Sunday, June 29, 2008

Brace Yourself

Michelle wanted me to share this photo with everyone to bring the magnitude of her surgery into full light. The picture is a bit blurry but you can clearly see the length of the incision. The good news is that the incision is very clean (no oozing or large spaces) and there is little to no swelling anymore. The doctors have done a wonderful job closing the sight, and once Michelle's hair grows back you won't even be able to see it. The bad news is Michelle wasn't expecting to go full-on punk rock hair! She's thinking of dying the nubs pink or something:)

The pain medication issue has still been a struggle, but a doctor from the neurosurgical team visited us last night and has come up with a new program for Michelle. Part of the issue is that Michelle has an extremely high tolerance to pain medications; let's put it this way, she's on enough pain meds right now to sedate a small village in Africa! This new pain management program combines "break through" with "long lasting" pain meds, as well as two different types of muscle relaxers. It is still early in this plan, but Michelle was able to get 2 or 3 hours of uninterrupted sleep last night, and is now resting again after breakfast this morning. She is still in pain, but we are hopeful that the doctors have her on a plan that will allow her to be discharged from the hospital tomorrow. Keep all your fingers and toes crossed!

Through all the pain this week, Michelle has truly shown the heart and spirit of a fighter. She hasn't complained about the pain or sought pity from anyone, despite her discomfort. Her strength and determination have been nothing short of inspiring. I can't imagine having to go through something like this and she has really impressed many people including me, her family, other Chiari patients on the floor, as well as the various health care providers.

Her room has been brightened by some beautiful flowers (which she loves!) and she has been watching her favorite movies to pass the time. Yesterday Michelle participated in one of the programs offered at this hospital called Pet Therapy. The dog they brought looked like a mini Lassie, and stayed with Michelle for a while which made her very happy. The other amenity they offered her was a clown-a-gram! Anyone who knows Michelle knows she is SO afraid of clowns, so she had to politely decline. Can you tell she's on the Pediatric floor? Family members have been with Michelle every step of the way, so she hasn't been alone for one minute.

Friday, June 27, 2008

Room 397

They have moved Michelle out of the ICU unit and into her permanent room. She is located on the 3rd floor, in the Pediatric unit, room 397, at the North Shore University Hospital in Manhasset, NY.

Unfortunately, Michelle has been in quite a lot of pain since yesterday. The doctors took her off the PCA pump (self administered morphine trigger) Tuesday and started giving her 20mg of oxycodone. The oxycodone, however, made Michelle really nauseous so the doctors changed her over to liquid morphine, but that isn't really helping with the pain. Michelle has an extremely high tolerance to pain meds and the doctors are trying to step her down too quickly. Hopefully we'll get that all sorted out today.

Michelle is also having some difficulty with getting any real food into her system because of her tree nut and gluten allergies. The hospital has assigned a dietitian to help manage her meals, but there were a couple of mistakes yesterday that left Michelle with nothing to eat but a boiled egg and some grapes. I talked to the director of the nutrition and food services department this morning and hopefully they will do a better job today with her meals.

Michelle has continued to work very hard to rehab her neck muscles; pretty much every time she wakes, she does her exercises. She is making great progress with her range of motion. Unfortunately, it is very difficult for her to rehab right now until they get her pain under control.

Michelle got a chance yesterday to read all of your comments for the first time and was lifted once again by the support and encouragement in those words. She thanks you from the bottom of her heart for being there for her during this difficult time.

Wednesday, June 25, 2008

Day 2 of the Recovery

Michelle has been moved out of the recovery unit and has been placed in the Pediatric Intensive Care Unit (the hospital didn't have any beds available in the Neurological ICU). She has a very private room that gets really dark and quiet allowing her to sleep.

The doctors have given Michelle some exercises to begin rehabilitating her neck muscles, and they are impressed with her range of motion. Michelle has to sit up every two hours or so in a chair for 30 minute intervals; and the rehab team gets her out of bed to walk around the floor 3 times a day. These bouts of sitting and walking definitely push Michelle's pain level higher, but are necessary to help her recover more quickly from the operation.

Michelle is resting right now and has been extremely touched by every one's love and support. We have been sharing your comments with her and they definitely bring a smile to her face.

