Congress needs to pass a bill to address the debilitating impact of the disease not only to the patients, but to the family and to society
After more than a year, I went to see my neurologist, a movement disorder specialist, Dr. Roland Dominic Jamora, the other day. As an X-Linked Dystonia-Parkinsonism (XDP) patient who has undergone Deep Brain Stimulation (DBS) procedure, he does not require me to see him except once a year to know how I am. It was his birthday the day before my wife and I saw him in his clinic. So, I sang happy birthday as I entered his clinic after being called by his secretary. He was surprised that I had a clear voice. I told him creative visualization for healing might indeed be working since that’s all I have been doing for the past month or so. He asked me if I had taken my maintenance medicine that day. I told him, I did not, in compliance with his instructions not to take any medicine when I go see him. That’s the instruction if I wanted my implant reprogrammed.
He put around my neck the holder of a programmer of my implant. The device hung exactly where my implant was. Then he took his iPad near his table. Then he said, if I did not come, my software would not have been updated. I felt my neck hardening, so I asked, what are you doing? Something was not right. Just wait a minute, he said, it is being upgraded. After awhile, he took the device from my neck and conducted the usual test of having my palms face up on my knees as I sit straight up, closing my eyes, counting 1 to 10, then asking me to touch my nose with my index finger then touching the tip of his finger with my same index finger, tapping of my hands, my feet, shaking my hand, etc.
Then he asked me questions based on the papers he was filling out as I answered him: how’s my sleep, my ability to swallow, etc and finally, in the scale of 1 to 100, how would I rate my health. Of course, 100!
While we were about to leave, he told me to drop by the Gamma Knife Center to see my neurosurgeon, then he instructed his secretary to call the Gamma Knife to let my neurosurgeon know that I was coming.
When we arrived at the center, there was the representative of Medtronics, the medical device company manufacturer whose device is implanted to every XDP patient. He explained to us that they now have the updated wireless charger after I complained to him that the skin of the wire of my charger peeled off. This new charger, he showed me, is larger than the size of my palm and has no longer a wire connected to a power bank. Instead, it has a separate blue receptacle for charging. He also informed us that soon, a new implant model would come out that is capable of reading what’s going on inside our brain and will automatically adjust the implant. He also showed us the cell phone-sized display that is used to control the new charger, which, according to him, will show what’s going on in the brain. He said that the display is modified for use only with the device. The patient can’t use it for the internet, etc, to protect itself from being hacked.
Finally, we were able to see my neurosurgeon, Dr. Jose Francisco A. “Jett” Aguilar, in whose name a minor planet 1993 FN41 was named as “7431 Jettaguilar” in his honor by a Paris-based International Astronomical Union (IAU). I asked him where in his miniature brain is the globus pallidus where he inserted my implant. When he pointed to its approximate location, I asked him if there were any scars or damage in my brain when he inserted the implant. He said our brain is like a jelly; the electrodes just slide inside. Besides, he said, during the MRI, they can already see the blood vessels which would cause bleeding when punctured. So, he avoided them.
Then, he asked me about the bill we filed in Congress. I went to Congress with my neurologist in July 2019. We asked for support for that bill from the Panay Island representatives. I remember distinctly that I was glad I was with my doctor because the representative from the lone district of Guimaras, Dr. Ma. Lucille Nava, was an infectious diseases specialist. So, she was able to ask her questions, which were answered by my doctor, in their medical jargon. But the Covid-19 pandemic hit and everything went to a stop. Before the pandemic, I remember going to Congress in September 2019 with Atty. Geraldine Acuña-Sunshine, one of the founders of the Sunshine Care Foundation, the only foundation based in Roxas City in Capiz that gives free medicines, checkup, groceries, and livelihood for XDP patient’s wives and family members.
Dr. Aguilar narrated how XDP was discovered in the 70’s when Dr. GH Viterbo from Roxas City referred five of the six cases of “dystonia musculorum deformans” to the neurology section of the Philippine General Hospital (PGH). It got the interest of doctors from Germany who sponsored around 10 patients to be brought to Germany for treatment. He said he was there during the first two operations to observe how it was done in the operating room.
At present, he told me, there is a High Intensity Focused Ultrasound (Hifu) machine in the PGH. It’s a high-tech machine which uses ultrasound to cause lesions in the brain. Since it’s ultrasound, there is no need to operate on the patient. He said, the lesioning is very effective in removing tremors from Parkinson’s patients. But, since there are many types of dystonia, particularly XDP which is generalized, the doctors found out that Hifu is not as effective as DBS with XDP patients.
Every procedure or medicine must go through an experiment or trial. As long as the patient is well informed and understands the risks and consents, there is no ethical issue.
He said, when they first performed the DBS in the Philippines, they waited for the results of the DBS that was done in the US. It was only after the results were confirmed to be good that they conducted the procedure in the Philippines.
Every medical procedure, before it undergoes implementation, passes through an Ethics Board for review before and after the procedure, to assess its effectiveness. Expectations are set with the patient. The doctor explains that the Hifu, for example, is still an investigational procedure.
The ‘investigation, experiments or trials’ may be the concern of someone I know. One perception is that without data for safety, XDP patients end up only as guinea pigs to experiments, with results that could be irreversible; also there could be studies that put patients into so much discomfort, but would not directly contribute or lead to the determination of cure for XDP. The other assertion, as my doctor states, is that doctors who have spent their lives in the service of XDP patients remain committed because their mission is to perform their duties as best as they can, not for publicity or for personal gain, but for the benefit first and foremost of the patients.
That it is important for Congress to look closely into the condition of XDP as endemic to Panay Island, but with cases already found in other places in the country and in many parts of the world, owing to migration, cannot be overemphasized. XDP is not only the affliction of the patient; its impact is a burden to the family and to society. A bill needs to be passed by Congress so that financial support, among others, can be made available for the procedure called DBS, for example, which remains to date the most effective remedy to such a progressive condition, but is highly prohibitive in cost even for professionals and lawyers like myself. DBS when done at the early stage of the disease can assuage the familial, social, and economic impact, or the XDP’s effects on the patient will surely be debilitating.
I asked my doctor how to bridge the divide among XDP advocates. He said with a smile, “Slowly.” He added, “We are all getting old. Let us set the division aside and work together for XDP patients.”
I promised to revisit our bill. I will keep in touch with Atty. Acuña-Sunshine who has more connections in the House of Representatives than I do. I reminded Doctor Jett of the pictures he took of me in the operating room, more than six years ago. He said he would look for them in the hard drive and send them to me.
Pilar, Capiz 