Tuesday, October 16, 2007

Your Health, Your Partner

As some of you may know, my wife FrankenKristin had brain surgery at The Chiari Institute (TCI) in New York two years ago to correct a symptomatic Chiari 1 Malformation. The need for the surgery stemmed from two car accidents that occurred in 2005. To read about that experience, follow the links on the right under the heading “There and Back Again: FrankenKristin’s Tale.” Since then we have been engaged in an ongoing battle with our health insurance company to cover her follow up care as well as a lawsuit against the driver in the first accident. We have yet to address the second.

By all accounts surgery was successful in alleviating nearly all of her Chiari related symptoms. At that time, the doctors indicated that she may also suffer from craniocervical instability given her anatomical structure and the whiplash she experienced. They indicated that this would not be clear until she had made a full recovery from the Chiari surgery.

Two years the Chiari surgery still remains a success However, after countless hours spent expermeinting with various treatment protocols, it is time to determine whether craniocervical instability is the source of her current situation and what we should do about it. Fortunately we had the support of her primary physician as well as the chief of neurosurgery at the flagship hospital in town to return to TCI for assessment. And yet, our insurer denied coverage contending that the diagnostic procedure TCI used is “investigational/experimental” and therefore not covered under our plan. Of course they informed us of our right to appeal. Which we did.

What follows is the statement I wrote last week in response to their decision. Enjoy.

To: Appeal Review Committee
From: Sven
Re: Appeal for coverage on behalf of FrankenKristin

It is on behalf on my wife FrankenKristin that I am writing this letter. Because of her current symptoms she finds it difficult to sit at a desk for any length of time. Since we’ve had insurance coverage through providers other than HealthPartners during the last two and a half years, her medical chart may not provide a complete representation of her current situation. Because of this, I am submitting the following statement in an effort to introduce additional information that should prove useful.

As you know, we are seeking coverage for care that is to be provided by The Chiari Institute in New York. The initial decision to deny coverage was based on the argument that invasive cervical traction is an experimental/investigational procedure. However, it is not solely for the purpose of invasive cervical traction that she is returning to The Chiari Institute, but rather to establish or rule out the presence of craniocervical instability and to determine the possible need for craniocervical fusion.

She had initially scheduled only the invasive cervical traction, due to time constraints and with the hope that she would assess the next steps in conjunction with her primary physician once we returned home - including the possibility of working within the network. However, given her current symptomology, the complexity of her surgical case and the recommendation made by the Chief of Neurosurgery at Regions Hospital it has been determined that The Chiari Institute is the only facility capable of providing care. Her intent to return to The Chiari Institute is based the following factors:

 The Chiari Institute is widely accepted within the neurosurgical community as the leading multidisciplinary center for the management and treatment of Chiari Malformation, syringomyelia, and other related disorders including basilar invagination, craniocervical instability, and spinal cord tethering. Dr. Thomas Milhorat and the team at The Chiari Institute are generally regarded as the foremost experts in the care and treatment of Chiari Malformation and related disorders. Their research and clinical practice represent the leading edge of care and treatment of these disorders and sets the industry standard.

 In 2005 FrankenKristin was diagnosed at The Chiari Institute as having a symptomatic Chiari I Malformation. She underwent posterior fossa decompression and C1/ partial C2 laminectomy the same year to correct this malformation and has found relief from many of the Chiari-related symptoms. Surgery was performed by Dr. Milhorat, Founder of The Chiari Institute and Chief of Neurosurgery at North Shore University Hospital, and Dr. Paulo Bolognese, Assistant Director of The Chiari Institute. Dr. Milhorat prepared a detailed operative report which described the nature of the surgery in the following terms:

“Posterior fossa decompression under color Doppler ultrasonography guidance consisting of a suboccipital craniotomy, C1 and partial C2 laminectomy, dural opening, microlysis of arachnoidal adhesions, bipolar shrinkage of the cerebral tonsils, expansile duraplasty implying autogeneous pericranium, and remodeling of the posterior cranial fossa with a titanium mesh/acrylic cranioplasty.”

 The pre-operative report noted the presence of retroflexed odontoid with pannus formation, Eagle’s syndrome, dilated central canal and possible craniocervical instability. It is for the signs and symptoms consistent with craniocervical instability, which she is currently experiencing, that she intends to return to the Chiari Institute for further evaluation. Craniocervical instability is defined as hyper mobility of the craniocervical junction where the head joins the neck. It is not uncommon to see this complication in patients, post-Chiari decompression, who have also experienced traumatic whiplash - which is the case for FrankenKristin. If the evaluation confirms the presence of craniocervical instability, the indicated surgical treatment is craniocervical fusion.

