Tuesday, April 27, 2010

Blessings and Curses

Yesterday in Cincinnati Dr. Kerry Crone, the neurosurgeon at Cincinnati Children's Hospital, flashed this image up on the large screen in his office, and I was stunned. We went on our trip knowing we might find out even more bad news because Dr. Crone suspected Danica might have a soft tissue condition of some kind based on things he read in her post-op report.  It was the 3D CT that painted this startling picture of our Danica's skull base and cervical junction. Something is horribly wrong.

I cried most of the long drive home yesterday as Danica screamed and thrashed in her seat from the negative effects of the nebutol. I hadn't eaten all day and was just shocked. Dan was focusing on driving in the pouring rain and traffic. We didn't really talk. We just had to process this newest thing. When I got home I had to work. I know it sounds crazy, but I am forever grateful for my telecommuting job that enables me to be here most of the time and flexible enough to provide and also care for all these medical appoinments, etc. Somewhere deep inside work has always been a good compass for me. It's something that keeps me going through the motions and doing the next thing. So, I struggled with Danica and finally got that exhausted baby aleep. Dan was already sleeping, curled up with his clothes still on, and I went downstairs and started to work.

I had written these words on a notebook during our meeting with Dr. Crone, and they kept staring at me while I ran lists:

Congenital Assimilation of C1 to the occiput

Open the dura, coagulate the tonsil, put in a patch

Fusion of the occiput to the C3

Rods and screws

Bone graft

Best alignment possible

8+ hour surgery, 14+ days hospitalization, halo cast 3-4 months

Limited rotation

I went on to write all the risks of each procedure which I won't share here and now. In a moment what was a consult about a scary second brain surgery became much more.

Tomorrow my dad will drive me to Cleveland to get the sum of Danica's paperwork and every scan she has had and we will send to Dr. Carson at John Hopkins. Today I sent an email to his PA letting her know we are now needing opinion on not just the brain surgery but also the spinal fusion based on the new CT. Dr. Crone kept using the word, "unstable" to describe Danica's current condition. He said a simple fall down the stairs or a car accident could leave her paralyzed. We need to move quickly but carefully these next weeks. We need wisdom to make the absolute best decision for our Danica.

Last night I screamed and cried and pitched a fit. I said over and over how I just can't do this. After I couldn't see straight I took an ativan and an ambien and fell asleep. Today the sunshine and the perspective from the rest and my Father drew me back to the truth. God makes no mistakes. He is good even when life is bad.

If you know me well I have probably gifted you the book "Cold Tangerines" by Shauna Neiquist or have quoted some of it to you in an email or a letter. The way Shauna writes is the way I think and journal and pray and try to live most of the time. In one of my favorite chapters she writes this,

"When you realize that the story of your life could be told a thousand different ways, that you could tell it over and over as a tragedy, but you choose to call it epic, that’s when you start to learn what celebration is. When you see in front of you is so far outside of what you dreamed, but you have the belief, the boldness, the courage to call it beautiful instead of calling it wrong, that’s celebration. When you can invest yourself deeply and unremittingly in the life that surrounds you instead of declaring yourself out of the game once and for all, because what’s happened to you is too bad, too deep, too ugly for anyone to expect you to move on from, that’s that good, rich place. That’s the place where the things that looked for all intents and purposes like curses start to stand up and shimmer and dance, and you realize with a gasp that they may have been blessings all along. Or maybe not. Maybe they were curses, in fact, but the force of your belief and your hope and your desperate love for life as it is actually unfolding, has brought a blessing from a curse, like water from a stone, like life from a tomb, like the actual story of God over and over. I would never try to tell you that every bad thing is a really good thing, just waiting to be gazed at with pretty new eyes, just waiting to be shined up and- ta-da!........there is something just past the heartbreak, just past the curse, just past the despair, and that thing is beautiful. You don’t want it to be beautiful, at first. You want to stay in the pain and blackness because it feels familiar, and because you’re not done feeling victimized and smashed up. But one day you’ll wake up surprised and humbled staring at something you thought for sure was a curse and has revealed itself to be a blessing-a beautiful, delicate blessing."

