grace led in life

It has been such a sweet thing to start to see more and more sunshine here in the PNW...
Springtime is here. Love the fresh air and sweet sunshine...

We've been overwhelmed and a bit out of sorts. Yes, some of that is lack of rest and a lot going on every day, but much of it is also just living out some really heart-wrenching and difficult things that weigh on our minds. It is so challenging to be daily thinking through medical decisions and carrying the weight of your little love struggling with their health and body and also try to make all the little day to day decisions of normal life as well. It often feels like an intense rollercoaster and the ups and downs of it are tiring. There is decision fatigue. We feel ourselves wanting to brace for the next crisis.
There is so much good in each day, too, and it can feel easy to be overcome by the hard things and miss the good things. Life can feel so unsettled and scary, BUT - we see God at work. We see him in so many details. We see him faithfully caring for our family in so many ways and through so many people. He is the solid rock we can trust while everything else feels chaotic and unsettled. 

God protects us and is our source of strength -
2 Samuel 22:2-3a The LORD is my rock, my fortress and my deliverer; my God is my rock in whom I take refuge, my shield and the horn of my salvation, my stronghold...

God is always with us and a comfort and support - 
Psalm 46:1 - God is our refuge and strength, and ever-present help in trouble. 

God is eternal and unchanging, a constant in his promises -
Isaiah 26:4 - Trust in the Lord forever, for the Lord, the Lord himself, is the Rock eternal. 

God is our refuge and ever-faithful - 
He will cover you with his feathers, and under his wings you will find refuge; his faithfulness will be your shield and rampart. 

I have an alarm for every morning that says "praise God and surrender" because I need the daily reminder. God is so faithful, every single day. Amid the hard, there are countless ways that I see him moving in life. It is evidence of his grace and mercy. 
Our hearts feel a bit of a tug-of-war between heartache and hope. It can feel unsettling for sure. And it is in that place that we search for something to cling to, right? Sometimes our circumstances are so heavy that they feel all-consuming, but we have a God that is ready to help us through it, and we can rely on him.

God has been such a constant. He promises to be faithful, and we see that every day.
Life is so hard, but he is so good. Our hearts feel grief, yet we have daily joy from the Lord. 

The past couple of months have been difficult. Our sweet girl has been a warrior, but her health has been so hard on her. We ended our last blog with Isla's start to her BiPap journey. She has been SO brave, and she was wearing her BiPap every night - all night! 

Lamby is on the same care plan as Isla 🤍

A few weeks later, Isla caught a virus. 
This was really hard on her and also very scary.
With her airway complexities, she cannot clear congestion and it will cause her to choke and not be able to breathe well. Her lungs are also high-risk, and her cough is very weak.  We had to call an ambulance a few nights into this virus, and head to the hospital for about 5 days. During her hospital stay, we realized Isla could not wear her BiPap while dealing with congestion - it was causing aspiration and choking for her, as well as causing her to swallow a lot of air, which was so painful. We advocated hard for her as we could see the BiPap was causing more issue than help. There is such a fine line in helping her breathing and healing from the virus and also trying to help her breathing from the decline of her medical condition and trying to keep her Co2 numbers lower. Hospital stays are so hard for Isla. She can't fully understand what is happening with her body, why she is at the hospital and all that is happening to her there. It is very scary for her. PTSD flares up. Thankfully, after about 5 days, she was showing enough improvement to head home and continue all the treatments at home. We are SO grateful for her team of doctors and for the equipment that we have to use at home (we don't take that for granted!!! Getting medical equipment approved and at home is not easy... so thankful we have what we do for our girl!) This virus was hard on Isla, and it was a slow recovery. We see the decline in her thoracic insufficiency as she struggled more than she has in the past with getting sick. 

