Over joyed!

Fall is in full swing. I am so glad that we are home to experience Fall in Bend. Although summer went by too fast, I am looking forward to the holidays. There is something about the crisp air, and fallen leaves that brings me joy. Although we were only gone for a few weeks, driving up to our house was a wonderful feeling. Samuel jumped out and said " I love my house"! Natalie was equally happy kicking her legs, and clapping. There is nothing more fulfilling than seeing your kids light up when they are home. Michael and I are overjoyed to be back with the kids. It's still hard to believe Natalie was discharged just 6 days after her surgery. This is nothing short of a miracle. God heard our prayers, and gave our family mercy. He protected Natalie every moment!

The weeks and days leading up to Natalie's surgery was incredibly hard. I feared losing Natalie. I couldn't imagine a day without her. It would have been easy to allow fear and worry consume every thought, but God's peace and guidance was our source of hope. God gave us the strength, wisdom and peace to know he ultimately is in control. As I write this I am reminded of all the other sick children right now fighting for their lives. Some of them waiting for organ transplants, going through chemo, struggling to breathe, and struggling with a multitude of health issues. This last week the CHARGE community lost two precious CHARGE babies. I can't imagine the pain and sorrow ripping through these families lives this very moment. I am not sure why some kids survive and others don't. It breaks my heart, and reminds me to hold dear to every moment with my precious children. In a moment it can be gone. Please take the time to love on the families you know with sick children. I can't tell you how much the support Michael and I have received has helped our family.

The first 48 hours after Natalie's surgery seemed to take forever. Thank goodness we took her to Lucile Packard. Dr. Reddy did yet another "first time" repair on her. She needed a patch to fix her pulmonary valve, however because her anatomy is not typical he couldn't place a patch in the typical place.  Dr. Reddy managed to go through the back of heart and place a patch. This repair confirmed that if we had not gone to Lucile Packard for her birth and first repair she would have ended up with a one-ventricle repair (half of a functioning heart).  Dr. Reddy has given Natalie a normal functioning heart. Although it is never a guarantee with a heart repair, I am hopeful she will not need another heart surgery for many years. The patch could last anywhere between 5-30 years. Dr. Reddy is a miracle worker, and my hero.

Of course Natalie's strong will and determination brought her through this surgery. After her first extubation Natalie's airway began to swell immediately. Michael and I watched as the doctor's decided what the next course of action was going to be. The odd thing is that Natalie managed to still have almost perfect oxygenation with just a small airway! The resilience and strength this girl has amazes me. After she was reintubated, Natalie went on steroids to help with the airway swelling. Of course all interventions end up with further interventions. The steroids caused a weekend immune system, which led to a tracheal infection. Luckily the infection was caught quickly, and she was given antibiotics. She bounced back quickly, and was ready to leave the CVICU. Did I mention my little hero only needed pain medication for the first three days! Spending just a few days in the step down unit we were sent home! What a different experience compared to the first time.

Now that we are back we are simply slowly getting back into a routine. Natalie has a cold, so we are watching her closely. Our primary care doctor and cardiologist could not be more happy with Natalie's health. She will continue to see the cardiologist for the next few months, and than hopefully every 6 months.

Michael and I have been encouraged from the support of all of our family and friends. It's not easy, yet somehow knowing we have so many amazing people loving our family makes these incredibly hard journeys a little easier. Thank you for everything!

Love,

The Pierce Family

Stanford bound

What a busy month around the Pierce household. Natalie is set to get heart surgery on September 15th. We have known she would need it for awhile now, but it still seems as though we are not ready. Maybe we will never be ready to take her. Giving her over to the surgical team is going to be gut wrenching, yet we know she needs the surgery. Last week she had one more echocardiogram, which showed an enlarged left ventricle. The plan is to either use a patch to fix her valve, or use a conduit. A patch is much more favorable as it will allow more time between her future repairs. A conduit will guarantee more repairs throughout her life.

She is bubbly, and as sweet as ever. Sometimes we can't get enough of her. She must be kissed a million times in a day. Her little toddler attitude is coming out, and we just love how demanding she is. Natalie is all about play, and is understanding simple instructions. She can clap when you ask, and is starting to give high fives. Signing is a slow process, but we are hopeful it will become more natural for all of us. Samuel seems to pick it up the fastest.