This is the story of God played over and over in our lives--in Danica's life. He continues to give life and strength for today and shining little miracles that light our way through even the darkest valleys. After Danica was born a verse I clung to was from Job 42:5, "I have heard of you by hearing of the ear but now my eye sees you." He is giving us healing of the eyes yet again to see the blessings through the curses in this journey.

Thank you for continuing to pray. Thank you for caring for us so well. Your support is part of what strengthens us when we are too tired and weak.

(Check my facebook for adorable pictures of Danica from her first hotel stay. Priceless.)

Friday, April 23, 2010

Heading to Cincinnati

Just a quick update to let you know we are heading to Cincinnati on Sunday night for a full day Monday to include a 3D CT scan of Danica's skull base and cranial junction (under sedation), a meeting with a team of geneticists and a meeting with Dr. Kerry Crone, the chief neurosurgeon at CCH. After reviewing Danica's scans and the op report from her November surgery he would like to explore the possibility she may have a genetic soft tissue disorder in addition to her Chiari. Because of a strange bone anomaly Dr. Cohen found and removed in her first surgery and her main presentation being tortocollis Dr. Crone believes it is prudent to perform these other tests prior to making any call on how a second decompression would be performed.

We also heard from Dr. Herbert Fuchs at Duke on Wednesday. He seemed very confident that if we open the dura, put in a dural graft and coagulate the tonsil there would be no more reason to remove any additional bone.

Based on three differing opinions and surgery methods we plan to seek a fourth opinion from Dr. Ben Carson at John Hopkins following these tests in Cincinnati. My sister in law's aunt Jo works at JH, and she was able to put me in touch with Dr. Carson's PA directly. The PA shared that of the four neurosurgeons at JH who perform Chiari surgeries they all have different mehtods. She confirmed how confusing it is to make a decision on what is the best surgical approach when opinions differ among the experts.

So many of you have kindly asked what we need. Very simply we need God to direct our steps to understand who is the right surgeon for Danica and what is the best surgical method to bring her relief and long term healing. Please keep praying! We also need measures of physical and mental and emotional strength to keep doing all this and working our jobs and taking care of the day to day things. We are tired and discouraged. Please pray for Danica as she undergoes more sedation Monday. It is a new hospital and new faces and a long trip for her. Please pray for Delaney as she stays here with my parents. She has been such a brave girl too and has made many sacrifices of her own through this last year.

Our Hope remains!

Sunday, April 18, 2010

Meeting with Dr. Cohen, other opinions and a gift

Wednesday I was able to drive to Cleveland to pick up copies of all Danica's records, the op report from her first surgery and discs with her scans on them to send to Duke and to Cincinnati Children's Hospital. Dr. Cohen graciously met with me to discuss questions Dan and I had following our personal research about the results of Danica's latest MRI. These are some things I was able to clarify/take away from our conversation:

1. He rarely "coagulates" or removes the tonsil in cases like Danica's. I understood he is firmly in the camp who believes brain tissue shouldn't be messed with unless absolutely necessary. When I asked him about research showing the tissue is damaged and results when other doctors do choose to go this route he agreed the tissue is not in good shape and results have not shown any long term negative effects.

2. He reminded me he had presented Danica's case to most of the chief pediatric neurosurgeons in the United States at a conference immediately prior to her surgery last fall. Danica's case is made even more rare because her Chiari is exclusively on the left side. He said most of the other doctors agreed with his initial surgical approach although there were some who told him they didn't think it would work. (I want to reiterate to you how humble and accessible and open this man is. Our meeting on Wednesday reminded me how grateful I am for his gift of helping our Danica and his advice now as we move forward.)

3. He commended our choice of seeking another opinion from Dr. Fuchs at Duke as well as suggested Dr. Kerry Crone in Cincinnati. As you can imagine pediatric neurosurgeons are a rather small and elite fraternity who rely on one another's research and expertise in many cases. It was helpful to know these were good choices at this stage.

Everything was overnighted to the other surgeons on Friday. I'm sure it will take a few days for them to get to the doc's desks and be reviewed and then for us to hear back from them. In the meantime we are trying to breathe a little and do some "housekeeping" to brace for what's to come.