Isla has continued to have daily fevers even while recovered from the virus. For the past month, we were noticing her hip having some more pain than usual, and by last week, she was starting to limp and not want to walk. We took her into the orthopedics urgent care at Children's and after x-rays, labs, and an ultrasound, we found out that Isla hip has fluid around it. We have her on anti-inflammatory meds and we have an appointment with an orthopedic hip specialist in a couple of days. We are hoping it is just temporary, possibly just from the virus, and that it will resolve soon. She lives with a lot of pain in her back, hips, shoulders, etc...so we need to get it looked into more. She is so strong! 

Isla has been slowly working on wearing her BiPap again. She has been quite nervous after the experiences with it in the hospital. Those scary struggles for her brought on a lot of anxiety with wearing it. Once fully better from the virus, she started with time on the BiPap machine during the day and now has been wearing the BiPap at night for part of the night. 
Her Co2 numbers have still been high but not trending higher than they have been in the past few lab checks. Isla was going to be having a BiPap titrating sleep study the day that she came down with the virus, so that has now been rescheduled for April 19th. Isla's pulmonologist in Philadelphia is fairly certain that she will need to be on settings double of what she is on now on, to try to maintain the breathing that she currently has. 
We are praying that Isla stays healthy for this upcoming sleep study, handles it miraculously well, and that we can gain very clear results to know exactly where her BiPap machine levels need to be to maintain her current breathing and how much of the day or night she is needing to wear it. We need this clarity. 

Despite all the health challenges, Isla still pursues each day with determination :) We have loved getting our warrior girl and fam out in the sun when we can...fresh air and vitamin D! Isla cannot always communicate her pain, but at times, she is able to say what hurts and communicate "too tired." We take many breaks through the day, but she doesn't want any of the challenges to fully stop her. She struggles with limitations. Her determination is really incredible, and I think to many people, she may seem "ok" or "better", but she is a warrior who is powering through her body doing a lot to try to stop her. We pray for her excitement for each day to not stop... for her determination to not waver. 

We loved celebrating Easter together and we are so grateful Isla could do a little of our tradition of painting windows. She still wanted to dye eggs for Easter and also do our tradition of an egg hunt. We were able to have a sweet backyard Easter lunch, and it was just such a blessed time. Precious family time! Isla still had a fever but was so happy to celebrate! Celebrating in life is one of our favorite things! 

Happy Easter! He is Risen!

Thank you for praying for Isla. She is currently at home, but here is a little synopsis of what has happened this week. (If you are needing more clarity into what is going on with Isla's medical needs as a whole, please read the blog prior to this one) 

We took Isla to the PICU on Monday (Feb 9) because her blood gas level (which shows CO2 retention) was so high. However, when we got there, she had bloodwork done again and her CO2 number was lower. Those results generally do not fluctuate that much within a couple of days. 
We had hoped that was good news...

Due to the complexity of Isla's needs, the Seattle Children's team wanted to be very thorough in testing, imaging, and communication prior to starting Isla on BiPap. 
We were also communicating with her team at Children's in Philadelphia. 
They all agreed that Isla's symptoms and test results are proving a decline with her thoracic insufficiency. We feel very sad. 

Isla had a CT and a "cinematography CT" to see her airway and lungs and to be able to watch her breathing through the CT. Her sleep study done in December showed her complex airway is causing moderate obstructive apnea. Her airway has many angles in it. Surgical intervention has not been an option so far. We will be in discussion with Isla's aerodigestive ENT here in Seattle to see if they have any insight to offer for her airway needs. 

The CT also showed that Isla's little lungs are not filling all the way when she breathes in. Her breathing is shallow, which also leads to her holding onto too much Co2 when breathing out.
She is still experiencing times of desaturation with her blood oxygen and times of tachycardia with her heart rate. If she is not at rest, this happens very easily now. 
The holding of Co2 is of high concern and causing her episodes of weakness, fatigue, some lethargy, etc. 

Isla was so brave! She held so still for so long!

Isla was started on BiPap Tuesday night. Unfortunately, there was an error with respiratory therapy that we were not aware of, and the machine used was not the one the doctors wanted Isla on for specific reasons according to what she is needing. There was also issue with her breathing not being in sync with the machine. 

