. I have two boys who came bursting into the world with personality and loud voices to tell me exactly what they needed. They were hungry for life. They were hungry for the world to be explored. Elsa came into the world quiet, subdued. She could barely take her first breath without assistance. She was too weak to cry, too weak to suck, too weak to tell me her needs. A doctor once told me an infant with prader-willi lacks survival skills and that was Elsa.

Back in my home it was a constant struggle to feed Elsa. Every three hours we would wake her small limp frame of 5lbs 15oz to force in her 50ml of pumped breast milk. She would barely open her eyes. She did not search for the bottle. Her small hands did not reach out in loving adoration of my touch. David and I had our techniques and skills of physical therapy to help her feed. We hold her chin and cheeks just right to increase sucking. We would stroke her neck to prompt swallowing. Tapping her bottle would promote her suck reflex. We would stimulate her with cold rags and deep massage to keep her awake. After 30 to 45 minutes she finishes a bottle and she was exhausted.

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I was referred to a lactation consultant for frequent weight checks and assistance with nursing. In the hospital I had a nurse try to assist me. I hated it. It was much easier with my boys who wanted to eat. They were starving from the beginning of life and have never let up! I nursed both of them for over a year. This time was so different. Elsa needed us to open her mouth for her. Even with milk dripping into her mouth she would not suck. I knew she was too weak to breastfeed. Once the nurse saw how weak and tired she was she told me I was right. My instinct with Elsa was right, yet again. I knew she would need to be bottle fed and I was very okay with that reality.

Every visit to the lactation consultant was dreaded. I knew Elsa needed to be bottle fed for her survival. She didn’t have a voice so I had to be that for her. We tried different holds. We tried a nipple shield. We tried a syringe drip on the nipple. She did nothing. She needed me to facilitate her suck. She needed me to facilitate her swallow. She lacked the coordination and motor control and patterns of suck, swallow, breath. She needed me to teach her how to eat. This was not going to be accomplished by breastfeeding.

After three visits with the lactation consultant and returning home to my husband in tears of frustration and feeling like I failed, I finally became bold. I told nurse I did not want to breastfeed. I told her I wanted to bottle feed Elsa and I was okay with this reality. I would continue to pump due to an overproduction of milk and wean from pumping after a month. I had painful plugged milk ducts every other day. I slept with pillows under my arms due to the pain. I took hot showers with deep painful massage over my breast each night. I would pump every three hours just to relieve the discomfort. I was so tired. It took over an hour to pump and feed Elsa. If not for my husband’s paternity leave I really think I would have had a mental breakdown.

I had eight gallon-sized bags of stored breast milk in the freezer by month one. From frequent travels with Elsa and stress I began to wean from pumping. After using my freezer supply, Elsa began drinking 24 kcal formula. I am so happy and relieved she is growing, not just surviving but thriving.

Breastfeeding is one of those hot topics in current affairs. Maybe because of the politics or maybe because of the push in our culture to be “all natural, clean-eating, and chemical and additive free”. I can’t tell you how ridiculous and frustrating this topic has become for a mother who has breastfed two children and bottle feeds another. We have all forgotten the purpose of breastfeeding. Is it not to sustain an infant’s life? Is it not to give our helpless and fully dependent child the food they need for survival? If one cannot produce breast milk, if one’s child cannot latch with a sufficient suck, what difference does it make if the child is breastfed? The real concern should be giving an infant food for survival. If that nourishment is from formula, then that infant will continue to thrive.

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Sure there is mom guilt. There is pressure from society and I know people look at me with a question in their head when they see a bottle. At this point I have learned to ignore them. I have learned that my daughter is growing and happy no matter what way she puts food in her stomach. I have also learned that I am in a better mental state not pumping daily. I am free throughout my day to enjoy my daughter. I can cuddle and play with her after she eats instead of pumping for 20 minutes. I have time to play superheroes with my sons. I can sleep for a five-hour stretch at night and partner with my husband to feed Elsa.

Not everything about motherhood is natural or instinct. Sometimes things don’t go as you planned. Mothers and infants need to learn from each other. Just as any relationship develops so does the relationship between a mother and infant. It takes time and it takes persistence.