They will be moving Michelle to a general room some time tomorrow, and when they do that we will post her room number and phone number on this site in case you want to send flowers or balloons.

Stay tuned!

Monday, June 23, 2008

The Surgery Was A Success!!!!!

Michelle made it through the surgery with flying colors!

The two neurosurgeons that performed the surgery said they achieved each of their objectives, without any complications. The primary goal was to coagulate or shrink the cerebral tonsils that were restricting the flow of Michelle’s cerebral spinal fluid; this restriction of CSF was the root cause of all of Michelle’s symptoms. Following the procedure, both surgeons confirmed that they were able to restore Michelle’s CSF flow to normal levels (probably to a level greater than she has ever experienced in her life). Great news!!!!!! They were also successful in creating a "patch" by harvesting a portion of Michelle's skull. This patch will help keep the cerebellum from falling down in the future.

We haven’t gotten in to see her yet, but will be allowed to visit her, one at a time, while she is in recovery. Michelle will remain there for the rest of the night and will be moved into the neurosurgery ICU, where she will stay for the next two days. Thursday morning she will be transferred one last time into a general room, and we hope that she will be fit to go home on either Saturday or Sunday.

The hard part for Michelle starts now, as she will be in considerable pain for the next four to five days. However, this pain will diminish with each passing day and with luck all of Michelle’s symptoms will be gone.

Thank you all for the love and support that you have given Michelle over the past few weeks. Your words filled her heart with joy and gave her the strength to endure the last few days, which were very difficult for her. I doubt if Michelle could’ve gone into surgery feeling more loved than she did. She is blessed with a wonderful support system and will need all of you during her recovery.

More to come, and thanks for staying tuned.

Tuesday, June 3, 2008

Many of you know, but for those of you that don’t, Michelle was recently diagnosed with Chiari I Malformation. She wanted to create this blog site to keep her closest family and friends up to date on her condition, surgery, and recovery. Chiari I Malformation is a very debilitating, rare disease and Michelle will need your support in the coming weeks and months as she battles through the surgery and recovery. Please feel free to post comments to this blog. Michelle will be able to read your comments, and they will help lift her spirits during this difficult time.

So how did Michelle get to where she is today?

Christmas day of 2005, Michelle was in a serious car accident in which she severely injured her neck and lower back. As a result of the accident, she underwent surgery in April of 2007 to repair damaged discs in her lower back and alleviate the nerve damage that had been caused by the herniated/ruptured discs.

In July of 2007, Michelle began to experience heart palpitations. These palpitations ranged from mildly uncomfortable to very debilitating; forcing Michelle to change her lifestyle. No longer could she consume caffeine or any other stimulants, and was generally unable to exercise at all. Even going out to run errands or attend social events became very difficult. There were times that the palpitations were so bad that she had to go to the emergency room. The ER doctors couldn’t pinpoint the cause of the palpitations, so Michelle sought the guidance from a couple different cardiologists to ascertain the cause and treatment for the heart palpitations. The cardiologists put her through a battery of tests: tilt table testing, wearing a heart monitor for an entire week while keeping journal entries detailing when symptoms came on to track her heart beats, Doppler studies of her carotid arteries in her neck due to the "tightening" feeling she was experiencing, blood pressure pills (that made Michelle extremely dizzy and severely dropped her blood pressure causing her to, once again, visit the ER), several heart tests, such as echocardiograms, and more. Unfortunately, none of the tests determined anything conclusive. A few doctors even suggested that Michelle was having anxiety and panic attacks due to her planning the wedding and prescribed her anti-anxiety pills, which completely infuriated her. Michelle’s only option at this point was to just deal with the heart palpitations and related discomfort.

Her condition began to worsen as time went on. In addition to the heart palpitations, Michelle began to experience vertigo, dizziness, trouble swallowing, ringing in the ears, visual disturbances, and fatigue. The vertigo forced her to leave a movie theater just five minutes into the showing because she became severely dizzy from the stadium seating layout of the theater. Everyday tasks, like drinking or eating, had become difficult because it was hard to swallow and non-descript foods appeared to trigger her palpitations. Fortunately, she was able to enjoy her wedding and honeymoon, although, not completely as a result of the multiple symptoms she was living with.