Practice wearing the BiPap masks to decide which one was most comfortable (Tuesday)

Stella dolly had a BiPap breathing mask, too. (Tuesday night)

ISLA WAS A WARRIOR and even though she was so scared and not fully understanding what was happening she asked for a "timer for morning" (to help her feel there was an end point) and boldly wore the BiPap ALL NIGHT LONG. There were constantly times of the alarm going off and while she felt so triggered by it, she continued to wear the BiPap and try to sleep. WE ARE SO PROUD OF HER! 

She had more labs done right away in the morning. We were sad to see that the Co2 was higher again and it was disappointing to find out about the incorrect machine being used. 

Isla woke up on Wednesday very tired - we could tell she was not just physically tired, but struggling mentally and emotionally, too. She was trying so hard to function while her brain was hitting fight or flight. We went outside for a walk in the sunshine; she loved it so much. But, as we came back in, her heart sunk and she was so scared. Medical trauma and exhaustion are so real. By early evening she hit her limit. 

sunshine and beautiful flowers! Such a gift from God!

Isla's medical team has been so kind and caring. They have been thoughtful and put all of Isla's needs as priority. They know how hard this is for her not only physically, but mentally and emotionally as well. The team really respected us as a medical family and were very open to us being a driving force in decisions. We advocated and they really listened. Being inpatient is really difficult for Isla. The plan was to stay inpatient, but with seeing how Isla was struggling and unable to process all that was happening, we knew that another night, starting the BiPap again, was going to be even harder and possibly create more trauma surrounding it. She is going to need this breathing support moving forward. This is going to become a daily needed thing. We did not want it to become even scarier and create a negative experience if we could help it. 
We, along with her medical team, decided to have Isla go home to feel more safe and secure. We are working with her team outpatient, and of course, if Isla gets worse in any way, we will head back to the hospital. 
Her labs were not the worst they could be, but not great either. It is a gray area/fine line...
But she was really struggling mentally and emotionally too. 

It is really difficult to make these decisions. We were told that a machine was being ordered, the team was discussing the best levels for Isla to start with at home, we will be working with a team in the sleep center to help Isla acclimate to the BiPap and to do a titrating sleep study, and if she shows any signs of getting worse, we will be back at the hospital.

Little ray of a rainbow showing up during a hard morning  remembering God is faithful in all his promises.

As soon as we walked out the doors of the hospital, Isla broke down. Big tears. The weight of not feeling well along with the heaviness that it brings mentally and emotionally is so deeply difficult. Having a rare syndrome and medical needs is chronic... it is life-long. The difficulties don't stop. Not being able to understand it all is scary. It's exhausting. She is truly a warrior. 

We've had communication with multiple doctors since arriving home Wednesday night. Unfortunately, nothing happens fast... so we are praying that Isla's machine will arrive very soon. 
We will be going in for labs again next week to check Co2 levels. We will be talking to Isla's surgeon in Philadelphia again this next week as well. 
Her surgical team in Philadelphia had called to schedule her next surgery, but planning for that is on hold for now as we help Isla's breathing and figure out all the complexities going on with her health - we need it to be safe to travel and do another surgery. 

Isla's body is tired. Things are shifting. Her brain still wants to go, go, go, but her body is saying much different. So hard for her to understand. We are limiting her activity and are anxious to get her going on her BiPap each night. We pray that her body will be in sync with the machine, that she will gain such good breathing help with it, that she will not have adverse side effects from it, and that her Co2 numbers decrease and don't cause any issues in her body. 

We are taking things a day at a time and watching her close. 

Please keep praying for Isla. Please pray for us as we communicate with so many doctors and make more decisions for our warrior girl. This is hard. We are struggling. It hurts so much to see your child suffering. We are holding on to Jesus - His faithfulness never fails - it is through him that we have HOPE. He is our comfort and refuge.