 

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Pink Dinosaurs

One of the best things about church, not only in your home town, but the big Church around the globe is that there are people everywhere praying and supporting you. Angela is one that after moving from Juneau to Anchorage continues to be a great support. She was there in the hospital as David, my husband, left to be with our boys overnight. She helped me feed Elsa. She let me sob in her arms. She let me tell her all the fears and anxieties I felt. She also let us invade her house for 3 weeks but I’ll tell you more about her later. She’s an angel and my very best comrade.

As the nurse came back into my room after taking Elsa for a weight check and more glucose testing, I could see her arms were empty. She carefully told me Elsa was sent to the NICU due to blood glucose levels of 38. She was lethargic, hypotonic (low muscle tone) and needed fluids. I was angry! I was upset I didn’t have her in my arms. I quickly called David and told him to come back.

Don’t forget, I just had major surgery. This was my third c-section and I was familiar with pain medication and the importance of rest after abdominal surgery. With Elsa away I couldn’t think of anything but taking care of her. I politely but anxiously asked the nurse to take the IV off of me and take me to her room. I was informed I would need to be back in my room, a level up from NICU, to receive pain medication every four hours. I didn’t care, I wanted to be with her.

I saw her in her little open air crib bundled and sleeping. Three nurses were in the room quickly putting cardiac leads, pulse ox monitor, and a blood pressure monitor on her body. One nurse was preparing an IV for fluids, another took her blood glucose. After two attempts to find a vein, the charge nurse for the night suggested giving her a bottle of formula. At that time I was slowly producing milk but she needed more. She was too weak to nurse so I pumped every three hours around the clock, syringe feeding her whatever 2-5cc I could pump out. Elsa was able to feed off the bottle with a bit of assistance to swallow. She was still learning coordination of suck, swallow, breath. As she was taking a bottle, the nurse suggested we wait and monitor glucose levels to avoid the IV. Two hours later her glucose was back up to 80! We could stay with syringe feeding breast milk and bottle feeding formula to keep her stable. I didn’t know how amazing this was until after her diagnosis. Most babies with prader-willi (PWS) have NG or G-tubes.

David came into the NICU to help feed Elsa and I went back up to my room for pain medication. My incision was hot. It was hard to move from sit to stand. I could not sleep on the pull out couch in the NICU.  After oral and IV meds, I promptly was pushed back down in a wheelchair to the NICU and sat by her crib praying. Praying she would stabilize, praying she could do this all on her own, praying I could get through it all.

Morning came quickly and I hadn’t slept all day or all night. I continued to pump and feed Elsa. I missed a few pain meds but I was so concerned with Elsa I didn’t feel much. I hadn’t eaten much so David suggested we go to the family room and eat breakfast. I remember crying so hard I could barely catch my breath as he pushed my wheelchair. I didn’t care who could see me. I didn’t care my face was red and swollen from lack of sleep. I was heartbroken.

We ate breakfast, me slowly pushing food in my mouth through sobs. David was so patient. He listened, he cared, he was a rock for me. A woman came into the family room to eat and I tried so hard to hold back tears. I couldn’t. She gently asked if this was my first baby, a question everyone asked me at this point. I couldn’t talk so David did the talking for me. I didn’t know her, I didn’t know her situation or how long her baby was in the NICU. She didn’t know me or my situation either. All she said was that she was in my spot just a few weeks ago. She said it gets better. It gets easier. You learn to help your baby any way you can. It would take me a long time, but she was right.

A few hours later with Angela, the family support coordinator stopped us in the hall. I was still sobbing. She asked what image we wanted outside of Elsa’s room. I thought of her brothers and all I could think of was a pink dinosaur. After going back up to my room for medication David and I walked by Elsa’s room and saw her pink dinosaur. She now has at least one outfit in each size with pink dinosaurs.   20180730_073045.jpg

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Three chickens and a farmer’s wife

You can frequently hear me refer to my children as chickens. Constantly running, jumping, and squawking, my boys are exploring the world with all five senses. My daughter just squeals with eyes wide open. My husband tends his home and office producing a crop of God fearing children and a wife in love with every move he makes. Come journey with me through the ups and downs of this unpredictable life!

“He will tend his flock like a shepherd; he will gather the lambs in his arms; he will carry them in his bosom, and gently lead those that are with young.” Isaiah 40:11ESV

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