 Following the direction of her primary care physician, Dr. David Caccamo, she has attempted a series of non-invasive treatments for these symptoms, within the Health Partners network, as well the network offered by our previous insurance provider, in an attempt to achieve adequate and lasting relief. To date none of the prescribed treatments has proved anything other than temporary.

 In an effort to explore additional treatment and possible surgical options, Dr. Caccamo contacted the Health Partners Assistant Medical Director, who consulted with Dr. Mark Larkins, Chief of Neurosurgery at Regions Hospital. Regarding the consultation, Kristin’s medical chart indicates per Dr. Larkins, that she should return to The Chiari Institute for follow up care. Dr. Larkins went on to say that Dr. Milhorat, is a world expert in Chiari Malformations and that there may be nuances in her surgical case that they would be best able to address. Dr. Larkins also felt that to see him or any other Twin Cities neurosurgeon would not be worth her time.

 Earlier this year a preliminary indication of craniocervical instability was made by Dr. Paulo Bolognese of The Chiari Institute through the review of various self-assessment tools as well as results from cervical extraction tests. Based upon these finding he recommended that Kristin return for a complete evaluation to establish or rule out the presence of craniocervical instability and the possible need for craniocervical fusion.

 In 2005 Dr. Mihorat presented research at the annual conference of the American Syringomyelia Alliance Project (1) where he highlighted the frequent occurrence of craniocervical instability in Chiari I Malformation patients and identified the proper course of diagnosis and treatment - before and after decompression. In that presentation he explains that craniocervical instability, which can occur post-decompression, is generally not provable by radiographic studies and that the only definitive diagnosis is through invasive cervical traction.

 According to The Chiari Institute, invasive cervical traction is a definitive test for establishing the diagnosis of craniocervical instability with functional cranial settling. The goals of invasive cervical traction are 1) to establish or rule out the diagnosis of craniocervical instability with functional cranial settling; (2) to identify patients who do not require craniocervical fusion, thereby avoiding an unnecessary surgical step; (3) to identify patients who are potential candidates for craniocervical fusion - in extraction and (4) to acquire precise radiographic and extraction weight measurements that can be reproduced at the time of the craniocervical fusion to maximize the likelihood of optimal outcome. It is policy at The Chiari Institute that all patients with clinical suspicion of craniocervical instability with functional cranial settling undergo invasive cervical traction prior to surgery.

 In determining the diagnosis of craniocervical instability, the presence of retroflexed odontoid with pannus formation combined with the previous posterior fossa decompression and C1/partial C2 laminectomy, complicate the surgical case to the degree that only someone with an expert level of experience with these conditions as well as intimate knowledge of her anatomical structure and previous surgical history should be considered. While it has been argued, albeit incorrectly, that invasive cervical traction is an investigation/experimental procedure, craniocervical fusion is not. Performing a fusion to address craniocervical instability is commonly accepted across the neurosurgical community. The use of invasive cervical traction by The Chiari Institute is an essential tool in establishing not only the need for surgery but also in acquiring precise indicators that can be reproduced at the time of surgery to maximize the likelihood of optimal outcome.

 Given the factors surrounding her previous posterior fossa decompression and C1/partial C2 laminectomy, the level of expertise and clinical success rate at The Chiari Institute, the nuances of her surgical case including the presence of a retroflexed odontoid with pannus formation, as well as the endorsement and referral of the Chief of Neurosurgery at Regions Hospital, it is clear that her current situation indicates that The Chiari Institute is the only facility capable of offering the proper diagnosis and treatment.

As you can imagine, each day is more difficult than the last. Up until the last few years, FrankenKristin has always enjoyed a physically active lifestyle and has been a role model for our children by actively engaging with them and promoting their enjoyment of physical health. Her current symptoms have left her unable to pursue most physical activity with our children. She experiences severe, sometimes debilitating pain almost daily which affects not only our family, but her work as well. She has been a Social Worker at Regions Hospital for almost 9 years, a career she truly loves. Although the job is challenging, and rewarding it is increasingly more difficult to make it through each day as her pain becomes more and more unbearable. Even though she is an on-call employee she very rarely accept extra shifts, as the pain at the end of each work day is prohibitive.

She has pursued and made a good faith effort to follow a course of pain management and physical rehabilitative treatments within the network, and now believes, with the support of nearly every medical professional she has seen, that it is appropriate to return to The Chiari Institute for further assessment and treatment. We acknowledge that insurance providers do not make these decisions lightly and yet we remain hopeful that she may secure the coverage necessary to restore her health. The prospect of traveling across the country for yet another surgical procedure is not easy for us, as it will require once again disrupting our lives and leaving our children behind. However, the potential for improved functioning and reduced pain will offset any short-term inconvenience. We pray that HealthPartners will provide the approval and coverage necessary for her treatment and future healing.