The next few months are going to be difficult, but we hold on to our hope in God. He has given us such an amazing daughter. We don't take a day for granted, and believe in his power. Please pray with us for wisdom and strength, and health for our princess. Pray that Samuel adapts quickly to his new routine, and Michael and I get the rest we need.Thank you for everyone who has supported and encouraged us. Natalie is such a joy and blessing to many. We will try to keep updates on the blog.

Love,

The Pierce Family

Summer fun

The last few months have been wonderful. Natalie turned one, and we had one amazing birthday party. It was such a huge celebration for us. A year ago today Natalie was discharged from the Lucile Packard Children's Hospital. I can't tell you how proud we are of our little girl. She has overcome so many challenges, and she still has the biggest smile on her face.

I am not even sure where to begin. She is doing really well. Natalie is almost 20lbs, which puts her in the 40th percentile. She says Dada, Mom, and a few other sounds. She rolls all over now, and stands assisted. These are huge milestones for her. As long as she continues to show such strong determination the sky is the limit.  She still does not love tummy time, but with incentive she will do it. Hopefully she will get herself into the crawl position soon. She is very determined and manages to find ways to get things she wants. She loves to dance and listen to music. She is even starting to sign a little. She is a Daddy's girl too. Michael adores every moment, and treats her like a princess. Samuel is still the best big brother we could ask for. He is all about helping baby sister now. He is asking more questions about Natalie's "boo boos", and I think he is understanding more about her impairments.

She failed her last swallow study, however we have decided to continue to feed her. She does great on thick puree, and wants to eat. She likes most food, and especially chocolate and whip cream! I hope to continue weaning her off the G-tube in the Fall.

The sad news is that were are heading back to California for another heart surgery. It's never going to be easy for us. Of course I keep thinking she won't ever need the surgery, but it's obvious her little heart can only tolerate so much. It's a good thing that we are going before flu season begins. Natalie is still not showing signs of heart failure that are obvious to us, however, the doctor's think her stamina should improve greatly. It's undecided what procedure will be done. There are few options, one of which will not require further surgeries. Michael and I are trusting her team of doctors. I remind myself that they have all done these procedures a thousand times, and know exactly what they are doing. I am not anticipating a long recovery, however, we are preparing to stay for an extended time.

Natalie is much more healthy, and we know how to manage her care. I hope we know all of her diagnosis, and have no major surprises.

Our summer has been full of wonderful memories. Natalie loves going on walks and to the park. It's sad summer is coming to an end, but we have taken advantage of all the fun Bend has to offer.

Thank you everyone for your support and encouragement. I know Natalie is such a joy to many. She is our inspiration and strength. I think about how much I love my baby girl, and I am reminded of God's love for his children. God's love is unfathomable to me, and I am ever so grateful for his kindness and grace. Many of you may ask how we are doing and how we cope. Our source of strength is from God. As we prepare to go to California, know that Michael and I believe God is faithful, and will guide us.

Love,

The Pierce Family 




 

Time is just flying by. Where did this year go? If I could stop time and hold on to Natalie's last month as baby I would. Of course I want her to catch up to her milestones, but I am quickly learning to stop wishing time would pass, and hold on to the moment. Her sweetness and precious giggles leave me speechless. I can't tell you how sweet her laugh is, or  how amazing it is to watch her play with Samuel. She loves her big brother more than anything. She is such a happy baby, and loves going out with the family. Being able to take her out more often has made such a difference for her and the whole family. Summer is here and we intend to live it up while it lasts.

So lately I have been asked a lot of questions about her delays. She is delayed, and is on her own clock and will achieve things as she can. She is determined and very smart. She takes in so much and nothing gets by her. I think her sensory impairments make her more in tune with her environment. She watches everything, and studies her environment intently. Natalie can sit unassisted for a minute, and she occasionally rolls to her tummy. Usually it's to see big brother or a favorite toy. She loves music, bath time, and going for walks.