One of the biggest issues right now is trying to really figure out what we owe for all Danica's medical expenses last year and getting Summa to follow through and pay their part. Besides one group of bills from early last year which Akron Children's allowed us to consolidate and make a monthly payment on we have a growing stack of "ignore" bills. Bottom line, one brain surgery is excrutiatingly expensive but now we are in a new year, have a new deductible, and are facing even more than we can imagine. As parents this has to somehow be put on a back burner, because we have to find Danica the best care at any cost, but it is still always there. When we begin to break under the anxiety and fear we only need to remember God's faithfulness to find some peace. We have so many stories we could tell of His gifts to us through the kindness of others over the last three very hard years, but I want to tell the newest one before I close.

Our most dependable car has needed new tires since last April when all this began. We have put countless miles on those bad tires driving back and forth to all Danica's appointments, physical therapy and to Cleveland. With these other opinions at Duke and Cincinnati we knew we were going to have to drive even further from home in the next month or so, and we knew the tires were bad enough we couldn't take a chance. Dan receives a quarterly bonus from his job which we would always use to try to catch up on bills or to take care of something larger like car repairs. His company cut those bonuses in half beginning in Jaunary. He gets the quarterly half bonus this Friday. We knew we were still short the amount needed for the two bad tie rods to be repaired and the four new tires we needed. I have been praying. I haven't told anyone about this need, just my God.

Last night my parents stopped over and my dad handed me an envelope with the exact amount we needed to add to Dan's bonus for the repairs and tires. This money was from a family who used to attend our church but moved away. They heard about Danica and our family and felt moved to bless us. This gift met our immediate need, and it was the perfect encouragement to us there is nothing our God cannot do. I know any discussion of personal finances makes some people uncomfortable, but it is a very real part of our life right now and of Danica's journey. Tonight we are trusting and resting and waiting and so very thankful.

Thank you to everyone for all your cards and emails and calls of love to us this past week. They mean so much. Most of all thank you for continuing to pray. Danica had a few rough times last week, mostly at night when she has overdone it and cannot rest. She had a really good day today and although her tortocollis seems to be getting worse she is still able to run and laugh and play. She will always be my hero!

Tuesday, April 13, 2010

Doing our research and listening to our gut

I know the three previous posts don't really scratch the surface of what we've been through with our little Dani J, but I hope they give you some background on her condition. It's hard to believe we have been on this journey for a year now.

Since Wednesday of last week we have been trying to get our bearings emotionally and also beginning the hard work of researching next steps. Dr. Cohen's doubt about doing another decompression so quickly, particularly with the C2 and C3 now involved, just stuck in my head and my heart. I instantly had a gut feeling we had to look for an option other than just removing more bone.

Some of you may remember there was a little boy, Josh, who was Danica's age and had his first decompression two weeks before Danica at Rainbow Babies by Dr. Cohen. I posted on a Parents of Chiari Children message board last week about Danica's need for another surgery and Josh's mom instantly posted back to me. We had been out of touch for several months because the kids were doing better, but now Josh had regressed and had a RE-decompression last Friday at Rainbow! I finally spoke to Jamie on Friday for two hours. This conversation gave me even more incentive to get several other opinions and explore other procedures before we move forward with Danica's next surgery. She shared how much harder this was on she and Josh the second time around. The surgery is even more invasive, requires a much longer surgery time and involves much greater risk because of opening the dura and then having to put a patch on her brain over the opening. The child then needs steroids to help them not reject the patch. This opening introduces very dangerous risks of leaking CSF fluid as well as meningitis. I just can not imagine putting us all through this again with only a small chance of it being truly successful and then finding ourselves back in the same place in six more months. I just can't believe that continuing to cut away Danica's spinal structure and making more room for her brain to fall and put pressure on her spinal cord is the only option.

