Haley's Hope for Health- CCI Surgery

Asking for support is never easy, but we are currently faced with a life threatening hardship. This post is our outward request for support, our plea for help, and our last ditch effort to achieve success in a situation that appears hopeless. Right now, our dear Haley is suffering from a multitude of debilitating conditions that are robbing her of her ability to live. Currently, Haley is completely bed bound and requires 24 hour assistive care. What was once a life of joyful activity and promising opportunity has devolved into a nightmare of daily pain accompanied by the constant fear that the pain will never go away. 
Haley has no ability to care for herself. We spoon feed her while she’s laying flat, hand bathe her in bed, and we physically lift her body onto her bedside commode so she can use the bathroom. Haley is declining daily and with each week that Haley’s condition goes untreated she loses another ability and gains another horrifying symptom. Haley spends her entire day in a dark room with an eye mask on and ear plugs in. Any amount of light or sound exacerbates the already excruciating pain she feels from her unstable skull sliding around, mechanically distorting her brainstem as the result of spinal instability. If Haley does not receive treatment soon, the conditions that have made her an invalid will claim her life permanently. 

Before Haley became bedridden from her illnesses she lived a life she was proud of. Even despite living with Myalgic Encephalomyelitis (M.E./CFS) for 5 years Haley had a career where she was giving back, working to assist the needs of those in recovery for addiction. Haley had a social life filled with the enjoyment of activities like yoga, book clubs, church groups and days at the beach. Most importantly Haley built a loving home life where she was excited to build a family with her now husband Danny. Haley’s severe Craniocervical Instability (CCI), Atlantoaxial Instability (AAI), Hypermobile Ehlers Danlos Syndrome (EDS), and Complex Chiari Malformation, have put all of those things Haley used to love about her life on a permanent hiatus.  
When paired together these ailments create a critically dangerous circumstance. To put it simply, Haley’s neck is like the game Jenga. With every day that passes, it’s as if another block is removed and her neck becomes more unstable. Eventually the connective tissue that supports her neck will completely deteriorate, and her already compromised spine will collapse underneath the weight of her skull. This end will come without warning, and every day that passes without treatment increases the risk of sudden death.
It is not just CCI and hypermobile EDS that keep Haley bed-bound. She also suffers from Hyperadrenergic Postural Orthostatic Tachycardia Syndrome, a condition that is caused by autonomic nervous system dysfunction, and aggravated by being in an upright position. Even seated Haley’s heart rate will jump from a normal 90 BPM (yes, that’s normal for someone with POTS) to an absurd 130 BPM. If Haley stands up her heart rate spikes to 150 BPM or higher and her blood pressure drops causing vertigo, vomiting, and fainting. Haley’s POTS is aggravated by brain stem compression due to CCI, and intensified by her Complex Chiari Malformation. Haley’s brain is slumping through the hole of her skull, causing a build up of spinal fluid, which doctors refer to as Intracranial Hypertension/pseudo-cerebral tumor. Essentially, Haley’s symptoms are similar to those of someone with a brain tumor.
The diseases, malformations, and physical ailments that Haley suffers from are a complex intermingling of medical abnormalities. These abnormalities are still not well understood by the broader medical community, especially in regards to how they interact with one another. Even so, there are a few specialist neurosurgeons in the world who have dedicated their lives to helping patients like Haley who suffer from Spinal Instabilities with rare comorbidities like EDS. These neurosurgeons have both met with Haley only to deliver the same diagnosis and solution: Haley needs two neurosurgeries, a Craniocervical Fusion and a Fossa Decompression. 

The surgeon Haley is scheduled to see in late April is one of the few neurosurgeons in the world who understands the techniques and practices regarding delicate cases like Haley. His specialized methods, and techniques, are pivotal to Haley having the best possible surgical outcome. Haley’s Ehlers Danlos Syndrome affects her joints, organs, skin and connective tissues, and her skeletal and muscular structure are compromised in such a way that an invasive procedure like surgery can be dangerous. This ultimately makes seeking the help of a true expert vital, if not a necessity. 

Insurance companies will do anything to deny complex medical coverage and they have used Haley’s Chiari Malformation as the primary reason to block her surgery. Haley’s insurance claimed that the Chiari Malformation Haley suffers from was not severe enough to warrant surgery, completely ignoring her other comorbidities. The insurance company’s denial completely ignored the opinions of the two expert neurosurgeons who evaluated Haley. The physical state of Haley, and her continued deterioration, indicates that her condition is advanced and dangerous. We are deeply troubled that insurance plans are written in a way that permits them to discard the opinions of expert specialist neurosurgeons, not allowing for exceptions and rare cases. We have exhausted our appeal options and now the only way to get Haley the surgeries she needs is through private funding.

The four procedures Haley needs are an Invasive Traction Test, an Intracranial Bolt Test, a Fossa Decompression, and a Craniocervical Fusion. These surgeries are highly invasive and as a result must be done in immediate succession. Haley’s procedures will take an estimated 16 hours over a period of two days. The cost of the surgeries alone are over $100,000+ and with the intensive recovery and hospital care the bill will likely reach at least $200,000. Haley’s case is such that the two neurosurgeons referred to earlier have made it clear that Haley needs to have these procedures done in order to save her life. Attempts were made on Haley’s behalf to explain this to the insurance provider but the expert medical opinions of these neurosurgeons were met with obstinance and skepticism by the insurance company. 

