May 19, 2012 marks a very special 1st anniversary for Josephine (Jo) Macaluso. It’s one she is happy to be alive and well to talk about and celebrate. Over a year ago, a rare medical condition suddenly turned Jo’s world upside down and we are honored that she was willing to share the details of her deeply personal struggle with us. “I think it is important for people to know they are not alone. The only way for this to happen is to share our experiences. Raising awareness is the key.” Here is her incredible story in her own words.
My story begins December 16th, 2010 at 2:45 pm. It was the day after my 41st birthday. I was driving and noticed when I looked to my left, my eyes could not focus and every image I was seeing appeared to be splitting. My assumption was that my prescription eye glasses needed a change. Simple, right? Not by a long shot.
As it turned out, my vision had not changed and the problem persisted. I have been a hospital pharmacist for almost 20 years, so I was very aware that double vision is not caused by anything benign. That’s when I knew I was in deep trouble.
My complete medical workup begins. I was scheduled for an MRI on January 6th and panic set in. Not only was I scared about what they were going to find, I have a horrible fear of doctors, needles and blood. I had never had an I.V. in my arm, never been in an ER (except while working) and never had any surgeries. But, I felt so much better having my tests and exams at the hospital I worked at for the last 5 years, St. David’s Medical Center in Austin, TX.
I made it through the MRI with a little sedation, a cloth over my eyes and the help of a caring tech who talked to me throughout the long table time. I finished and was sent home. As soon as I walked in the door, my phone rang. It was the hospital telling me that I needed to come back for a CT scan. I hadn’t even had a chance to cut off my patient I.D. wristband yet. So, back to St. David’s I went. This time after the scan, they wouldn’t let me leave and had me wait in the lobby instead. I paced the whole time. They now needed more MRI images.
At this point, I still had no idea what could be wrong with me. But, you know you have something uncommon when the radiologist has your images opened along with a Google search. The next several weeks involved me seeing lots of doctors and “ologists” in all different specialties, to get an accurate diagnosis and to manage my worsening vision and headaches.
On February 18th I saw neurosurgeon, Dr. Douglas Fox. He knew exactly what I had… a cavernous malformation, also called an angioma, which is the result of abnormal blood vessels that form raspberry-like clusters in the brain and spinal cord. Cavernous angiomas are estimated to occur in approximately 1 out of every 500-600 people, which is approximately 0.2% of the general population. For at least 20% of those with the illness, cavernous angioma is hereditary and in some of these cases people can have multiple angiomas, some too many to count. If angiomas bleed they can produce seizures, neurological issues, headaches, and hemorrhages.
Dr. Fox was familiar with this condition because he did a residency with one of the top angioma specialists in the country. I thought, “Hallelujah, I finally have a diagnosis.” But, despite having clarity, it was nothing to celebrate. Having a symptomatic (a.k.a. bleeding) angioma was rare, and it was even rarer to have one in the brainstem area… I had both of these conditions, as it turned out. Lucky me!
Let me give you some perspective on what that means… the smallest of intrusions in the brainstem can result in significant, and potentially life-threatening, symptoms. The nerves that transverse the brainstem control basic, involuntary functions such as respiration, gag reflex, heartbeat regulation, body temperature, pain and heat sensation, and hiccupping as well as other voluntary functions including eye movement, swallowing, facial muscle control, walking and speech. In a nutshell, the functionality of almost every critical system in my entire body was being comprised by this angioma that bled.
Wait & See
With all that, my doctor’s approach was to wait and see, simply because there were very few options. The only current treatment is surgical removal of the angioma, which is complex; so complex that only a few neurosurgeons in the world will even attempt brainstem angioma resections. If left alone, I was told that the blood from my angioma should, in theory, reabsorb and my symptoms should lessen. But, the odds of a re-bleed were as high as 30% per year for the rest of my life. Being a poker player, I usually make decisions based on understanding odds and probability. I didn’t like those odds. After each re-bleed the symptoms get progressively worse and the healing takes longer, if it happens at all.
At this point, my vision was so bad that I had to wear the ever-so-attractive eye patch. Trust me, it’s not easy to pull off pirate chic! The angioma was sitting on my 6th cranial nerve that controls the lateral eye movement. As the weeks went by, I was terrified as my range of single vision became nonexistent when using both eyes. My left eye was deviating in towards my nose causing complete double vision. Without the patch it was like looking through a kaleidoscope.
So, I started seeing a wonderful eye muscle specialist, Dr. Melinda Rainey, who recommended Botox injections in my eye muscle. Long before Botox was used to treat wrinkles cosmetically it was used to treat strabismus, also known as crossed eyes. Crossed eyes occur when the muscles on one side of the eye are weaker than those on the other side, causing the eyeball to be pulled towards the stronger muscle. Botox works to relax the overactive muscles in order to bring the eye back into proper alignment. I made a total of three trips to the operating room to get the injection. The Botox was not going to “cure” me, but would allow more time for my 6th cranial nerve, which was damaged by the angioma bleed, to heal. Thank goodness for anesthesia!
In the meantime, my overall health continued to deteriorate. The head pain was intense and I was sleeping long and deep. New symptoms were developing and I had all the signs of a stroke; slurred speech, numbness and partial paralysis on one side, facial drooping, loss of coordination and difficulty swallowing liquids. I also had severe increased sensitivity to sound. It all got so bad that I was barely able to function and had to stop working. I knew my angioma had bled again.