Following the honeymoon, Michelle returned to New Jersey and attempted, for the first time since her back surgery, to go back to her job as an RN in the emergency room at Newark Beth Israel Medical Center. While giving every ounce of energy to her job, Michelle ultimately was forced to discontinue working at that hospital because the rigors of the job were too much to bear in light of the symptoms; nearly passing out several times on the job because of the palpitations and dizziness.

In early January of 2008, another symptom reared its ugly head, severe headaches. From a severity and occurrence standpoint, these headaches immediately went to the top of Michelle’s list of symptoms. There were numerous occasions that she had to confine herself to a dark room, devoid of any light, and try to gut it out in silence. Over the counter pain medication had no effect on her headaches. Michelle went to her primary doctor to address the "migraine" headaches. Her primary doctor could not put her finger on anything but ordered Michelle to get a CAT scan of her brain to see if there were any neurological problems. The results of the CAT scan led Michelle’s primary physician to order Michelle to get an MRI of the brain because she suspected something was wrong, but didn’t want divulge anything until an MRI confirmation could be delivered. In fact, she notified Michelle of these unfavorable findings by leaving a voicemail on her cell phone, which made Michelle wrought with fear. At this point, Michelle was in so much pain so suddenly, she thought her doctor was about to deliver a terminal blow. When she finally got in touch with her physician, the doctor gave her a vague explanation, and said, "We will discuss more when the MRI results are in."

The MRI results came back in the middle of February and they confirmed what the primary doctor suspected, Arnold-Chiari Malformation, or Chiari Malformation. Very little is known about Chiari Malformation, so the primary physician ordered Michelle to go see a neurologist. In the meantime, the doctor prescribed Michelle some pain medicine. Unfortunately, the next several weeks were punctuated by nothing but frustration. The neurologist that Michelle saw failed to make a connection between her symptoms and dismissed her condition as nothing more than migraines, and even laughed at her when she insisted on him delving more into the finding of Chiari Malformation on the MRI. He prescribed her migraine medication and told her to check back with him in six months. This was a road that Michelle had been down before, suffering from "migraines" as a teenager, and she knew that there was more wrong then just migraines. In retrospect, the "migraines" that she experienced as a teen may very well have been an early onset of Chiari Malformation symptoms, which went into remission until the head trauma from her car accident. This is all speculation, of course, but it makes sense. Furthermore, the pain medication her primary doctor prescribed was having little to no effect on her headaches.

Michelle got onto the computer and began researching Chiari Malformation intently; learning any and all information that was available regarding this rare disease. It was at this time that she found the Chiari Institute, a unique and world renowned center for treating this disease. Getting an appointment with the institute was no easy task, but with a little persuasion from a distant relative of Michelle's we were able to get her an appointment on April 2, 2008. Michelle received a complete and thorough medical exam from a physician’s assistant, a neurologist, and a neurosurgeon. The institute utilized their own imaging center to take MRI’s, CAT scans, and Doppler images of Michelle’s brain and brain stem. Dr. Paolo Bolognese delivered the diagnosis of Chiari I Malformation. In addition to the Chiari diagnosis, Michelle was also diagnosed with POTS, EDS, and possible Tethered Cord Syndrome (see definitions below). While no one of these diseases directly causes Chiari Malformation, a great number of patients that suffer from CM I have been found to have the other rare syndromes as well. Chiari I is a congenital disease, which would likely explain the migraines that Michelle experienced in high school. However, the migraines and other symptoms were re-activated, if you will, as a result of the trauma she experienced from the car accident back in December of 2005; coming on slowly, one at a time almost.