In all our research we found several pediatric neurosurgeons who believe cauterizing or removing the cerebellar "tonsil" (the part of the brain that is exposed) is optimal over continuing to decompress. Their research shows the tissue is already damaged and not functioning properly anyways. This is very controversial because the fundamental tenant of most neurosurgeons is to preserve brain tissue at all cost. In Danica's case it makes complete sense that we address the cerebellar slumping of the brain rather than continuing to take bone and make more room for the brain to be "swallowed" up by the spinal column. One of the surgeons who performes these tonsillectomies is Dr. Herbert Fuchs, Chief pediatric neurosurgeon at Duke. I was able to talk to his attending yesterday at great length about Danica's case and their approach to cases similar. They do many "re-do" surgeries for children who have had a failed first decompression. (By "failed" I do not mean that the choices made by the neurosurgeon in the first surgery were not appropriate. All of these surgeries have a chance of working or not working and only time will tell. Particularly in a child as young as Danica the chances were she would need further intervention at some point.) She said opening the dura is ALWAYS necessary in a second surgery but most likely intead of removing any bone they would cauterize the cerebral tonsil. Sometimes they will even reconstruct the C1 (and C2 if applicable) if too much bone was taken in the first decompression.

It gave me hope that we may be able to find someone who is able to help us beyond just another decompression and do something that will give Danica more lasting relief from her symptoms as she grows. We are not completely clear on the long term affects of removing this part of her brain. We know there will be extensive therapy following surgery to help her with the motor development issues. We feel like this is still superior to not knowing what kind of damage is otherwise occuring with the brain being pinched and growing pressure on her spinal cord.

The next step is getting Danica's scans and operative report from Rainbow and Fedexing them to Duke. Dr. Fuchs will then call us to discuss. We also have sent an email to Dr. Cohen asking him about this procedure and some other things regarding moving forward. Based on Dr. Cohen's response we may seek a third opinion.

So, that's where we are today. It's the strangest feeling to have all the real life stuff going on around you when you are facing something this huge. Our biggest need right now is for prayer. We need direction to the right doctors and wisdom to know what we should do next. As we look into Danica's little face everything else fades away.

I will post updates here as we have them. Thank you to everyone who has continued to love and support our family through this.

Danica's MRI results . . . Chiari continued

Last Wednesday, April 7th, Danica had a follow up MRI prompted by the return of some of her old symptoms as well as some new issues. This is my facebook post from that day.

Thank you so much to everyone who was praying for us today. Danica was so brave. She barely even cried when they put her IV in and couldn't wait to see the "big tunnel." She was so funny waiting for Dr. Cohen to come back from Case Western. She asked Helen, Dr. Cohen’s assistant, “Where IS Dr. Cohen?”

The results of the scan are not good. If you don’t remember the details of her Chiari or her first surgery you can see the note in my profile from last October, “Danica’s brain surgery is scheduled.” She had a brain decompression in November of last year. Her symptoms were dramatically reduced and we have enjoyed several months of seeing her run and jump and play with very little pain. About a month ago we started to see old symptoms return as well as some new ones. The scan showed that her Chiari has descended past the C1 vertebrae which was partially removed in her first decompression and is now being pinched by the C2 and the C3. Dr. Cohen does believe she needs another decompression. There are many risks associated with going back in and removing more bone and opening the dura. Because of Danica’s young age and rapid development by removing bone in those two vertebrae we could actually create more space for her brain to “fall” further into her spinal cord. On the other hand the amount of pressure she is experiencing now is damaging long term. There are not really any good answers. We have decided we will watch her for the next month and will have a follow up. Dr. Cohen believes if we can wait even a few months more without causing any irreparable damage it will improve the outcome of a second surgery.

I’m sorry if this is not very explanatory. I am so very tired and shell shocked.

Please continue to pray for our sweet girl. Please pray no permanent damage will be caused as we watch her the next month or so and that she will not experience further regression that would necessitate an emergent surgery. Please pray for Dan and I. The burden of making these huge decisions is almost overwhelming. We need wisdom. Please pray for grace to keep living the day to day when this weighs so heavily on our hearts and minds. Please pray for our finances. We do not have any idea how we will continue to move through this mounting and ongoing debt.

Our HOPE remains.

Danica's Brain Surgery is Scheduled

This is a facebook post from October 21, 2009, a month after Danica was diagnosed.