Heart wise she is doing good. Our last echo showed the same results as the previous ones. Although it is still bad, she seems to be doing well. As she becomes more active, she will put more strain on her heart.  Her Surgeon and Cardiologist plan to wait until she shows signs of heart failure before surgery. Michael and I anticipate surgery before late fall, or next spring. Of course we could be wrong and it may be a lot longer or next week. It's really up to Natalie, but I don't see Dr. Reddy waiting much longer, and would prefer repairing her heart before Flu season kicks up again. Her last few monitor observations showed elevated QT levels (this could cause heart failure) so she is currently being weaned off her arrhythmia medication. The original plan was to keep Natalie on arrhythmia meds till she was two, but now the doctors are thinking it may be best to get her off them. Hopefully this works because there are just a few more drugs that she could go on.

It's confirmed that she has profound hearing loss in her left ear. Our doctor's don't believe a hearing aid will make that great of a difference for her left ear. The plan is to purchase a softband BAHA so she can get as much sound to her right ear. They are very expensive, so hopefully our insurance will cover most of it. Her right ear is still within the normal range. The plan is to do hearing tests every few months to track any changes. CHARGE kids experience various degrees of hearing loss, which can also change over time. Natalie's hearing loss is a combination of conductive and neurological, so the trick is going to be treating the conductive loss as much as we can.

Our Opthamologist confirmed that Natalie has a malformed optic nerve in her left eye. Although her MRI showed no optic nerve Coloboma, it is obvious that her eye definitely works harder to focus.  It's likely she will start wearing glasses in the Fall. Her right eye seems to be 100% normal. Patching seems to be impossible, as she always finds away to pull off the patch.

She still sees all the usual therapists. They all love her of course, and are amazed how much she is improving. Unfortunately, Early Intervention (EI) stops through the summer. Our Physical Therapist through EI is amazing, and has been instrumental in her growth and development. Natalie is so blessed to have experienced and caring therapists working with her. 

Her head shape is improving a lot. Yay!. So grateful the helmet is helping. It's not fun traveling to Portland every other week to get the helmet adjusted, but at least it's improving her plagiocephaly. She will wear the helmet for a few more months at least.

Now for the biggest change...She is being weaned off of her G-tube. At first I was so nervous, but after 5 weeks of weaning, she is eating all of her meals during the day orally. Drinking is still challenging, but she is improving day by day. Over the last two weeks she gained 5oz. Go Natalie Go. She is our little rock star. If I have learned anything this past year is to trust your instinct, and pray. Pray and believe that God is faithful, and through him all things are possible. Natalie is doing so much better in this area than anyone would have thought. Of course we know CHARGERs defy the odds. I am still hoping by her first birthday she be off her G-tube completely.

Life at the Pierce household is ever so exciting. Terrible 3s are at the height. Raising a busy three year old keeps us busy. Samuel just started preschool for some much needed time away from mommy and daddy. It's been really good for him to be around other kids,  and mommy and Natalie get to go to appointments by ourselves.

There are no words to describe how blessed we are. A year ago we had no idea what to expect with Natalie. We didn't know if she would even come home from the hospital. I wish that I could see into the future and know for sure that my baby girl is going to be fine. I guess this is why I have faith, to believe in what is not seen, and asking fervently for God to make Natalie whole. I don't see CHARGE syndrome and all the crazy medical issues that come all with diagnosis. I see a beautiful baby girl made in God's image. It's still hard to believe God gave her to us. What a gift, and privilege it is to be her mommy. 

Blessings,
The Pierce Family 



  

Joy beyond measure

My heart is full. My family is growing strong, and our little girl continues to defy the odds. I am hesitant to be excited, but I am rejoicing during the good times. Life is constantly crazy, and it never feels like it's going to end. I am not sure even sure what it was like before two children. All I know is I wouldn't trade our life for anything. Yes the future is unknown, but we put our hope in God. He is faithful, and so merciful. Michael and I are beyond ready to be involved in the church community. Samuel is thriving, and adores Natalie. 