We appreciate your attention in this matter. Please contact me if you require additional information.



(1) A video of the presentation can be found on The Chiari Institute website: Click on Ehlers-Danlos Conference.


Sven said...

Today we found out for in-network coverage. Hooray!

Attila The Mom said...

Yippeeee!!! That was a really great letter. I'm so sorry you guys are going through all this. :-(

kim said...

yeah! what Sven said!!

so that means you were approved yes?

Terri said...

I am so glad to hear that you were approved. I too had Posterior Fossa Decompression and I would LOVE to chat with your wife, but I cannot find an email address on here for you. If you get this. please have her, or you email me at tmacford@yahoo.com I am curious about the instability issue... I think I have it, but I am only 8 weeks post op.

Good luck and thank you in advance!!


Charnel said...

I run a support group for people with chairi and EDS. CCI is a common problem for those of us with Chiari and connective tissue disorders. I would like to invite you and your wife to our group so you might get any info on the fusion you want prior to surgery.
Charnel Jerome

Anonymous said...

My son is 18 and possibly having some cranial instability issues. I would love to talk to your wife if she feels up to it. My email is graytongirl@gmail.com . My son and I have started a support group and it's been a great journey.

Chiari friends,

Anonymous said...


My name is Frankie and I just had my trip to TCI last week and they have diagnosed me as well with cranial cervical instability. They want me to use the aspen vista collar and at home traction. If that works well, they want me to come back and do the test to see when the pain stops by doing a procedure and then they would want to do the fusion to my spine to keep my head from moving anymore. At this point I am very scared and nervous but would like to speak with you more about your wifes symptoms and see if we are alike. I too, am unable to stand the pain at the end of the day and my life has gone from active to inactive over the last two years. Looking forward to hearing from you and bless you for supporting her so faithfully, it means so much to us wives.

Susan S. said...

Thank you for posting your letter. I will use it to guide me in my upcoming insurance battles before embarking upon my TCI surgical procedures!

Susan S.
Coping with Chronic Illness Through Cycling

Anonymous said...

Hi Sven.
I have read your story and in many ways it sounds like ours. I went to TCI for the same reasons. An answer. And I thought they were the best. I have had 2 invasive cervical tractions in an attempt to prove that I have instability so they could do the fusion surgery. Sadly at the time I was diagnosed, there were 3 of us in a support group all diagnosed with the very same thing. I thought it was odd. I didn't listen to that inner voice and had the fusion surgery. Aside from the nightmare that followed postop, I have progressively gotten worse. After getting absolutely no response from inquiries to TCI for my symptoms, I found a good neurosurgeon locally. After thoroughly reviewing all my imaging, records, op reports, and symptoms it was determined that I should never had the fusion surgery at all. This type of fusion is for people with internal decapitations. And even then, it is far too big a fusion for that. The problem then is that the fusion puts a great amount of downward pressure on the cervical vertebra and leads to destruction of vertebra below the fusion which will result in the need for repeated fusions to correct it. It has contributed to increased headaches, muscle spasms around the hardware, chronic fatigue, numbness and tingling down the arms into the hands, feet, weakness in the legs, etc. Given that the symptoms are now attributable to the fusion itself, it was not worth the cost, pain, and suffering not to mention that they literally scrape all the muscles off of the bones to do it and then lay that muscle on top of the hardware creating constant irritation. I now have to have the hardware removed in another serious operation.

When they cut into the 1st and 2nd cervical vertebra with the posterior fossa decompression they actually weaken the main joint that supports the head. At the very least, fusion should only support those 2 vertebra. Not the head AND neck. God made our heads and necks with the ability to move for a reason.

Please...if any of you are hearing this diagnosis, please do yourselves a favor and really research it and get a 2nd or 3rd opinion.

Fedja said...


I have gotten familiar with craniocervical instability issues last year. I have some kind on instablity in that area.

I would be interested in following issues:
- What were the symptoms after accident of you wife?
- How instability was diagnosed?
- Was is seen in regular MRI?
- What was done in the surgery?
- What is situation now?

If answer to those questions is in blog-texts, could you tell me in which blog tests. Because of my injury I am not able to sit for very long and I can't go through all the textes.

t. Fedja from Finland
fedjairene at gmail dot com

Anonymous said...

I am the same problem I had the CCF surgery and now have alot more problems now and had to have alot more surgerys because of it. I am thinking of having all of the hardware removed. good luck to all