Suggestion on the Timing of the Neuro-Psycho Test for DBS

More than three years ago on July 14, 2017, my neurosurgeon, Dr. Jose Francisco “Jett” Aguilar of Cardinal Santos Medical Center, drilled two holes just over my forehead where he inserted the tips of my brain implants connected to the battery embedded in my right chest. I was awake during the procedure which my doctors called Deep Brain Stimulation (DBS). I was awake because as Dr. Aguilar inserted the implant, my neurologist, Dr. Roland Dominic Jamora, was constantly checking if the position of the implant affected my speech, my vision, and my hand or movements in general. He asked me to follow with my eyes the light of the flashlight he was waving from left to right and back again. He also asked me to speak to know if my speech was garbled or not, etc. 

With my neurosurgeon Dr. Jose Francisco “Jett” Aguilar at the finish line during the 5k marathon dubbed as the Utakbo at the Veteran’s Memorial Hospital on November 19, 2017.

Before I was brought to the operating room for the surgery, a metal restrainer was put in my head. I was prohibited to take my medicines, which were muscle relaxant, to at least lessen the pulling of my neck which caused my head to turn painfully and involuntarily. The restrainer had clamps on each ear. That clamp was inserted in each ear that no amount of toothpaste-like pain reliever could take away the pain. The restrainer was to stop my head from moving. I remembered crying and asking myself what wrong had I done to deserve the pain after the restrainer was put in place. Then, if my memory serves me right, I was placed inside the MRI machine to determine which part of my brain in relation to the restrainer is my globus pallidus, a structure in the brain involved in the regulation of voluntary movement. This is where the two electrode-tips (implant) would be placed. These MRI pictures were thoroughly studied. Inside the operating room, Doc Aguilar and perhaps another doctor from across the room would shout numbers to each other in relation to the depths and perhaps angles the electrode was being inserted.

After the implant was properly placed, the anesthesiologist told me to wear the mask with oxygen so that I can breathe properly. I knew that was not oxygen rather anesthesia meant to make me sleep so that my doctors could insert the wire under the skin of my head going through my neck, to my chest. Next, they inserted the battery beneath the skin on my right chest and connected the wire directly to my brain. Finally, they stapled my skin to bring them together to close the wound.

With my neurologist, Dr. Roland Dominic Jamora in one of my regular check-ups in his clinic

They were a team of doctors doing my brain operation. But these are the two doctors known to me since I had to beg my neurosurgeon on the operating bed to drill slowly as he might damage my brain. On hindsight, I hope he was not insulted with my reminder. He said, I didn’t have to worry because the drill he was using was a special drill and not something that could be bought in a hardware store. I heard the cracking sound of my skull as he drilled holes into it.

In the head restrainer I was talking about

I also had a regular checkup with Dr. Jamora first, to turn on my implant after about a week, and to fine tune the settings that were appropriate for me. The fine tuning of the implant settings took about a month. He would set my implant at a certain level and asked me to go home and observe, and return again to him to report how I felt. I saw him regularly almost every week, until he got the settings right.

But the rest of their team were silent workers whose names I could not remember now.

Still unconscious in the ICU from my operation.

Anyway, before that fateful day on July 14, 2017, I was subjected to myriads of tests. First, I had to have a genome test to make sure if I had X-Linked Dystonia-Parkinsonism (XDP) in my genes. Before the test, I had to undergo a pre-test counseling. I asked the geneticist why do I need counseling. She said, it’s because there are patients who are in denial, and no matter how long she explains to them the procedure and the possible results, they have a very hard time understanding it. It’s as if they don’t hear a thing. 

I was also required to undergo a post-test counseling to help me understand the result, especially if I had the bad gene in me. Fortunately or unfortunately, my genetic exam result of the presence of XDP was preceded by the symptoms that positively indicated I had XDP even without the gene-test result. So, I asked to be exempted for the post-test counseling, which the geneticist graciously did.

With Metropolitna Mountaineering Society Founder Long Henson, former President Roy Lopez, and Angela Hendrix in my hospital room

It was easy. A man has an XY gene. A woman has an XX gene. If a man and a woman have a child, say a male child, the Y gene of the man and the X gene of the woman will be inherited by the son. Now, if the woman has a defective X which causes the XDP, the child may either inherit the healthy X gene or the defective X gene. So, that child has fifty-fifty chances of having XDP. Now, a man who has XDP has a defective X gene. If he sires a son, that son will inherit the Y not the X from his father. So, a male child of an XDP male patient is free from XDP. The problem is when the XDP man sires a female child. Since a female has an XX gene, the defective X gene of the man will be inherited by his daughter. That daughter will be an XDP carrier. But since it is seldom that women suffers XDP, this daughter of his might pass it to her children. The verb is might because there is a fity-fifty chance of inheritance as explained above. If the children inherit the healthy X from the mother, good for them. If otherwise, it means suffering.

It’s because I understood this concept that my geneticist excused me from the post-test counseling. Also, I have seen my brother suffer with XDP and I buried him when he died. So, I have accepted the fact of XDP should I have it in me.

The staple wires used to stitch my wound closed

I also had to undergo a psychiatric counseling to match my expectation with the results of the operation. I was informed that only 80 to 90% of my symptoms will be gone or will be taken care of by the implant. The rest will remain. And that the implant will only delay the progress of the disease and not stop it. In short, the implant is not a cure.

I also had another test with a cardiologist to know if my heart was beating just enough to still make me love my wife after operation. If I had no heart problems, the operation would go through.

But the one examination that I loathed the most was the neuro-psycho test, where I was made to solve puzzles, asked to repeat words that were spoken, and so many more questions. What is loathsome about that examination is the fact that my neck was twisting and I wasn’t feeling well (that’s why I had to undergo a DBS), yet I was mercilessly subjected to hours of examination.

The test was to measure my memory, problem solving skills, etc prior to the operation. After the operation, I should be tested again to find out what has changed.

I recently talked with my doctor that that exam was useless because naturally, with so many distractions from twisting neck and other XDP symptoms, I could not concentrate. Naturally, I would get a low grade. He said, it was meant as a benchmark. If I wasn’t tested before the operation, with what result will they compare my exam result after the operation? 

Under the Dipolog Cross on July 10, 2018, almost a year after my operation, after 10 days of biking from Pasig to solicit brain and blood for XDP study

With all due respect to my doctors, I would like to suggest that they do the neuro-psycho test after the operation when the patient’s implant has been fine tuned and his condition has stabilized because he will be in a better position to take the tests. He will have less distractions from XDP symptoms. Then, they should let us take the exam regularly, say once or twice a year to know if our memory and problem solving skills have improved or deteriorated as a result of the DBS.