We are still doing all the usual doctor and therapy visits. We are starting vision therapy this month. Natalie is extremely lucky to have a such a great team of doctors working with her. They fight for everything she needs and more. Last month we have a team of nurses who went out of their way to make sure Natalie got her RSV shot. Words do not describe how thankful we are. She is loved, and one of the nurses always says she is Natalie Peoples... (that is the last name of her doctor). It's great knowing that Michael and I can trust their advice and direction, and that they all truly care for her.

Our trip to Portland went well. We managed to coordinate a CT, MRI, ear tubes, lower airway scope, and a sedated echocardiogram.,..and they said it couldn't be done! Things do happen when you are persistent. I am glad that Natalie shouldn't need any other procedure in the near future, outside of heart surgery. We gathered a ton of information, most of which confirmed what we already expected and knew.

Her CT showed that she does not have semi circular canals in both ears. She has chonal atresia, which will be repaired this year.

Her MRI provided a lot of valuable information about her cranial nerve abnormalities. I am not not sure how accurate the scan was, but in a nut shell the results are great. Her brain is generally normal in morphology, and signal intensity...She basically has a normal range of white matter. There is no fluid collection, or masses, and she does not have hydrocephalus that was originally diagnosed. With CHARGE syndrome there are numerous cranial nerve abnormalities, and Natalie definitely has the vast majority of them. She does not have her olfactory bulbs, no left cochlear nerves, her facial nerves, and nerves 9, 10, and 11  are  hypoplastic. She is also missing her semicircular canals (her balance organ) in both ears. I am sure many of you are asking what all of this means. Like so many CHARGE kids they overcome so many difficulties. Things that we take for granted, like walking, eating and drinking, smiling, and talking are more challenging for CHARGE kids. The good news is that they find ways to compensate. Natalie is already showing us that she is willing, and wanting to experience life at its fullest.

We recently did another hearing test. According to the MRI Natalie shouldn't be able to hear out of her left ear, but she has had responses on her ABRs. Basically, we have no idea if she may actually have a small nerve that was not seen on the MRI. . Her hearing out of her right is normal. She will continue to wear her BAHA until we have more information about her left ear. She may not need a hearing device if there is she does not hear out of her left ear. Kids with CHARGE often experience varying degrees of hearing loss throughout life. It is a constant management of their conductive hearing loss. Knowing that she can hear us is such a joy. We will continue to do more hearing tests, and decide in a few months what Natalie will require long term. Michael, and I strongly believe that it is in Natalie's best interest to learn sign as a second language. We hope that she will use both spoken and sign language.

Did I mention she is so cute in her pink helmet. She is tolerating so well, although she has not slept in it yet.  We will get her up too 23 hours eventually!.

She loves yogurt, and is slowly improving on eating. She is showing anticipation for food, which is a huge difference than the previous months. She is showing us what she can tolerate. In time she will be a fully oral fed baby!

Spring is near

It's almost Spring time! I can smell it in the air. After an extremely long winter, we are all ready to spend time outdoors. We all have cabin fever, and can't wait to take the kids out to the parks, pool, and take a mini family vacation. That's right...VACATION. We so need some fun time. I got the o.k from the doctor as long as Natalie is healthy. We don't plan to go far, but I will take whatever we can get. Maybe a trip to the Oregon Coast is around the corner.

Where to begin....Natalie is getting so big, with a whopping 17lbs. I just love her chunky cheeks, belly, and legs. If she only realized how adorable she is. She is catching up to many milestones. Although her head bobbles, and she is not totally sturdy, she can sit unassisted for a bit. She reaches for almost everything, and just adores her big brother. She smiles mostly at him, and occasionally at Michael and I. She is definitely a Mommas girl. She reaches out for me to pick her up all the time, and cries if I leave the room. Yes I hope the phase doesn't last forever, but right now I am relishing in the baby love.

Can you believe she is 8 months old? She has been home for 6 months, and so far we haven't had any major hospitalizations. Just another month and the Flu and RSV season will be done. RSV is at the height right now in Bend, so we are taking extra precautions. We're currently fighting to get her last RSV shot. It seems that our Oregon Health Plan is more of  pain than a help. Providers are very reluctant to give Natalie service. It took over a month to finally get a helmet for her. We will have it fitted next week. It's pink, and I of course we will get some cute stickers for it. 