Despite the overwhelming evidence supporting a Posterior Fossa Decompression surgery, Dr. Bolognese wanted Michelle to try two different things. The first was to begin taking an osmotic diuretic called Diamox. This pill could potentially alleviate some of the pressure in Michelle’s brain, which might rid her of the terrible headaches by helping her expel some of the cerebral spinal fluid (CSF) that is being prevented from properly draining out of her brain due to her CM I. The second thing that Dr. Bolognese wanted Michelle to do was to get urodynamics studies. This test is designed to determine whether or not a patient has a neurogenic bladder, or a bladder that isn’t functioning properly. If Michelle was found to have a neurogenic bladder then it would confirm the Tethered Cord Syndrome diagnosis. Tethered Cord Syndrome requires its own surgery; however, it is much less risky than a Posterior Fossa Decompression surgery. Sadly, the Diamox made Michelle feel absolutely horrible and the urodynamics test was inconclusive. Dr. Bolognese still states he has an underlying suspicion that she does in fact have Tethered Cord Syndrome, and wants to continue to monitor it closely in the future. We met with Dr. Bolognese one more time on May 22nd and Michelle decided to proceed with the Posterior Fossa Decompression surgery.

So what does Michelle have in front of her?

Michelle’s surgery is scheduled for Monday, June, 23rd at 7:00 AM. The entire surgery will take between 8 and 9 hours and she will be in the hospital for 6 days total, without any complications; fingers crossed! Michelle will be at home recovering for at least 3 months before she can begin resuming normal activities and work. The prognosis is good, most of her symptoms should go away entirely, and she should be able to live her life like any other adult.

The surgery itself is very delicate, as you might imagine, however, the Chiari Institute is the only place in the world where two neurosurgeons scrub in on the surgery. They do not use resident doctors or physicians' assistants to perform the surgery. This method greatly reduces the risk factors involved with such a complex surgery. Before her actual surgery, Michelle is scheduled for a smaller surgical procedure in which they will put her under anesthesia, and insert two titanium screws in each of her temples. When she wakes, she will be connected to traction by those screws, which will attempt to elongate her spine. Why you may ask? They suspect that in addition to her posterior fossa being malformed, she may also have another area contributing to the compression of her brain. The 2nd cervical vertebrae has a long bone that extends upwards called the odontoid. Usually it extends up at a 90 degree angle, but sometimes that bone can actually tilt backward towards the brain stem, causing a sandwich effect of the brain stem between her posterior fossa and the odontoid. The traction will attempt to alleviate some of the symptoms by bringing the odontoid back to its proper position. If that is the outcome, the surgeons will have to operate on that area as well. Before her scheduled PFD surgery, Michelle will have an area in the back of her head shaved outlining the area the surgeons will be operating on, much to her dismay! The neurosurgeons will be making an incision from Michelle’s first cerebral vertebrae all the way up to the crown of her skull, roughly 8 – 10 inches. They will then go in and cut open the dura, which is the protective, sterile membrane that surrounds the brain stem, brain, and CSF. After this, the neurosurgeons will coagulate the part of Michelle’s cerebellum (the cerebral tonsils) that have been forced downward into her spinal canal by the malformed posterior fossa. Once the tonsils have been coagulated, or shrunk back up out of and through the foramen magnum back into its proper place in the skull, the neurosurgeons will then harvest a part of Michelle’s skull, near the crown of her head. They then will open up the malformed posterior fossa, and use the harvested skull piece as a sort of patch to make the space that houses her cerebellum larger, to mitigate against the cerebellum descending in the future. The removed portion of skull regenerates, and will actually grow back in time. Once these steps have been done, the neurosurgeons must complete the delicate task of closing the dura and other membranes that were opened to gain access to the cerebral tonsils. This is a critical step because any small holes in the stitches could lead to life-threatening infections.

Your prayers and best wishes are certainly needed. Michelle will be in considerable pain following the surgery because the doctors will have to cut completely through all of the muscles, tendons, and other connective tissues in her neck to gain access to the surgical sight. If the recovery weren’t enough, Michelle has also recently discovered that she is deadly allergic to almonds, hazelnuts, and other tree nuts, along with a severe allergy to wheat gluten; a problem that has forced her to visit the ER about 5 times since January. Michelle will have to navigate very carefully around what she eats for the rest of her life; just another one of the many perks of being a Chiari Malformation patient. She has been following a very strict Gluten/nut-free diet the past 5 or 6 weeks. These allergies will not alleviate after the surgery, as was secretively hoped; as her doctor states, "Once a Chiari patient, always a Chiari patient."

Please stay tuned to this website for additional updates as they become available and thank you to everyone for your love and support for Michelle during this trying time.