Thank you to so many of you who have been praying for us and encouraging us through the last six months. In late April Danica woke up with a very crooked neck and pain (tortocollis) and since then we have been through lots of physical therapy, scans, botox, and finally an MRI on September 15th that revealed a Chiari malformation. Chiari Malformation is a serious neurological disorder where the bottom part of the brain, the cerebellum, descends out of the skull and crowds the spinal cord, putting pressure on both the brain and spine and causing many symptoms. Since the call with her results from the physiatrist we have done research on our own but have been kind've waiting in limbo for appointments with the neurologist (yesterday) and neurosurgeon (this coming Monday) at Akron Childrens. Although we were told she has the condition they did not give any details about the severity, etc. We knew because she has quite a few serious symptoms affecting her development that surgery was going to be in her future. We also scheduled an appointment in early December with a neurosurgeon at the Cleveland Clinic known for his work with Chiari patients. Since then I have not been able to eat much or sleep. I have felt like I am holding my breath just waiting to know how much more we have to deal with, and if can they fix her. Danica also began waking in the night screaming in pain and complaining of more pain since the botox injection on the 15th, something that was intended to help not hurt her. It's been a very long and tiring month.

Two weeks ago my friend Beth sent me an email with a flyer for a fundraiser for a little boy, Nicholas, who has Chiari and they needed to raise money to help him have surgery. I hadn't had any contact with someone else who was facing this with their child so I called the number on the flyer and eventually made contact with the mother on Monday night of this week. She went through their own journey and of course his case is different from Danica's in many ways but the three similar things are they have Summa PPO incusrance like we do, they saw Dr. Bartkowski, the neurosurgeon at Childrens, and Dr. Luciano, the neurosurgeon we have the appointment with in December at Cleveland Clinic. Not all neurosurgeons are experts in all disorders so when a hospital has one neurosurgeon--like Children's--it just makes sense he may not be an expert on Chiari. One thing in particular that raised my eyebrows was a comment from the neuro group when they called with the results of the MRI that Danica's tortocollis was probably not related to the Chiari. WHAT?!?!? My instincts told me this Chiari was exactly the cause and after my own research through many medical journals found it is one of the symptoms especially in pediatric cases. Her son had a new "less invasive" decompression from Dr. Luciano at Cleveland Clinic a year ago and now he is facing the need for another surgery. She said the Clinic was not warm and fuzzy like a chldren's Hospital and that his bedside manner was not good at all if that was important to us. So, I was feeling a little discouraged after our talk but also empowered to ask better questions and be the best advocate for Danica possible.

Sorry this is so long--I am getting to the point! Yesterday we met with the neurologist which was kind've a waste of time. She didn't even explain the scans to us, and it kind've came out of her own mouth that she didn't know that much about Chiari and the neurosurgeon would need to explain. Well, I had the name of a neurosurgeon at University Hospitals Rainbow Babies, Alan Cohen. They call him the "King of Chiari." I called his office thinking I was just not going to feel good about these two other appointments and needed a third opinion and totally expecting them to tell me his next appointment was in late December or even January. Well, from the moment his receptionist answered the phone I had this amazing calm feeling. I told her a one minute synopsis of Danica's history, and she put me on hold and came back and said, "You have waited long enough, I can double book you tomorrow with someone else. You need to see Dr. Cohen. Just bring her scans." Thank you God!

Today we went, and it was like no other doctor's appointment I have ever been to. He went over each picture and explained that Danica's Chiari is rare in that only one side (the left side) of the brain is 10 mm below where it should be. (Most people who have Chiari have it on both sides) He showed us how the flow of her CSF (spinal column fluid) to the brain was almost non existent on the left side. And then he said it makes perfect sense that she would pull her neck to the right to adjust the impingement on the left--trying to release the pressure and pain it was causing. The botox injection which was intended to relax that muscle on the right was actually relaxing her neck back into a painful position that was pinching the area especially in certain positions. I can't tell you how good it is to have something logical come out of a doctor's mouth--a real reason for all this pain and her deformity. So, he said her condition although not emergent was serious because something like a car accident or a fall or other injury could cause her to become paralyzed so we need to be very careful with her and get a decompression scheduled. I won't bore you with the details of decompression. It's scary but can be very successful. So he got out his schedule and we chose Friday, November 13th. She will be in the ICU following surgery for 24-48 hours and then in the hospital 5-7 days. She will have another full MRI under sedation the Wednesday prior to check her entire spinal cord for any pockets of fluid that can sometimes accompany this diagnosis. As we left the hospital and walked out into the gorgeous day I didn't know whether to laugh or cry, but I felt hope.