We are only doing an Echocardiogram every other month. Yay! We are watching her heart closely. She is not showing any signs of heart failure, and is thriving. For now we are rejoicing, and are so grateful that she is getting stronger.  Please know that it can be anytime. Heart failure usually occurs quickly. It can be weeks, months, or even a year. It's still very difficult to say.  As long as Natalie continues to grow and get stronger we are in better position for her next surgery. 

We leave for Portland next week. Natalie is getting tubes placed in both ears to elevate fluid build up. I am hoping that her hearing improves in both ears, and she will feel better. Her left ear has had a constant effusion since she was one month old. She has been treated twice for ear infections as well. We are also trying to schedule an MRI. It's challenging to due multiple procedures because Natalie requires a special Anesthesiologist. Please pray that her procedure goes well, and she comes off sedation fine.

We are attempting our first g-tube weaning trial. I have no idea how successful we are going to be, but I my gut feeling is that we must try. She is not nearly as interested in food, so I want to capitalize on any remaining interest that she has. Her stamina and coordination has improved greatly, so we are going to see what she can do. The most challenging part is going to be teaching Natalie to eat when she is hungry. She has never really experienced hunger. Our plan is to reduce her nightly feed so she wake up hungry. Of course we can only push her so much, but I am confident that she can do it. If she loses more than 10% of her weight we will stop and try again the following month. I am both nervous and excited. It's going to be hard to let her be hungry, but it's the best thing for her long-term.
We also are going to be doing even more therapies. We are going to begin vision therapy, and do outpatient physical therapy at the hospital. It's going to make us even more busy, but she really needs more care. We are wanting to focus on building her core muscle strength and improve her head control.  The extent of Natalie's vision loss is still not known. She is compensating to see to her left, but we are noticing her left eye is slower to focus. I am looking forward to working with a vision therapist to see how we can improve and strengthen her left eye. Good things take time...

We are going back to church next month! I have never gone this long without being in a church community. I am grateful that we God has kept us reasonably healthy but I sure do miss the fellowship of other believers.

Please pray that we remain healthy this month, and Natalie will get her RSV shot. Also that her tube surgery goes well next week. We will be gone from 17-20th.

Love The Pierce Family

Heart Healthy

Our little girl's heart is so complicated. I am so grateful for her repair, but it is making it difficult to read her echocardiograms. Typically, velocity is measured to calculate a gradient, which illustrates the severity of Pulmonary Stenosis. For whatever reason, Natalie's gradient calculations range greatly, making it difficult to get an accurate assessment. Dr. Reddy and our cardiologist decided it would be best to measure her right ventricle output, as these measurements are more consistent with her Cath. They are also watching to make sure her valves are not leaky. To say the least I am confused, and we are still waiting. We will do echo's monthly, and likely a sedated one in the coming months. It sounds as though we may be able to put off surgery for awhile, but you never know. Things change quickly. We're  also meeting with our electrophysiologist next week. We will be discussing her arrhythmia's medications, treatment, etc.

She is battling a cold right now. She is strong, and managing fine. It does seem like we are suctioning constantly! She started antibiotics today to clear up another ear infection. Hopefully she will be feeling better soon.

Did I mention she is a whopping 16lbs...My goodness. My little chunky girl. Although she doesn't go out much she still wears all of her adorable outfits, shoes, bows, and hats! She is dressed better than most.

I can already see sibling rivalry. She takes Samuel's toys, and Samuel thinks everything is his. Of course she can't fight back yet, but I know one of these days she will.

Michael and I are in full swing, and managing well. We found out that she didn't qualify for Developmental Disabilities. All I can say is they said no to the wrong family. I will apply, and reapply, and reapply until she gets it. She will really benefit from it, and I am determined for her to get it. There reasoning is terrible, and completely incorrect. They said that although she has CHARGE, it doesn't mean she is cognitively delayed. Ironically enough she qualified for OHP, the Oregon Health Plan. What a relief and answer to prayer. Many of our costs, like hearing aids, co pays, and prescriptions should be covered now. I am grateful that God continues to equip us with the skills and means to take of her.

