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
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.