So, praise that we found this condition early (some people suffer for years without proper diagnosis) and for the timing of finding this doctor and his willingness to see her so quickly! Specific prayer requests would be for Danica's continued pain and safety until she can have the surgery. Please pray for our strength and health going into November so we can follow through as scheduled. Dan and I are working a lot and not sleeping much at all. We are trying to go away Friday night to rest. Pray this can really happen, and I can feel relaxed enough to leave Danica with my parents. Pray for sweet Delaney and her peace as we are away from her so much. She has been through so much beginning with our separation when I was pregnant with Danica, and it's hard on her. She has her own anxiety about all this. Lastly pray for the financial stress all this brings. We have learned over the past years that the only thing that matters is people. We need reminded to put all that obligation on the back burner and just focus on getting Danica well. Dan has very little time he can take off and I do not get paid if I don't work so just the logistics of surgery are difficult.

We know God is good even through all this. Even when we don't feel it we believe it! Love to you all!!! Your notes and sweet gestures through the past months have been such a blessing and at the perfect moment would give me the strength to keep going to all the appointments and getting up in the night.


This is a post from my personal blog on September 20, 2009. Danica had been suffering for months and finally we had a name for the ugly monster causing her so much pain. They called it "Chiari."

Our little Danica had an MRI on Tuesday that revealed a Chiari malformation in her brain. What this means in simple terms is she has a gap where most people's skulls are closed and so some of her brain is sticking out into her spinal column. Some people have this condition without symptoms, but in Danica's case she is presenting many of the most severe symptoms very early on. In many ways I think I felt initial relief knowing there is a real answer or cause to the torticollis and other issues she has been experiencing. But as I have researched and talked to several doctors over the last few days I am realizing the road ahead for us is not an easy one and includes some form of neurological surgery to try to give her relief and the best chance for normal development. I'm back to holding loosely.

On the front of Danica's baby book is a quote from Roy Lessin.

Just think,
you’re here not by chance,
but by God’s choosing.
His hand formed you
and made you the person you are.
He compares you to no one else—
you are one of a kind.
You lack nothing that
His grace can’t give you.
He has allowed you to be here
at this time in history
to fulfill His special purpose
for this generation.

Beginning March 23, 2007, the day I found out I was pregnant with this little girl, God has been making a way for Danica and preserving her life in miraculous ways. Friday Dan emailed me the verses from Psalm 139 I clung to while fighting for her those long months laying in the hospital. "For you have formed my inward parts; You have covered me in my mother's womb. I will praise You, for I am fearfully and wonderfully made; Marvelous are Your works, And that my soul knows very well. My frame was not hidden from You, When I was made in secret, And skillfully wrought in the lowest parts of the earth. Your eyes saw my substance, being yet unformed. And in Your book they all were written, The days fashioned for me, When as yet there were none of them." These are Danica's life verses now.

Do I believe God makes no mistakes? Do I believe He lovingly formed this child's skull and brain how we find it today to fulfill His purpose in her life and in ours? Do I believe we lack nothing God's grace can't give us including strength for today and the days ahead? My verse for this week has been Mark 9:24 "I do believe; help me overcome my unbelief."

I'm afraid. I'm so very tired. I know that trusting God with my child is perhaps the hardest thing He will ask me to do. As He grows my faith and asks me to rest in His promises. I will fall. I will have moments of anger and confusion. I will want to quit and walk away.

Oswald Chambers wrote, "Living a life of faith means never knowing where you are being led. But it does mean loving and knowing the One who is leading. It is literally a life of faith, not of understanding and reason — a life of knowing him who calls us to go." Knowing a God who is unchanging and will do everything He says He will do is the only way I will navigate through the next weeks and months. I believe.