Even though the social stigma that once surrounded swallowing your pride and asking for assistance has all but vanished, asking for support is still hard. We are asking for your support because we have not given up and we are committed to finding a solution for Haley’s aggressive medical problems. Join our team and help us achieve a goal that has become impossible to achieve alone. 

Asking for support is never easy, but neither is providing it. We are overwhelmed by the generosity and assistance you have blessed us with and we are extremely grateful. Throughout this process our greatest source of hope has come from the willingness of friends and strangers alike to say “yes, I will support Haley.” Whether you can or cannot support us at this time, we want you to know we are still grateful for you. We are grateful that you took the time to read this page and to learn more about these ailments.

We ask that you pray for Haley, specifically that you pray for God to open doors for healing. This is the prayer we recite on Haley’s behalf: 
“Father, please open the door of healing for Haley. Please open the hearts of those who can help, and inspire them to do so. Please grant peace to Haley and to those who support her. Please bless us with the understanding and acceptance that prayers are answered in your time, and not always as we expect. Please guide my actions and use my time, treasure, and talents to answer the prayers of others. We open our ears to hear your response. We open our eyes, bearing witness to your mercy. We hold out our hands to accept your healing grace, and in doing so, we open ourselves to the gifts of faith and the abounding hope it provides.

 Amen.”

Read below for more information on Haley’s co-morbidities and journey:
Haley Holt graduated from Lake Norman High School in 2008 and graduated from ECU with honors in May 2012.  Her life was full throttle ahead and she was ready to hang the stars.  She moved to San Diego, CA in June 2012 to "live her best life".  She had an amazing career as a Social Media Strategist. She met Danny McCarthy, the man of her dreams. In October 2015 she was sick for 10 days with flu like symptoms.  Those flu like symptoms never went away and her body never recovered.  Haley was diagnosed with Myalgic Encephalomyelitis (M.E.)/CF.  It is a  devastating and debilitating complex illness, with no cure. At first she was able to manage the symptoms with lifestyle changes, many doctors and treatments but they began to worsen.  She had extreme fatigue, widespread body pain and weakness, brain fog, dysautonomia and extreme migraines. After her and Danny's wedding in October 2018,  she started rapidly declining. She was diagnosed with and hyperadrenergic Postural Orthostatic tachycardia Syndrome (POTS) while still suffering with ME/CFS.  The symptoms worsened with each passing month  - severe migraines, eye pressure, brain swelling, extreme muscle fatigue and uncontrollable tachycardia. She had to quit working and will probably never be able to go back. Now she lives her life in bed, lying flat 95% of her days with Stoli (her dog) by her side.   Her legs will no longer carry her body nor her heart handle her body being upright so she needs a wheelchair to be mobile and oxygen to help her breathe. She has numbness in her extremities, vertigo, cognitive dysfunction, sensory sensitivity and sleeps 14-17 hours a days.  Despite Danny playing Superman, she now needs daily in home care and nursing.

Haley & Danny, along with family & friends, made the trip to Silver Spring MD and on October 6, 2019. 
After many tests, Haley was also diagnosed with the following additional spinal/neuro illnesses

- CCI (CranioCervical Instability) with with cerebellomedullary syndrome
- Atlanto-axial instability with presyncope, diffuse hyperesthesia and hyper-reflexia
- Foramen magnum stenosis with symptoms of CSF obstruction
- Intracranial hypertension
- Basilar Impression and Cranial Settling
- EDS (Ehler's Danlos Syndrome) 


Some of her symptoms stemming from the latest diagnosis are -  
* heavy headache: a constant to near constant headache that can be described as feeling like the head is too heavy for the neck to support (feeling like a “bobble-head”)
* pressure headache: an impairment of CSF flow causes intracranial pressure which would be aggravated by “valsalva maneuvers” such as yawning, laughing, crying, coughing, sneezing or straining.
* Dysautonomia: brainstem compression can lead to a dysfunctional autonomic nervous system (the involuntary regulator of all body functions). Symptoms of this include, but are not limited to:tachycardia (rapid heart), heat intolerance, orthostatic intolerance (low blood pressure when standing)
syncope (fainting), polydipsia (extreme thirst), delayed gastric emptying, chronic fatigue, neck pain, facial pain or numbness, balance problems, 
muscle weakness, dizziness and vertigo, vision problems, difficulty swallowing, ringing in the ears and hearing loss, nausea, impaired coordination,
downward nystagmus (irregular eye movements) and paralysis.

The life that Haley lived and loved is not the life she is living now. While her faith and soul are strong - her body is very, very weak.

But we have hope &  faith (and with God's healing hand) that the amazing team of doctors that we are working with can help Haley to be able to live a happy life !!!

Haley needs your help with the ongoing medical expenses. 

We thank you in advance for your support, love and prayers.


At the end of the day, all you need is HOPE & STRENGTH -
HOPE  that it will get better and STRENGTH to hold on until it does.
author unknown

I can do all things through Christ who strengthens me.
Philippians 4:13