Under The Knife
Still seeking help for what was happening to me, my case was then reviewed by world renowned neurosurgeon Dr. Robert Spetzler at Barrow’s Neurological Institute in Phoenix, AZ. He and his team determined I was a candidate for the brainstem angioma resection surgery. If there was any doubt that I had to proceed with surgery, this new bleed within 3 months of my first one answered that.
May 2011, we have a surgery date set, but I was getting worse as the days approached, and we were advised to get to Phoenix immediately. I was only able to walk a few feet and needed a wheelchair most of the time. I first met Dr. Spetzler two days before surgery. He looked me squarely in the eye (pun intended, as my left eye was covered with the pirate patch) and told me the risks and statistics. While he impressively had performed more brainstem angioma resections than anyone else in the world, his grand total was still only a couple hundred surgeries of this kind because of the complexities and risks. His best hope was that I would come out of surgery the way I went into it. His job was to get the angioma out as safely as possible. I was warned that coma, stroke, paralysis and death were potential consequences. But, I did not have a choice at that point; I was so sick and felt if I survived the surgery, that I would just face whatever followed.
May 19th was my surgery day. I was nervous, but ready to proceed. The surgery lasted many long hours as my family waited anxiously in the waiting room. I awoke in recovery and immediately noticed I was able to move the fingers on my right hand. Pre-surgery, I was unable to even sign my hospital consent forms.
My first surgery and hospital admission lasted a total of 48 hours. This was pretty much record time for a brainstem angioma resection. I was prepared mentally for a much longer stay. I didn’t need intravenous pain medications, was eating well (I’m Italian… we can eat through anything) and had stablized neurologically. I had no pain and felt great. I really did. I was back at the hotel for a few days to rest before flying home to Austin. Just to make sure I would be OK for the travel day, we did a test run to a local Phoenix casino and I got to play some blackjack for an hour. I was on the mend!
Back in Austin, with my cane, I start my physical therapy at St. David’s Rehab. I was well enough that I did not need to be admitted. Dr. Thomas Hill, my post-surgical neurologist, set me up with physical therapist, Natasha Johnson. Despite feeling much better overall, I had some balance issues, coordination problems and my vision was very impaired. I spent many hours with her as well as on my own getting stronger. My home still has charts and visual exercises posted everywhere. I try to spend an hour a day doing eye exercises. Natasha sends me reminder texts to make sure I am doing my eye work. She has been incredible in my healing process. I can’t stress enough how supportive all my co-workers and the staff at St. David’s are to me.
At my 6 month post-op mark, I got a good MRI report. “Things could not look any better.” I got a pat on my back and was told to get on with life. It was a strange feeling when I left the neurosurgeon’s office.
Despite that good news, I soon realized that my body was not quite done causing me issues just yet. In November 2011, only 6 months after my brain surgery, I wound up having another surgery… this time to remove a suspicious nodule that was found on my thyroid during one of my neurological workups earlier in the year. So, I get though that and then just over a month later, I end up in the ER on December 31st, the last day of my hellish year, with a new onset cardiac arrhythmia (I had a sudden, unprovoked heart rate of over 200 beats per minute). As I was being rushed to the “crash room”, all I could think was that I was NOT going to die now after surviving all I did this past year. I survived (again) and triumphantly shouted, “Goodbye 2011!”
Moving Forward & Giving Back
The great news is that my vision has improved tremendously and as the weeks and months continue to go by, I have gotten a larger field of single, focused vision. Today, I am almost starting to feel like my pre-December 16, 2010 self again, which is incredible. But, I am also very aware that I will never be quite the same again. I have good days, many great, but I also struggle with some bad days. Folks with brain angiomas often liken the condition to an “invisible” disability. We live with chronic issues as a result of either having the surgery or having periodic bleeds. While we may look fabulous to others, many of us suffer residual problems that not everyone can see. We just know we are different physically, mentally, emotionally… guess that makes us special in some way.
I know the odds of my angioma coming back are very slim, but my fear will probably never go away. When that fears starts to creep up on me, I draw strength from a line in the Melissa Etheridge song I Run For Life that really resonates for me, “It’s a blur since they told me about it, how the darkness had taken its toll. And they cut into my skin and they cut into my body, but they will never get a piece of my soul.”
I am proud to say that in January 2012 I was elected as a Board Member of the Angioma Alliance, a wonderful support group I joined when I was first diagnosed. Diana George, an Angioma Alliance peer supporter who happened to live in Austin, contacted me when I joined. She came to my house several times and held my hand and let me vent my frustrations. She and the entire group have been a continued source of strength and information for me.
Now, it’s finally my turn to give back to others affected and spread the word about the Angioma Alliance, whose mission is to raise awareness and to find a cure. It is a non-profit organization created by people and families affected by this rare disease. Donations for research are always welcome and you can learn more at AngiomaAlliance.org.
I am grateful each and every day for all my blessings. I am also so thankful to my family and loved ones, co-workers and friends who stood by my side, prayed for me and continue to be there during my long road to recovery.
Women You Should Know would like to thank Josephine for allowing us to tell her story with the hope that it brings some much needed attention to this medical condition and the good work of the Angioma Alliance.