We continue to move forward and are even starting to talk about future plans. Did I just say future plans. Yes I did! It feels good to plan, and dream. Although Natalie still needs to spend a lot of time indoors, we are excited to get Samuel involved in some activities. I am also planning on volunteering for the Red Cross again.

Today I got a package from the kids at Lava Ridge Elementary, where she is a Sparrow. All I can say is how sweet kids are. Raising a special needs child is frightening, especially knowing that her peers may not accept her. These kids have already accepted her as their friend. I am in awe, and look forward to her school days. And hey if kids do tease her she has her big brother to look at for her!

She had her first belly laugh. These moments are priceless, and I cherish each of them.Watching her develop, and make milestones is a big deal. She is persistent, and as sweet as sweet can be.

She loves sucking on mango and apple slices. We're still working on tasting foods. I realized that as long as we consistent and give her opportunities to try food in a fun manor she will eventually consume enough. I was imagining that she would consume her daily calorie intake via rice cereal, and purees...and than I remembered she is only 7 months old and it she will when she is ready. I have no doubt that she will be an oral eater.

We're planning on tubes, and another MRI in the next few months. As we know more I will keep you posted. For now enjoy these moments of celebration with us.

Blessings,
The Pierce Family 

Getting so big!

I might be the worst person to ever do a blog. I always plan on updating, but time escapes me these days. I often wonder how parents with 2+ kids can do it, or twins...yikes. Some weeks are more difficult than others. I can't imagine what Natalie feels like, going from one appointment to the next. Our new "normal" has become a routine now, and we seem to be getting the hang of everything. Most of the clinics know us by first name basis, and accommodate our needs. Natalie has seen a ton of doctors, and they have a really good idea of her symptoms and future interventions. I am so grateful for our doctors in Bend, Portland, and Palo Alto. It makes it so much easier knowing that Natalie is in good hands. I know many of them have a special interest in her, and watch her closely.

We are in holding pattern for now. It could be anytime when we go back to Palo Alto for her next open heart surgery. She is not in any obvious heart failure and growing strong. I'm hopeful that she will continue to grow and get strong before we go back. We are also waiting to hear back about getting Natalie a helmet. It seems intimidating to commit to it, but if our insurance will cover some of it we will likely move forward and get her one. She will have to wear it 23 hours a day for 3 to 4 months. Between wearing her BAHA, eye patch, and helmet she wont have any time without something attached to her.

I wish I could describe how cute she is. Her voice quality is improving so much. She isn't cooing as much as we would like, but each day she is vocalizing more and more. She loves to watch Samuel run around. He is becoming more and more interested in playing with her. He likes to show her all of his cars, trains and planes. He still has moments of jealousy, but it's obvious he adores her to pieces. He constantly says " I love my baby", and "my baby is alright".  She is rolling side to side, and is still working on her head control. She doesn't mind tummy time and reaches out for toys. Feeding is still a slow process, but we are making progress. She can handle a tablespoon per feed. We are hoping to get her to 2 tablespoons twice a day before her next swallow study, which will probably be late next month. I don't want to rush into a swallow study unless I know she is can pass it.

I am constantly reminded of how blessed we are. She is our little miracle baby. I laugh when therapists and doctors are so surprised to see her progressing as much as she is. I just say that is my girl, and you watch! There is nothing this little one can't accomplish!

The power of prayer and faith keeps us going. I don't know what the Lord plans for this little one, but I do know he has his hands are on her. I am excited to see God mightily at work with her, and our family. There are many moments where I question God, and the craziness of our lives, but after each day passes I reflect and have such peace and assurance that God is in control. He knows Natalie better than anyone else and is giving us the wisdom and skills to take of her. What a privilege it is to take care of her, and Samuel.

I know so many of you wonder how we are doing, and managing everything. To be honest we are tired, and overwhelmed, but keep on going knowing that we have so many supportive family and friends helping us. It's truly incredible, and I know we simply could not take care of Natalie without the support from you! Thank you a million times for your thoughts, prayers, encouragement.

Love,

The Pierce Family